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81–046 PDF 2003

S. HRG. 107–592

BIRTH DEFECTS: STRATEGIES FOR PREVENTION

AND ENSURING QUALITY OF LIFE

HEARING

BEFORE THE

SUBCOMMITTE ON CHILDREN AND

FAMILIES

OF THE

COMMITTEE ON HEALTH, EDUCATION,

LABOR, AND PENSIONS

UNITED STATES SENATE

ONE HUNDRED SEVENTH CONGRESS

SECOND SESSION

ON

EXAMINING PUBLIC HEALTH ISSUES RELATED TO BIRTH DEFECTS, FOCUSING ON STRATEGIES FOR PREVENTION AND ENSURING QUALITY

OF LIFE

JULY 26, 2002

Printed for the use of the Committee on Health, Education, Labor, and Pensions

(

(II)

COMMITTEE ON HEALTH, EDUCATION, LABOR, AND PENSIONS

EDWARD M. KENNEDY, Massachusetts, Chairman

CHRISTOPHER J. DODD, Connecticut

TOM HARKIN, Iowa

BARBARA A. MIKULSKI, Maryland

JAMES M. JEFFORDS (I), Vermont

JEFF BINGAMAN, New Mexico

PAUL D. WELLSTONE, Minnesota

PATTY MURRAY, Washington

JACK REED, Rhode Island

JOHN EDWARDS, North Carolina

HILLARY RODHAM CLINTON, New York

JUDD GREGG, New Hampshire

BILL FRIST, Tennessee

MICHAEL B. ENZI, Wyoming

TIM HUTCHINSON, Arkansas

JOHN W. WARNER, Virginia

CHRISTOPHER S. BOND, Missouri

PAT ROBERTS, Kansas

SUSAN M. COLLINS, Maine

JEFF SESSIONS, Alabama

MIKE DEWINE, Ohio

J. MICHAEL MYERS, Staff Director and Chief Counsel

TOWNSEND LANGE MCNITT, Minority Staff Director

SUBCOMMITTEE ON CHILDREN AND FAMILIES

CHRISTOPHER J. DODD, Connecticut, Chairman

JAMES M. JEFFORDS, Vermont

JEFF BINGAMAN, New Mexico

PAUL D. WELLSTONE, Minnesota

PATTY MURRAY, Washington

JACK REED, Rhode Island

SUSAN M. COLLINS, Maine

BILL FRIST, Tennessee

JOHN W. WARNER, Virginia

CHRISTOPHER S. BOND, Missouri

MIKE DEWINE, Ohio

GRACE A. REEF, Staff Director

PRISCILLA HOBSON HANLEY, Minority Staff Director

(III)

CONTENTS

STATEMENTS

FRIDAY, JULY 26, 2002

Page

Dodd, Hon. Christopher J., Chairman of the Subcommittee, opening

statement .............................................................................................................. 1

Reed, Hon. Jack, a U.S. Senator from the State of Rhode Island, opening

statement .............................................................................................................. 4

Cordero, Jose ́ F., M.D., Director, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, U.S.

Department of Health and Human Services, Atlanta, GA ............................... 5

Prepared statement .......................................................................................... 7

Pote, Mr. Harold, President, Spina Bifida Foundation, New York, NY; Dr.

Nancy S. Green, Medical Director, March of Dimes of Birth Defects Foundation, and Assistant Professor of Pediatrics, Albert Einstein School of Medicine, New York, NY; and Fred Liguori, Parent, Granby, CT ........................... 17

Prepared statements

Harold Pote ................................................................................................ 19

Nancy S. Green, M.D. ............................................................................... 25

Fred Liguori ............................................................................................... 32

(1)

BIRTH DEFECTS: STRATEGIES FOR PREVENTION AND ENSURING QUALITY OF LIFE

FRIDAY, JULY 26, 2002

U.S. SENATE,

SUBCOMMITTEE ON CHILDREN AND FAMILIES, OF THE

COMMITTEE ON HEALTH, EDUCATION, LABOR, AND PENSIONS,

Washington, D.C.

The subcommittee met, pursuant to notice, at 9:40 a.m., in room

SD–430, Dirksen Senate Office Building, Hon. Christopher J. Dodd

(chairman of the subcommittee) presiding.

Present: Senators Dodd and Reed.

OPENING STATEMENT OF HON. CHRISTOPHER J. DODD, A U.S.

SENATOR FROM THE STATE OF CONNECTICUT

Senator DODD. The subcommittee will come to order.

Thank you all for being here this morning. Let me just announce

ahead of time that I think we are going to have one or perhaps two

votes at 10 a.m., which will disrupt the hearing a little bit; but my

hope would be to complete your work with us, Dr. Cordero, and

since I do not have to vote immediately—I can vote after the vote

starts—so we will have about 40 minutes or so. And then I will

come back right after the vote, and we will continue the hearing.

So just to announce ahead of time that we may have some disruption of the orderly flow of events.

But I am very grateful to all of you for joining us, those of you

who are in the audience today as well as those who will be testifying, and family members and friends who have come out for this

hearing of the Senate Committee on Health, Education, Labor, and

Pensions, and the subcommittee which I am very proud to chair,

the Subcommittee on Children and Families, which I have been

proud to be associated with during my entire service here in the

United States Senate for more than 20 years.

The title of today’s hearing is ‘‘Birth Defects: Strategies for Prevention and Ensuring Quality of Life.’’ Today the Subcommittee on

Children will examine two very crucial questions—first, how do we

best prevent birth defects, and second, how do we best support

those born with birth defects and the families and loved ones who

work to assure their care.

We should begin initially with an examination of the problems

presented by birth defects. Birth defects are the leading cause of

infant mortality in the United States and affect more than 150,000

infants born each year. In many cases, children with birth defects

face a lifetime of disability. They are the leading cause of death in

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the first year of life. Sadly, the parents of one out of every 28 babies receive the terrifying news that their child has a birth defect.

Both genetic and environmental factors can cause the more than

4,000 known birth defects. However, the causes of about 60 percent

of birth defects are currently unknown.

We have a chart here on my left which will highlight some of the

points I am about to make. Each and every day in the United

States, more than 400 children are born with a birth defect. Tragically, in the same day, 17 infants will die due to a birth defect.

Another 78 infants will lose their lives before they reach their first

birthday because of a critical birth defect.

The wonderful pictures that surround the dais today show children—Nicholas Liguori, of Granby, CT, whom I just met and who

said, ‘‘Hello, Senator Dodd’’—I appreciate that, Nicholas; I told him

I have a brother named Nicholas as well, so it is a great name— Nicholas is with us today and is the son of Fred Liguori, whom we

will hear from shortly on the second panel. You are also looking at

a picture of Gregory Pote, carrying the Olympic torch. We are

proud to have you with us, Gregory. Greg is the nephew of another

witness, Hal Pote, who will also testify on our second panel.

