Cover Page

####### I N D E X

###### 

###### SOCIAL SECURITY ADMINISTRATION

######### POLICY CONFERENCE

###### Growth Disorders in the Disability Program Friday, November 18, 2005

####  Page

#### Introductory Remarks

by Patricia Jonas, Assistant Deputy

Commissioner, Office of Disability

and Income Security Programs, Chair 4

by Glenn Sklar, Associate Commissioner,

Office of Disability

Programs, SSA, Co-Chair 7

**[Session One: Growth Impairments in]{.underline}**

**[Ex-Premature Infants]{.underline}**

by Monte Hetland, M.D., Pedriatic Medical

Officer, Central Office, SSA, Moderator 11

**[CDC Growth Charts 2000]{.underline}**

by Zuguo Mei, M.D., M.P.H., CDC 13

**[Presentation]{.underline}**

by Anna Dusick, M.D., Indiana University

School of Medicine 30

**[Presentation]{.underline}**

by Toni Whitaker, M.D., University of

Tennessee Health Science Center 49

**[Presentation]{.underline}**

by Maureen LaRosa, NICU, Grady Hospital 65

**Audience Questions and Discussion Session** 71

**[Session Two: Linear and Weight-Related]{.underline} [Growth
Impairments]{.underline}**

by Monte Hetland, M.D., Pedriatic Medical

Office, Central Office, SSA 111

**[Growth Disorder in the Disability Programs:]{.underline}**

**[Linear Growth]{.underline}**

by John Parks, M.D., Emory University

School of Medicine 112

**[I N D E X (Continued)]{.underline}**

**[Page]{.underline}**

**[Session Two: Linear and Weight-Related]{.underline}**

**[Growth Impairments]{.underline}**

**[Growth Disorders in the Disability Programs]{.underline}**

by Bess Schoen, M.D., Emory University

School of Medicine 123

**[Presentation]{.underline}**

by Heather Taylor, Parent Participant 136

**[Presentation]{.underline}**

by Paul Nrabosky,

Little People of America 142

**[Presentation]{.underline}**

by Alexandra Philips, Child Participant 144

**[Audience Questions and Discussion Session]{.underline}** 150

**Keynote: "\-\--" indicates inaudible in the transcript.**

### M O R N I N G S E S S I O N

(9:00 a.m.)

***Session One: Growth Impairments in***

***In Ex-premature Infants***

***Introductory Remarks***

###### by Patricia Jonas

MS. JONAS: Good morning. I think we are all about settled here and you
have something to munch on for your enjoyment here. I am Pat Jonas. I am
the Assistant Deputy Commissioner for the Office of Disability and

Income Security Programs, and I am welcoming you here on behalf of the
Social Security Administration to this outreach conference. And today's
topic is on growth impairments.

Some of you were here yesterday and saw the dynamics of how this works.
We basically have panels of medical professionals, consumer advocates
and representatives. Then we will take a break, come back, have a period
for questions and discussion. And then we will have a lunch break and do
the same thing in the afternoon.

One thing I want to sort of emphasize here is the discussion piece. I
think yesterday's conference went very well. Most of the period after
the speakers was focused on questions to the speakers. And one thing
that I think we missed a little bit was some discussion amongst the
folks from SSA about your challenges in implementing. I think we want to
hear from you as well. This isn't just about soliciting specifics,
although that was an important part of this. This is also about, you
know, how do we make this work for people who have to actually
adjudicate the claims. So I will sort of poke at a couple of you during
the break, and see if we can get you to engage in a little bit of
discussion.

One of the things I wanted to say before I turn this over to Glenn in a
second, was also, you know, so what happens as a result of these
conferences? You know, we have asked you to come here. I think yesterday
was a great session, with a lot of important discussion. So what happens
next?

Well, there is the immediate piece of what happens. The folks from the
Office of Disability Programs

have a task to perform here, going back and rewriting the specific
listing itself. But other things happen sometimes as a result of these
conferences as well. And again, it goes to the point about hearing the
full dynamic of the challenge about this particular policy.

In some of the outreach sessions we have heard some things which were
very similar to yesterday's discussion, with regard to, for example, the
importance of this \-- some specialization going on with regard to the
particular body system, in terms of more people, more general
practitioners going out and seeking assistance of specialists with this.
And also the importance of the almost 24-hour care for the individual
patient, especially children, for this.

And other outreach sessions on other impairments have had similar issues
that have come up. So we hear some common themes, and that goes into our
thinking about our disability program as well. So although it may not be
a specific issue for a change in the listing, it may generate some other
issues.

And as an example of things that happen, as a result of one of the
outreach sessions, we sat down and thought about a demonstration
project. And we alluded to that a little bit yesterday, but I wanted to
say just a little bit more about what that means. You know, SSA has the
authority to do demonstration projects where we are basically sort of
switching the roles. Yesterday we talked about, well, it might take an
act of Congress to do something \-- I am looking at Dr. Becker here. I
heard it.

(Laughter.)

And I think we think so, too. And in some cases, in order to make that
case for something, we have to have some information. I mean, we talk
about making changes to our Social Security Program. We typically talk
about regulatory changes where we have the authority to do that.

But when there is an issue that requires some legislative changes, we
can go forward with those, but I think it requires more than just a good
idea. So we use those demonstration projects. So that is a way that we
make some practical changes or propose that, using that particular
procedure. And that came directly out of one of the outreach sessions.

So it is an important project, an important issue that we are doing just
individually on a listing. But again, the more dynamic these
discussions, I think the more helpful they are to all of us.

And Glenn, we will turn it over to you.

**by Glenn Sklar**

MR. SKLAR: Good morning. And again, thanks for joining us. I recognize
many familiar faces from yesterday, so for us that makes it a little bit
easier so we won't have to completely backtrack. But what I would like
to do is walk through some of the major themes we heard yesterday, for
the benefit of those who weren't here, and at least give you a sense as
to some of the main issues that were put in play.

As Pat mentioned, it was a great session, and in many ways it is clear
we have a huge challenge in front of us, almost a complete re-think of
the existing listing from so many different levels.

The first panel was very clear on the fact that diabetes in and of
itself is not a disabling condition. What you really need to look to is
the end organ state. And we certainly have some work to do there in
capturing some of the potentially damaging impacts of diabetes that
aren't in the existing listing, for both type 1 and type 2.

There are also some questions about the theoretical underpinning of the
listing for type 1 and

type 2 diabetes as well. In the listing right now we do use issues such
as hospitalization as a proxy for disability, and many folks said that
is really not a useful standard in 2005, or the number of
hospitalizations for DKA \-- and hopefully I will get this right \--
diabetic ketoacidosis. Am I close, Monty? And it goes back to the issue
of, when you are crafting policy you want to make sure your policy not
only makes sense at the medical level, but also from a public policy
perspective, and what types of behaviors do you want to support, and
drive.

So we have a lot of work ahead of us, particularly on the diabetes side
of the house, with both type 1 and type 2. We are reminded with type 2
not to forget about the impending explosion, really, in type 2 cases, in
that with an increase in obesity in America, with an aging population,
that we can't forget about type 2, even though our listing really talks
about type 1 diabetes primarily.

We also heard some wonder personal experiences that really brought the
disease to light, particularly from Ms. McGrath. She was very open,
explaining how 30 years ago, how difficult it was to manage her
diabetes. And I can tell you, the standards probably about 20 or 30
years old, reflecting that timing, and how again we are measuring
essentially urine output, and how today, with technological advances and
better knowledge, better glucometers, test strips, better insulins, that
work even better, one can achieve much greater control of type 1 and
type 2 diabetes. So our standard needs to reflect that.

We also heard about some of the major studies that have been done,
explaining how better control leads to much better outcomes. So we
really need to take that into account as well, and perhaps come up with
a more flexible model for putting out what we call our listings, or
criteria.

Again, it is not good enough to do this every 10 or 20 years, when
technology is changing in six-month,

12-month and 18-month increments. So really our whole strategy here has
changed, in that what we would like to

do \-- across the board, not just with endocrine disorders, but with all
the 14 different body systems that we are working with \-- is do a
complete flip of the entire set of listings by 2007, where they will all
be at a base line state that we are comfortable with that it
incorporates the latest medical information \-- and then have a more
targeted approach in the future, whereby you won't have to change a
whole listing, but could come in with a more targeted approach where
just a particular piece may change, based on new technology. And if any
particular body system has shown how important it is to keep up, it is
the endocrine system.

In the afternoon panel, again, the themes were very similar. Though
endocrine disorders are not in and of themselves disabling, one needs to
look at the consequences of the disease, and that we need to make some
very significant changes in our standard, because it is quite outdated.
In fact, it was pointed out that some of the tests we use, many labs
don't even run those tests anymore.

So there is a lot of work ahead of us. But we are up for the challenge,
and we really want to embrace a new model whereby we have a huge amount
of input on the front end, and we can really keep up and keep pace with
technology, and keep these listings moving, to keep them as up to date
as possible. So conferences like these are invaluable to us.

So with that, I am going to turn it over to

Dr. Hetland, who is going to introduce our first panel, and also
introduce a couple of the folks in the room who you might be seeing and
hearing from from Social Security throughout the conference. Barry
Eigen, our Program Policy Officer, is in the back. And Ms. Kathy Lively,
who is a Division Director for our listings area as well. So, with that
\--

***[Session One: Growth Impairments]{.underline}***

***[in Ex-premature Infants]{.underline}***

**by Monte Hetland, M.D., Moderator**

DR. HETLAND: Good morning. Thank you, Mr. Sklar.

I am the Pediatric Medical Officer in the Central Office, dealing with
medical policy affecting children.

This morning's topic, to give you a little bit of background, is
focusing on the very small, premature infants, which if you think about
the function example 7

and 8, basically any child, whose family meets the financial criteria,
obviously, who has a birth weight under 1200 grams is a function equals
allowance until the first birthday, and then the CDR is mandated at that
time.

We also allow, under functional aids, children who are born between
birth weights of 1200 and 2,000 grams, and who are SGA. And this is SGA,
meaning small-for- gestational age. And we have arbitrarily set that at
the third percentile. And we will hear some discussion of that this
morning.

Let me introduce our first panel this morning. Our first speaker is Dr.
Zuguo Mei, who is a physician and clinical epidemiologist at the CDC,
and was instrumental in helping to publish the 2000 growth chart
revisions. That is the most recent, and a totally new concept to many of
us who are still struggling with the concept of using BMI, body mass
index, in assessing growth.

Our second speaker will be Dr. Anna Dusick, who is a developmental
pediatrician, and an associate professor of clinical pediatrics at the
Indiana University School of Medicine. She also is involved in follow-up
clinics at the Riley Childrens Hospital in Indianapolis.

Our third speaker will be Dr. Toni Whitaker, who is also a developmental
pediatrician. She is an assistant professor in the Department of
Pediatrics at the University of Tennessee Health Science Center. She has
also been involved with NICU follow-up clinics, as well as with our AUCD
projects, and she will be sharing some of that feedback with us.

Our last speaker, Ms. Para Ward, unfortunately had a family emergency
overnight, so she is unable to join us. However, she spoke with Maureen
LaRosa, who is the clinical nurse who is involved in the NICU follow-up
clinic at Grady Hospital, and she will be sharing some of the thoughts
that Ms. Ward was going to share with us this morning.

After our four speakers are done, we will take a short break, and then I
will ask them to come up and we will have a discussion. And I agree, I
would like to hear more from the audience. So, Doctor.

***[CDC Growth Charts 2000]{.underline}***

**by Zuguo Mei, M.D. M.P.H.**

DR. MEI: Good morning. Thank you for inviting me to this very important
conference. I feel sad, you know, my talk today \-\-- introduction about
the new CDC growth chart.

When we said it is new, it is relatively new. It is actually, we start
the year 2000. So I just give you a little bit of background.

(Slide)

My presentation, we are focus on those five area. First, I give you a
little bit of background, and then I will give you what those growth
chart based on, what is the reference population. And then I will give
you a brief introduction about the body mass index, which called BMI.

I am also giving you a comparison, what is the BMI compared to the
weight for stature, and then \-\-- I give you some example.

(Slide)

First I want to introduce a concept, what is the percentile \-- do any
of you know "what is the percentile" means? So by definition, percentile
ranking the position of individual by indicating what percent of the
reference population the individual would equal or exceed.

Hope you can understand better than me, since the English is my second
language.

(Laughter.)

Okay. I will give you example. So for example, for five years \-\-- for
weight for age of child, if you look at the weight for age of child. His
weight, it is at the 25^th^ percentile. What this mean? This means that
at five year or so the girl compare to the population of 100 \-\-- old
\-\--, it is 25th percentile weight more or less than 25 others. But 75
weighed more than she does. Does this help?

Okay.

(Slide)

And secondly I want to say who is going to use the growth chart? Mostly,
it is, you know, first category it is pediatrician, mostly. And the
second, it is a health professional. And the third it is research.

(Slide)

And secondly I want to introduce you to another two definition or
concept. First, it is standard; the second it is reference. What is the
reference between those two? When we see a standard, usually we see it
is a normal or optimal growth. So it is ideally. So, if you trying to
say it is a standard, the more apply to \-\-- healthy children, with low
problem, low chronic condition, and also follow all the feeding and care
recommendations.

So if we do the reference based on those definition, we can call it is a
standard. But however, it is not very easy to get those kind of data. So
the second one we call the reference. Reference \-\-- actual growth. It
is based on representative sample, and include both poorly nourished and
unhealthy children.

So basically when you say if it is a reference, it is just applied to
most recent, you know, representative data to describe what is the kids
looks like.

(Slide)

So when you understand this two concept, then I will say what is the CDC
2000 growth chart stand for? Does the CDC 2000 growth chart, we apply as
a standard, or as a reference?

So will I say there is some key departure from standard, \-\-- 2000
growth charter we include children with chronic condition, because there
is no way we can exclude all those chronic condition based on the data I
will show you later. And also not all the children follow feeding and
care recommendation. So it is not sure standard.

(Slide)

But also it is not a \-\-- reference, because we excluded a very low
base weight infant, which is less than 1500 gram, because those very low
birth-weighted children, definitely growth differently.

And also we exclude the NHANES, which it means the National Health and
Nutrition Examination Survey, the \-\--, the \-\-- data from six and up,
above, because we observe the dramatically \-\-- trend in weight. Those
kids definitely getting more heavy. So we exclude those. I will show you
later the data.

And also I will show you we use some much older data, so it is not a
truly reference either.

So people may be asking, "Why you doing this? You are not a standard,
but also not a reference. Why you doing this?" The reason, because in
\-- clinically, in the pediatrician's office, where you evaluates each
individual cases, the doctors, pediatricians, always treat them like a
standard to compare with. But for research, for other health
professional, they always use them to compare with, from one population
to another population, more like as a reference.

So that is why we said, you know, it is not \-- it is between the 2000
growth chart. It is not a \-\-- standard; but it is not \-\-- reference,
either. Okay?

(Slide)

So right now I will tell you what this reference the data based on.
Majority of the data from six months to 20 years old are based on the
five, cross-sectional, National \-- later I will present to you Health
Examination Survey, which is \-- National Health and Examination Survey,
two which is in the early '60s, and the three \-- it is in the '66 to
'70s. On the NHANES I, II, III, which is National Health on the
Nutrition Examination Survey I, II, III.

Most of the data is from those five survey data combined. Under some
exception, because we don't have enough sample for the young infant,
from birth to six months. So we use some national birth certificate
data, and also two states, you know, birth months. And also a \-\--
subset of CDC Pediatric and Nutrition Surveillance System data to just
increase the sample size from birth to six months. And a majority from
six months on are based on those five national representative surveys.
Okay? That is all the data come from.

(Slide)

On the reference population for the CDC growth chart, we said it is
racial and ethnic diverse, include all those race and ethnicity. And we
have two set of growth chart. The first set is for infant from birth to
36 months. And the second set is for children from three years old to 20
years old. And also include both breast-feeding and formula-feeding
infants. Okay?

(Slide)

And as I said before, it is not a truly reference, so the first one
would exclude is very low birth rate infant, which is less that 1500
gram who are excluded from the first set, from birth to 36 months child.
Okay.

And the second one is to weigh the data from

six \-- from infants three. After age six the weight data has been
excluded, because the \-\-- trend in weight. So \-\-- in both weight-for
age chart, and the BMI for age chart.

(Slide)

I just want to show you why we exclude. Because you can see here, from
both NHANES I, II, III, which is the I, II, III, for each age group, if
we include the NHANES III you can say the age are just problems of \-\--
weight, what looks like. You will see from boys six to 11 years old,
NHANES I, II, III, the problems. And from 12 to 17 you can see how much
the jump, if we include the NHANES III data. And also from girls.

So that is why we decided, you know, to exclude them. Otherwise if you
include them, they automatically, you know, \-\-- the reference
population we are underestimate the problems of overweight. Okay?

(Slide)

And also we said the CDC growth chart are for all racial and ethnic
group combined, because there is \-- sufficient evidence show you where
\-\-- inference \-\-- to be contributing more to the valuation of growth
than genetical inference, particularly for children less than five years
old. Okay? I will show you that data later.

And also we do have enough sample \-\-- data to do a ratio and \-\--
chart. That is another reason we didn't develop a, you know, separate
chart. And also the impact of race and ethnicity on BMI-for-age is
unclear. So that is why we don't develop those, you know, as a specific
chart.

(Slide)

One example, we actually look at the data \-- it's from the CDC
Pediatric Surveillance Data \-- looking particularly Asian group from
\-- most of them refugee population, come to the States in the early
'70s, to look at their children. We can look at the problems of low

height-for-age. You will see compared to the white population, you can
see in the earliest '80s, those children, the problems of low
height-for-age almost, you know, more than twice higher than the white
kids.

But as the environmental improvement in their family, look at those
children later. You can see it is almost the same as white. So that is
where we said how environmental, economic improvement can improve the
general growth of young children.

(Slide)

And also we didn't develop, you know, feedings specific, particularly
breast-feeding \-\-- formula feeding. We know, you know, feeding, it is
a factor of our growth, particularly in weight, because evidence shows

breast-feeding kids, most likely during infancy they gain less weight
than formula-feeding kids. But however, we can't divide those
specifically. One is the \-\-- side of the issue, and the second one it
is a practical issue. For example, how you are going to \-\-- if you
develop one set of chart for breast-feeding children, another set for

formula-feeding kids, what the \-\-- of breast-feeding kids, which child
you are going to use.

So that is why we didn't develop it, you know. We know there is a
difference, but we can't develop it because that restriction. One is a
\-\--, another it is a practical issue. Okay?

Okay. Before I turn to the next slides, how many of you, I will ask you,
have seen a growth chart before?

(Display of hands.)

Oh, a lot. Yes, because of the \-- great. So that will make my job much
easier.

(Slide)

So the next one I just want to show you what the growth chart looks
like. Here it is the \-- maybe you can see in the doctor's office \-- it
is combined, you know, for children from birth to 36 months; that is for
boy. The

Length-for-age and the weight-for-age, because we combine those two
chart to save paper.

You will see that is the weight-for-age, the XX says it is age, from
birth to 36 months. The other \-- second set is \-\--for-age, because
from children \-- we call them \-\-- because we measure the children
lying down, instead of standing up, so we call the \-\-- lying down,
call them

\-\--. For standing up, we call that either standing height or stature.

So the four sets there you can see, we have two boys in English system
and the metrical system, inch and the centimeter, and the pound and the
kilo and age. Okay?

Since most of you see a chart before, so I don't need to give you more
explanation.

And that is for boys. For girls we have a different color. You see here
the same chart for girls.

On the next one is the head circumference for children from birth to 36
months, and also for

weight-for\-\--. So the XX here, it is \-\--. You can say it is either
in centimeter or inch.

So the difference is that here the XX is age; but here, instead of age,
it is \-\--. So it like a relative weight to your \-\-- or height.

(Slide)

And the second set is for children from two years old to 20 years old.
So we have the weight-for-age from two years to 20 years old. And I also
have a standing height, we call it stature, so it is a stature-for-age,
also from age of two to 20. Okay? That is also for boys.

(Slide)

On the new one it is body mass index for age. So I will show you how we
calculate the body mass index. But just give you a sense how the curve
looks like, you will see from age of two, that is a downward side, and
the \-\-- going up is age. Okay? Just keep this in mind.

(Slide)

So the indicator we used for nutritional status for \-\-- we used fifth
percentile for the low end, and we used 95th percentile for the high
end. For example, for stunting, for shortness, we used the \-\--
stature-for-age if it is less than a fifth percentile. And underweight,
we either used \-- for young children we used the weight-for-months, or
for older children we used the body mass index for age, used the less
than fifth percentile.

And also for up here for overweight we used \-\-- or the 95^th^
percentile. We also have a category based on the BMI-for-age if the
percentile between 8\-\-- to 9\-\-- percentile, we call that at risk of
overweight. Okay.

(Slide)

So now I will give you a little bit of background, what is the body mass
index? Body mass index, or BMI, actually is not a direct measurement
indicator. It is a calculated indicator. It is based on your weight and
height. So you calculate for me your weight, convert it to kilo, and
divide it by height in mete squared. So it is a calculated indicator.

And the BMI is an effective screening tool, because it \-\-- relative
weight. So it is the relative weight to your height. But it is not a
direct measure to your body fat. So we \-\-- it is a screening tool; it
is not a diagnostic tool, particularly for overweight or obesity. Okay?

And also for children, BMI, it is age and gender specific. You know why?
Because for children there \-- this increasing weight in age, and you
simultaneously also increase kids are getting taller, so that is why it
shall be age and gender specific, compared to adults. Because for
adults, your height is relatively stable, so your only thing to
calculate the BMI is your weight. If your weight is going to change, you
BMI is going to change.

(Slide)

So, here just example how for children the BMIs change with age. So I
give you example from under 95^th^ percentile for children at two years
of age, four years old and nine years old and 13 years old, and you can
see the change in BMI, you see here, from 19.3 to 25.1. Okay? So the
children, because they are both weight and height, it is changing with
age; that is why that BMI is changing. Okay?

(Slide)

The advantage for use the BMI, because maybe lots of people were asking
why you switch from a, you know, much more for meaning, you know,
weight-for-stature indicator, to a BMI indicator? The reason \-- the
most reason I can explain to you, it is for tracking purpose, because a
BMI has been used for adults for so many years. And we trying to use the
same indicator to tracking from childhood for adulthood. So that is the
major reason, it is consistent with adults' index.

(Slide)

And also \-- here I can show you some tracking published the paper. It
is tracking BMI \-\-- from birth to 18 years old, with percent of all
the way the children who are obese at age of 25, which is young
adulthood. So you can see the tracking, you can see much clearly all the
children, if about the 95 percentile of BMI, definitely have a much
higher chance to be obese at young adulthood.

So that is why the big advantage, you can track him from childhood to
adulthood, by use one single indicator.

(Slide)

Another advantage to switch to BMI for age, because there's studies
showing BMI-for-age, it is related to health risk. There is some
correlation with, you know, cardiovascular disease risk factor. And also
it is related to lipid level and high blood pressure in middle age.

(Slide)

And also for young children, particularly for children less than five
years old, we just published a paper to show it is very comparable. So
it doesn't matter which indicator you are going to use. You use the
weight-for-stature, or use the BMI-for-age. It is very comparable. So it
doesn't matter. That is why it is relevant. For young children you can
either use weight-for-stature, or

BMI-for-age.

But also some studies show the BMI-for-age, because it is not a direct
measure of body fat, but however,

from studies showing to compares relatively \-\-- body fat measure, for
example, the \-\--, so it is very close \-- it is showing a very high
correlation between BMI-for-age, and, you know, percentile body fat.

(Slide)

And I also show you the shape of

weight-for-stature to BMI-for-age, it is different. You can see from the
\-- I think I run out \-- okay, it's come back \-- so that is the
weight-of-stature occur where you see it is consistently increase with
height, but a BMI \-- firstly you have a downward, and then going up.
Okay. The shape, it is a little bit different.

(Slide)

So now I will give you some example. \-\-- for the relationship from
those indicator to another. First I give you a young infant, a
three-years-old boy. His length is 26.8 inch, which by calculation is
length for age of percentile, is close to the third percentile which is
2.9 percent you see here. So you can see the pointer here. It is below
the fifth percentile.

On his weight, it is 17. \-- 17 pounds five ounce. It is also on the
third percentile. So maybe people asking, if the same child, his
length-for-age, it is on the third percentile, and also his
weight-for-age, it is on the third percentile, what is relative weight
compare to his

length? Shall it be also on the third percentile? Or shall it be
different?

Actually, his weight-for-length percentile is the 43.7 percent. You know
why? Because the length-for-age and the weight-for-age, it is compare to
a population in the same age, 10 years old. You are shorter; you are
lighter, compared to the reference population. But however, since you
are both shorter and lighter, you compare your weight to your relatively
shorter status, you know, it is not so bad. Right? So that is why the
weight-for-length percentile, actually it is not on the third
percentile, which, actually it is on the, almost in the, in, you know,
almost close to the average. Okay?

Any question about this?

(No response)

Okay. Good.

(Slide)

The second example I will give to you is the same chart. If his weight
remain the same, but his height, or length are taller \-- for example,
his length for age, it is about the average \-- which is 52 percent,
okay? but his weight remain the same, on the third percentile, what his
weight for length looks like?

(Slide)

See here, 1.4 percent. Because it is simple; compared to the same age,
three years old, he is taller, he is about the average, but he is much
lighter compared to the 10-months-old boy. His weight-for-age is about a
third percentile. But if you \-- his weight, since he is so light,
compared to the relative \-\-- taller, the \-\--, of course his
percentile will be much worse. So this is why only the

1.4 percentile. Okay.

(Slide)

And the third example I will give to you for older children, almost
three years old, 35-months-old boy, his length almost on the third
percentile, and also his weight, it is around third percentile also. So,
what his

BMI-for-age percentile looks like? You see the difference before. The
weight-for-length, it is about 43 percent, right? From the example 1.
See here, right? It is the length about the third precentile, and the
weight-for-age is about a third percentile. But age/weight-for length is
about 43, or 44 percent.

And here, the example 3 I gave to you is

length-for-age is about a third percentile. Weight-for-age also about a
third percentile. But if you use the new indicator, border mass
index-for-age of percentile, it is only 22 percent. Why is that? Because
that before the weight-for-length, it is like two-dimension; you only
compare your weight to the same height or length; nothing you do with
age. Okay? So it is most likely, kind of like aging-dependent. Okay?

But here, border mass index-for-age, it is like a three-dimension. Here
my calculation is also relative to weight. You calculate that from your
weight in kilo, divided by height mete squared. And also compare to your
age.

So here, \-\-- are both on the third percentile. You calculate the \-\--
relative weight. But you also need to compare to your age, 35 months. So
that is why your \-\-- 43 percentile, you are only like half, 22
percentile, because compared to your age, you are not doing very good.
Okay.

That is why either understanding the

weight-for-length or weight-for-stature it is like a

two-dimension. It is like age-independent. However, the border mass
index for each is like a three-dimension, both consider your weight,
height and age. Okay?

(Slide)

And the last example I give to you, the same

35-months-old boy, if that is his height, it is about average. But his
weight remain the third percentile. What his BMI for age looks like?
Even much worse, because you are about the average height, but your
weight, it is so light. When you compare it to your relative weight, and

also compare it to your age, you are much worse. Okay?

(Slide)

So, if you need more detail on the CDC growth chart, we have a web site
on the CDC.govgrowthchart. Also included are training material, and also
have a \-\-- about the BMI calculated \-- how the BMI calculated. And
also have a BMI table. And you can download the growth chart from the
CDC web site, that looks like. And also they have a special section
using the CDC Growth Chart for Children With Special Health Care Needs,
problem mostly, you know, apply to what will be more important to you
guys. I think I will end my presentation here. Thank you, very much.

(Applause)

***[Presentation]{.underline}***

**by Anna Dusick, M.D., NICHD**

DR. DUSICK: Good morning. I am pleased to be here today to address the
policy on growth impairments in ex-premature infants. And I would like
to thank Dr. Hetland for this invitation to talk to you this morning.

(Slide)

I would like to start with commonly used medical terminology, and share
with you the current concepts for guiding growth after a premature
birth. So let's start with prematurity, just to make sure we are on the
same page. And Dr. Mei has already referred to many of these terms, so
we will go through them rather quickly.

Term birth, medically we consider that to be

40 plus or minus two weeks of gestation. Pre-term birth, then, is less
than or equal to 37 weeks of gestation.

(Slide)

Further terminology that you have heard a bit about, the term low birth
rate, or LBW \-- and you will see that in the literature a great deal
\-- is less than 2500 grams, which for those of you who think in pounds
and ounces, is five pounds eight ounces.

Very low birth weight, or VLBW, is less than

1500 grams, and that is about three pounds, five ounces.

And the third classification you will see commonly in the pediatric
literature, is extremely low birth weight, and that is less 1,000 grams,
and two pounds three ounces. And this is the population that I study.

(Slide)

So where does the assignment of gestational age come from? The
assignment of gestational age is based on three things: mother's
menstrual history, and on an

old-fashioned term, the estimated date of confinement, or the EDC, from
usually fetal ultrasound, from the obstetrician. And then finally from
the physician's examination after birth, using the Ballard score.

So what is the Ballard score? It is a maturity rating scale based on
physical and neuromuscular criteria and weeks of gestation. Medically
the term "small" for gestational age in the pediatric literature is a
birth weight less than a 10^th^ percentile for gestational age.

(Slide)

Now that the use of the terminology is established, let's review what we
know about growth in the premature infant still in the NICU, or the
Neonatal Intensive Care Unit, before discharge. Numerous studies reveal
that prenatal infants grow at a slower rate after birth than they would
if they were still in utero. The lower the birth rate, the greater the
degree of growth retardation after birth. And most pre-term infants, at
36 weeks of gestation \-- that would be postconceptional age \-- are
less than the 10th percentile on fetal growth curves.

Now, fetal growth curves are something that

Dr. Mei did not address, and currently with the National Institute of
Child Health and Human Development Neonatal Research network \-- which I
will tell you about \-- we are using these growth grids from Alexander.
That is the standard that we are using. And there are lots of
discussions about which standards to use, and that is currently the one
that we are using.

So what does this mean? You grow better in the womb than you do in the
NICU. And that has been commonly known.

(Slide)

So this postnatal growth retardation in premature infants is caused by
several things: Inadequate calorie and nutrition intake. Increased
energy expenditure. Gastrointestinal intolerance, which limits the
feedings that the baby can have. Oromotor dysfunction, with the
limitation of feeding, their intake. Gastroesophageal reflux.

In the NICU we know that growth retardation is associated with increased
complications of prematurity, including chronic lung disease, and late
onset sepsis, or infection.

(Slide)

So we have set the stage that most premature infants are growth
retarded, as compared to term infants, when they go home. So let's talk
now and focus on what happens after discharge, and that, of course, is
the purview of the follow-up clinics, the general pediatricians, the
specialty staff that follow the infants after they are discharged from
the NICU.

For those of you who don't work in pediatrics, premature infants tend to
go home, I would say, usually about two weeks from mom's due date. So
keep that in mind. That is about average. Some children who are sicker
would go home later than that, but in general when a premature infant is
born, mom says when can they come home, it is a few weeks after mom's
due date.

(Slide)

So let's talk, then, about the follow-up. Now, follow-up recommendations
vary from center to center. And I think you are going to hear perhaps
what Dr. Whitaker's clinic does, and it may be different from my own
clinic.

The follow-up programs may include primary care. They can include
specialty care, or some of the follow-up programs are strictly for
research. It is different, depending on the staffing, and the needs of
that community, and of course the money of the facility and all those
other kinds of considerations.

(Slide)

So, infants are recommended for follow-up who are high risk. Well, what
is high risk? There can be biologic risk, or \-- for example, I am going
to go through some biologic risk. They may have had interventions in the
NICU. For example, they may have had \-- been on a bypass machine
because their lungs were very, very, very sick. Those children may have
special needs, and so they are certainly followed up. Other
interventions. Maybe they got a tracheostomy while they were in the
hospital.

And so these children have other problems and they are going to need to
be followed up. So there may be any kind of interventions.

And the last thing is a social or environmental risk. And those tend to
be included much more in those kinds of clinics that have primary care
availability.

Pre-term defined in the literature that I have quoted here, the
Guidelines for Perinatal Care from the American Academy of Pediatrics is
considered a birth rate of less than 1500 grams, and 32 weeks of
gestational age. That is the high risk biologically pre-term population
that the Academy has defined.

(Slide)

So let's look at the group with the birth rates less than 1500 grams.
And I don't think I have a pointed.

(Pause.)

Oh, we are all out of batteries. Okay. All right.

So here are the healthy people, 2010 data, and this chart is looking at
fetal weight and subsequent death per 100,000. And this is based on 1998
\-- thanks

Dr. Levitsky \--

(Pause.)

(Slide)

Okay. This is the group that we are really focusing in the most on, or
what healthy people 2010 is focusing in on. Okay. So the table shows
three groups: your less than 2500-gram birth weights; your 1500- to

2500-gram group; and then your less than 1500-gram \-- which I am
already telling you people consider high risk. Okay?

We have all deaths under year; deaths that are birth to 28 days \-- I
think this is getting weak, too.

(Pause.)

Yes, we will go with this one, okay.

This is what we call neonatal death, which would be birth to 28 days,
okay? Typically these children are still in the NICU. And then 28 days
to one year \-- I am destined not to have one of these, I think. Okay.

So this population of high risk premature infants, and this box here, of
late death, is the box that we are trying to impact, and what I am
addressing very much today, and why I showed you this chart.

(Slide)

I would like to tell you a little bit about our experience and what we
do, just to kind of set the stage from \-- about growth impairments. My
own follow-up program at Indiana University covers four hospitals. We
include a county hospital in that, and there are a university hospital
and two private hospitals.

We have two outpatient sites, with weekly clinics. We have maternal and
child health funding, and we do use patient billing. And our population
is 65 percent Medicaid eligible. And we do have a neonatal nurse
practitioner, who is my clinic coordinator.

(Slide)

So who do we follow? I have already told you that it is not standard who
\-- which kids are considered high risk by different clinics. But who do
we follow at my clinic?

We have 684 infants in my 2004 count. Our criteria for enrollment are a
birth rate less than 1500 grams, children who are neurologically at risk
\-- that would include, for example, the term baby, who is born with
birth dysphyxia or hypoxic eschemic concephalopathy; they would be
considered at risk. An infant who may be greater than 1500 grams who has
meningitis in the NICU, would then need to be followed. Children with
multiple congenital anomalies. Children who received extra corporeal
membrane oxygenation, which is the heart/lung bypass machine that they
may be on for their lung disease. And then on occasion there are some
other kids that we will have by request of the neonatologist; for
example, a child with a heart defect may not be something we are looking
at in terms of multiple congenital anomalies, but the child may be
medically fragile, and that neonatologist may say that we need to follow
up. That is pretty much how we decide, or divide up our population.

(Slide)

So what do follow-up programs do? Monitor growth. Manage unresolved
medical problems \-- and you will hear more about that from Dr.
Whitaker's talk. Detect abnormality or delayed development. Provide
early intervention and habilitation. Discuss neonatal safety. Educate
parents. Coordinate care and insure follow-up. And refer to other
community resources.

(Slide)

When? When do you follow the kids? There is no hard and fast rule. In my
own clinic, I follow them whenever they need to be seen. There are
children that are medically fragile and come every month. There are
children, for example, they may be on home ventilation, mechanical
ventilation at home; they come every month. There may be other children
for whom, they are failing to thrive, and we will talk a lot about that.
I may see them a lot at first, and once things get rolling, we spread
out those visits.

But in the consensus statement from the NIH, which I have listed here,
that Dr. Vohr authored, these are the recommendations: In infancy, very
much like pediatric visits, between three and four months, six to nine
months, 12 to 14 months. In toddlerhood, 18 to 24 months, 30 months. And
then in pre-school, three years, four years, and then again at five
years.

\` If you look at the follow-up literature, you will see that there are
certainly clinics that will follow the kids up until adolescence. And
Dr. Hack, in Cleveland, is even reporting 20-year outcome of premature
infants.

But typically, many, many centers, if the kids are growing well and
developing well, they will then discharge them from their clinics as
they get older.

(Slide)

So were those corrected ages, or uncorrected ages. That is always the
question. So typically those are corrected ages. So how long do you
correct for prematurity? I looked through the literature \-- I know what
common practice is, but I looked through the literature to find some
help with this. And again, in that NIH consensus statement that was
published in Pediatrics in 2004, Dr. Vohr lists two to two-and-a-half
years, or 24 to 30 months. Jordan listed three years, in a very sound
publication.

And I just had a conversation with Dr. Hack the other day, and there is
some data, and some people have decided to correct up until
eight-and-a-half years, more for development than for growth. In my own
clinic, we use three years, or 36 months.

(Slide)

So let's turn out attention to the growth graphs used to evaluate the
ex-premature infant. And I would recommend using the NCHS growth grids
that you just heard about this morning.

I am recommending the use of them because prematurity is not a
disease-specific state. For example, children with Down's syndrome may
have other growth measures or growth curves. But prematurity is not a
disease-specific state. And especially after what we learned this
morning about the reference data, you want to look at the children
according to the reference population.

These children have the potential for growth according to their genetic
potential. If we compare them to other premature infants with growth
failure, we will

under-represent their growth delay.

If you were to use the IHDP growth grids, you will then compare them to
other children growing poorly, and say, "Aha, they indeed do not have a
growth delay." If you accept abnormal growth, then you are going to
eliminate the efforts to support appropriate growth.

(Slide)

Let's move on to a case \-- since you have done such a nice job of
previewing this, I will go through this. This is a former patient of
mine. Little Lauren had a birth rate of 775 grams. Her gestational age
was 27 weeks. Mother had the H-E-L-L-P syndrome, HELLP syndrome, and
that is why Lauren was born prematurely.

Her Apgar scores were 1, 3 and 6, and certainly her first \-- her middle
Apgar score certainly puts her at some risk of long-term sequelae. She
had respiratory distress syndrome, and she was ventilated for 20 days.
She was on oxygen at 36 weeks, which many will use as the definition of
chronic lung disease. And she has apnea and bradycardia in the NICU.
This probably sounds familiar with anybody who deals with preemies;
could be anybody.

For a term-corrected age I saw her in clinic. She had chronic lung
disease, and was still requiring oxygen. She had improved oral feedings,
but she had a little mild hypertonicity or spasticity.

(Slide)

So here is her grid. And I followed her out until she moved at 30
months. This was a wonderfully compliant mother. We did have a
discussion about her feeding practices and the baby's weight here. But
let's take a look at the weight graph for corrected age. This is the
term equivalent. And here she was at two-and-a-half months. And here we
are at seven-and-a-half months. And here she is \-- okay? \-- moving
along very nicely.

This period here of liner growth, she actually grew faster than a
typical population, and she had plenty of calories to do so, and she
went ahead and pretty much stabilized and maximized her linear growth.

(Slide)

Here is her head circumference. Her head circumference has done nicely.
And here is her

Weight-for-length ratio. She was over the 97 percentile here, and mother
was so insistent on her growth, she would not remove the extra calories
from her diet. Eventually as the girl was doing much more self-feeding
when she got older, you know, into the 18-, 20-month range, you could
see that this stabilized a little bit. Okay? So this is my expectation
for growth with good follow-up care, a child with relatively few
long-term complications, and normal developmental outcome.

(Slide)

So as physicians, we don't look just at the growth grid, and just how
they plot on the growth grid for corrected age, but we also can
calculate the average gain per day by their gender, and we take a look
at how they are growing. That is how I was able to discuss with mother
that her baby was growing a little too fast, at least at first.

So this table shows the 50^th^ percentile based on the NCHS growth
charts in weight gain in grams per day. When I see that baby back in my
clinic after, and I see you discharged as an infant, I will calculate
how many grams per day they are gaining, and I will look at them. No one
looks at one set of measurements on a growth chart in providing care.
You need to look at several to look at your trends, and you need to look
at several to see the actual health and nutrition of the baby.

(Slide)

As I said, many premature infants need to catch up. So catch-up growth
refers to rates of growth over time, over a time period, that are
greater than the usual growth rate. The earlier the catch-up growth
occurs, the better. However, we know that frequently ex-premature
infants have poor growth, for multiple reasons that you will hear in the
next talk.

(Slide)

This graph is a cohort of extremely low birth weight boys, less than
1,000 grams at birth, for corrected age at 18 months. The median weight
\-- we saw them actually between 18 and 22 months, and we have
superimposed their weight on the old NCHS curve. The new one really
wasn't out when I was putting this together.

So here is the median. This is the fifth percentile, and this was the
95^th^ percentile. Now, in this population, not every child, or patient
had the same

follow-up, rigorous work with their growth or their intake or their
diet. This is measured from our network across the country, and so in
general we could see that the medians were around the 10^th^ percentile,
and certainly these are the kids down here that you all are finding
eligible for your Social Security.

On the new grid, it frame-shifts downward even more, and I am sorry I
don't have a picture of that.

(Slide)

So I would like to tell you a little bit about where some of that data
came from, and show you some more recent data, some newer data. Recently
we did a presentation on catch-up growth in the extremely low birth
weight infant, in early childhood. And as Dr. Hetland said, I am part of
the NICHD Neonatal Research Network. There are 17 centers across the
country, and we are follow-up centers, NICUs, and follow-up centers,
that look at our outcome of extremely low birth weight infants

(Slide)

The objectives of our growth study were to

determine the incidence of catch-up growth from infancy to early
childhood in extremely low birth weight infants, and to determine
factors related to improved or poor growth over time, and to examine the
relationship between growth and developmental outcome in the extremely
low birth weight

pre-school child.

(Slide)

Our methods included a follow-up protocol at both 18 and 30 months,
including the same items: interview for medical history; growth
measurements of weight, length, head circumference, anorol developmental
examination, and the Bayley Scales of Infant Development, Second
Edition.

(Slide)

Our subjects included 745 matched paired samples. Every child was seen
at 18 and 30 months. The birth weight was 790 grams, plus or minus 128
grams. The gestational age was 26.5, plus/minus 1.9 weeks. The small for
gestational age, based on less than a 10^th^ percent of fetal weight was

15 percent. Male gender, 45; oxygen use at 32 weeks, 45 percent;
prenatal steroids, 82 percent; proven necrotizing NO colitis, 8 percent;
intercranial hemorrhage, Grade 3 or 4 was 11 percent; and any
periventricular leukomalasias was

4 percent.