These are children who are, thankfully, very much with us. It is

because of people like Greg and Nicholas that we are here today

to talk about this issue. So the question is not only how do we best

prevent birth defects, but also how do we best support those born

with birth defects and the loved ones who work so hard to provide

for their care.

I know that every parent here with us today will agree that

there is no greater gift for a parent than that received upon the

birth of a child. As we also know, with such a great gift comes

great responsibility. However, for parents of a child born with a severe birth defect, such a great gift brings with it even more responsibility.

Every parent wants the very best for their child. Parents of children with birth defects are clearly no different. The blessing that

comes with the birth of a child is clearly no less joyous for parents

of a special needs child. However, it is only reasonable to accept

that parents of a special needs child may require more assistance

in order to ensure that their children receive the supportive services that they require and deserve.

I believe that as policymakers here in the United States Congress, we have responsibilities as well. Our responsibility should

lead us to enact effective, helpful systems of care that not only prevent birth defects but also provide aid to those with birth defects

so they can achieve all that their abilities allow.

Thankfully, we have made progress. Since 1960, the number of

infants dying due to birth defects has been cut by more than half,

which is great news. We now know that there exist some simple

measures that can greatly decrease the chances of birth defects. We

must work to ensure that all women of childbearing age are aware

that there are certain, often very easy steps that will help to ensure a safe and healthy pregnancy.

For example, we know that a modest 400 microgram daily dose

of the B vitamin folic acid could prevent 50 to 70 percent of all

cases of spina bifida and other neural tube defects.

3

While encouraging as these numbers may be, we also know that

about half of all pregnancies are unplanned and that in order to

be effective, women must be taking the required dose of folic acid

before they get pregnant. And while studies indicate that the message stressing the critical importance of folic acid is currently being

heard, we all know that behaviors do not change overnight. I believe that if we continue our educational efforts, the desired

changes in behavior will occur, as evidenced by the fact that the

number of women who smoke during pregnancy has dropped approximately 30 percent since 1989 alone. However, we must continue to work to spread the lifesaving messages that truly prevent

birth defects.

Efforts to prevent birth defects are tremendously worthy. In fact,

they should be strengthened. And while the importance of birth defect prevention cannot be overstated, the fact remains that there is

no one action that will prevent all birth defects.

While progress has been made both with regard to birth defect

prevention and efforts to better the lives of those living with birth

defects, this is not to say that more cannot be done. In fact, Congress can and should do more.

The Birth Defects Prevention Act of 1998 authorized the Centers

for Disease Control and Prevention to conduct programs aimed at

the prevention of birth defects through surveillance, research, and

education. With the authority given by the BDPA, the National

Center on Birth Defects and Developmental Disabilities at CDC

provides funding to 28 States to develop, implement, and/or expand

community-based birth defects tracking systems, programs to prevent birth defects, and activities to improve health services for children with birth defects.

However, the BDPA’s authorization ends this year. In fact, I look

forward to working with my colleagues, both Democrats and Republicans, Senator Bond in particular, to reauthorize this important legislation and maintain our commitment to preventing birth

defects and ensuring the quality of life for those living with birth

defects.

I am also encouraged by the efforts underway to create a National Spina Bifida Program. I believe that such a program could

greatly improve our efforts to prevent the incidence and morbidity

of spina bifida and other neural tube defects. A National Spina

Bifida Program would also have the effect of greatly improving the

quality of life of the more than 70,000 people presently living with

this often severely disabling birth defect.

Thankfully, advances over the years in medical treatment such

as the development of in utero surgery have allowed children with

spina bifida to become adults living with spina bifida. I believe that

a National Spina Bifida Program would provide vital resources to

help these individuals lead full and rewarding lives.

It is my hope that the discussion we are going to have this morning will provide us with an outline that will lead us to advances

in both prevention and the development of helpful systems of care

for those living with birth defects.

So it is with great optimism that we begin today’s hearing.

Again, I want to thank all of our witnesses, particularly the famiVerDate 11-MAY-2000 10:48 Mar 06, 2003 Jkt 000000 PO 00000 Frm 00007 Fmt 6633 Sfmt 6633 S:\DOCS\81046.TXT SLABOR3 PsN: SLABOR3
4

lies who have come down with Nicholas and Greg to be part of this

program today to shed some light on these important issues.

I see that my colleague from Rhode Island has joined us, and I

thank him very much for being here and will turn to him for any

opening comments or thoughts that he might have.

Senator Reed?

OPENING STATEMENT OF HON. JACK REED, A U.S. SENATOR

FROM THE STATE OF RHODE ISLAND

Senator REED. Well, Mr. Chairman, you said it so well that I

cannot add very much at all.

I want to welcome Dr. Cordero. Obviously, this is a topic of great

concern. We have made progress over the last several years, but we

can do much more.

I would note that your interest in children goes way back; it is

no longer theoretical. Mine is strictly theoretical, so I want to just

commend you for holding the hearing, and let us get on with the

witnesses.

Senator DODD. Since you have asked, Grace’s pictures are right

here.

[Laughter.]

Senator REED. Usually I do not have to ask.

Senator DODD. She was born a little more than 10 months ago

and brought great joy. I often say that when I reach into my wallet

to take out the pictures, there is a 50–50 chance that my AARP

card will also drop out.

[Laughter.]

So I am an older father.

Let me introduce our first witness—and I thank my colleague

from Rhode Island for his comments.

This morning, we are going to hear from two panels, as I mentioned at the outset. Our first witness is Dr. Jose ́ Cordero, Director

of the National Center on Birth Defects and Developmental Disabilities at the Centers for Disease Control and Prevention.

Throughout his very distinguished career, Dr. Cordero has been

a strong and very effective leader in the fight against birth defects.

He is a pediatrician and joined the CDC in 1979, first working in

the Division on Birth Defects and Developmental Disabilities, and

later serving as deputy director of the National Immunization Program.

Since the National Center opened last year, Dr. Cordero has undertaken the critical work of saving the lives of infants through the

prevention of birth defects and developmental disabilities.

He is also a recognized leader in developing effective policies to

support those who are born with birth defects.

I thank you, Dr. Cordero, not only for your presence here today

but for many, many years of commitment to this issue. You truly

honor us with your presence, and we are anxious to hear what

ideas, thoughts, and advice you can give this Congress.

5

STATEMENT OF DR. JOSE ́ F. CORDERO, DIRECTOR, NATIONAL

CENTER ON BIRTH DEFECTS AND DEVELOPMENTAL

DISABILITIES, CENTERS FOR DISEASE CONTROL AND PREVENTION, U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES, ATLANTA, GA

Dr. CORDERO. Thank you. Good morning, Mr. Chairman, Senator

Reed.

I am Jose ́ Cordero, Assistant Surgeon General and Director of

the National Center on Birth Defects and Developmental Disabilities.

Thank you for the opportunity to be here today to discuss public

health issues related to birth defects prevention and ensuring quality of life for individuals who have a birth defect.

Mr. Chairman, as you mentioned, more than 150,000 infants are

born with a birth defect each year in the United States. Birth defects are the leading cause of infant mortality in the U.S. Babies

who do survive often experience lifelong disability. In addition to

experiencing physical impairments, 7 percent of children with a

birth defect also have a serious developmental disability such as

mental retardation, hearing loss, or vision impairment.

The challenges that confront children over the course of their

lives, including access to medical care and preventive services, the

development of secondary conditions, and social isolation are critical public health issues.

With the establishment of the National Center on Birth Defects

and Developmental Disabilities in 2001, efforts related to birth defects, developmental disabilities, and the health of people with disabilities have been given new visibility.

Although the Center is fairly new, CDC’s commitment to these

issues is not. Since 1968, CDC has made birth defects monitoring,

research, and prevention a priority. In 1985, we initiated a similar

program for developmental disabilities, and in 1988, we established

our disability prevention program and dedicated ourselves to promoting wellness for the 54 million Americans who have a disability.

When it comes to the prevention of birth defects and developmental disabilities, we have already seen a great many public

health successes. We have virtually eliminated the mental retardation, hearing loss and vision impairments associated with congenital rubella syndrome, as well as mental retardation from Hib meningitis, which I must say when I was a pediatrician was a very,

very common cause of mental retardation.

We have seen drugs which caused devastating birth defects, such

as thalidomide, denied entry to the United States market or now

tightly controlled.

Another emerging success story is the result of research which

shows that the B vitamin folic acid prevents serious birth defects

including spina bifida and anencephaly.

In 1992, the U.S. Public Health Service issued a recommendation

that all women of childbearing age should consume 400

micrograms of folic acid to reduce their risk for these birth defects.

CDC and the National Council on Folic Acid, a coalition of nonprofit organizations, took action to provide women with information

about folic acid. The members of the National Council, the Spina

6

Bifida Association of America, and the March of Dimes will also be

testifying here today.

In 1996, the Food and Drug Administration also responded to

this compelling scientific information and required all enriched cereal grain products to be fortified with folic acid by January 1998.

The results of these public health information and fortification efforts are beginning to bear fruit.

Data published last week from 24 population-based State birth

defects surveillance programs indicate that the prevalence of spina

bifida has decreased 31 percent from 1995, before fortification, to

1999, after fortification.

These results are extremely encouraging as are the data emerging from other CDC-supported projects, suggesting that folic acid

may also prevent or might have a role in preventing other birth defects.

Epidemiologic research like this is the foundation of our efforts

to identify new causes of birth defects and developmental disabilities. For example, CDC supports Centers for Birth Defects Research and Prevention in seven States. These Centers are undertaking the National Birth Defects Prevention Study, one of the

largest studies ever conducted on the causes of birth defects. This

study will provide the Nation with a continuing source of information on potential causes of birth defects. CDC is also supporting

similar research programs for developmental disabilities.

We are very optimistic about our ability to use science to identify

new causes of birth defects and developmental disabilities and the

ability of the public health community to translate the science into

action.

However, until all causes of birth defects are identified, and prevention strategies are implemented, some children will still be affected. Many of these children will share the experience of tens of

millions of other Americans who are living with a disability.

Great health disparities currently exist among people with disabilities. For that reason, CDC is supporting research related to

health promotion and wellness among people with disabilities. One

promising research project involves a community-based wellness

program known as Living Well. This program has been shown to

reduce disabilities due to secondary conditions by 37 percent and

to reduce physician visits by 45 percent. We are expanding this

program to a number of States.

Finally, CDC recognizes the importance of ensuring that people

with disabilities have access to the specific types of medical treatment and care that are required as a result of their disability but

also access to preventive health services. For example, CDC is

working to ensure that women who use wheelchairs have access to

mammograms and cervical cancer screening.

However, there are many challenges ahead of us. We can take

great pride in the fact that individuals with disabilities are living

longer. But the health care system is not adequately prepared to

deal with the consequences of our success. Most physicians have

little experience providing care to adults with spina bifida, cerebral

palsy, Down syndrome, or other similar conditions.

Young people who have grown up with spina bifida or other birth

defects often experience serious medical complications during their

7

teenage years and need assistance dealing with both physical and

social challenges associated with their disability.

It is clear that there is a need to educate health care providers

on the issue of disabilities across the lifespan. We have a long way

to go, but we are already moving forward. I think we can and will

do much more in the future.

I appreciate the opportunity to share with you this information

on CDC’s activities to prevent birth defects and developmental disabilities and promote quality of life for people with disabilities. It

is our goal to ensure optimal health and wellness, beginning before

birth and continuing throughout the lifespan. To achieve this goal,

we undertake epidemiologic and prevention research, implement

tracking and monitoring programs, and partner with organizations

who share our mission.

I hope you find this overview useful, and I will be very happy to

answer any questions that you may have.

Thank you.

[The prepared statement of Jose ́ Cordero, M.D. follows:]

PREPARED STATEMENT OF JOSE ́ CORDERO, M.D., M.P.H.

I am Dr. Jose ́ Cordero, Director of the National Center on Birth Defects and Developmental Disabilities at the Centers for Disease Control and Prevention (CDC).

I am a pediatrician and geneticist, with 29 years of public health experience related

to birth defects, developmental disabilities and children’s health.

I am very pleased to be here today to discuss the extremely important public

health issues related to birth defects prevention and ensuring quality of life for individuals who have a birth defect. More than 150,000 infants are born with a birth

defect each year. Many of these babies do not survive, and birth defects are the

leading cause of infant mortality in the United States. For those babies that do survive, they often experience lifelong disability. In addition to physical impairments,

birth defects are closely linked with a variety of cognitive and developmental disabilities—7 percent of children with a birth defect also have a serious developmental

disability. The challenges that confront these children over the course of their lives,

including access to medical care and preventive health services, the development of

secondary conditions and social isolation, are critical public health issues.

With the establishment of the National Center on Birth Defects and Developmental Disabilities (NCBDDD) in April 2001, CDC has been given increased visibility for its efforts in these areas. However, while the organizational structure for

NCBDDD is relatively new, CDC’s commitment to these important issues is not.

Since 1968, with the development of the Metropolitan Atlanta Congenital Defects

Program, CDC has made birth defects monitoring, research and prevention a priority. In 1985, we initiated a similar program for developmental disabilities. Over the

years, efforts in these two areas were focused on two primary goals—the prevention

of birth defects and developmental disabilities for which causes have already been

identified and the identification of causes for those which have not. However, we

also recognized that the public health community’s responsibility does not stop with

prevention. We cannot afford to ignore the public health needs of the 54 million

Americans who have disabilities. As a result, in 1998, we established our disability

prevention program and dedicated ourselves to a prevention research, monitoring

and intervention program aimed at identifying and implementing strategies that

will eliminate challenges to optimal health and development and promote wellness

for all Americans, including those who have a disability.