(Slide)

So this is a graph showing the weight percentiles at 18 months and 30
months in this set of children. And certainly color-coding this less
than third percentile group is what we are looking at today. And you can
see at

18 months 27 percent of these children were less than the third
percentile. And many of these children had caught up by 30 months. And
that was just statistically significant.

(Slide)

The length percentiles, however, did not show the same change. And so
here is your less than third percentile for length, and here it is again
at 30 months, and there was no difference.

I used the 10^th^ percentile, because, again, in the neonatal
literature, small for gestational age is less than the 10^th^
percentile, and so for some kind of consistency that is why we continue
to do that in this outcome data.

(Slide)

In head circumference, less than the third percentile was about 12
percent. And we did see some improvement. We saw some improvement by 30
months.

(Slide)

Because we had such a large sample set, we were able to do a regression
analysis on factors that were associated with the weight less than
10^th^ percentile at 30 months, and we found that birth weight in
decreasing

100-gram intervals was significant. The smaller you are, the less you
weighed at 18 months in general. White race was a factor; male gender,
being small for gestational age at birth; and having cerebral palsy of
moderate or severe disability were related.

We controlled for many, many, many other factors, and I have a slide if
you really want to see the rest of them.

(Slide)

We also looked at the Bayley scales of infant development. There are two
scores that children get: a mental developmental index \-- and that is
what I am talking about here, the MDI. And we looked at their weight
outcome at 30 months to see if it had any correlation. And here is the
weight that is less than the 10^th^ percentile.

If you look at the mean of this mental development mental index, you
will see that it indeed is less; the median is less; the percent of
children who are greater than minus two standard deviations is
significantly higher, as well as those that are less than three standard
deviations, again, significantly higher. So these children are doing
more poorly.

(Slide)

When we look at the PDI, or the motor scale \-- the P stands for
psychomotor; makes it confusing, but this is the motor scale, or motor
ability, again, with your weight less than the 10^th^ percentile. We do
see a difference in the motor scale, difference in the mean, and in the
percentage of children who are minus 2 or minus 3 standard deviations on
this scale.

So it appears in the ex-preemie that growth is good, and growing better
is associated with better performance.

(Slide)

So in summary, we did see catch-up growth between 18 and 30 months in
weight greater than 10^th^ percentile; head circumference greater than
10^th^ percentile. But there were no changes in the length
relationships.

Extremely low birth weight pre-school children then with a weight less
than the 10^th^ percentile have lower mean daily mental developmental
indexes and lower mean psychomotor developmental indexes.

These are the centers that are included in this neonatal research study
and the network.

I am going to close with just this little vignette, and this is
Christie, born at 870 grams. She was 24 weeks of gestational age. She
was born at home. Her outgrowths were 2 and 3. She had respiratory
distress syndrome and chronic lung disease, and she had a posterial
fossa bleed in the brain.

At 22 days, corrected age, we saw her in clinic. She had chronic lung
disease, and was still on oxygen. She, too, had some oromotor dysfuntion
and some hypertonicity. Kind of sounds like that last baby I showed you.

(Slide)

Well, here is Christie's growth grade. And when we saw her initially as
a three-month-old, she was doing fairly well. By the time we saw her
here at 10 months, you can see that her weight had dropped off quite a
bit. And interestingly, as a result, her linear growth had also slowed
down.

We were able to do some changes in health care, feeding practices, high
caloric density, and make some intervention. And there she is, having
responded very nicely to that. You can even see an increase in her
linear growth. And she went on to do quite well.

(Slide)

You can see the effect that it also had on head circumference at that
age, which is why we worry about this so much. And sure enough, she was
able to show catch-up, and look, she is on the very same percentile that
she had been. And we were able to see her go on and do quite nicely.

(Slide)

Here is the weight-for-length ratio that Dr. Mei was referring to, and
this was our \-- obviously our trouble spot there. That is why we look
at the entire graph over time.

Thank you.

(Applause)

***[Presentation]{.underline}***

**by Toni Whitaker, M.D.**

DR. WHITAKER: Good morning. I am also quite happy to be here to
participate in this important discussion. I have some overlap with what
has been told to you already, but hopefully enough to keep your \--
enough differences to keep your interest. But I think the overlap is
nice in that we are emphasizing some of the same points. I have a little
different take on some of it, but it is nice that Dr. Dusick brought up
so many of the medical conditions that I want to talk about.

(Slide)

I also want to provide a little more emphasis on the developmental
problems that premature infants have. And certainly, as you know, the
reason we are here is because we have this low birth weight listing.
Prematurity itself is not a listing, or listed impairment, but the low
birth weight is. Babies born prematurely and with low birth weights have
such a variety of other problems, as Dr. Dusick had introduced.

I want to try to link some of those a little more closely, and just keep
us attuned to how many different sorts of problems these babies may
have.

My focus personally is more on the developmental side, but there are
plenty of links to go around, with weight, growth, weight gain over
time. I have chosen this extremely festive crayon background to remind
us continually that babies will need these continued developing

neuro-developmental skills to have good cognitive outcomes; but also to
grow and be able to do the other things that babies do.

These babies have enough problems that a broad approach to their
reassessment is important over time as well.

(Slide)

Some of the medical conditions \-- Dr. Dusick had a slide that listed
some of the problems that babies would have while they are in the NICU
early on. Those problems don't necessarily all go away when they leave
the NICU. They may have had significant medical problems while in the
nursery that may or may not continue to be significant in and of
themselves, but that still makes a dramatic impact on the baby
potentially.

Neurologic injuries, obviously a big problem developmentally. Medically,
these may feed directly into problems with weight gain, growth over
time.

Some fairly common neurologic injuries that babies may sustain in the
nursery include cerebral hemorrhage, stroke; they may have excess fluid
in the brain, which impairs brain development. They may have infections
early on, and meningitis would be an infection of the brain, or of
cerebral tissues. They may have seizures as a result of some of these
other things as well.

Dr. Dusick also alluded to problems with breathing, respiratory
problems, chronic lung disease. Early on this might be a baby simply
needing oxygen, needing some support while their lungs develop. That is
to be expected, because premature babies don't have fully developed
lungs. If this goes on and if it is severe enough, this may be a
persistent problem even after they leave the nursery.

Certainly requiring supplemental oxygen early on is common. If they have
been leaving the nursery still requiring this oxygen, that indicates how
severe that may be. However, if a baby does get off of oxygen, he still
may have enough underlying problems that it interferes with how well
they are able to do medically and functionally, even if the oxygen is
not there. It may be a little difficult to decide sometimes when that
becomes an impairment, if the big items aren't there

(Slide)

Other medical conditions may include congenital anomalies. Some of the
congenital anomalies may be the reason the babies were born early in the
first place. Problems with the baby may lead to problems with the
pregnancy, may lead to early birth.

Some of these are fairly easy to spot if it is a heart defect, or other
major organ system involvement. Those, again, would probably be listed
impairments early on, may or may not seem significant early on, but
could cause functional impairment down the line.

Congenital perinatal infections continue to be relevant. And again,
meningitis would be one infection that would directly affect the brain
developmental outcomes. But other just general infections can certainly
weigh heavily on how well the baby does overall, as would other major

medical \-- I have listed just a couple of organ systems here. Certainly
the list could be longer, depending on what body system was affected.

Vision and hearing loss, also fairly common in premature babies, again,
varies in degree. May be something fairly mild, but this is difficult to
measure in a baby how well they hear and see. This may be something that
is better assessed at later times. It is very common in premature babies
to have such problems.

(Slide)

These conditions can relate directly to feeding. Let me just talk a
little bit about how premature infants, or infants feed in the first
place. When babies are born at less than about 34 weeks gestation \-- no
hard and fast rule here, but they don't really have the neuro-motor
abilities to suckle well, to nurse, to take a bottle. They will build up
that ability over time. They will begin to learn how to suck. They still
may have trouble, significant trouble, coordinating their suck ability
with swallowing and breathing. So every time you add one more medical
problem on top of that, that may interfere just a little more, so it is
an additive effect with each bit of trouble.

So early one, babies born in a 20-something-weeks' gestation, less than
34 weeks' gestation, they are commonly given a little nasal gastric
tube, little tube down their nose that goes to their stomach, and they
get their food in that way. That is easy to do, you know, it allows them
to grow while you get them big enough so that they can do it by
themselves. Certainly if there are enough problems, they may have
intravenous nutrition as well.

Even when you get the food into them, the premature infants may have
trouble digesting it. They have immature gastrointestinal systems. They
may have some spitting up as a result of gastroesophageal reflex, can't
keep it down. That is fairly easy to measure if you are putting it in
them, so you will just \-- you can measure how much you put in; it is
not easy to measure maybe how much comes back out. So it may be
difficult to figure out how much a baby is getting in.

And this would not necessarily just be in the nursery, but after they go
home as well. Parents are having a lot of spitting up with the babies
because of this immature GI track. They may not quite know how to
quantify that to determine if they are getting in enough and keeping in
enough to grow.

Gastroesophageal reflex might also be known to us as heartburn, could be
potentially painful thing. Infants aren't very able to tell us when they
are having pain due to heartburn. That is essentially what it is, and if
this is painful enough, this may lead to some aversion to feeding if
they are having pain after every feeding.

Parents can adjust fluid volumes. If the baby is doing a lot of spitting
up, they may give less, they may give smaller amounts more frequently.
But that is going to take a fair amount of work on the parents' part to
figure out how much to give at what time to make the feeding optimal,
making follow-up, close follow-up with physicians very important.

(Slide)

Again back to neurologic development \-- and I have sort of said this,
but wanted to repeat it again \-- the babies have to be neurologically
old enough, so to speak, in order to make these feeding transitions and
have enough feeding skills to grow.

Oromotor abilities, or the way they coordinate the muscles of their
mouth to do the feeding, like I said, is a developmentally progressive
thing, so they will learn the skills. However, they are premature babies
with enough medical complications and specific problems with these oral
motor muscle controls, that they are not able to do a good job with
that, even as they grow older. So that has to be really closely
monitored to be sure that they can continue to get enough calories in.

And again, this is not just a tiny baby problem. This can be a problem
as the babies grow older. They are supposed to move on to taking solid
foods, rice and sweet potatoes and wonderful things. Then they are
supposed to move on to table foods, and eat off the table and pick up
their own things. They have to have the developmental skills to do each
one of those steps.

Even simple developmental things like holding your head up, and being
able to take in those foods, premature babies have often developmental
lags, and they are not able to do these things sometimes as soon as
parents expect them to. And so there may be a disconnect in what the
parents will expect, making it, again, very important that they have
good developmental follow-up, so that these things can be coordinated,
explained to the families, so that there will be good anticipatory
guidance provided to families, so that they can lead these babies, and
feed them enough to get them to grow.

(Slide)

Medical conditions may specifically hinder growth. Dr. Dusick alluded to
this, also. Intake my be limited if there are certain problems that
prevent them from getting the food in the first place, neurologic
dysfunction being the number one, in my opinion. Babies with abnormal
muscle tone and control, and if severe this might be called cerebral
palsy, if there are enough problems with the abilities, the baby's
ability to coordinate, maintain appropriate muscle tone.

Just because a baby doesn't quite have enough problems to have a
diagnosis of cerebral palsy, doesn't mean the problems may not be there,
though. They still may have enough trouble that it interferes with how
they are able to take in nutrition.

Breathing difficulties such as the bronchopulmonary dysplasia. Continued
need for respiratory care may interfere if the baby has to choose
between breathing and eating. If there is enough trouble that they are
not able to do both at the same time, they may have to choose the
breathing, which would make sense, but there may be some limits as to
how much they can take in if they tire out too early with their feedings
because of breathing, or because of, say, another congenital problem,
such as a heart defect.

Genetic syndromes also can limit the growth potential just by their
nature, that would be a limit of their genetic potential.

(Slide)

Just as conditions may limit how much a baby can take in, they may cause
the baby to burn excess energy or calories after the calories are in
them. So getting the calories in is not the only problem necessarily.
Certain disease states or conditions will cause them to burn up that
energy faster, so that may mean that a baby will need extra calories
above and beyond to search for that catch-up growth. But those are the
very babies that may have trouble getting these calories in in the first
place. So just a little list there.

(Slide)

Developmental disabilities link, again, both to the medical conditions
that may be common in premature infants, as well as to their growth
problems. It is widely known that developmental disabilities are much
more common in low birth weight, and small-for-gestational age infants.
These babies may have diagnoses early on that will allow them to \--
that will show that they have a functional impairment, based on the
medical conditions, but possibly not depending on severity, so you have
to just keep watching these developmental conditions over time to see
how much they interfere as well.

Select developmental problems include just general delay; cerebral
palsy; language problems; mental retardation; learning disabilities.
Delays, possibly cerebral palsy, could be diagnosed in the first, second
year. Unless the cerebral palsy is very severe, that is not going to be
diagnosed in the first months of life; that is going to take a little
while, usually.

The language disorders; mental retardation; learning disabilities are
much later diagnoses, usually. Calling attention to the fact that we
need to think when we are deciding how to reassess babies born at low
birth weight, when would be an optimal time to do that? It may not be at
the one-year mark, because there are less good ways to measure these
outcomes at the one-year mark, whether corrected or uncorrected age is
used.

Obviously, also the earlier you can identify some of the sources of
problems, as with growth, the developmental problems earlier that are
identified, the better, because you can make the interventions that will
be

long-lasting.

(Slide)

I have also the pleasure of presenting some information from AUCD, and
AUCD, if you are not familiar with them \-- us, is the Association of
University Centers, representing nearly a hundred university centers
specializing in developmental disabilities.

We are also presenting separately some other information, more on the
developmental side, and I didn't want to get too far off into
developmental issues, but wanted to call attention to a few findings.
But this is to be presented separately to Social Security.

(Slide)

AUCD has had a collaboration with Social Security Administration for the
past six years. A series of case reviews was done on low birth weight
infants around the

time \-- or just after the time of their continuing disability reviews
at that one-year mark; they ended up being somewhat later than that, of
course.

Saw nearly a hundred kids in 2000 and 2004, and this is just a
compilation of some of the findings. Again, there were more findings,
but this seemed relevant to the topic at hand today.

We did multidisciplinary assessments with these babies. Some children
are already receiving services, both for medical problems, which would
include growth, but also for developmental problems, usually in the
birth to three state programs.

Despite this, a huge number of additional kids were found to really be
thought to benefit from enrollment in some additional services.
Eighty-plus percent of \-- now, some of this would have included in kids
that already were in services \-- 80-plus percent of these kids, low
birth weight, small-for-gestational infants, were thought to need
developmental services. A significant percentage \-- around the 50
percent mark \-- were thought to need medical services.

And this is just to call attention to these babies have lots of problems
potentially. They may or may not have had some listings for some of
these other medical conditions. Initially they may have just been in
with low birth weight to start with, but continued to have significant
other problems.

(Slide)

Part of the AUCD case reviews was about the issue of correction. Dr.
Dusick brought up the issue of correction for prematuring. This varies
somewhat. I will give you the quick version. I could talk a while on
this, but \-- little passion of mine, but I am going to limit to one
slide.

In general what you are looking at with correction is adjusting the
baby's age for the degree or prematurity. Until that degree of
prematurity doesn't seem to matter anymore. So if a baby is four months
early, when they are 12 months old, they are really going to act, look,
be growing more like \-- what did I say \-- eight-month-olds \-- 12
months, yes.

That same four months, when they get to be

36 months old, is a much smaller percentage of their total, so it seems,
you know, there is some point in there where it becomes less important.
When exactly is that point where it becomes less important? Now, that is
up or discussion.

Research findings vary somewhat. They vary between growth and
development. They vary between different streams of development, say
motor skills versus language skills versus fine motor skills or thinking
abilities.

There is also some inconsistency in clinical practice, so that one
center, or one clinician might correct a baby's age for growth, and/or
development up to two years. Others may correct to three. Even within
the state birth to three, early developmental intervention programs,
practices vary somewhat, and eligibility criteria varies state to state.
Dr. Dusick and I were discussing before we got started today, her state
will classify a child as being eligible for these early intervention
services if they

are \-- correct me if I am wrong \-- 20 percent behind in two or more
areas, or 25 percent behind \-- I am using the word "behind" loosely
here \-- 25 percent in one single area.

Tennessee, where I am from, uses 25 percent behind in two or more areas,
and 40 percent behind in one single area.

So there is a big gap in there in Tennessee \-- and other states, too; I
don't want to just pick on Tennessee \-- but there is a gap of kids that
wouldn't be served in Tennessee that would be served in Indiana. Each
state varies. It would be lovely \-- I would love for us to have some
sort of coming together \-\-- I know you are not exactly the group.
Wouldn't it be great if all the states could serve the same sets of
population.

It would also be nice \-- I mean, this may be a step we can work toward
with this particular group \-- if those criteria for eligibility could
match Social Security's criteria. I don't have that answer, though. I
just want to bring it up as a topic potentially for discussion.

The risk of correcting too long is that you might miss some true delays.
If you correct and say, "Oh, well, you're two years old now, but you're
really acting \-- you really, if we correct your former year
prematurity, you should only be acting like a 20-month-old, and
therefore you are doing okay." That might miss a true developmental
disability or growth problem in a child that you correct too long. If
you don't correct for the prematurity, the risk would be that there
would be a \-\-- delay, when really the baby just needs some time to
catch up. So again, I don't have the answer to this, but I want to call
attention to the fact that there is not real consistent use. I do have
one possible solution.

Within the AUCD's policy recommendations \-- and again there is a longer
version of this coming \-- but the basic facts that were concluded,
basic thoughts concluded, were, as I said several times, these babies
\-- low birth weights, small-for-gestational infants \-- are at very
high risk for multiple medical conditions, including poor growth. They
have often functional developmental impairments. They really deserve
broad reassessments at the time that they are reassessed.

(Slide)

One possible solution of being able to broadly assess these babies
somewhat better, is to extend that diary date, or the time for their
continued disability review, to a later time \-- again, the number is
not necessarily set in stone in my mind about what would be an optimal
time, but the one-year reevaluation does limit the amount of information
you could give on that baby's true functional status \-- which would
include broad assessment of all developmental abilities, including the
growth and the other medical problems.

If they were reassessed later, that might reduce the need to worry about
the correction factor, that I have already said is sort of problematic
if everybody is not using it consistently. It would also be really
important for the very smallest babies \-- not necessarily all of them
potentially, but the very smallest babies, who are at most risk.

(Slide)

I have just a couple of quick points on health care. Critical to all
babies who have multiple medical problems is access to appropriate care.
Dr. Dusick showed the growth chart of the child who had poor growth
starting, and then had medical interventions and did better. What if
that baby didn't have access to good medical interventions? That growth
probably would have gotten worse and worse.

So, just in summary I would like to emphasize that babies will need good
care from potentially a variety of different folks, their primary
physician, to follow their growth, to determine who else they may need
to be sent to, specialists which might include other physicians,
nutritionists. Critical to all of this would be access to good insurance
to get the access to the care that they need.

(Slide)

And just a final slide on one more push for developmental services. They
should have access to the early intervention programs to make the most
of their development. If they are developing well, they will probably
have some links to better growth as well.

Some of the direct services, developmental direct services, might
include specific things tailored to feeding difficulties, such as
occupational therapy, other services that could specifically impact
growth, just based on feeding alone.

And thank you, very much, for your attention.

(Applause.)

***[Presentation]{.underline}***

**by Maureen LaRosa**

MS. LaROSA: Good morning. I don't need a pointer, because I don't have
any slides for you. I didn't prepare to speak this morning, because I
was here for moral support for Ms. Ward.

And she is a mother, who, her child is in our clinic. And like the
clinics you have heard about today, our clinic is very similar to that.
And I am a nurse who works in the clinic, and I called Ms. Ward because
she hadn't arrived, and unfortunately they had a medical emergency last
night, so she wasn't able to come, which is, of course, one of those
things that happens with families who have high risk children, and just
hard lives.

I am going to try to do justice to her conversation with me on the
phone. I asked her about what she wanted to speak about to you, and what
she wanted the Social Security Administration to understand about her
life.

Brandon was born at 25 weeks. He was one pound and 12 ounces when he was
born. We follow children from four different hospitals in the Atlanta
area. He was born outside of our usual pickup, but we have very similar
pickup criteria to what you have heard about; but we also have a
referral in, so if a pediatrician or family hears about our clinic, and
they have a child who meets our admission criteria, we will take them
in. So Brandon came to us just before he was a year old.

He had had a very extensive, long hospital stay. He was in the hospital
for about five months. Mom took him home, and the hospital has done a
very good job teaching her about his feeding. He was a very poor feeder.
He had had very severe lung disease. He didn't grow well in the nursery
because of the high caloric burn-off from his lung disease. And they had
given her CPR training.

So she took him home, and eight hours later he stopped breathing, so she
had to do her little CPR and call 911, and he was back in the hospital,
and came to one of our hospitals, and then they referred him to us. He
had a severe sepsis and was just quite sick.

Brandon has had a difficult course. He is now three. He is weighing 26
pounds. He unfortunately did not have that lovely growth curve that you
have seen, but we do have a number of children who do this, and that is
that they are poor growers. And this is a very wonderful mother; she
works very hard at trying to fee him. We have had occupational
therapists work with him, speech therapists work with him, nutritionists
work with him.