PREVENTION WORKS

When it comes to the prevention of birth defects and developmental disabilities,

we have already seen a great many public health successes. Through immunization,

we have virtually eliminated the mental retardation, hearing and vision impairments associated with congenital rubella syndrome, as well as mental retardation

from Hib meningitis. We have seen drugs which caused devastating birth defects,

such as thalidomide, denied entry to the United States market or tightly controlled.

Removing lead from gasoline has meant that far fewer children have blood lead levels that might adversely affect their intelligence, behavior and development. These

8

successes have been achieved through close coordination and collaboration between

Federal agencies—including CDC, the National Institutes for Health, the Food and

Drug Administration, Health Resources and Services Administration—and with the

private sector.

More recently, we have seen the results of many years of prevention research pay

off when we learned that increasing consumption of the B vitamin folic acid resulted

in significant reductions in the rates of neural tube defects—a category of common

birth defects which include spina bifida and anencephaly. In 1992, the United States

Public Health Service issued a recommendation that all women of childbearing age

consume 400 micrograms of folic acid to reduce their risk for these serious birth defects.

As a result of this recommendation, CDC immediately began The National Folic

Acid Campaign to provide women with information about folic acid. It is especially

important for women to know that in order to prevent these birth defects, folic acid

must be taken before conception and during early pregnancy. Educational materials

have been developed stressing the use of folic acid prior to conception and these materials are provided to State and local campaigns. The National Council on Folic

Acid, a coalition of non-profit organizations, has been established to assist in this

effort. Two key members of the National Council, the Spina Bifida Association of

America and the March of Dimes, will also be testifying here today. In 1996, the

Food and Drug Administration also responded to this compelling scientific information regarding folic acid and authorized manufacturers of enriched cereal grain

products to fortify these products with folic acid. All such products were required

to be fortified by January 1998.

We may be beginning to see the results of these public health education and fortification efforts. Data published just last week from 24 population-based State birth

defects surveillance programs supported by CDC indicate that the prevalence of

spina bifida decreased 31 percent from 1995 (pre-fortification) to 1999 (post-mandatory fortification). Additionally, the 1999 data from the National Health and Nutrition Examination Survey (NHANES) on folate blood concentrations reveal substantial increases among women of childbearing age. The trend in 1999 already exceeds

the Healthy People 2010 goal to increase the median red blood cell folate level to

220 ng/mL among nonpregnant women 15–44. Further research, which includes not

only measuring rates of birth defects but also includes measuring women’s folic acid

intake, is needed to confirm that these downward trends are a result of the fortification and public health education. However, these results are extremely encouraging,

as are the data emerging from other CDC-supported projects that suggest that folic

acid may also play a role in preventing other birth defects, such as imperforate anus

and abdominal wall defects known as omphalocele.

RESEARCH HOLDS NEW PROMISE

Epidemiologic research is the cornerstone for our efforts to identify new causes

of birth defects and developmental disabilities. Translating these epidemiologic findings into effective public health strategies through applied research represents the

next step to making prevention a reality. CDC has underway a broad range of research, including both epidemiologic and applied, that will be providing the foundation for our next prevention success stories.

CDC’s Centers for Birth Defects Research and Prevention, first established in

1996 and formalized with the passage of the Birth Defects Prevention Act of 1998

(Public Health Law 105–168), serve as a cornerstone for these efforts. The Centers

were established in 7 states with nationally recognized expertise in birth defects

surveillance and research. CDC supports and coordinates the overall activities of the

Centers, and participates as an eight study site in the National Birth Defects Prevention Study (NBDPS). This study—one of the largest ever conducted on the

causes of birth defects—is a key part of the program of research for the Centers.

The study will provide the Nation with a continuing source of information on potential causes of birth defects and will serve as a mechanism for identifying new substances in our environment that are harmful to developing babies. In addition to

participating in the NBDPS, each individual Center is undertaking its own centerspecific research agenda on issues including nutritional, environmental and behavioral factors associated with birth defects, financial and other costs of birth defects,

and birth defect prevention research.

CDC is also supporting similar research programs for developmental disabilities.

The Children’s Health Act of 2000 mandated the creation of centers for excellence

in autism and developmental disabilities epidemiology, and in 2001, CDC established CADDRE—Centers for Autism and Developmental Disabilities Research and

Epidemiology. This collaborative network of four centers plus CDC will collect and

9

analyze information on the incidence, correlates, and causes of autism and related

developmental disabilities.

Our collaborations on research to identify causes for birth defects and developmental disabilities are not limited to institutions in the United States. CDC has initiated a very important collaboration with Denmark to help improve our understanding of the potential causes of cerebral palsy—a condition which affects 10,000

babies born in the United States each year. As part of this collaboration, we will

be undertaking a case-control study of the relationship between infection in pregnancy and cerebral palsy, using cases identified in the Danish Cerebral Palsy Register and archived newborn blood samples. Denmark has a unique combination of

national public health data systems that is not found elsewhere in the U.S. or

abroad. These unique Danish data systems can all be linked by a universal personal

identifier. Thus, they provide much important information (such as health, medical,

and sociodemographic information) to carry out epidemiologic studies of reproductive

and developmental outcomes. Also, because these data systems contain information

on large numbers of individuals over long periods of time, studies can be made with

an unusually high level of statistical power. For reasons like these, such collaborations are invaluable in providing timely and relevant information that can be put

to immediate use in the development of prevention strategies.

Other prevention-oriented research currently supported by the CDC includes Legacy for Children and Project Choices. Legacy for Children is a program consisting

of randomized, controlled, longitudinal research projects being implemented at the

University of Miami and the University of California at Los Angeles. These projects

examine the potential for improvement in child developmental outcomes through

interventions designed to influence parenting behavior. The early years of life—especially from birth to age five—are crucial for cognitive and social/emotional development. Parents play a critical role in their children’s development and are responsible for their children’s environment. Children develop within the context of their

family and their development is affected by the nature of the relationship with their

parents. Previous early intervention efforts have typically included early education

for children in child care centers and social or mental health services for the parents

in their role as adults, with only marginal attention devoted to parenting behavior.

The interventions initiated by CDC focus on parenting behaviors and the relationship of the parent with his or her own child. Results from these projects will be

available over the next few years and will help determine what are the critical components of early intervention programs for children at-risk for developmental delay.

Drinking alcohol during pregnancy poses a serious threat to the health of the unborn child. While overall alcohol use during pregnancy has been declining since

1995, rates of frequent drinking and binge drinking remain high and thus continue

to pose a risk for many women. Interventions aimed at reducing alcohol consumption must begin before pregnancy because birth defects associated with prenatal exposure to alcohol can occur in the first 3 to 8 weeks of pregnancy, before a woman

even knows that she is pregnant.

In an effort to reduce the number of children born with alcohol-related birth defects, CDC is working to develop and test interventions to identify women at high

risk for an alcohol-exposed pregnancy and help them to reduce their alcohol intake

and postpone pregnancy until they stop or limit their drinking. Project Choices is

the centerpiece of CDC’s research efforts aimed at preventing alcohol consumption

during pregnancy. CDC has demonstrated that women in certain community-based

settings (such as alcohol and drug treatment centers, jails, and primary care centers

serving low income populations) have a sevenfold higher risk for an alcohol-exposed

pregnancy. However, information on how best to reduce this risk was lacking. To

address this data gap, CDC initiated Project CHOICES: a multi-site collaborative

study involving three universities (Nova Southeastern University, University of

Texas at Houston, and Virginia Commonwealth University). Results from the first

phase of the study found that two-thirds of all women who were at risk prior to the

intervention were no longer at risk at the time of the 6-month follow-up visit. The

intervention is now being tested more rigorously through a randomized clinical trial.

MONITORING OUR PROGRESS AND MEASURING OUR SUCCESS

As more causes for birth defects and developmental disabilities are identified, and

prevention strategies are implemented, it will become even more important for the

public health community to have good information regarding the number of cases

of birth defects and developmental disabilities. Only by having such information will

we be able to ensure that our programs are having their desired effect and make

any needed adjustments and improvements. In addition, monitoring rates of birth

defects and developmental disabilities over time also enables us to determine whethVerDate 11-MAY-2000 10:48 Mar 06, 2003 Jkt 000000 PO 00000 Frm 00013 Fmt 6633 Sfmt 6621 S:\DOCS\81046.TXT SLABOR3 PsN: SLABOR3
10

er rates are varying over time or in different geographic regions. Such variations

in rates may provide clues to additional causes or possible need for concern.

CDC is working toward the establishment of comprehensive monitoring and tracking programs to accomplish these objectives, and currently CDC:

• Conducts model surveillance programs to monitor the prevalence of birth defects and developmental disabilities in the metropolitan Atlanta area.

• Provides financial and technical support for State-based birth defects surveillance through cooperative agreements with 35 states, as well as through a collaborative effort of State programs known as the National Birth Defects Prevention Network that collects and disseminated State-based birth defects surveillance data.

• Supports the Fetal Alcohol Syndrome Surveillance Network (FASSNet) in five

states.

• Supports the Autism and Developmental Disabilities Monitoring Network

(ADDM) in four states.

• Funds 30 State-based Early Hearing Detection and Intervention (EHDI) tracking programs.

• Continually monitors folate in women through the NHANES.

• Works with States to improve the timeliness and quality of reporting of birth

defects through the vital statistics systems.

ENSURING HEALTH, WELLNESS AND QUALITY OF LIFE COMPLETES THE PICTURE

While we are very optimistic about our ability to use science to identify new

causes of birth defects and developmental disabilities and the ability of the public

health community to translate this science into public health action, we know that

our prevention efforts will never be 100 percent successful. For example, we estimate that folic acid has the potential to prevent 50–70 percent of all neural tube

defects. There are still other causes of neural tube defects that remain unknown.

Until the causes of all birth defects are identified and prevention strategies are implemented, close to 3 percent of all babies will still be born with a birth defect. Most

of these children will share the experience of tens of millions of other Americans

who are living with a disability. For that reason, CDC has also placed a high priority on research focused on promoting health and wellness among people with disabilities.

Working with universities and other research organizations, CDC conducts research to develop a better understanding of the varied and unique health conditions

of people with disabilities. CDC seeks to identify health risks and protective factors

and to measure and develop effective health promotion activities to prevent the secondary conditions related to living with a disability. CDC is currently funding 11

projects in academic settings that develop new methods for promoting health and

wellness among people with disability. One particularly promising research project

will be rigorously testing a community-based wellness program, known as ‘‘Living

Well,’’ which has previously been shown to reduce disability due to secondary conditions by 37 percent and reduced physicians visits by 45 percent over 6 months. This

empirically grounded health promotion intervention, delivered by community-based

consumer-controlled independent living centers, offers a model for both improving

health and independence of adults with physical disabilities, as well as containing

medical services costs within a managed-care context. The new study will evaluate

the model with a larger, more representative sample and a more rigorous design.

Additional data on costs and preferences will be developed to conduct a cost-effectiveness assessment. The researchers will also look at how adults with physical disabilities can maintain and improve their health after participating in a health promotion program to further enhance cost-effectiveness.

In addition, CDC is working with State health departments to improve their capacity for implementing programs to address the public health needs of people with

disabilities. Currently, CDC is supporting such activities through cooperative agreements with 16 states. Specific programmatic efforts include improving State surveillance activities, conducting health promotion interventions, developing and monitoring policies affecting people with disabilities, and facilitating partnerships between

State- and research- or service-based agencies. These programmatic efforts do not

focus on specific types of disabilities, but rather they address common issues in a

variety of cross-cutting disability domains including communication, learning, mobility, and personal care/home management.

Finally, CDC recognizes the importance of working with the health care delivery

system to ensure that people with disabilities have access not only to the specific

types of medical treatment and care that are required as a result of their disability,

but also access to important preventive health services. For example, CDC has

worked to ensure that women with a mobility impairment who use a wheelchair

11

have access to mammograms through the Breast Cancer Early Detection Program.

To ensure this, CDC works with State programs to purchase special equipment. In

addition, CDC is working to ensure that people with disabilities have access to information that will help them reduce health risk behaviors such as tobacco use and

sedentary lifestyles.

However, there are major challenges that remain ahead of us. While we can take

great pride in the advancements that have been made in increasing the life expectancy of individuals with disabilities, we also must recognize that the health care

system is not adequately prepared to deal with the consequences of our success. The

life expectancy of an individual with Down Syndrome has increased from 1 year to

49 years since the late 1960’s. Just several decades ago, very few children with

spina bifida survived at all. Because these changes have occurred over a relatively

short period of time, most physicians have had no experience dealing with an adult

with these types of conditions. In 1994, SBAA and United Cerebral Palsy sponsored

a symposium entitled ‘‘Preventing Secondary Conditions Associated with Spina

Bifida or Cerebral Palsy.’’ Then, as now, one of the major issues of concern to people

with spina bifida and cerebral palsy, their families and their health care providers

was the issue of transition from pediatric care. The need is particularly acute in

teenage years. These are difficult years for any young person, but for a young person with a disability, such as spina bifida, they can be even more challenging. For

example, teenagers with spina bifida often experience serious medical complications.

In addition, these teenagers often need assistance in dealing with both physical and

social challenges associated with catheterization, skin care, and cleanliness. Social,

educational, and vocational support are also essential to ensuring full participation

as an adult.