He is now in special needs pre-school. He does run around; he walks; he
talks. He is delayed. But he still has to be tube-fed 10 hours every
night as a supplement to the fact that he just does not tolerate eating
much food, and so he doesn't. So that helps keep him going.

Mom wanted you to know that she did find that going at the one year for
reevaluation for continuation of SSI, she did not find that to be a very
difficult process; she was able to manage that. And she is still
receiving SSI for him.

She is very grateful for that money. That has really helped to hold her
family together. Because of all of his health issues and the amount of
time she has to put into trying to feed him, she is unable to hold a
job. I know she just can't do it. And every time she thinks he is about
ready to be able to manage, things happen, and she has had to pull back
again.

This year \-- we had discouraged day care or any kind of group care
because he really couldn't handle infection very well. This year we were
able to, you know, help her to get him into the public school system,
and he is doing like three hours in a pre-school program, and they are
helping with his feeding and trying to work on that, also.

She did mention that there are, you know, when you have a child who
doesn't eat, you don't sleep much. She, you know, with the 10 hours of
feeding at night, she says he sleeps in her room, and she keeps one eye
on him all the time, because she is just afraid that something will
happen with one of the tubes, or he will get caught up in something. And
it is very anxiety-producing, which does \-\-- feedback.

And then you have a child who you bring home and then they die, and then
you have to resuscitate them, and they come back to you; and then they
still have all these problems. It is very hard not to be very anxious,
and that anxiety, and they are not growing; you think people are
evaluating you and judging you. So you have to try and force them to
feed \-- some of these parents spend enormous numbers of hours trying to
make these children eat, which only makes them \-- you can't control a
toddler and what they put in their mouth, so that is a setup, making
sure that they don't eat. And no matter how much we work with the
parents to try and back off, they are just so anxious. That makes it
much more difficult for them.

She felt that there are lots of services, but it is \-- even here in
town we have the Marcus Center, and they have a feeding clinic for very
severely malnourished children who don't eat well. They have a very
intensive program. But you have to have a family who can be there every
day for extended periods of time to work with them. It is very
behavioral, and it is expensive.

So then if you are not able to do that program, or your child is not
that severe that you go that route, you are doing it sort of lots of
pieces, with lots of different people's advice and help. It is difficult
to navigate.

One of the things we have noticed as far as your one-year review in our
clinic with a lot of our children, is the kids who are not severely
growth retarded, they are just sort of eking along, they are doing okay
with drinking a bottle, that right around one year, when you go to put
those kinds into table food, everything falls apart. And we see a steady
decline in growth on some of these children, because they have so many
oromotor issues.

A lot of these children, because of all the negative things that have
happened in their mouth, they gag very easily; they have had the reflux,
they associate eating with pain; they just \-- they choke easily; they
won't let you brush their teeth. So they don't like textured food. And
so just when they are running around and need to increase calories, they
really start having some feeding issues.

So when you are doing that one-year eval, sometimes you are going to
miss a group of kids that four months later are really going to start
sliding on their growth curves, because of a lot of the oromotor issues
that you have.

We work with occupational therapists and speech therapists. I don't know
about other places, but here in Atlanta they are find, good OTs and good
speech therapists, that can work on these issues with the families. And
so, you know, we \-- there is always hunting for people who can help
with this.

Parents don't like it when their kids won't eat, and they won't take
textures, and again, that sets up a bad cycle between the parent and the
kid and eating. We probably have, what most of the literature shows, is
about 25 percent of our low birth weight kids will end up having some
problems with feeding and will fall off the growth curve.

Ms. Ward has continued to work with Brandon. And he is a very charming
little child, and she knows that he is going to be delayed, but is
working very hard with him on these issues, and continues to do
everything that everyone asks her to do. She still, you know, just gets
him to grow at a very poor rate, but he does consistently grow, and he
probably will at this point be expected that he will always be very
growth-retarded because his potential has probably been damaged at this
point from not being able to grow for such a long time. But you know,
she continues to try.

So \-- all right?

(Applause)

DR. HETLAND: I have about between 10:45 and 10:50. Why don't we
reconvene at five minutes after 11:00, and we will have our discussion.
Thank you.

(Recess)

***[Audience Questions and Discussion Session]{.underline}***

DR. HETLAND: Thank you, this morning, for trying to enlighten us. I
think the audience \-- certainly I did \-- I have been doing growth
charts since I was a pediatric resident, and it is technically very
challenging to plot a child correctly, and do the appropriate
corrections for prematurity. And so when we try and to that as a
program, it is not easy, and likewise, doing our CDRs at 12 months of
age, I think we are hearing may not be the best idea.

So I am hoping that we have more of an interaction today, because I
would really like to hear more from the audience, particularly those of
you who are in both the regional office and in the DDS, in terms of what
problems are you seeing when you try and do a CDR on these

ex-preemies? and questions that we can ask our audience.

Dr. Dusick was most polite in not explicitly saying, pointing out that
we are not, as a program, using the most up-to-date growth charts.

(Laughter)

DR. HETLAND: But that has been our theme for the last two days, we are a
couple decades behind. Okay?

Likewise the correction, R. Palms says to correct for prematurity for
weight up to 12 months of age, but up to 24 months for developmental
age. And why that came into effect, I don't know, but it seems to not be
very consistent.

I am going to open it up for questions for the audience for our
panelists. And likewise our panelists may ask questions of us as an
audience.

Mr. Eigen \-- oh, forgive me. For the benefit of our transcriptionist,
every time you in the audience speak, please identify yourself. She has
her back \-- she doesn't see who is waving their hand and talking. I
will except, though, ex-speakers do not have to identify yourself. I
don't have to identify myself. Everybody else, do it.

MR. EIGEN: My name is Barry Eigen. I am the Policy Officer in the Office
of Disability Programs. We are the people who write these rules.

I have a couple of comments, preliminary comments, and then I have a
question for Dr. Dusick about a couple of your slides.

First of all, we tend to talk in abbreviations. For those of you who
don't know what some of the abbreviations are, TOMS is an abbreviation
for the manual that our adjudicators use. It is a gigantic manual that
our adjudicators in the state agencies that make disability
determinations for us use to follow our instructions.

CDR stands for continuing disability review. And some of you may not
know this, but there is actually a law about low birth weight children,
and when we have to do continuing disability rules, the Social Security
Act requires us to do a continuing disability review at age 1, unless at
the time we first allowed a child, we can determine that a later date is
more appropriate. But basically we are supposed to do them at age 1
whether we think it is right or not.

The third thing, I just want to point out to everybody about this AUCD
project is that they have submitted their comments to us, and those
comments, the report of their comments is on Social Security's web site,
so if you go to www.socialsecurity.gov, there is a place to click called
Our Program Rules. And then on that page there is a place to click
called Comment on Developing Rules, and from there you can figure out
where to find their comments. It is a very interesting document, which
includes data about this project you heard about this morning.

Okay. So here is my comment to \-- my question to

Dr. Dusick: You had two slides about the Bayley, and just before you had
the slides, you had another slide that

said \-- I can't remember what it was about, but it was something \--
but you made the point that you had data that was sufficiently refined
that you could break it out into 100-gram increments to see what the
relationship would be, and whether there was a relationship as you went
down the way.

You didn't say the same thing about the Bayley slides, and I was
intrigued by them, particularly the developmental one, because it showed
that kids below the

10^th^ percentile \-- I am making up the number, but it was around
20-something percent, or a couple of standard deviations below the mean,
or had some other significant score finding, and I wondered whether you
had the data broken out by 100-gram increments for those kids, so that
we could see whether, as the kids got lighter, got smaller, the
developmental scores would either get worse, or stay the same, or
whether there was any relationship.

DR. DUSICK: That is an excellent question, and we have not run that data
by birth weight group. But I am writing the manuscript now, and I will
take that under advisement to include in there.

(Laughter)

DR. HETLAND: I had the same question.

DR. FERNOFF: I'm Paul Fernoff, a medical geneticist here at Emory, here
in Atlanta, and one of the Atlanta Regional pediatric consultants.

A couple things. We use, we tend to use currently the less than
1200-gram category, it is automatic, and then the 1200 to 2,000 grams,
NSGA. And it seems to me most of the folks in the neonatology world tend
to use the, for their definitions, less than 1500 is the extremely low
birth weight.

DR. DUSICK: Very low birth weight.

DR. FERNOFF: Very low birth weight. So one of the things would be to use
the categories where we have some outcome data to back up some of the
rationale if there are some changes in the rules \-- because I am never
quite sure where that 1200 came from, but it would make more sense

to \--

DR. HETLAND: Search me.

DR. FERNOFF: Okay.

(Laughter)

DR. DUSICK: The World Health Organization divides things in 250-gram
increments, and you might see some of their outcome data, look at the
1250-gram infants.

DR. FERNOFF: I don't remember exactly where the original ones came from.

And let me ask about \-- again, the real world \-- in terms of reviewing
the claims at the regional level \-- and Monte and I discussed this a
little bit \-- on these

one-year diaries? and my impression is some of these children do get
reviewed really at a year the DDS gets them, and I am always amazed that
at that time many seem to be doing okay \-- but of course we don't have
really the developmental status that we need at that point, too.

On the other hand, some of the children, even though they are supposed
to be reviewed at a year, may take two years, three years, by the time
they are found and reviewed. So one suggestion would be, using low birth
weight as a \-- especially the extremely low birth rate babies, and
those, the SGA babies, too, we know those children are at the highest
developmental risk, and by really allowing a disability period long
enough to really assess them \-- whether it is two years or three years,
whatever that is \-- and then really becoming more of a proactive \-- we
were just talking a little bit about it \-- because at that point it
assures that those children are going to be able to stay in a care
system where they can get the optimal care, and it really gives it more
of a preventative kind of approach to what we are trying to do, I think,
\-\--, so \-- my suggestions.

DR. HETLAND: Dr. Becker.

VOICE: \-\--.

DR. HETLAND: Oh. You are next, then.

VOICE: \-\--

DR. HETLAND: Wait, wait, wait, it is not on.

(Pause)

MR. FORCE: Jud Force, from the Federal DDS. I was around when that
standard was written, and it actually was a standard that was
recommended by an expert panel of professionals, pediatricians,
development pediatricians, people how have worked with children with
very low birth weight. And this was written actually as part of the
response to the Supreme Court's requirement to rewrite a standard for
children.

And some of the experts wanted actually an automatic allowance at 1500
grams. Others on the panel wanted the standard written at 1,000 grams.
And 1200 was a compromise.

(Laughter)

DR. HETLAND: Okay. How about the SGA? How did we end up at three percent
when the rest of the world uses 10 percent?

MR. FORCE: Well again, I think that \-\-- experts were using literature
which would suggest 10 percent being the standard, others feeling that
three percent was the \-\-- standard, and the agency accepted the third
percentile rather than 10 percentile for small-for-gestational age.

DR. EIGEN: \-\--

MR. FORCE: And as Barry is pointing out, I think in terms of it being
the severity standard, feeling that a third percentile was preferable to
the tenth percentile.

DR. HETLAND: And the third percentile is more than two standard
deviations from the mean.

MR. FORCE: That is right.

DR. HETLAND: Also Dr. Fernoff and I had a conversation that using the
10^th^ percentile for SGA, that some of those are completely normal kids
who may just be twins, or have other reasons for being suppressed while
they are still in utero, and by \-\-- age, that those kids \-- since
they are basically normal \-- will have caught up growth-wise. However,
if we use the third percentile, we are more likely to be catching the
children who have chromosomal disorders, or congenital infections, or
other reasons where they will have ongoing problems.

So it is a smaller net, but focusing, I think, on the more involved
population.

Dr. Becker \-- oh.

DR. DUSICK: Monte, I have a question. What fetal growth grid is used as
the standard for determining that third percentile, or the 10
percentile, for that matter.

DR. HETLAND: Jud?

MR. FORCE: I believe we were using the Ballard neonatal growth chart,
which actually, as I understand it, was taking figures from the CDC, or
NC \-\-- growth chart. It really was not a population of prematures that
was followed from birth to 12 months, as I understand it. The Ballard
was based on more the standard growth charts that were in place at that
time.

DR. DUSICK: Yes, the Babson and Benda curves, if I am not mistaken.

DR. HETLAND: I think that is right, and \--

DR. DUSICK: This is a subject of discussion in neonatal groups as well,
of which standard to use.

DR. HETLAND: Which would you recommend?

DR. DUSICK: Well, currently \-- we talked about this long and hard with
the NICHD, and we are using the Alexander curves. They are based on a
U.S. population. And the big drawback is there are no head circumference
measurements, and \-- but that has been a limitation with many of those
charts.

DR. HETLAND: Dr. Whitaker, what do you use in your clinic? Do you know
off the top of your head?

DR. WHITAKER: By the time they come to me,

we use a corrected age and plot them on the standard CDC charts.

DR. HETLAND: And you do the same in your \--

MS. : \-\--

DR. HETLAND: Okay.

MR. : \-\--

DR. HETLAND: You don't have a mike. We don't who you are.

MR. BUDA: I'm Frank Buda. I work at the regional office. I thought that
programmatically we are supposed to use whatever the treating source
uses, so that if we get a chart and it is using an Alexander to
determine just

small-for-gestational age, that we are supposed to use that. We got a
chart, and they were using the Ballard. We are supposed to go with what
they are using. I thought that was how the program worked.

Now, if not, then we need to know we are supposed to use either one or
the other. But the way I thought we were doing it was to go with what
the treating source uses. Are we going to change that, or \--

DR. HETLAND: I think we have to \-- we are talking about apples and
oranges in terms of in one sense you saying what graph to use as to how
to identify SGA infants at birth, and then maybe growth during the first
year of life. But I think that for after the first year, our \-- for
growth impairment it says use the most recent CDC-generated growth
curves. And so if you have a chart that the doctor was not using the
most recent, I would have some programmatic problems with that.

MR. BUDA: But do we have the program statement on the SGA of what we
should do, because I thought that whatever chart the treating source,
the neonatologist was using, was the chart that we went by.

DR. HETLAND: I don't know that that has ever been programmatically
spelled out, but it would certainly make sense.

Yes?

DR. DUSICK: I have a comment to make about that. The treating physician
would use the term conventionally SGA to mean, though, less than the
10th percentile, rather than less a third. And so I think if the program
is going to have a different definition, they will have to choose a grid
to use, or you may want to rephrase that term, and not use what is
considered a standard nomenclature in neonatal literature. And that is
probably the point that I was making just by convention. So extremely
small-for-gestation, or something like that.

MR. BUDA: I was talking about the grids that are in the chart. In other
words, we often get a record that has a copy of whatever standard they
are using, and then we can plot, or look at how the weight was plotted
on that standard. That is what I was referring to.

DR. DUSICK: Okay.

DR. HETLAND: Ms. Connell?

MS. CONNELL: This is Regina Connell. I work in the Office of Medical
Policy. There is a chart in the PALMS, the operations manual, that
indicates what we would calculate to be under the third percentile,
based upon the weeks of gestation. So there is a chart in the PALMS that
says, "If you are at 36 weeks gestation, and you are under this
particular weight, then you are under the third growth percentile for
that particular child."

DR. HETLAND: Dr. Becker.

DR. BECKER: Well, I have three questions. The first would continue this
discussion. The most important thing in the growth is the outcome, and
the outcome which probably affects that child the most would be the
developmental outcome.

Certainly in my earlier years in nutrition, there was a major \-- the
best correlation to outcome was the change in heads circumference. And
so we talk a lot about growth and weight gain, but would head
circumference be a better predictor of where that baby is going, and
should that be used? I mean, you did show the change in head
circumference correlating with growth, but it does sometimes, and
doesn't other times. And that will lead into my next question.

DR. DUSICK: Probably Dr. Whitaker and I can both comment on this.
Because of pathology, because of neurologic insults, it is difficult to
always use head circumference.

The data that I showed you for MDI and PDI do hold out for head
circumference. I just didn't choose those graphs to show you, but they
do.

You have to consider a couple more factors, and by and large, when the
head circumference is growing in a percentile range, usually between the
10^th^ and the 90^th^, that has shown the best for outcome.

When we look at head circumference clinically, we are also looking,
though, at rate of growth. Too rapid growth could mean hydrocephalus.
That wouldn't necessarily, you know, be good developmentally. On the
other hand, certainly if the growth flattens out, and we don't see the
growth, usually there has been prior damage.

So it is more difficult to use head circumference, but we do clinically
a lot. Okay. Medically we use less than the second percentile as the
term microcephali, and there are some good genetic papers that talk
about that.

And it is much easier to use now that we have the new NCH growth grids,
because they go down lower than the old ones did.

DR. WHITAKER: I agree. Clinically it really may matter, and we do see
\--

DR. HETLAND: Could you speak a little more in the microphone.

DR. WHITAKER: We see the correlation clinically, but I agree, it is not
necessarily an all or nothing. For each individual child it could be
very different, but should be a correlation generally.

DR. HETLAND: I think in the PALMS, if you have a child whose head
circumference is more than three standard deviations from the mean, that
is an allowance because it is thought that that is consistent with what
will be microcephali, and ultimately mental retardation. But we are
talking really, really small.

DR. BECKER: Well, the major reason I asked that is from the pediatric
endocrinologist point of view coming in at the other end of the
spectrum, there is a lot of interest now in fetal and \-- not fetal, but
neonatal \-- maybe even fetal nutrition, and neonatal nutrition, and the
opposite problem, not under-nutrition, but over-nutrition, and a major
cause of Type 2 diabetes maybe later on rather than Type 1.

So there are certainly those children who are not meant to be taller and
fatter, and probably the most important thing as we look at growth is
the BMI or weight/height ratio, whatever.

So how would you bring in those children that we, as pediatricians \--
and I plead guilty \-- have overfed our children in order to get
catch-up growth in patients who shouldn't get catch-up growth, who we
now know are destined to get the metabolic syndrome, if that exists, and
diabetes?

And I was just wondering if the head circumference is growing normally,
and the patient is still relatively short, whether that might separate
those out.

DR. DUSICK: That is the question, I think, of many of our \-- the
subject of many of our research studies.

One, I look at the linear growth, what I showed you today was that
between 18 and 30 months many of the children have reached their growth
potential. There are plenty of older follow-up studies that have shown
that linear growth can continue on into adolescence, that the children
may still be having catch-up linear growth. I look very closely at
linear growth.

I also use the weight-to-length ratio over time, and in my clinical
practice I don't want to see these weight-to-length ratios that are as
high as that first girl that I showed you. In general we do keep them
usually between the 10^th^ or the 25^th^ percentile, is clinically at
our institution in general what we are looking at for weight, for
length, in the little ones.

We talked privately, Dr. Mei and Dr. Whitaker and I talked privately
before we sat down here about infants \-- and especially under one \--
the weight of the head, and the head circumference becomes important in
the body weight measurement. And that is why sometimes your
weight-for-length measurement cannot be useful, because you may have a
child who has too big of a head, or you may have a child who has a very
small head. And so the weight-for-length ratio may not be helpful to
you.

In terms of are we overgrowing some children and leading to long-term
complications, I think that a lot of those studies I would like to see
done better on this population, and I think we are still looking at
that.

DR. BECKER: Yes, and the betterness and part of the problem, being the
mentor of some people in my division who are trying to \-- not
prospectively, but mostly retrospectively, is that it is difficult to
separate out in manuscripts a small-for-gestational age baby and a low
birth weight baby. And they probably have very different outcomes. And
so \--

DR. DUSICK: Then maybe the NICHD neonatal \-\-- would be the perfect
place to do that prospectively.

DR. BECKER: Yes.

My next question is for Dr. Mei, and that relates to the growth curves.
I have never understood why there are no BMI charts for
under-two-year-olds.

DR. MEI: It is a very good question. Why does CDC \-\-- growth child
\-\-- for body mass index for ages start at age two, instead of, you
know, from birth to \-- with other curves?

The reason \-\-- decided to start at age two, it is for majority for two
reason. One reason, during infancy the BMI, it is very unstable. So they
are rising during infancy very quickly. And then after 11, 12 months,
and they drop very quickly. And then, you know, from the curve I show to
you from two to 20 years old, also have a downward side, and then come
back.

So for statistical, you know, technique, if you are trying to \--
because we smooth all the curve from beginning to the end, it is very
hard to smoothing a curve like \-\-- to actually, precisely capture the
pattern. So that is \-\-- more \-\-- under our, you know, and more
advanced technique to capture the growth.

Under the second one, the reason we start at age two, it is \-\--
clinically you switch from imagine kids lying down to standing up.
Usually they switch at 24 months. Because the body mass index calculated
your weight in kilo, divided by standing height, \-\-- it is actually
standing height. But if you imagine the kids lying down, the difference
would be lying down would be higher, the measurement, usually from
NHANES data \-- it is not consistent. From NHANES one, the difference,
it is about 1.2 centimeter. So imagine the kids lying down were
potentially 1.2 centimeter taller than you imagine standing up.

So, but from NHANES 3, the difference, it is like a point 8. So in order
to, you know, consistently, you have to do some adjustment for the
height. You either imagine lying down, or standing up. So that is why
major \-\--

DR. BECKER: That would be true for weight-for- height, too. It is a
problem we have in everything we do.