It is clear that, in addition to developing programs that address the public health

needs of people with disabilities, there is a need to educate healthcare providers on

the issue of disability across the lifespan. Addressing these issues will also provide

an opportunity to begin to prepare our public health and health care systems for

the challenges presented by the aging of the American population. I look forward

to working toward future successes in these areas, and recognize that achieving

them will require broad-based partnerships and collaborations with research, public

health, medical and voluntary organizations. We have a long way to go, but we are

already moving forward. I think we can and will do much more in the future.

I appreciate this opportunity to share with you this information on CDC’s activities to prevent birth defects and developmental disabilities and ensure quality of life

for individuals who have experienced disability. Our goal is to ensure optimal health

and wellness—beginning before birth and continuing throughout the lifespan. To

achieve this goal, we undertake epidemiologic and prevention research, implement

tracking and monitoring programs, and partner with health care and voluntary organizations who share our mission. I hope you found this overview useful, and I will

be happy to answer any questions that you may have.

Thank you.

Senator DODD. Thank you very much, Dr. Cordero, for your

work. Of course, the work of the National Center is tremendous,

and we are very grateful for the efforts that have been made and

the initiatives that have been undertaken.

I have a number of questions. Just picking up on one of the last

points you made about the need to educate health care providers

and the problem that exists out there with the lack of understanding among the physician or health care provider population, what

specific recommendations would you make? We are going to be reauthorizing legislation here, and if there are some things we could

recommend as part of our efforts that could help close that gap, I

would think that that would be one of the critical things we could

try to achieve.

Dr. CORDERO. Yes, Senator. There are two areas that we are actually approaching at CDC. One is that we have entered into a collaborative agreement with the American Academy of Pediatrics to

educate physicians about the challenges in the first 18 years of life

of working with individuals with disabilities, particularly spina

bifida and others.

12

Second is that CDC is developing, in collaboration with the National Institutes of Health and the AHRQ, to look at a consensus

on what are the specific activities that can be done to help individuals with spina bifida. This consensus conference will address

issues of medical care, issues of functional limitations, and also social and activities of daily life. I think that will identify both what

are the research gaps and what are the things that we can implement currently to help individuals with spina bifida.

Senator DODD. When do you anticipate those recommendations?

Dr. CORDERO. The meeting is scheduled for April or May of 2003.

Senator DODD. The reauthorization expires this year, so any

thoughts you have ahead of time would be helpful if we are going

to reauthorize before the expiration. I do not want to have to get

to this in the next Congress. We could end up losing a year or so.

It is going to be tough this fall with so much on the agenda, but

this will be very important to get reauthorization. I anticipate wide

support for us, so if you could give us some ideas specifically on

how we can do that, it would be helpful early on.

Dr. CORDERO. Right. We will be happy to provide more specifics

in writing for you.

Senator DODD. Would you, please? That would be very, very

helpful to us. And I can see heads nodding in the audience, so I

am sure we will get some additional suggestions on this.

What I will do is ask a couple more questions and then turn to

my colleague from Rhode Island. As I announced ahead of time,

Jack, we will then break to go over and vote, but we will at least

finish with Dr. Cordero. So I will watch the clock, and I may have

to submit some additional questions to you in writing.

I wonder if you could give us some idea as to why these numbers

continue—they are still large numbers. We know that we have

done a lot with folic acid, yet the numbers still seem high in the

case of spina bifida among certain communities. I wonder if you

could shed some light on that.

Second—and I will ask both questions and then turn to my colleague—what has the CDC identified as some of the environmental

causes of birth defects, and what is the National Center doing to

provide information about these causes so that people have a better

level of education about what to avoid and what to be careful

about?

Dr. CORDERO. Let me answer the first one about the numbers in

the case of spina bifida. Actually, I think we have made great

progress, as I mentioned, with the over 30 percent decline in spina

bifida comparing before and after fortification.

We have made some progress in educating women and families

about the use of folic acid, but we realize that we still have challenges in reaching some communities, particularly the Hispanic

community. We are actually working to develop a more aggressive

campaign to educate women in Hispanic communities about the

use of folic acid. We have actually started a project in Miami and

San Antonio on how we can be sure to reach the Hispanic community, with messages but also working with physicians and the communities in those areas to be sure the message gets out and that

it is translated into action.

13

Senator DODD. That is important. What about the environmental

issues?

Dr. CORDERO. CDC is also working with the Center for Environmental Health and the National Institute for Environmental

Health Sciences in actually funding a number of projects and centers looking at the area of birth defects and developmental disabilities.

As I mentioned, we are funding seven Centers for Birth Defects

Research and Prevention, and these centers are actually conducting

the largest study, one of the largest ever, and there, we are hoping

to identify new causes of birth defects, including environmental

areas.

In terms of what we can recommend today, I think the important

things are, one, that women begin to take folic acid— Senator DODD. When they are of childbearing age.

Dr. CORDERO [continuing]. Of childbearing age, yes—and also,

that they actually stop using alcoholic beverages before pregnancy

begins, quit smoking, and also, that they review with their doctor

what kinds of exposures they have in the workplace to ensure that

they are not exposed to chemicals and other things that could be

a problem for them during pregnancy.

Senator DODD. Are there lists of these things, for instance,

chemicals or substances in the workplace—there are known lists of

these materials, aren’t there, that women who are pregnant or are

thinking about getting pregnant should not be around?

Dr. CORDERO. Yes. Actually, one of the challenges that most

women have when they seek information is finding one place where

they can get all the information. And actually, one of our fundings

for this year is a center that would provide information. This is a

center in California which would be able to answer women’s questions about what are the kinds of exposures, so if they have specific

questions, they can be answered. I think that that is something

that hopefully, we can expand in the future.

Senator DODD. And these health clinics—last Monday, we opened

a dental division—we have 11 public health clinics in Connecticut,

but they are all over the country, and they are growing, and they

are very successful. It seems to me that for the audiences that we

are talking about here, those places would be a very good place to

start, given the fact that so many people who may not be as wellinformed about the problems could start getting that information

when they show up at these health clinics.

Dr. CORDERO. I agree, Senator. I think that that is an area that

we can look into, how we can expand there.

Senator DODD. Yes. I have about 100 more questions for you, but

let me turn to my colleague from Rhode Island, Senator Reed.

Senator REED. Thank you very much, Mr. Chairman.

Thank you, Dr. Cordero. When we were doing the Children’s

Health Act of 2000, I became aware of and interested in the Fragile

X problem. Is CDC doing any work with respect to Fragile X?

Dr. CORDERO. Senator, we have been working with a number of

colleagues on Fragile X for a number of years. We actually funded

Dr. Stephanie Sherman, who is one of the world experts on Fragile

X, to conduct a study looking at two questions—one, what can be

done to help individuals with Fragile X, and two, can we begin

14

screening for Fragile X in the near future. At that time—this was

a few years ago—the answer was that it was not yet the time for

that. And we are working with colleagues, reviewing those questions, and especially what we can do to identify children with Fragile X early and then, what are the interventions that may actually

work.