DR. MEI: Yes. But that weight-for-height, that we separate the chart,
weight for ounce, and the weight for stature.

DR. BECKER: And what about the Ponderal index that the British use?

DR. MEI: The \-- yes, Ponderal index, means the

\-\--, go to \-\--, right?

DR. BECKER: Right.

DR. MEI: Yes \--

DR. BECKER: \-\--, I mean, would that help us in evaluating?

DR. MEI: We actually evaluate this from the literature that is showing
actually the border mass index, it is the \-\--, it is the best,
particularly consistent with \-\--.

DR. BECKER: My question regarding the growth charts, I recognize that
the NHANES III was excluded. That is what you said this morning, I
think, right?

DR. MEI: Only partially. Only in the weight date. But the height and the
month data still in the \-- in the, you know, months-for-age, weight-for
\-\--, and you know, weight for age until age of six. Because the only,
you know, impact after age of six for \-\--, weight-for-age after age of
six, and the BMI for age after six.

DR. BECKER: Then why are the 90^th^ percentile and the 95^th^ percentile
on the new growth charts for weight so much higher than they were in the
old ones?

DR. MEI: So the new one, you mean, some are higher than the old one.

DR. BECKER: In other words, they can be fatter and still \-- what was
more overweight before is not as overweight now.

DR. MEI: Yes, that is very good question. But also it is due to all the
date we combine, because the '77 date it is mostly only used \-\-- one
data. But the new growth chart we combine all those five together. And
some of those data in NHANES II you can see the problems of \-\-- I some
older kids in \-\-- do slightly increase. So that is maybe \-- another
thing is due to the smoothing technique. We used a different smoothing
technique, so that shall be slightly difference in the curve.

DR. HETLAND: On the weight-for-length measurements, the curves for the
first two years of life, is that data including or excluding premature
births, and how they were growing, as well as children with chronic
disease?

MR. MEI: We \-- because in the NHANES data we don't have the
gestation-for-age, so we \-\-- to exclude the very low birth weight,
which is less than 1500 gram, for all those curve, months-for-age,
weight-for-age from birth to 36 months, and the weight-for\-\--.

DR. HETLAND: So is it inappropriate for us to be using weight-for-length
for these premature infants, and assessing their growth in that way, or
should we just stick with weight-for-age, which can be corrected more
easily?

DR. MEI: I would say maybe you \-\-- combination, because each indicator
only adjust to a particular issue. For example, the weight-for-age only
consider your weight and age. But the weight-for-months is considered
just a example I gave to you. Your weight-for-age is on the third
percentile, also months-for-age also on the third percentile, but when I
look at your weight-for-months, it is actually close to the average.

DR. HETLAND: Okay.

DR. DUSICK: I think the problem is in looking at just the few data
points that you are going to have from term-corrected-age to 12 months,
because if a child is growing poorly \-- let's say length is less than a
third; weight is less than a third \-- the weight-for-length indeed will
be within the normal range. It may be that they are just starting their
ascent, and they are just beginning their catch-up growth \-- and as you
can see, we saw catch-up growth into toddlerhood and almost at three
years of hood, and you may just be crossing that line where they are not
yet pushing their linear growth to their genetic potential.

Okay.

And so when we look at clinically, I am looking at mom's height, dad's
height, sibling's height; where the baby is now; how does the baby eat;
what do I think their needs are, and I am looking at all of that. And a
snapshot is difficult; it is very difficult.

DR. WHITAKER: And I agree, your snapshot may be better with more
information, so having that extra type of information could help. It may
not be always as conclusive as you would like, but you would at least
have some different information from different aspects.

DR. HETLAND: I will throw this out as a bombshell. In the NPRM for
Digestive for Children, 10508 for malnutrition, it proposes \-\--
malnutrition judged by weight-per-length less than a third percentile,
for children under two; and for children over two, using the BMI, less
than a third percentile. And I am hearing here some concerns about that
that may not be the best way to go.

Pros and Cons. But I mean, we have already published that as meaning to
implement that. We may need to go back and change that.

Yes?

MS. BROWN: Nancy Brown, from the Regional Office. I guess my concern \--
and I am not a statistician, so I don't really know how meaningful this
is \-- but in most cases when we are looking at child's impairment, we
are comparing this child to the child without an impairment. But you are
telling me the growth charts are \-- have in the population that you are
considering children with chronic conditions; that is part of the
population, is that right?

So is that really appropriate, or should we be looking at a different
percentage or something if we are really comparing this child to this
population that does include children with chronic conditions?

DR. HETLAND: Tough question.

DR. MEI: Indeed, it is a tough question, because ideally that is why
\-\-- through the concept in the beginning, you know, standard verse
reference.

So ideally where we have data sets, have all the list of the \-\-- from
the chronic disease condition, whatever, you exclude them. And then
another set include them, to see how much impact, how much difference
than which one you decide you are going to use.

But in the reality, in the data source we have, we don't have those, you
know, privilege to doing this.

DR. HETLAND: Just as an aside, one of our afternoon speakers, Dr. Bess
Schoen, is a pediatric gastroenterologist, who takes care of kids with
failure to thrive, particularly children with HIV, who are failing to
thrive. And I believe she is also going to be talking about how she uses
BMI in assessing these children with special needs.

Yes, Dr. Becker.

DR. BECKER: I just wanted to comment on your question about
malnutrition.

DR. HETLAND: Please do.

DR. BECKER: I started my life in nutrition, before I did endocrinology.
And I think one of the concepts that we have that malnourished children
are thin, is incorrect. Malnourished children may be thin very early on
in their nutritional deficits, but once they reach equilibrium, which
means that the body is going to try to adapt, and the first thing it
does is stop growing, because a lot of other body functions are more
important than growth. So you will get a very malnourished child, who
will end up normal weight-for-height. And so being underweight for
height is a very early, maybe after a few months, six months of
malnutrition. And then they will be, according to these growth curves
there will be absolutely normal BMIs, or absolutely weight-for-height,
but then under-nutrition has not been corrected.

DR. HETLAND: Thank you. We will take that to

heart.

Do you have questions for us? Like why are you doing this?

(Laughter)

DR. HETLAND: Yes, please?

DR. WHITAKER: That is a good question.

(Laughter)

DR. HETLAND: I am curious, in any of your NICU patient population that
you are following, have you had problems getting your kids \-- the
preemies who were getting SSI benefits, have any of them been ceased at
a year of age, or have there been problems getting them back on the
rolls.

I know AUCD had some findings when they were looking at charts.

DR. WHITAKER: We certainly do see kids that have been ceased, and
sometimes that is not necessarily the fault of the organization; it may
be the fault of how the families are able to get their services and
support. So sometimes it is a case of needing help to get these things
accomplished, and knowing how they can get their children evaluated. And
I think evaluated completely, because growth keeps a lot

of \-- growth problems certainly can continue and keep a lot of them in.

But I personally see more \-- a lot of kids with developmental issues.
And I think families are struggling to get good \-- complete evaluations
of all the different things at times. Growth continues to be a problem
for many of them, though.

MS. LaROSA: I think we have the same experience, that we have some
families who, you know, are denied, and then we have to work to have \--
to help them to get back on if we really feel that they need that extra
help.

DR. HETLAND: Were these kids that were already on the rolls and getting
ceased, or ones that were applying for the first time?

MS. LaROSA: Some that ceased, and then we would try and, you know,
petition to have them re-enrolled, if that was what we felt was in the
best interest of the child.

DR. HETLAND: And so during that time frame potentially there is a lack
of medical insurance that may go along with that?

MS. LaROSA: For a small percentage of our families, yes. Some of our
families, because of their income, they will qualify for the Medicaid.
But we do have some families that have, you know, insurance that doesn't
cover, or they don't have any insurance, and so once they lose that
Medicaid, then they are in difficulty.

DR. HETLAND: Dr. Whitaker?

DR. WHITAKER: I agree.

DR. HETLAND: Mr. Sklar?

MR. SKLAR: I just wanted to follow up on that theme, too. Yesterday
during the endocrine disorder session, we certainly heard a lot about
the importance of access to medical equipment, and access to medical
expertise, and if you are lucky enough to have it, and lucky enough to
get insurance, and you have the right technology, and a great pediatric
endocrinologist, you can have a really good outcome; and if you don't,
you are just a setup for a potentially acute or chronic problems.

In this instance, with extremely low birth weight,

do we have those types of coverage issues and real spotty coverage, or
is this one of those situations where a hospital is just not going to
turn away an infant, and Medicaid is going to be more likely at least to
provide more even coverage across the whole patient population than with
many other types of pediatric problems?

DR. WHITAKER: I think if you mention specifically hospital, if you wait
till the point where it comes to a hospital yes or no, the hospital
probably won't turn them away, but they really need the preventive care,
the primary care, and families without insurance are very reluctant to
seek this out to start with.

Yes, there may be some physicians, primary physicians, as well as a
specialist, who might see them, but it is such a stumbling block if they
can't get past the front desk when they ask, "Do you have insurance?"
That prevents probably a lot of the preventive care.

So, sure, when they get to the point where they need a hospital, that is
pretty far down the line though, and this would be much better handled
early on, rather than later.

DR. DUSICK: I am running into some problems with some of our families
who are working, and they may be the working poor. Dad changes jobs
because it is going to provide more money per hour and a salary, and
oops, now this child has a prior existing condition and can't get
coverage. Now he makes more money and so he doesn't qualify for other
things. And so this can be a problem.

We can look at children with special health care needs services, but
they, of course, have guidelines as well. So that is one problem I see.

The other problem that I see, in a state like Indiana we see children in
our follow-up clinic who may come from three-and-a-half to four hours
away. Certainly if they receive Indiana Medicaid, Indiana says, "You may
live closer to Chicago, but we want to keep those dollars in the state;
you need to get on down to Indianapolis and see the doctors there." And
that certainly puts a strain and a burden on these families.

There are parts of the state that don't have a lot of pediatric care. It
may be from family practitioners, other primary care providers, that may
not have the knowledge about early feeding problems. They may not be
looking at growth with as fine-of-a-tooth comb as we are. And so I see
those problems particularly in rural areas.

MS. LaROSA: And I think what we find is that with some of the families
who have the insurance gap, if their child is delayed enough, you can
get the Babies Can't Wait and they will help provide your services. But
if you have a young child who is having feeding disorders and you want a
therapist to work \-- it is hard, you know, to be that delayed when you
are that little. And so you may not qualify for Babies Can't Wait, and
if you don't have insurance, then you can't get any therapies. You know,
"Our hospital will take care of you, but our therapists expect to get
money."

MR. EIGEN: This is Barry Eigen. I just want to add a gloss to that. I
happen to have AUCD's report with me, and this is one of the things they
track. I should warn you that the numbers of low birth weight children
we looked at was very small. It was only about 87 kids altogether, in
two separate years, and they weren't randomly selected.

But even so, while about three-quarters of the kids were receiving
medical services, for all the other kinds of services, the numbers
dropped off significantly, for probably \-- we don't know for sure, but
probably for the very reasons you have been discussing. For example,
only about a third of the kids were receiving psych services. Depending
on the year, the number was like 12 percent, versus 24 percent for
occupational therapy. Even physical therapy was quite low. And \--

DR. HETLAND: Barry, what is the age range that you are talking about?

MR EIGEN: These are kids who are approximately one-and-a-half years old.

DR. HETLAND: Eighteen months, okay.

MR. EIGEN: Most of the kids are about a

year-and-a-half; some are a little younger, some a little older, but
basically.

One of the things AUCD noticed was, for early intervention records, the
centers that reviewed these cases had recommended EI services for about
80 percent of the children in 2004, but only about 13 percent of those
kids actually were currently enrolled; and furthermore, that was about
half the number that had been enrolled in the first year that they
looked at cases for us. So there was actually a decline in the access to
early intervention in this very small group of kids.

DR. HETLAND: Yes?

DR. BUDA: Well, that in a way \--

DR. HETLAND: Who are you, please?

DR. BUDA: Oh Frank Buda \-- in a way was one of my concerns. You all run
follow-up clinics for low birth weight and very low birth weight
preemies. When we do our assessments \-- of course, growth is a very
concrete thing. You can look at it, look at the rate of change, and see
what is going on with their weight and their height, however, you want
to assess it. It is very concrete.

But we really need to look at the whole child, and we do. Do you provide
developmental assessments, some guide as to whether or not this child is
keeping up with his

age \-- in other words, to provide us some data, so that we can look and
see if this child is developing his skills in a normal rate.

DR. HETLAND: Wouldn't that all be captured in your Bayleys that you are
doing in clinic?

DR. WHITAKER: I am sure. And some follow-up programs, as you said, may
do Bayleys or different \-- there are certainly other tests, too, that
can be very useful \-- may do them at different times. So depending on
how soon you got that information back, I mean, even by the time you got
it, it may still not be the right information, but \-- I think the issue
with the correction at the one year is real important, because if you
see them at one year \-- this is assuming they come in, say, right on or
near their 12-month birthday \-- if they are four months early and they
used this example before, now they would be acting like an

eight-month-old baby, but the numbers of things that you can concretely
measure in an eight-month-old baby are fairly limited.

And so even if you can get those scores, that may or may not reflect,
you know, some years worth of what is going to happen with them. That
would be my concern

about \-- certainly the more scores, the better, the more information
from different aspects you can get, the better.

There also may be big differences in how well they can do certain
different sorts of things, like how well they sit up, how well are they
crawling around, versus how well they are starting to use language. But
an eight-month-old typical eight-month-old \-- and I know we are talking
about your 12-month-old, but a typical eight-month-old, if you correct,
is not going to be saying a lot. They might say, "Mama." They might say,
"Dada." But they are not going to have enough other words and easily
measured things at that point.

DR. DUSICK: Developmental pediatricians use a concept of developmental
quotient, and even if we don't have standardized tests that we are doing
in the office, with taking a history, observing the child, doing a

neuro-developmental exam right there in about a half an hour, we do come
away with something called the developmental quotient, which would be
the age of performance, divided by the corrected age, and then
multiplied by a hundred. And it gives you kind of a number. It is not an
intelligence quotient, but it is an estimate of development.

So for example, if the baby that she mentions is acting like an
eight-month-old and is corrected age of eight months, multiply that by
100, you get 100 percent; that child's developmental quotient is 100
percent. That is a common shorthand that we use in communication, and
that can be one way that we determine even early intervention services
for kids. We look at different streams of development, gross motor, fine
motor, language and social skills to be able to do that.

The problem can be, if you were to use a developmental quotient in
setting a standard \-- we have looked at this in the NICHD Neonatal
network \-- is whose scale of achievement do you use in setting this?
The Bayley scales of infant development are frequently used in the
medical literature, because it is a precise test; it is rigorously
controlled; it is recently normed; there are standards to which it is
given; there are times items. And that is a much \-- it is just a much
finer grade to look at things. We do know that the older you are when
you are tested, the more likely it is going to hold for the long term.

DR. WHITAKER: Another point on developmental pediatricians, not every
low birth weight,

small-for-gestational age baby is going to have access to all the
testing that is best for them, so regionally or locally you may have
less information to work with, depending on who is doing assessments.
The birth-to-three programs \-- called different things; what is this,
Babies \--

DR. : \-- Can't Wait.

DR. WHITAKER: Different names in different

states \-- they have folks that will probably do some of those
assessments. They may be single types of evaluations like only a
physical therapist would have done evaluation, or only a language,
speech language person \-- that would be a little less common in the
younger babies. But there may be less complete information if you have
to piece it together that way.

DR. HETLAND: I am just thinking, I don't think it actually has been
implemented in print as national guidelines, but certainly we are
thinking about making recommendations that when doing these preemie
CDRs, asking the DDSes to explicitly ask, is the child followed in a
zero to three program, or an NIC follow-up program, and to specifically
ask for that information.

While it technically is not a treating source \-- the way we tend to
think of it is primary care \-- there is a lot of information there,
including usually developmental testing that has already been done, in
which case then the DDS does not have to go out and pay for it by
getting a \-\-\--psych CE to obtain the testing results.

Yes, Regina?

MS. CONNELL: Regina Connell. I think it was you, Dr. Dusick, who
mentioned children with special health care needs, that concept, that
label. And I was \-- I believe it is a federally used term, and I was
wondering whether children with low birth weights, or very low birth
rates, or extremely birth weights are automatically included in that
group, or does it depend upon their medical status, if there are other
things that make them included or not included in that group.

DR. DUSICK: Honestly I don't know all of their inclusion criteria, I am
sorry. I have a memory chip saying it is probably very low birth weight,
but I don't know that for sure.

DR. HETLAND: Dr. Fernoff.

DR. FERNOFF: I don't know about that, but \-- these birth to three
programs that are nationwide \-- but in Georgia I know the low birth
weight baby \-- I don't remember what, automatically included in that.
There are a number of categories, and I suspect in most states they have
some cutoff for low birth weight, so you should be able to assess, if
its children can be assessed, and then there are the therapists. That is
another problem with who is receiving services, because it may not be
that they don't have access to the service, but \-- as we know in rural
areas and whatever there are tremendous shortages of some these
therapists around, so that the children are not getting speech and
language, or \-\--, something like that.

MS. LaROSA: Right. And in Georgia, the fact that the Babies Can't Wait,
they are admission criteria. That is helpful to you, and you will get
some information. But depending on when they looked at the child,
frequently we have children that we follow, and they looked at the child
and they are not going to offer services because they looked so young.
And then we see, you know, problems emerging three, four months later,
and then we send them back for an evaluation by them. So it also just
depends, you know, it is hard \-- it is hard to gather, because it is
all \-- I mean, we cover four nurseries in the city, and there's tons
more out there, children who are not getting any services for
developmental follow-up. So it is hard.

DR. WHITAKER: I have recently looked at a \-- just briefly glanced at a
list of different state requirements or eligibility criteria for the
birth to three programs. They do vary. They don't all include
specifically low birth weight or premature infants. Tennessee, for
example, doesn't use the weight so much, but the 30-week gestational age
cutoff. And some states might include low birth weight and/or preemie
babies based on their biological or medical risk, but not all of them
necessarily do. They have to have a documented developmental or other
problem before they can get in.

DR. DUSICK: Yes, while IDEA, Part C does encourage states to have these
programs, there are states that can opt out of accepting federal money
under this, and not have a program, number one.

And Number two, they do not have to use biologic risk categories. They
can wait until the child is already showing developmental delay before
having services. And the level at which that delay is set is set by the
states, and they do vary quite a bit.

DR. HETLAND: Dr. Force.

DR. FORCE: Jud Force, from the Federal DDS.

In another life I worked for Children's Medical Services in Maryland,
which is a Title 5 program under the Social Security Act, and special
health care needs actually came from the Bureau of Maternal and Child
Health, which administers the Federal Title 5 program.

And as you were saying, Dr. Whitaker, it really is up to the state Title
5 program to define who would be included in that special needs care
population, and some states would include low birth weight, others may
not. And this would particularly relate to states who still have what
they call a purchase of care program, where they will actually authorize
and pay for certain types of specialty medical services. And the
financial eligibility for these services, again, varies from state to
state.

In most states, though, the financial eligibility for the services that
they might purchase for children with special health care needs would be
higher than the Medicaid eligibility standard.

DR. HETLAND: I am curious if there are any judges in the room, do any of
the CDR cases ever make it up to the ALJ level? Do you see any of them?

JUDGE SPIVEY: Yes, I am Verley Spivey. I'm the Hearing Office Chief
Administrative Law Judge at Atlanta North.

Yes, we do see them. One of the things that is interesting to me is it
is very important for you to resolve your questions in terms of your
criteria, but in terms of making a decision about under which listing
that you want to proceed under, it is very useful to sort of separate
the, what I call resolvable and nonresolvable issues.

The reason why that is important is if you've got

a \-- and a completely unresolvable issue is likely to meet the criteria
for a listing, you can actually avoid the subsequent CDR. If you've got
a situation where the person is low birth weight, and they might come
back in, you have a situation where you do the review on that. And if
that is the case, it may \-- very well likely they'll miss something,
the case will be appealed, and it will come to me, and we will look at
it. And fortunately Dr. Buda will help me out. And generally we will
resolve the issue.

But if you've got one, if you've got an issue where you've got a choice
between two listing possibilities, and you've got one that is clearly
not going to resolve, then that \-- when it comes up for the CDR and it
is reviewed, you know, if the person is \-- clearly has this issue, it
is not necessary to do the CDR. You don't inconvenience a person who is
disabled, and you don't spend a lot of administrative money.

So I mean, I do see the issues, and the one comment I have is that one,
that if you've got a choice between several listing possibilities,
always include the nonresolvable ones, because it will avoid the CDR
ever coming up to me in the first place.

DR. HETLAND: Thank you. CDR is continuing disability review, meaning
when do we go back and reevaluate the claim to see if there has been
medical improvement, which is the statute requirement for whether or not
to continue receiving SSI benefits, or to cease at that time because
medical improvement has been documented in the file. That is a CDR.

I think we are going to wind up now, and I would like to thank our
speakers for flying in and helping us out. I have five after 12:00. I
would like to reconvene in an hour from now, at 10 after 1:00, okay?
which is a few minutes early, but we are on a tight schedule this
afternoon. Many of us need to make our flights on time, so I am going to
hold people to the schedule that we have this afternoon. Thank you.

(Whereupon a luncheon recess was taken at 12:03 p.m.)