Let me say that we are about to publish a paper in the Morbidity

and Mortality Weekly Report that has actually been looking at the

delays in diagnosing Fragile X. I think that that is going to give

us some information to then move into additional actions.

Senator REED. Thank you, Doctor.

This line of questioning raises the issue of the impact of genomic

research, generic research, on birth defects and to what degree

CDC or NIH is pursuing the breakthroughs that we have seen in

genomics.

Dr. CORDERO. I think genomics offers a great opportunity for,

one, finding more causes of birth defects, especially looking at the

genetic environment interactions, which is an area that we had

very little opportunity to look at before. We are working very closely with our Office of Genomics at CDC, as well as with the NIH,

in exploring areas where we can use the genomics knowledge to actually find causes of birth defects. In fact, several of our centers—

a center in Iowa is actually looking at the role of genes in cleft palate, and also the interaction of cleft palate and genes in causing

these serious birth defects.

I think it is the future, and we expect and will continue through

our Centers on Birth Defects to do more research and fund more

research in that area.

Senator REED. I know the Center on Birth Defects is preparing

a study about the topic. It seems to me that there are so many different ways to approach the issue, since it is very complicated, but

without being overly simplistic, there are some basic public health

initiatives—education, good nutrition—and then, there are some

more sophisticated issues in terms of genetic predisposition, environmental factors.

Within your study, are you also going to send up here or devise

a strategy that would approach the public health problems in terms

of the comprehensive way, and let us know how much it would

cost, and then lay out a strategy to deal with the more complicated

issues of genetics and environmental? Is that something that you

are contemplating?

Dr. CORDERO. The answer is yes. Actually, I think that what you

are outlining is very much the approach that we are following in

terms of addressing both the issues of birth defects and also when

we apply genetics into it. For whatever we do—let us take the example of even spina bifida—we actually need to educate the public,

we need to educate the health care professionals, and then determine how the intervention actually works and how we insert also

the laboratory work that needs to go into it.

I agree, and I think that that is exactly the process that we are

following, and as we get more into the genetics of diseases, I think

we will have more of that. But the public health effort to educate

and track and also conduct more research will be the basis of what

we will continue to do.

15

Senator REED. Thank you very much, Dr. Cordero.

Thank you, Mr. Chairman.

Senator DODD. Thank you, Senator Reed.

I would ask you if you could, just in response to Senator Reed’s

questions, if you are going to get us some early information in the

next month or so where you include this kind of information. As

I said, Doctor, you have a very willing and anxious Senator here

on this issue. Senator Bond offered the original bill back in 1998,

I guess it was—he is going to try to come by this morning, by the

way—but we would very much like to get as much of this information as we can so that when we craft the reauthorization legislation, we can accommodate as many of these ideas as possible in

that bill.

Dr. CORDERO. We will be very delighted to provide more information in writing.

Senator DODD. And before we race to make the vote, I did not

get to the questions on the additional costs. I mean, $1 million is

not an outrageous number when you start talking about the cost

of continuum of care. That is a staggering figure. And obviously,

these disabilities do not choose people based on economic circumstances and their ability to afford it; they are blind to that. So

we need to be talking as well about how we can provide some really

meaningful support so that with the wonderful new breakthroughs

now medically, people can live very productive lives for a long time,

and to enhance their ability to do that and support their caregivers

and their families has got to be a critical piece of any discussion

in this area. So I would be very interested as well in any ideas you

can suggest and give to us on how we can be more supportive of

the families out there and the individuals themselves, so they can

have lives that are fruitful and productive.

If you would make that a part of your recommendations to us,

I would appreciate it.

Dr. CORDERO. We certainly will, and especially the care of individuals with birth defects is a key area that needs to be addressed.

Senator DODD. Yes. Again, I will submit some additional questions to you in order not to hold you up, and we will come right

back as soon as the vote is over.

[Questions of Senator Dodd were not available for inclusion at

press time, however, copies are retained in the committee file.]

Dr. CORDERO. Thank you.

Senator DODD. The subcommittee will stand in recess for a few

minutes.

[Recess.]

Senator DODD. The subcommittee will come to order.

My apologies to all of you for being disrupted with the vote. Let

me thank all of you again for being here.

I am going to submit for the record a statement from Senator

Bond who will be unable to join us this morning but, as I mentioned a while ago, was the author of the authorization in 1998. So

his statement will be included in the record.

[Statement of Senator Bond was not available at press time,

however, copies are retained in the committee files:]

Senator DODD. I am pleased now to introduce our second panel

of witnesses and thank all of you again for being here.

16

First, we will hear from Mr. Hal Pote. Mr. Pote is the president

of the board of directors of the Spina Bifida Foundation, which is

affiliated with the Spina Bifida Association of America, an organization dedicated not only to eradicating spina bifida but one that

is admirably working to better the lives of more than 70,000 individuals living with this birth defect.

In addition to his responsibilities at the Foundation, Mr. Pote is

a regional bank executive at J.P. Morgan Chase Bank. He is the

proud uncle of Greg, whom I met before the hearing and who is

with us here today and whose picture is up behind me here, carrying the Olympic torch.

Mr. Pote, we thank you for joining us today. You are very gracious to be here, and I commend you for the wonderful work that

you are doing.

Next, we are going to hear from Dr. Nancy Green, who has recently been named the medical director for the March of Dimes, an

organization that we are all familiar with for its long record of lifesaving work. Since its founding in 1938 by President Franklin Roosevelt, the March of Dimes has proven itself an invaluable national

resources to the Nation’s public health.

Dr. Green received her M.D. from Columbia University College

of Physicians and Surgeons and currently serves as associate professor of pediatrics and cell biology at the Albert Einstein College

of Medicine in New York.

We congratulate you on your new position and look forward to

you working with this committee in the coming years.

Our final witness is Fred Liguori from Granby, CT. I am pleased

to have you here, Fred, coming from our State. Fred and his wife

Jill have a son, Nicholas, whom I have already mentioned and

talked about, a fine young man.

We also have up here on the dais a picture of Nicholas riding his

tractor. He is reading diligently over there with his mother at this

particular point. Nicholas has spina bifida, and Mr. Liguori will

testify to some of the unique needs that Nicholas has.

I have already discussed some of the costs that can hit a family,

and we are anxious to hear your ideas and thoughts, and again, we

are very grateful to you, as well as to you, Hal, for being here. I

am always impressed when people are willing to come forward in

a public setting and talk about these matters. It is not easy to do

so, and I am glad that you have brought family along as well so

we will get a chance to meet them—so these are not just numbers

and statistics and data, but we in the Congress here and our staffs

and members who can be here, those who are watching on CSPAN,

which is covering this hearing today, can appreciate that this is

more than just about data and numbers. It is about real human

beings and real needs and what we can do cooperatively to make

those burdens lighter, and to see that we maximize the potential

of each one of these wonderful God-given gifts of life.