**[A F T E R N O O N S E S S I O N]{.underline}**

(1:15 p.m.)

***[Session Two: Linear and Weight-Related]{.underline}***

***[Growth Impairments]{.underline}***

**by Monte Hetland, M.D., Moderator**

DR. HETLAND: I would like to get started again. For those of you who
perhaps weren't here this morning, I am Dr. Monte Hetland. I am the
Pediatric Medical Officer in Baltimore dealing with medical policy for
children receiving SSI.

Our focus this afternoon is going to be on the growth impairment
listings, not specific to premature infants. And for those who would
like to reference, the

100-body system listing is on page 171 for growth impairments. And on
page 209 is the 10508 malnutrition under our digestive body system. So
that is page 171 and page 209.

And then to give you a little bit more to do, if you look at our outline
for today, the second attachment, Attachment B, these are the six
questions that we have posed to our professional speakers this
afternoon, dealing with our asking the questions, are our regulations up
to date? Do they capture the population that we should be shooting for?
Do they miss anybody? How can we recommend? How can we update them?

Our first speaker will be Dr. John Parks, who is a professor of
pediatrics, and a pediatric endocrinologist. He is chief of the section
of endocrinology at the Emory University School of Medicine.

Our next speaker will be Dr. Bess Schoen, who is I pediatric
gastroenterology. She is an assistant professor in the Department of
Pediatrics, also at Emory.

And our third speaker will be Ms. Heather Taylor, and our fourth
speaker, substituting for Amy Miele, will be Paul Nrabosky, who is a
member of the Little People of America.

After such we will take a short break, and then reconvene for questions.

***[Presentation]{.underline}***

**by John Parks, M.D.**

DR. PARKS: Okay. And also we will have Alexander Philips, who is a young
woman, is also challenged around the issue of growth, and she will be
participating in our panel discussion, and addressing questions as part
of the discussion now.

(Slide)

When we think about growth disorders and growth issues, there are some
things that are simple, and there are some things that are more
difficult. For example, it is easy to tell \-- you have a known
measurement and an age \-- how to tall the child is compared to their
piers.

We can often \-- not all the times \-- figure out why they are
relatively small, or at the other end, relative tall. We can sometimes
find a way of improving growth weight, but it is much more difficult to
have any conceptual agreement on criteria for disability.

(Slide)

There are general definitions for disability, and I just wanted to point
out the apposition of these criteria, as they apply mostly to functional
testing, and see how they would fit in using height-for-age as a
standardized test or measurement.

When it comes to functioning in cognitive areas \-- and Dr. Buda, please
correct me if I misunderstood that \-- it seems like there is a
distinction between marked \-- which is generally in the two- to
three-standard deviation score range, and extreme, which is greater than
3SD. Is that an appropriate way to think of height issues?

(Slide)

Well, under the 100.00, the criteria, or at least the things that enter
a determination are the heights, current and three previous
determinations, plodded on an appropriate growth chart, with heights of
parents and siblings. And at this point I think the \-- just the
description introduces a bias, and a prejudgment, saying, "You do that
to exclude short statures as a familial characteristics rather than as a
disease." That was the first thing I ran into that seems to me a bias
rather than a scientific judgment.

Bone age or other imaging is of course among the diagnostic studies, but
do we really need to introduce diagnostic studies, or are we measuring
against standards?

It says that the criteria are \-- for growth problems are applicable
until closure of the major epiphyses. In other words, growth issues are
only a problem while you are still growing, for a criterion when you are
still growing. And I think you will hear today from people in that very
situation, who are grown up, and short adults, in a world where
everybody is much \-- almost everybody is much bigger. So I also think
that even from the go that is introducing a bias that is difficult to
defend.

(Slide)

Extreme short stature, I just \-- I didn't know whether Alexander was
going to be here today, but I showed another young woman, who is 23. Her
height is equivalent to that of a five-year-old. Her skeletal maturity
is that of a 10-year-old. She has not signs of puberty, and she and five
of her brothers and sisters have a genetic defect of pituitary
development. In her case we can know down to the molecule and to the
base what has caused her short stature.

(Slide)

If you just saw her by herself, you would think she is a perfectly
normal-looking eight-year-old, or six, or some \-- depending on how much
experience you had with students, children, grandchildren, you would
assign an age to her, and it wouldn't be 23. She is shown with a
23-year-old medical student of average size, and it puts it into
perspective.

(Slide)

Objectively, you can use growth charts. And I want to spend a moment or
two on the growth charts. The ones that are currently in use in the
United States were issued by the CDC in 2000. They represent data
collected in national health and nutrition examination surveys, NHANES
and its predecessors over the last 40 years. There has been no change in
height. There has been a major change in weight, which as you probably
heard this morning, there was a political decision not to use current
reference, but to exclude all 1990s weight measurements from the weight
charts, so that the weight charts are the way we were, as referenced;
the height charts are the way we were and we are, and we haven't changed
in the last 40 years.

Now, you can see at the extreme right the height of the 23-year-old
woman in relation to the norms. You can see Alexander's height; you can
see a red unknown. And how do we use these? Well, current criteria talk
about the third percentile. Here the third percentile is shown as the
lowest line on this CDC chart.

This is for all practical purposes \-- even if you use little dots for
plotting, rather than for display, that is close enough to minus 2SD to
be comparable. Some charts, the data certainly allows calculation of a
third SD. They are not generally available. Most of the growth charts
that pediatricians and endocrinologists use in their offices are
distributed as re-printings, re-formattings of the chart by various drug
and nutrition companies.

I would like to take the liberty of asking your help to pass out charts
that do show minus 3SDs, so that people can kind of put themselves on
those charts. One is mail, one is female, take whichever you want.

(Laughter)

And you will see that the average height in an adult male in the United
States is 69-1/2 inches, average height of a female is 64-1/2 inches, a
five-inch difference.

The fifth percentile in men is 65 inches, and in women it is 60 inches.

So there are different degrees of short. The main reason I pass this
out, besides being able to place yourselves on the chart, is to show
where minus 3SD, equivalent to the lowest 1/10^th^ of one percentile
lies.

(Slide)

Okay. With that in mind, let's look at 100.02, what would be marked
problems with growth. In this case it doesn't stand alone as a height,
but needs to be related to another additional specific medically
determinable impairment. And it would be a fall of greater than 15
percentiles in height, which is sustained, or a fall to a persistence of
height below the third percentile, roughly minus 2SDs.

(Slide)

Extreme could be a stand-alone, not identified as being related to an
additional specific medically determinable impairment \-- and impairment
is different from diagnosis, as has become clear to the physicians
attending these sessions.

The criteria there are a fall of greater than

25 percentiles in height, which is sustained. And it really doesn't say
anything about height in number two. It says a bone age which is more
than two standard deviations below the mean. And I think that is a very
inappropriate thought.

(Slide)

I also don't like the falls in height percentile. If you look at a
marked fall of 15 percentiles, it could be from the 75th to 60^th^. Is
that a marked growth impairment? Dr. Schoen may have her opinion on
that, but I don't think it is. Severe. A fall from the 50^th^ to the
25^th^, in this instance over two years. Is that marked impairment? No.
Is a fall from the 25^th^ to the first marked impairment? Yes.

(Slide)

Look on the back of your growth charts, and you will see something that
is equivalent to the speedometer in our car; not how far you have
traveled on your growth trip, but how fast you are traveling. The units
are centimeters

per year multiplied by 10, and think of miles per hour. Through most of
childhood, the average child is growing around six centimeters a year.
Think of that as cruising at 60, just for comparison.

What do you need to do? How much do you need to slow down to go from the
75^th^ to the 60^th^ over two years? That is a criterion for the first
stage of growth impairment. What do you need to slow down to 52. How
slow do you go over two years to go from the 50^th^ to the 25^th^?

4.2 centimeters per year. How slow do you have to go? An equivalent
dropping of 25 percentiles under severe growth impairment to go from the
25^th^ to the first in two years. You have to slow down to one
centimeter a year \-- the equivalent of 10 miles an hour \-- between
seven and nine years of age.

The numbers are slightly different depending on which part of the growth
chart you look at. And I am suggesting that if you are going to retain
change of percentiles, they should be plotted not as change of
percentiles \-- which really means different things on different parts
of the chart \-- but on a height velocity chart. And the very fact that
I had to introduce a proprietary version of the growth chart in order to
have these references, means that one would have to consider having
other references available, not just to physicians, but to anyone else
who would review the growth data. As it is, I would submit that the
change of percentiles as currently expressed is not an appropriate
criterion.

(Slide)

So these are suggestions. I don't think \-- I think it is a bias to
exclude short stature as a familial characteristic rather than as a
disease. We will see instances today in real life experience where,
sure, it is a dominantly, \-\-- is a dominantly transmitted condition.
Are you outlawed because you have inherited a medical disorder from a
family member? No. But equally important \-- but also on the same score,
the polygenic inheritance that makes people take after their parents
should not be an exclusion, if we are really talking about the
handicapping aspects of short stature.

Bone age or other imagining, I don't think is required for telling how
short someone is. It is a characteristic, but not important in terms of
disability. Criteria applicable to a closure of a major ephiphyses. When
you stop growing and you are short, you stay short. When you stop
growing at an earlier age, you are shorter.

So I don't think that belongs in there.

(Slide)

Marked. A fall that is greater than 15 percentiles in height which is
sustained, or fall to a persistence of height. I would suggest
substituting two or more years of growth at a rate below the 10^th^
percentile for growth rate for age, or something else keyed to that
standardized measurement.

And second, fall to below the third percentile, you might consider
substituting minus 2SDs.

Extreme. Fall greater than 25 percentiles, you are familiar with my
argument against that terminology. I would suggest substitute two or
more years of growth at a rate below the third percentile. Of course,
for younger children it would have to be less than two years.

Bone age, I think is irrelevant. And if you are going to talk about
severe impairment because of height

per se, then include some criterion for height. Arbitrarily I have
suggested minus three percentile, or the lowest \-- the shortest in a
thousand, the lowest .1 percentile.

Weight-for-height. Dr. Schoen is going to talk about one of the
questions was specifically, do the

BMI-for-age standards adequately reflect nutritional assessment of
weight-for-height? I would submit that on a population basis are useful;
on a clinical and an individual basis it can be extremely misleading.
BMI is related to height, not to age. So what do you do with someone who
is an atypical height-for- age?

Second, it gives no indication to the parent or to the family how much a
child would need to gain or lose to be in a different percentile, or how
long they would have to grow without a change in weight to go from
obesity to a lower category for weight.

So that BMI has its purposes, but I think it is faulty for individual
assessment. CDC chose to compile, but not actively distribute the
weight-for-height. In the old areas the weight-for-height went out to
about an average height of an 11-year-old. In the current it goes to a

six-year-old, to the average height for a six-year-old. So it is
basically a \-\-- is it two or three years? two or three years,
weight-for-height, and there is effectively a two- to six-year
weight-for-height, weight-for stature, which is available, and should be
available to examiners.

Overall, just some generalizations in relation to one of the questions
posed. Persons with disproportional growth appear short for
chronological age, are they treated as being much younger than they are?
Are they misclassified for age? We will hear about that today.

Persons with hormone deficiencies appear to be younger than their
classmates, and are more often misclassified as younger children, \-\--
as to whether they belong in a particular school setting. And we will
hear more about that today from the individuals.

There is more difficulty in helping the very small child achieve
age-appropriate roles and goals than with a child of normal stature.

Shortness as a result of chronic illness serves as a mark of disease
severity. One may need different criteria if you are thinking of it as a
marker of disease severity rather than a handicap in and of itself.
\-\--. It is renal failure, \-\-- enteritis \-\-- imperfecta, other
conditions and their attendant problems.

Axis to medical care. How does axis to medical care influence issues of
growth and short statute? There are three really simplistic ways to
think of it. One, it influences the likelihood of arriving at a correct
diagnosis. It influences likelihood of receiving appropriate treatment
to improve growth, whether it is to addressing a systemic illness,
correcting a hormone deficiency, or some other measure. And it should
increase the likelihood of receiving appropriate interventions, advice
in the school and home setting, to reduce the impact of disabilities
related to growth impairment.

So that will be my part, and reserve the rest for the question period.

(Applause)

***[Growth Disorders in the Disability Programs]{.underline}***

**by Bess T. Schoen, M.D.**

DR. SCHOEN: My name is Bess Schoen. I am on the GI faculty at Emory, and
I take care of a lot of kids with nutritional problems, in the fact of
other medical diseases.

(Slide)

And I am going to talk a little bit today about growth as a proxy for
adequate nutrition. This is a little different from the approach that
Dr. Parks took. And growth is the result of a lot of things, obviously
genetic, but also the endocrinal hormonal factors that Dr. Parks talked
about.

But my side of this is the nutritional aspect.

When you have normal growth, it is a sign of basically good health and
good nutrition. And on the other hand, impaired growth, when it is
associated with inadequate weight gain, may be a sign of
under-nutrition.

Impaired linear growth, like Dr. Parks mentioned, may not be a
disability in itself. But the disability comes from the consequences of
under-nutrition, and that may manifest in a lot of ways that we will
talk about today, such as the attainment of poor or inadequate motor
skills, or development, or even interference in the immune system.

(Slide)

When we go through the categories that you have in your reference book
\-- Dr. Parks has already talked about this, but I only wanted to make a
couple more comments \-- these criteria of fall of greater than 15
percentiles in height, which is sustained, or fall to a persistence of
height below third percentile, in the absence of familial short stature,
or some type of endocrine disorder, both of these may be appropriate
slags for under nutrition in children.

(Slide)

That category, though, 100.02 was in conjunction with another existing,
preexisting medical condition. 100.03 is the growth impairment without
another specific medically determinable impairment.

Now, the standard here is higher, and I wasn't really clear on why it
is, but they require more severe growth retardation than they do in the
other category. And maybe someone can explain that to me afterwards.

In the population that I see, if I see a child who has growth
impairment, and I can't find anything else going on, that may imply that
there are some psycho-social factors as a reason why the child isn't
getting enough food, or they not getting enough calories, or whether it
is a behavior issue, that maybe the child is refusing. But those are red
flags to me.

The delayed bone age \-- and I want to ask

Dr. Parks about this later \-- but in the kids I see with malnutrition,
the fact that the bone age is delayed may be actually a good sign for
me, that our intervention \-- they have growth potential, they have
catch-up potential. That may be a good sign that we can actually
intervene and have success.

(Slide)

Now, go to 105.08, the failure to thrive. I am just repeating here the
definition that I saw, the inability to gain and maintain adequate
weight. Many children with failure to thrive show signs of developmental
retardation and physical and emotional decompensation; that is pretty
broad.

And the question was asked to me, what degree of weight growth would we
consider disabling? And I think that is the degree that prevents a child
from attaining motor milestones, cognitive skills, or from attending
school on a regular basis.

And this can be caused \-- and we see this not infrequently in the
clinical setting \-- by weakness or loss of muscle mass, by fatigue, or
again, recurrent infections.

The failure to thrive policy in younger children, up to age two, has
several criteria here. The age. And then failure to thrive in this age
group is a medically determinable impairment. I am not sure if I
understood what that means. And then they have duration criteria.

And I just wanted to make a comment that when you have failure to thrive
expected to last \-- or having lasted 12 months, or expected to last 12
months, with at least six months as measurements, that is a long time in
children under two years. And I think we should consider that. You can't
let a child in that age group go for six months to a year without
considering that there is some disability there.

The weight criteria obviously are here below, fall of 15 percentiles, or
persistence of weight below the third percentile. And I would like to
just make the comment that we need to be considering those, I think,
earlier, rather than giving it that much time.

(Slide)

Now, I have several suggestions for the section on the digestive system,
and that is the 105.00, and under that came the 105.08. This was my
first foray into policy, and I found the way these were written a little
bit confusing, and I think they jump around quite a bit. So I maybe can
make some suggestions for organizing them a little bit.

Disorders of the digestive system can lead to malnutrition in a variety
of ways. They can interfere with a child's ability to ingest nutrients;
can interfere with the absorption; or lead to loss of nutrients, as in
the case of no absorption or diarrhea.

And we see a lot of kids who have diseases that are associated with
rapid consumption or utilization of nutrients, kids whose metabolic
rate, or by the fact that they are having so much inflammation in the
body, they are consuming calories at enormous rates. And as an example,
I take care of a lot of kids with HIV, and also inflammatory valve
disease, and they fall into that category where their nutrition
requirements are tremendous, and way beyond anything you would expect in
another child that age.

(Slide)

This section requires documentation of a GI impairment. Now that is very
appropriate, and the studies that are included in that section \-- I
didn't repeat them all there, in your paper there \-- I agree with all
the studies that are mentioned. I do want to say that that is the place
I think where you, if you want to include stool malabsorbtive studies,
they belong here rather than where they are, in the section we will get
to later.

In the growth retardation and malnutrition, they make the point that
correctable conditions, where surgery can protect the problem, do not
count, and that this isn't appropriate.

And then there is a section on multiple congenital anomalies that I
won't comment on.

(Slide)

Now, one of the slides, .01, is the categories of impairment in the
digestive system. And they jump around quite a bit, and I would like to
suggest that we start with mouth, and go to the other end, and think
through the GI track sort of in order.

And the first is 105.03, which is esophageal obstruction, or diseases of
the esophagus. But the current listings jump over the stomach, and I
think we should perhaps include diseases where there is a loss of the
gastric surface area. Those are likely going to be surgical issues. They
are rare in kids, but they do exist.

I am also going to suggest another category for small bowel disease, and
this is a big deal, and has changed quite a lot since I think your
original \-- or since the last time these listings were written. Small
bowel disease has taken over GI practice, and these are problems where
there is a loss of the surface area, or the absorptive area of the
bowel. The big one I am thinking about is short bowel syndrome.

I just came off hospital service two weeks ago. I had six kids on my
service with essentially no intestines. These kids have incredible
functional losses. They are

IV-nutrition-dependent. They are hospitalized for months to years at a
time. They have lots of disabilities that relate to their indwelling
catheters, recurring infections, chronic liver disease, that goes with
the use of IV-nutrition. And that is a category of illness that just \--
I don't see it in there at all.

And it certainly needs to be addressed now, because the pediatric
surgeons are creating more and more short bowels as children \-- as
premature \-- of course, I wasn't here for the section on prematurity,
and you may have discussed this \-- but we are seeing more and more
necrotizing enterocolitis and other congenital anomalies, and those
children in years past would not have been saved. They are routinely
saved now, with almost no remaining gut.

So this is a category I think we certainly should include.

There is a section, 105.07, for chronic inflammatory bowel disease.
Certainly belongs there. Our goal with those kids is to get them
completely functional, with the use of therapy. And most of the time we
do. But there are individuals who are quite disabled by inflammatory
valve disease.

(Slide)

I added a few others. Again, I don't know \-- and

when I got in here today, I did notice there is a section on cystic
fibrosis, but I don't know if there is a part that relates to the
pancreatic insufficiency \-- which is certainly a big segment of cystic
fibrosis \-- and pancreatic insufficiency, which comes from other
causes.

And then I noticed that there is a section for liver transplant. We now
are doing small bowel transplants; not a lot, but I personally have two
patients in my practice who have been transplanted, and the same
criteria, I think, for liver transplant can apply here, that those kids
may be disabled early in the course. Once they become stabilized, then
certainly the goal is not to have any functional disabilities anymore.

(Slide)

The section on chronic liver disease, the way it was written includes
rather than individual diseases, for some reason it has biliary atresia.
Inoperable biliary atresia is not compatible with life, and those kids
have to be transplanted. And then they, of course, would fall in the
transplant category.

Chronic liver disease, I think, is basically defined by these things
that were mentioned here, and I did suggest a little bit of a change in
the way they are \-- in the order in which they are listed.

The section on liver transplant, we discussed.

And then 105.08, I think that the numbering or these things can be in a
little bit more organized fashion.

And malnutrition section, due to demonstrable GI disease, causing a fall
of 15 percentiles in weight or persistence of weight less than a third
percentile, and one of the following. I think some of these are a little
passé. I don't think anybody really does stool fat excretion. It
requires a paint bucket where you collect three days' worth of stool
samples. It is unwieldy; it is difficult to do; it is nasty. Nobody does
it anymore.

The persistent hematocrit of 30 percent or less, despite prescribed
therapy, iron therapy, certainly seems appropriate.

Serum carotenes are also really not used anymore, and there are other
things we can use, spot tests in the stool, or in the serum, that I
think would be more helpful than these particular tests.

Serum albumin of three grams per desi-liter or less, can be appropriate
to use, but we do frequently see that kids who are chronically
malnourished, because they have catabolized their muscle, will have a
falsely elevated serum albumin. So that is not always helpful.

(Slide)

What do we think \-- or what do I think about the adequacy of these
listings? Well, I think that they do identify most kids who could be
considered disabled from failure to thrive or malnutrition. We thought
\-- and I did this with our HIV nutritionist; we kind of discuss these
things \-- we thought also that some of the anthroprometric measurements
might be helpful, such as fat for mass, which looks at muscle mass by
the mid-arm circumference. Or subcutaneous fat stores measured by the
tricept skin fold measurement. That may be going a little too far in
terms of requirements for large populations. But these would pick up
kids who have some evidence of loss of muscle mass or fat stores, who
don't fit the growth criteria.

(Slide)

Dr. Parks talked about weight-for-length, or weight-for-height, and body
mass index. We have been using those because that is what has been
available to us. And I think in terms of under-nutrition, we see that
the DMI scores seem to correlate pretty well with the

weight-for-height scores, as we have used them in our HIV population.

Certainly body mass index less than 50 percentile for the age may be a
good reflection of acute malnutrition, and we look at those and utilize
those. One thing I wanted to point out that we have seen in our HIV
population, but also we see in other chronic diseases \-- and people
have documented this before \-- that when you have kids with nutritional
stunting, where the height is stunted to stay commensurate with the
weight, in those kids, to achieve catch-up growth in them, we found in
our population that you have to push the BMI way about 50^th^ percentile
\-- sometimes to 75^th^ percentile \-- before the catch-up growth will
come.

So you have to get them chubby before they start to grow. And so
sometimes I think using these very, very low BMI criteria may not help
kids with nutritional stunting.

(Slide)

In the etiologies of impaired growth, each of the diseases obviously
affects development and functioning in different ways. Just the example,
the inflammatory bowel disease, which usually in our population has its
onset in the pre-teen years, and interferes with the growth spurt, with
sexual development, and in turn interferes with

psycho-social adjustment \-- and those are some big functional
disabilities for those kids.

But all of the causes of failure to thrive and malnutrition, if you
document malnutrition, share some common effects, and that can be
cognitive and motor development affects on them. Sub-optimal school
attendance or performance in school. Increased susceptibility to
infections. I keep coming back to that, that this is what you see
functionally in the clinic.

And impaired wound healing. I wanted to bring this up, because we see a
good number of kids with problems like cerebral palsy, who require
orthopedic surgeries, and require different kinds of surgeries. And we
see those kids having complications of malnutrition that manifest when
they have surgery, that is in the fact of getting infections, or poor
wound healing, or superior mesenteric artery syndrome, that all come
back to underlying nutrition.

(Slide)

I put this up, that \-- you don't need to worry about this slide. But
this is a slide that \-- this comes from Tracy Miller's chapter on HIV
and nutrition from one of our nutrition textbooks. And I really like
this slide, because it shows that the immune effects of malnutrition are
almost identical to the effects of the HIV virus on the immune system,
almost identical. And so what we see in kids with HIV or AIDS is that
they have a compounding of their immune deficiency based on
malnutrition.

And we have shown in our population that kids who start their anti-retro
viral therapy in better nutritional condition, have a better response,
have a better viral load response to their anti-retro viral therapy than
kids who were undernourished at the time of starting it.

I just wanted to point this out, because the

malnutrition affects these kids in more ways than just having them be
small. This is where the functional problem is.

(Slide)

Does the lack of health care insurance, or access to quality medical
care impact the course of impaired growth? Yes. Lack of insurance or
access to quality care prevents or delays diagnosis of the underlying
conditions which cause failure to thrive. This is \-- I am saying all
the same things Dr. Parks did \-- prevents or delays diagnosis, prevents
our ability to intervene with supplementation to correct the problem.

It allows kids to fall into this cycle, that I see in the clinic not
infrequently, where you have a child who has failure to thrive, picks up
every little infection that comes along, and loses his appetite, and
then gets less and less nutrition. Sometimes the way we have to
intervene there is to give nutrition in a way that may not be my mouth,
maybe they require tube feedings through the nose, or through the
stomach. And sometimes that is the only way to break that cycle.

Lack of insurance allows marginal cases of failure to thrive to get
worse and worse. And then it ends up costing the health care system more
by \-- ultimately more, by paying for preventable complications, of
surgeries, or infections, or hospitalizations that would otherwise not
have happened.

(Slide)

I wanted to make the point that one of the reasons I was glad to be able
to participate in this is that in our setting, we feel frustrated
frequently that in our state Medicaid only pays for nutritional
supplements if the kid has a G tube, and not everybody has a G tube.

And we have \-- there are lots of kids who could be drinking calorie
supplements and they could do much better, but they can't any support
for paying for them. And some of the traditional insurance payers don't
pay for supplements at all. And in an underprivileged population, they
don't have any way to afford these things.

So I wanted all of you to keep those things in mind, because that is a
place where we really can intervene.

Thanks.

(Applause.)

***[Presentation]{.underline}***

**by Heather Taylor**

MS. TAYLOR: Hi. My name is Heather Taylor, and I am here to tell my
story about myself and my dwarf children. I am one of 200 different
types of dwarf. I am an achondroplasia dwarf. I have two children that
are four and six, and they also have achondroplasia. We have a
disproportionate short stature with shortness of limbs, and have average
size trunks. Our average height is about

four-six, men and women. All dwarves are handicapped, but some more than
others.

I grew up with average height parents, and when I was born they knew
nothing about my disability. Until I began school, I thought I was a
normal child. I was teased excessively about my shortness, and as a
result, it affected my schoolwork and self-esteem. As I grew older, I
had no \-- I had to deal with the discrimination with obtaining a job.

I did not have the complications and surgeries of so many dwarves have
today. As an adult I have had spinal stenosis, and I have had surgery
for it.

My daughter, Hannah, is in first grade. She is in the 95 percentile on
the achondroplasia growth chart. On the average pediatric height chart,
she is the equivalent of three-year-old. At this time there are no
indications of medical issues that affect her at this time.

My daughter, Olivia, is in Pre-K. She is in the five percentile on the
achondroplasia chart. She has numerous medical problems. Due to her
small nasal passage,

her adenoids were blocking her airway. She is experiencing sleep apnea
all night long. She was not sleeping during the night due to the lack of
oxygen. Due to the shortness of the tubes between her ears and her
throat, there were chronic ear infections, which may led \-- which led
to hearing loss, also tubes. Also as a result, there was a speech delay.
She is under the care of a cranial specialist and will be monitored for
future reconstructive surgery. Due to extreme cranial problems, Olivia
is in speech therapy for an indefinite period of time.

That is just to give you a little background about my family.

Some of the challenges that I have to overcome as an LPA parent with LPA
children: One of my \-- one of my biggest challenges is teaching them to
appreciate themselves, and develop pride and accept what they are and
who they are, and their disability.

Because of their disproportionate stature, a number of psychological
problems can arise. There's lots of things I have to do, and I'm just
going to list all the things that I do. I have to, with the school
system I have to avoid activities that could be harmless to them per se,
recreational activities.

My six-year-old is in first grade and she has PE. The kids run laps
around the playground. My daughter can run one lap to the kids' three
laps.

Install practical aids. Lower light switches on the wall. Door knob
extenders. Stools, potty chairs, adaptive furniture. Temporary or
permanent pedal extensions for driving in the future, on their cars.

Insuring schools have appropriate equipment to accommodate their
shortness, as far as stools under their feet. My Pre-K four-year-old is
so petite that she has a stool that she gets on the potty, but she's so
little she falls inside the potty. So we had a \-- the county has put in
a special potty for her, so that she does not fall through the potty.

Lowering sinks so that she can wash her hands. Reaching paper towels. If
my daughter is not helped by another student or an adult to go to the
bathroom, she can get into the bathroom, but she cannot get out because
she cannot reach the handle on the door.

Social challenges. Social challenges is a major, major thing. My
daughter on a daily basis is six years old.

Comes home exhausted, not only because she is trying to do her
schoolwork alone, and walk the halls, and sit in like the regular
six-year-olds, but she's been teased, called names. She says \-- I've
always told my children that God makes everybody different and we're
here for a reason.

When my six-year-old says, "I want to talk to God. Why did he make me
different? Why can't I just be normal?"

This is affecting her. They key thing, she's a straight A student, up to
this year. She is \-- it's \-- the teasing and the verbal issues that
she's getting is now starting to affect her schoolwork.

I am constantly up at the school. I have educated all of the teachers.
I've answered any questions. I answer questions any of the parents have
about our stature, to help educate them, so maybe that the world will be
a little bit nicer to my children.

They are constantly verbalizing their age. My daughter \-- the
four-year-old is very petite, and she has a speech impairment. Her
communication is not very well, and she's constantly being asked, "How
old are you?" All of the kids in her class are extremely taller than
her. They're always asking her, "Why are you here? This is big school.
Babies are not allowed."

Finding appropriate clothing for my children is especially a challenge,
especially for my four-year-old. She is about a Two-T, Three-T, and to
find four-year-olds adequate clothes that aren't one-sys, and do not \--
they're not baby clothes, is a definite challenge.

Shoes. We have the widest feet. It's definitely a challenge. We can't go
to WalMart and buy shoes. We have to go to the specialty stores because
we are a double E shoe size.

Financial issues. There's always financial issues because we have costs
of medications; we have costs of the co-pays, and the travelings to the
doctors. We have

costs \-- alteration costs; you go to buy clothes, but you still have to
have them altered.

Medical challenges. Selecting a pediatrician familiar with dwarfism.
There's not a lot of pediatricians out there that are even familiar with
dwarfism.

There are several medical issues that I have in the future that my
children could possibly have. Compression of the brain stem. There needs
to be \-- during the surgeries that my children have had, and will have,
I have to make sure that the anesthesiologist does special
considerations for general anesthesia. I you hyper-extend their neck,
you could damage their spine. If you give them too much anesthesia,
because of their age, and not because of their height and weight, you
overdoes my child.

Motor delay in walking. All of the motor

skills \-- my daughters did not walk until they were 16 months; my
youngest, 17 months. Crawling, they never crawled. Their arms and legs
were too short to \-- they scooted.

Low blood sugar. Dwarfism, we have low blood sugar. So their nutrition
is very, very important. Also their weight. I monitor what they eat,
because if they put on too much weight, which puts on too much stress on
their knees, which will develop a physical walking issue.

I have to watch for their joints, joints too large for limbs because
they pinch their nerves.

I have yearly \-- my children have yearly sleep studies, because of
sleep apnea issues.

Annual orthopedic checkups. I have to monitor their head growth, because
they have unusually large heads, like myself.

Water on the brain, which they are usually born with. I think that if
they aren't born with it, I don't think that we run the risk, but it
could be possible.

Compression of the brain stem.

Those are all of the medical challenges. And I guess that's all I've got
to say.

(Applause)

***[Presentation]{.underline}***

**by Paul Nrabosky**

MR. NRABOSKY: Hi. My name is Paul Nrabosky, and I am with \-- I am a
member of The Little People of America, and I was, I guess, asked in an
impromptu to speak, because another person wasn't able to make it with
our group, Amy Miele.

Little People of America is an organization that has been around for
about 50 years now, and I have been involved for about 18 years, a
former chapter president, and just an active member at this time.

I'm going to make this quick, and speak in general times. Obviously a
lot of people with the medical profession are very up on a lot of these
issues more specifically, I guess.

Obviously people of dwarf stature have problems with physical barriers
at home and out in the public. Most dwarves are confronted with, are
there physical disabilities, such as hearing, breathing and orthopedic
problems.

We are also faced with social, psychological issues as well. This
includes acceptance from the

average \-\-- society, which leads into acceptance of ourselves, from
ourselves.

Many people come from rural areas, and from families who can provide
only limited economic support. With many different types of dwarfism, we
have different medical conditions. Most of us do not have medical
benefits, and thus we need help from Social Security Administration.

While SSA is looking at assistance to trimming policies, we ask that you
take everything into consideration, from problems we face at an early
age of personal development, to as we age and our medical conditions may
force us to seek other employment, or obtain other economic support.

Most people of short stature are very headstrong, and want to be as
independent as possible. However, at times we simply need some financial
assistance to help us gain, or maintain that independence.

We all know that any assistance provided at an early age can eliminate
or prevent future medical and social problems. Thus this assistance can
have a tremendously positive impact on our quality of life, and on a
long-term positive impact for society.

I want to thank you now for your consideration for giving us, people of
short stature, an opportunity to be heard.

(Applause)

DR. HETLAND: Okay. I want to thank our speakers very much. I have 2:15.
We will reconvene at 2:30.

(Recess)

***[Presentation]{.underline}***

**by Alexandra Philips**

DR. PARKS: Alexandra Philips is a young woman that I have known for some
years, who has been challenged around growth issues. And just in
starting \-- well, she has a different form of short stature from
Heather, in that hers is endocrine-related, incompletely treated, but
\-- she lacks several pituitary growth hormones.

But I noticed early during Paul's and Heather's presentations that
Alexandra was nodding her head a lot.

(Laughter)

Which things do you remember, Alexandra, that seemed to ring true to
you?

MS. PHILIPS: Well, the wide feet, of course. My feet are very wide. I
can't wear narrow sandals or boots, because they will hurt my feet like
crazy.

And clothes, it's very hard for me to find clothes, because I just
learned how to sew last year so I am not altering and making some of my
clothes.

DR. PARKS: That is an important observation. I don't know how many
people really have those skills anymore.

(Laughter)

It is even more rare than still knowing how to cook in this society.

(Laughter)

All right. Are there any questions directed specifically at Alexandra?
How old are you? What issues do you face at school? Do you think
changes, differences or issues as an adult are going to be very
different from being her own age? Anyone want to pursue that line of
questioning.

(Pause)

DR. HETLAND: Okay. Well, I will ask you the questions.

DR. PARKS: Okay.

DR. HETLAND: How old are you, and what were some of the same issues that
you were not in your head in agreement with out other speakers?

MS. PHILIPS: Well, I just turned 16 in August, so I guess I'm almost
16-and-a-half in February?

A lot of my issues are being teased in school. At first it would bother
me because I didn't know how to respond to it. But my dad taught me a
lot about how to joke back with them, not about me, but make fun of
them, because nobody is perfect.

(Laughter)

And they did, when \-- this year, because I just started in a new
school, and I didn't know how to fit in, and so everybody was shocked,
"Wow, I can't believe you're in the 11^th^ grade," like \-- it's not
new. Everybody \-- or most of the people that are in 11^th^ grade here,
I don't see why it's different with me.

And when I came here and told people that I was 16, I'm in the 11^th^
grade, people were, "Okay," I'm like wow, that's a different response,
because I'm usually hearing, "Whoa, what happened \--" it's really
crazy. So that's most of the problems I have that you've heard of.

DR. HETLAND: I also heard you talking with

Dr. Becker and sharing some stories about how driving is an issue for
you.

MS. PHILIPS: Yes, I really am paranoid, and so when I'm driving, I get
really scared, because I don't want to accidentally hit somebody and
die, because I know that the airbags come out, it can probably snap my
neck. And I know that once your neck is snapped, you're gone. And that
worries me a bit.

And so sometimes I really don't like to drive, but when I do, I'm just
going around the corner to school, and from my house, because it's only
a mile away. And that's as far as I drive.

DR. HETLAND: How about our other speakers? What adaptations do you use?

MS. TAYLOR: I was sharing that we, you know, at the LPA there is a web
site that you can go on and there is apparently a paper that you can
print off about the airbags, and you can take it to your mechanic, and
they can disconnect the airbag. Because the \-- I drive with no
extensions. I'm able to reach my pedals. But I am also up on the airbag,
and if somebody to rear-end me, or I to

rear-end them, that airbag is going to completely bash my face. So it is
\-- it's an issue.

I'm able to reach my gas, my brake, I'm able to reach my steering wheel.
I'm even able \-- I'm able to turn my steering wheel. There are several
LPs that we do know that cannot; they have to have a special steering
wheel put in the car, because the steering wheel is so big, their arms
are so short they cannot turn the steering wheel.

There are adaptions where their car seat has to be elevated up so they
see over the dashboard. There are \-- well, there are a lot of
accommodations for driving. There are several issues.

MR. NRABOSKY: A lot of LPs have to go out and economically find ways to
re-adapt their cars. One woman

in \-- I was being told by Heather, a woman that lives in the Carolinas,
South Carolina, she's real short, and she has to spend about \$20,000
just to modify the car so that she can drive it.

MS. TAYLOR: She just graduated from college with a Masters, and she is
probably about three feet, I mean, she is very, very, very little, and
her arms are very, very short. She carries a stool with her everywhere
she goes, even if she goes into a restaurant, because she could not \--
she could not even get in one of these that we are sitting in.

Her parents bought her this new car for graduation, as a graduation
gift. Well, she goes to take it to have it, the accommodations. It's
\$20,000, and they can't \-- they don't have the money to \-- they can't
afford to make the modifications that she needs. They're trying to help
their child \-- their graduate be independent and have her car and be
able to go to places that she needs to go. And her car is sitting in a
shop, waiting for the accommodations.

DR. PARKS: And I think you can see, even from your perspective, that
people of short stature \--

DR. HETLAND: Can you sit up a little bit more, at the microphone.

DR. PARKS: People of short stature differ in their body proportions,
that Heather and Bess are about equal in sitting height. And that \--

MS. TAYLOR: Our trunks are normal.

DR. PARKS: Yes.

MS. TAYLOR: Our trunks are normal height. Our arms and legs are this
short.

DR. : \-\--

MS. TAYLOR: Achondroplasia, that is one type, though.

DR. PARKS : I know.

MS. TAYLOR: That is one type of \--

DR. PARKS: I know. And I was just making the \--

(Laughter)

DR. PARKS: I was just making the point that body proportions differ,
even in people with skeletal dysplasias.

Okay. I think we could \-- and that would have impact on what is
difficult to use in an average size world, and what is easier to use.
And height is not the sole determinant in that area.

I guess I would like to \-- I think it is appropriate to open the more
general issues now.

***[Audience Questions and Discussion Session]{.underline}***

DR. HETLAND: We will open the field. Please remember to identify
yourself. I have someone here from vocational people. I am sure you have
vocational questions you would like to ask her panel \-- hint, hint.

MR. PHILIPS: I would like to ask a question.

DR. HETLAND: Okay. Please.

MR. PHILIPS: Were any of the panel \--

DR. HETLAND: Excuse me. You must speak with a microphone so that it can
be transcribed. And identify yourself.

MR. PHILIPS: Oh, thank you. My name is William Philips, and I am the
parent of Alexandra Philips.

And a burning question for me \-- I have several questions, but I will
limit it to just one question \-- and I would like for all of the
panelists, you know, to sort of jump in and answer the question. I was
always curious in terms of the social issues that when you go out in
public, when you deal with people, how is that, how is it dealing with,
you know, just a common day going out to the shopping mall, going out to
where you have a large population of people, and the reactions are \--
how are people's, or the population in general, their sensibilities to
your stature, and to your right to privacy?

MR. NRABOSKY: I guess I'll start, okay?

MS. PHILIPS: Go ahead.

MR. NRABOSKY: I think \-- like Alexandra was saying, in that when she
moved I think people are more accepting in society, I think society in
itself is just reflective of how it is changing, and a little bit more
accepting of people of different stature.

And it varies from place to place. More rural communities, you are going
to get more looks, because it is much more rare to see someone of a
different stature.

But I think that \-- how we deal with it? We all have different ways, I
guess, but some of just ignore it; some of us, if it's a child, I mean,
I'll accept it more from a child, and I'll be glad to educate them. When
it's an adult, then I kind of start to feel like it's their
responsibility, and I'll just ignore them \-- unless they truly come up
to me and say, "I don't understand," or "Help me to understand." Or \--
and some of them will ask me questions. And I don't mind, you know,
talking to them to help educate them. I just feel it is our part to help
people learn, I mean, that is society's part.

And sometimes, though, I'll tell a child, "Well, God made me that way,"
you know, "this way," I mean, because that's \-- sometimes \-- it
depends the age of the child, you know. And sometimes I've even teased
them, at Christmastime I'll tell them, "Well, you know, did you always
wonder who's been telling Santa who's been naughty or nice?"\
(Laughter)

MS. TAYLOR: For me per se, to be honest \-- honestly, I'm 35 years old,
and when I go shopping at the grocery store, to the mall, to the, you
know, I walk out of this hotel, people look at me everywhere I go. There
are comments made everywhere I go. It's part of my life.

I don't recognize it unless you get in my face. And unless you get in my
face and insult me, I don't recognize it. If you have a question, I'll
answer it.

Now, my children, on the other hand, my children are little people,
also, and we go to the mall as a family. And I've been a little person
all of my life, so I've kind of, I guess, had my own way of dealing with
the \-- the looks, the stares, the comments. But I have two children,
and being a parent, as most of you all probably are, being a parent,
when somebody is looking at your child, and

saying \-- saying something about your child, those are fighting words.

But you have to be really careful how you present yourself in front of
your child, because what kind of example as a parent would I be if I
allowed them to insult me by what they said about my child? I have to
set an example for my children, and try to console them when their
feelings have been hurt, and answer their questions, and tell \-- and
try to explain to them why people say things that they do, why do they
stare at them, you know.

And basically sometimes, you know, my child will get her feelings hurt,
and all's I can do is sit there and hug her and cry with her, and tell
her, "You know, well, it is going to be okay, and it's okay that your
feelings are hurt, you know, and it's okay."

But this is a part of our life. And we either accept it, and we learn
how to deal with it, or we grow up miserable and angry, and we want to
hide ourselves in our rooms, you know. That's all I have to say, I
guess.

MR. PHILIPS: Well, Daddy, to answer your

question \--

(Laughter)

I know you don't take me shopping a lot, but it's really stressful,
because Mom really doesn't understand what I go through. She doesn't
understand that people look, and just stare me up and down.

And sometimes I don't think about it, and sometimes I really don't feel
like being bothered. They still are so persistent. You know, when a
child that is my height, or taller, says something to me, I mean, it
really doesn't bother me, because I'm like, well, they don't know any
better anyway. But if an adult does it, I'm like, that is very ignorant.

DR. : Thank you.

(Laughter)

MS. PHILIPS: People want to label me and claim me, "Oh, you're a dwarf,
you're a midget." "I'm not. I'm just short, \-\--. And if you can't
accept the fact that I'm telling you I am 16, and I'm like this, and I
don't have a deficiency, that's your problem, because you don't want to
listen to me."

(Applause)

MS. PHILIPS: And something that you didn't

know \-- this happened recently, like Tuesday, I think. A lady at my
cafeteria is so busy, wanted to know, "How old are you?" "What grade are
you in?" "Are your parents like this?" "Why are \--" she didn't even get
my food ready. And by the time \--

(Laughter)

MS. PHILIPS: You know, by the time she finally got the order right, it
was time to go to class, and I'm not going to miss class for food. I
just was hungry.

And people just are so \-\-- about it. They come out of the blue, "How
tall are you?" "Hello. How are you?"

MS. TAYLOR: "What is your name?"

MS. PHILIPS: Yes. "How tall are you?" Like "Can't I go to glass first?
Can't I do what I have to do before you question me?" Like, "Why are
you're worried about me?" "Get yourself straight first."

(Laughter)

MS. PHILIPS: Yes, "Get a life."

(Laughter)

MS. PHILIPS: It's like people, they'll point and laugh, and I'll stop,
and like, "You are so rude. \-\-- and get some." It's like \-- because
it's very obvious that they don't have any. And I grew up where \-- to
respect everybody, no matter how they look on the outside, but how they
are on the inside.

I'm not a very judgmental person unless I know that person, and so I
can't say automatically that person looked at me and said, "I'm not
going to talk to her." I'm going to talk to anybody, because I'm just a
social butterfly, and that's how I will stay.

(Laughter)

(Applause)

MS. PHILIPS: Thank you.

MS. TAYLOR: My daughter has a T-shirt and it says, "You laugh at me
because I'm different, but I laugh at you because you're all the same."

DR. HETLAND: Touché.

I have a question for Dr. Schoen. Could you explain a little bit more to
the audience here, how do you get short bowel syndrome?

DR. SCHOEN: Well, most of the children that we see have it as the result
of an accident, or a surgery for a congenital anomaly, or because of
this disease called necrotizing enterocolitis, which causes essentially
an infection, and eschemia or a loss of blood profusion to the
intestine. And so some length of intestine is lost, and generally it is
quite a lot.

And what you try for \-- just to talk about therapy for a minute \--
because those kids have so little, absorbtive surface, they can't absorb
their nutrition, they have to be on IV nutrition. But we try different
strategies to get the bowel actually to grow. And in young children, and
especially in pre-term infants, they do have some potential to enlarge
the bowel. There are some surgical strategies for lengthening the bowel,
but ultimately those children either die or they get bowel transplants.
And sadly, many of them still die.

MR. NRABOSKY: Is this something that is genetic in any way, as caused
from a genetic issue, or \--

DR. SCHOEN: Generally not. That is a possibility, but it generally does
not have to do with genetics.

MR. HETLAND: Well, I will point out to our audience that in our POMS we
say that failure to thrive can be used as a medically determinable
impairment up to age two, but no long. And after that we don't say
anything. And I have had to field many questions about, well, why is
that? I don't have an answer. So it is one of the things we need to be
working on.

DR. PARKS: Do you have a recommendation,

Dr. Schoen?

DR. SCHOEN: Well, you know, failure to thrive is really \-- in terms of
the name is really only used in infancy and toddlers, and they switch at
two or three to malnutrition. And it seems like that is exactly what
happens with your recommendations here, because they shift over at that
age to the whole criteria for malnutrition. And I don't know that that
is necessarily inappropriate. It seems almost more nomenclature.

But you are saying is that it stands alone in infants. Why can it not
stand alone in children and teens?

DR. HETLAND: I don't have an answer for that.

DR. SCHOEN: Yes, and I \-- you know, I think the onus is always on us to
try to find out why it is. Is there another medical cause for it, which
is, you know, what we spend our days trying to figure out.

When we don't find anything, that puts us back into the category of are
there psycho-social issues that are the cause? But in situations like
that, it seems like in a way it does stand alone. Of course the whole
point is to correct it, and not to have a permanent disability in that
category.

DR. HETLAND: You quite appropriately pointed out that some of our
criteria under the 105.08 listing are not longer performed, such as
carotene and \-- I forget what the other one is, \-\--. Do you have any
recommendations about what could substitute for that, along with \-- I
think we used hematocrit and albumin.

DR. SCHOEN: Right. I think what you are trying to look at with fecal
fats and carotene are mal-absorbtion. And there are a lot of \-- there
are a number of different diseases that we didn't even talk about today
that have fat \-\-- absorption. Nowadays we really use spot tests more,
just a single sample for fecal fat, which doesn't need to be a
collection of several days. And there are stains that can look for fat
globules. There are studies that look at the broken down fatty acids.
There are tests of mal-absorbed sugar, such as the PHD acid level of the
stool. You can look at sugars that come out in the stool. The breath
tests, that look, again, at mal-absorbed sugars.

So \-- and those are non-invasive, and they are much easier than the old
collection of the large quantities of stool.

DR. HETLAND: One suggestion I have heard is that we should potentially
move weight growth disorders to our growth impairment listings under
100, and that that might make it easier for adjudicators to look at it
in that context.

I am curious as to whether people think that would be helpful or a
hindrance, in terms of ease of using our listings.

(Pause.)

Nobody wants to say anything.

MS. BENDANN: Monte, I am not going to respond tot that.

(Laughter)

DR. HETLAND: Who are you?

DR. BENDANN: I'm Janet Bendann, Childhood Policy, NFSSA.

And Dr. Schoen, I wanted to ask you to go back to something you just
said. This is a very elemental question. Failure to thrive from birth to
two, are there multiple etiologies? Did I hear you say that if it
persists after age two, there might be some question as to psycho-social
factors involved? Did I mis-hear that?

DR. SCHOEN: No, that doesn't have anything to do with age. When we call
a child failing to thrive, or malnourished, we want to find a cause for
it. And sometimes we will, you know, we just don't come up with any
reason.

And certainly when you do an initial history, you are looking for clues.
Has the formula been prepared incorrectly, or is there some reason why
the child is not being feed at appropriate intervals? And obviously

looking \-- if there are signs of vomiting, is there diarrhea, someplace
where the calories are lost?

But in some children \-- and this happens frequently \-- you just can't
come up with any reason. We see some children who just don't want to
eat, and it is almost like a cycle, where if we intervene for a period
of time, and give them nutrition through a tube for a period of time, as
they start to feel better \-- and some of this probably has to do with
gastric capacity, too. As you begin to fill the stomach, they actually
begin to have an appetite again, and they can gradually begin to take in
the calories they need, and you take the tube out. But there is not a
specific diagnosis for that.

Now, your question was, does the age or the label, failure to thrive,
versus malnutrition \-- that doesn't have anything to do with
psycho-social issues. It is just when you can't find \-- if you can't
find any other cause, you think about what else could be going on as a
reason for inadequate calories.

DR. BENDANN: Do you at any age between birth and two think about
temperament issues, or attachment disorders?

DR. SCHOEN: We do, and we have difficulty finding people to help us with
those problems.

DR. BENDANN: You mean in the field of psychiatry, or \--

DR. SCHOEN: Exactly.

DR. BENDANN: \-- or pediatric psychiatry?

DR. SCHOEN: That is something that I don't know if my colleagues would
want to comment on that, but \--

DR. BENDANN: That would seem like a rich ground for research.

DR. SCHOEN: We have a lot of difficulty finding mental health providers
in our city who can take on those kind of patients. And I would love to
hear comments about that.

DR. PARKS: It is ironic that infant feeding in a medical setting,
problems of feeding often go to the speech therapist, who has
cross-training in this area. But it is from a different background,
around \-\--, swallowing \--

DR. : Mechanical.

DR. PARKS: Mechanical, rather than interactive \--

DR. SCHOEN: We have \-- in Atlanta we have the Marcus Institute, that
you may have heard of, that Bernie Marcus brought to our community, and
they have a feeding program there. But \-- it helps in some kids. They
do have a psychologist, but there has been some turnover with that. It
is part of the Kennedy-Krieger program.

But unless you go through that feeding program, it is very difficult in
Atlanta to find help with those kind of problems.

DR. HETLAND: Dr. Dusick?

DR. DUSICK: Yes, I would like to address that just a little bit. In
infants, much failure to thrive is multi-factoral, so with that infant
we are wondering about attachment, is there maternal depression? Is
there access to formula, or are they diluting it to make it stretch and
last the whole month? Are there adequate feedings? Are there adequate
numbers of feedings?

Oftentimes, in most \-- usually what I see in my clinic, it is some of
all of that. And it takes pediatricians, primary care providers,
possibly OT, speech. We get a lot of referrals for poor growth and poor
feeding. And it is usually, like I say, multi-factoral.

Now, early intervention programs, I think, are really coming to the
forefront in learning and recognizing feeding as an adaptive disorder,
and looking at that. While there is not a specific domain, they are
looking at it. And so there are more people, I think, that are being
able to help families with early infant feeding.

DR. PARKS: Is it fair to say, though, that that has not reached the
point of being a marketable medical specialty, where you get the best
interaction is with a team approach that generally requires contracts or
grants to support the interdisciplinary team?

DR. HETLAND: Yes.

DR. HARPER: I am Charles Harper from the Atlanta Regional Office. First
just a comment for Alexandra: You might as well get used to those
questions. I am at the other end of the spectrum and people still \--
strangers will get on the elevator and say, "How tall are you?" "Do you
play basketball?" So \--

(Laughter)

They don't care. You know, people are going to ask you weird questions.

Heather and Paul, I wanted to ask you guys how your conditions have
affected you vocationally? Have you been able to find employers that
would accommodate, and were you able to work, or have you been able to
find that you couldn't work?

MS. TAYLOR: I have worked. I have not worked in four years, but my
background, I \-- as you all see, I'm a talker, and I love people. So I
was in management, and I supervised employees. So yes, I was able to
work. Did they accommodate me? No.

You know, I'm pretty independent. I took a stool in, and I had a stool
that sat under my feet at a desk. Because of my \-- I have a back issue,
and \-- at this point, and it is hard for me to sit for a length of
time. But before my back issue I was able \-- I sat at a computer, and I
put like a crate or a stool under my feet so I would have more back
support. I was able to work pretty much, yes. They didn't, you know,
there was nothing \-- there was nothing they did to accommodate me.

I got talked down to. I got patted on the head by my boss, "Good job,"
you know. I got, you know, you have \-- the social pressures, the social
pressures; not the respect, you know, as I was, I guess, a supervisor,
and I did not get the \-- I got talked down to, instead of respected, as
my position, because of my heighth. That's happened in several different
jobs that I've had.

MR. NRABOSKY: I \-- I guess I \-- at a younger age I think I got a lot
of disrespect in some ways, but I've never really \-- as far as
accommodating me, places of employment that I've been with haven't had
to accommodate me too much, although later in life I did get a special
chair, a special keyboard for my computer, a foot pedal. But I don't
even need those nowadays, because I have had bilateral hip replacement
about three-and-a-half years ago, so my physical condition has changed
dramatically.

But I \-- other than being of short stature, I'm fine. I probably am \--
I am just one of \-- I'm on the taller end of the spectrum of short
people, I think, and I just haven't been confronted with a lot of the
issues. Discrimination is out there, it's in society in general, and
sometimes it does happen in the work area.

Although as a teenager, in high school \-- Alexandra \-- I was probably
one of the most popular kids in the school, and it actually helped me
out socially a lot.

(Laughter)

So, and I was involved in things that \-- I mean, I wrestled in high
school, and I was in musical plays and things like that, and so I just
\-- I think that my social stature \-- my physical stature helped my
socialization skills.

MS. PHILIPS: Well, even though I am in a new school, I am popular, too.
I don't think it's because I'm short; it's because I just have a vibrant
personality.

(Laughter)

And as you can probably see, I am a wrestling manager. I don't wrestle
because I'm too little. And you know, that doesn't have nothing to do
with it; I'm weak. I am in a lot of clubs. I am in Latinos Anitos, which
is Latinos United \-- I'm not really Latino, but I just wanted to be in
it to learn the culture. And I'm in Spanish Club, which is basically the
same thing, but you go and do more outings, and go places. And the other
club, you really don't, you just learn the culture. I'm in CIA, which is
Christians in Action, and I'm really in that.

I am in Drama Club. We haven't done anything yet, but I am in a play,
and the major club I am in is Video Production, which is like Fox Five;
I'm a reporter, and this week I made Member of the Week, because I was
working on three stories, and I edited one of my other worker's stories,
so I've earned that title.

(Laughter)

(Applause)

MS. TAYLOR: Just to say one more thing about the jobs, I have gone \--
and I'm sure that you have, too, in your life \-- on several job
interviews on jobs that I wanted to work. I wanted to be a flight
attendant so bad. And I'm just too short, they told me I'm too short.

At one time I wanted to drive a UPS truck. I mean, you know, there's
been several jobs out there, and dreams that I've had that I've wanted
to do, but I could not do because of my size.

DR. SCHOEN: Just related to that, when you said you wanted to drive a
UPS truck, how do you reach the pedal in your car? You said that you
can.

MS. TAYLOR: Well, you know, I reach mine. Now the car that I had before,
I had extensions. It depends on the car, you know, it depends on the
car. My seats move all the way up. Now, keep in mind I'm up on top of my
steering wheel, and it's not \-- I'm not driving safely, but I'm doing
it; I'm not driving safely. But I can reach my pedals; I should have
extenders. Actually, I should.

MS. PHILIPS: To tailgate what you said,

Ms. Taylor, I do have dreams like that. I did want to be a singer, and
I'm still striving for that goal. But since

I sound like I'm about two years old, my voice isn't that developed. So
I'm not singing that much, even though I am still singing.

But my biggest dream right about now is to be a model, and you know you
have to be a Amazon to be a model.

(Laughter)

But I do have a modeling contract with Film and Fashion World to be a
commercial model, for like TV commercials and magazines. I don't do
runway; I can't do runway. But I am achieving my goals right now.

DR. HETLAND: I would like to ask our panelists, we have asked you a lot
of questions today; are there any questions you would like to ask us?

(Pause)

Okay. Go ahead.

MR. NRABOSKY: With the information-gathering here today, and with other
things you had yesterday, is there a determination of how long it is
going to take to come up with changes in the policies and, you know, do
you have a time line set on when you'll get this \-- the changes to the
policies?

DR. HETLAND: Well, I would love to say in six months, but I think from a
practical standpoint, in terms of getting everything written, it
probably will be 12 to 18 months. This is our last outreach. We have
had, I think, five or six of them over the last year and a half. And all
of them need to be re-written. So, some will be easier than others, but
we have gained a lot of information. It will make our job easier in
terms of how to re-write this.

MS. TAYLOR: Will you also do more research on, say, achondroplasia
dwarfism? Will you also do more research to help determine your
policies? Do you all do the research? Do you all do, like \-- what is my
question?

DR. SCHOEN: You have expert panels as you write it, other than just
these gatherings.

DR. HETLAND: We do have some expertise within the system that we are
able to \--

MS. TAYLOR: Consult with.

DR. HETLAND: Right.

MS. TAYLOR: I have tons of literature.

(Laughter)

DR. HETLAND: Okay. But we take all the help we can get.

MS. PHILIPS: I have a question \-- are you finished?

DR. HETLAND: Are we done now this afternoon?

MS. PHILIPS: No, are you finished with what you were saying?

DR. HETLAND: I am finished, yes.

MS. PHILIPS: Okay. Dr. Parks, I know you said something about an
inhaler, or something, as a growth \-- a growth hormone, I guess, and
you and I both know how needle-phobic I am. When is that coming out, so
I can get hold to it?

DR. PARKS: Alexandra and I and her family have a long history, and some
of her shortness is due to a lack of growth hormone. And you heard
yesterday that the usual endocrinologist response to hormone deficiency
is to say it is simple, no problem; if somebody is missing something, if
they can't make it, they can take it; we can give it to them. But
Alexandra has not been able to stay on growth hormone shots for very
long at a time, even though we tried.

Is it fair to share that \-- you raised it that we have tried five or
six times.

MS. PHILIPS: Uh-huh.

DR. PARKS: And is looking forward to an alternate delivery system that
will probably not be available for growth hormone until you are in your
20s, at which time you may still have some potential for growth, you may
have a metabolic need to growth hormone as an adult.

So I can't \-- it sounds like I am doing the same sort of political
avoidance that you have heard in other contexts, but \--

(Laughter)

Of course, we share your goals, but we are also frustrated at the time
it takes to get them achieved. So, the answer is, I don't know.

(Laughter)

DR. HETLAND: Dr. Mei, last question?

DR. MEI: \-\-- Zuguo Mei from CDC. I have a question for Dr. Park. In
your presentation, you did compare the weight-for-stature, or
weight-for-height chart for body mass index for age-of-child.

I fully agree with you, because the weight-for-stature, or
weight-for-height chart, it is much easy to explain to parents, you
know, and also very easy based on your height, you know, you have to
gain how many pound or kilo to reach whatever the average.

But you said that BMI for age is somehow, it's misleading. So I wonder
what the misleading you are \-\--.

DR. PARKS: If you look on the back of your growth charts at BMI-for-age,
you will notice that it increases as you get older. In fact, body weight
\-- and it doesn't remain constant. The advantage of the BMI is it can
be \-- the charts can be carried over into adulthood. The disadvantage
is that it saves \-\-- the second order derivative calculation, it has
no physical, takes a lot of education to get a physical meaning, and
does not lead to direct recommendations about degree of change, degree
of weight change and degree of BMI change.

The other thing is that weight actually increases as more than the
square of height, somewhere between the square and the cube. For example
\--

DR. MEI: \-\--

DR. PARKS: \-- a BMI for a person six-three would read falsely high,
compared to a younger person. A ponderosity index, or \-\-- index,
weight over height cubed, would misclassify me as being, you know,
thinner than I am.

And in childhood, the impact is that when I am looking at BMI for age, I
look at BMI for height age, to sort of keep height as a constant
variable. For the average \-\-- of the population, it is fine to do BMI
for age. For the individual whose height is far away from average, short
or tall, the BMI for the age at which most children are at that height
is better. For example, we could take the

23-year-old woman, who is 127 centimeters, and calculate her BMI, and it
would be \-- and as I recall, it is about 16; 16 would be seriously
under weight for an average size adult. But it is precisely average for
a five-year-old to

six-year-old who is her height.

DR. MEI: You mean if you simply just plot on the weight \--

DR. PARKS: If you plot on an adult BMI chart someone as short at that
young woman, or with achondroplasia, or with incompletely treated growth
hormone deficiency, like Alexandra, your plot is having a very low BMI,
but \-- for age, but would plot as having a pretty normal BMI for height
age.

DR. MEI: So in your opinion, you are more in favor, you know, for young
children, just to use the

weight-for-height.

DR. PARKS: I think weight-for-height is not subject to those
restrictions. It is published and available, easily, between ages two
and six, and I would suggest that it is a superior instrument for
judging proportionality of weight during that age, \-\-- age.

DR. MEI: Okay. I understand, because basically that is why we use the
\-\-- chart, weight-for-stature, approximately from age two to less than
six. Because one big reason it is for the U.S. \-\-- special program for
woman, infant and the children, which is a weight program, so for
children less than five you have one indicator consistently used, under
age of five.

And also for tracking, if you are tracking the young children, you can
tracking by weight-for-ounce, weight-for-stature, or from, you know,
birth to less than \-- approximately less than six years old. And also
if you are trying to tracking from childhood to adulthood, you can use
the BMI-for-age from as early as age two to adulthood.

DR. HETLAND: Okay. I just wanted to follow up in terms of the reasonable
expectations about when the new proposals will come out. The fact that
the two of you,

Dr. Parks and Dr. Schoen, have come with very explicit recommendations,
will help us tremendously in terms of starting grounds. In other
outreaches, we have not necessarily reached such concrete
recommendations, and so that helps us a lot.

(Applause)

DR. HETLAND: Also, once we as the Office of Medical Policy get done in
terms of coming up with the changes or proposals, then it has to go to
the actuarials for them to figure out, quite honestly, "Is this going to
cost more, or less, or the same?" Then it has to go to the government
lawyers to be looked at, and they have several months to look at it.
Then the Office of Management and Budget gets 90 days to look at it, and
they inevitably come back on the 89^th^ day and ask us questions that we
then have to scramble to ask.

(Laughter)

So, I would love to have it done faster, but I just wanted you to
understand that it is not all within our purview. Okay.

Thank you, very much for our speakers today.

B\` (Applause)

Before I turn things over to Mr. Sklar, I want to remind you, please
fill out the evaluations and turn them in. Also, those of you who would
like to receive copies of the Power Point presentations, or the
transcription \--

MS. : Just the Power Points.

DR. HETLAND: Just the Power Points, okay \-- please leave your name and
information with Tracy, our coordinator.

Thank you, very much. Mr. Sklar \-- Mr. Sklar is waving off. Thank you,
very much. Have a safe trip home.

(The meeting was adjourned at 3:32 p.m.)