So we thank you for coming today, and we will begin with you,

Hal, and move right down the table in the order that I have introduced you.

17

STATEMENTS OF HAROLD POTE, PRESIDENT, SPINA BIFIDA

FOUNDATION, NEW YORK, NY; DR. NANCY S. GREEN, MEDICAL DIRECTOR, MARCH OF DIMES BIRTH DEFECTS FOUNDATION AND ASSISTANT PROFESSOR OF PEDIATRICS, ALBERT

EINSTEIN SCHOOL OF MEDICINE, NEW YORK, NY; AND FRED

LIGUORI, PARENT, GRANBY, CT

Mr. POTE. Thank you, Senator.

Mr. Chairman and Senator Reed, you are very gracious with

your remarks. I want both of you to know how pleased we are to

have this opportunity to be here today, so thank you very much for

that.

I am here representing the Spina Bifida Association of America,

the Spina Bifida Foundation, and our whole spina bifida family,

and I respectfully request that my testimony be included in the

record.

As you mentioned in your opening remarks, Senator Dodd, spina

bifida is a neural tube defect that results from the spine failing to

close properly. More important, it is the most common birth defect

in the United States that results in permanent disability, and as

you mentioned a minute ago, there are currently 70,000 families in

our country who have been affected by spina bifida.

Our Association is a national voluntary health agency working

on behalf of all of these folks and their families through education,

advocacy, research and service. We are about 30 years old, and

today we have 60 chapters and serve 100 communities around the

country.

As you mentioned, Senator, my personal passion for this issue

comes about through my relationship with my nephew, Greg, who

is here today. He is a terrific kid, and I love him deeply, but he

has been dealt a tough hand. He has had several major surgeries;

he has another one scheduled for next month. On the other hand,

as you can see from that photograph, he is clearly the most famous

member of our family, and while that was very early in the morning, Senators, I am sure that both of you saw him on television

when he was carrying the torch.

[Laughter.]

While prevention of spina bifida is a critical public health issue,

and we are fully committed to the range of activities that can reduce the incidence of spina bifida, in the interest of time, I will

focus my remarks on three other important issues, and my written

testimony will deal with prevention. And Dr. Green, I am pleased

to say, in her role here representing the March of Dimes, will be

focusing on prevention in greater detail.

The three issues are the following. First, quality of life. As a result of this neural tube defect, most babies and children with it suffer from a very broad range of challenges, including physical, psychological, and educational challenges. These include paralysis, developmental delay, numerous surgeries, and living with a shunt in

an attempt to ameliorate their condition.

After decades of poor prognoses and short life expectancy, due to

recent breakthroughs in research and treatment, more children

with spina bifida are becoming adults with spina bifida, and that

is the very good news. This then provides a new range of chalVerDate 11-MAY-2000 10:48 Mar 06, 2003 Jkt 000000 PO 00000 Frm 00021 Fmt 6633 Sfmt 6601 S:\DOCS\81046.TXT SLABOR3 PsN: SLABOR3
18

lenges including education, job training, independent living, and

health care, now prolonged for secondary conditions.

Even if we are successful in preventing the majority of spina

bifida cases in the future through folic acid and supplementation

of grain, our Nation must still take the steps to ensure that the

thousands of individuals living with spina bifida today can live full

and healthy lives.

To that end, improved secondary prevention interventions and

treatment therapies must be identified, developed, and delivered to

those in need. Dr. Cordero referenced some of the things that we

are working on in conjunction with his organization, AHRQ, and

others.

Secondary conditions associated with spina bifida—and by the

way, these secondary conditions are more complicated for a person

with spina bifida than they are for anyone with any other birth defect—can include neurological disorders, paralysis, bladder and

bowel control difficulties, learning difficulties, depression, social

and sexual issues, latex allergy, obesity, and skin breakdown.

For example, our children with learning disabilities may have

difficulty paying attention, expressing or understanding language,

and grasping reading and math. So a response there—early intervention with children who have experienced learning problems can

help them considerably prepare for school.

With appropriate, affordable, and high-quality medical, physical,

and emotional care, most people born with spina bifida will live a

long and productive life. Ensuring access to the full range of necessary and appropriate services is essential to improve the quality

of life for those born with spina bifida.

The second topic, which you have referenced already, Senator, is

cost. It is in fact estimated—and we do not yet have hard data on

this, but that is part of what we are doing—that the lifetime cost

of care for individuals with spina bifida very often exceeds

$500,000 and very often exceeds $1 million, as you mentioned. And

Senator, as you pointed out earlier, when you look at that milliondollar cost and then you think back to Dr. Cordero’s testimony, the

decline in spina bifida incidence is happening in the socioeconomic

groups where you would expect to first see it. The challenge is that

folks who have greater socioeconomic challenges are also the folks

who are not seeing a decline in spina bifida incidence, and they are

in fact the folks who are least able to afford these burdens of medical cost and probably least likely to have the private health insurance that can help ameliorate that situation.

Nationally, we think this is a $750 million per year issue, and

we know that the Social Security Administration is paying out $82

million a year to folks who are affected by spina bifida.

The third issue that I would like to comment on deals with the

range of choices available to families when they learn of a diagnosis of a fetus with spina bifida. Historically, that family had two

choices—first, to carry full term, to have a beautiful child but a

child who would be faced with a lifetime of challenges; and the second option was to terminate the pregnancy voluntarily. There is

perhaps now a third option developing from in utero surgery, and

through the work being done now at three hospitals, it is very

early, and we are now supporting the work being done to go back

19

and look at the results, but it appears that there is some encouraging evidence coming out that, for example, perhaps 30 to 50 percent of births of folks who have received in utero surgery would result in their not requiring a shunt. So there could be some very exciting things coming out of in utero surgery, but we need support

to make sure we understand that and that those best practices can

be promulgated to the community of surgeons as well.

I would like to close by first thanking Dr. Cordero for his testimony and for his leadership. There are two specific messages that

we would like to leave with this subcommittee.

The first is that the National Center for Birth Defects and Developmental Disabilities is critical for our community. It was a very

important step forward when this Center was founded and Dr.

Cordero’s leadership was established. We would urge that it be

funded at a level of $115 million this coming year. I think that is

about $25 million more than the funding it received last year. We

are well aware that this is a budget with lots of challenges, but we

would love to see that kind of support.

Then, secondly, as you indicated in your opening comments, Senator, identifying $3 million of that to support the Center of Excellence for Spina Bifida would be something that would also be very

important to us as well.

So in closing, let me again thank you on behalf of our community

and for the 70,000 families for the leadership that you have shown

and the work that your subcommittee is doing, and thank you for

the opportunity to be here.

Senator DODD. Thank you very much for very fine testimony. I

look forward to working with you some more on this.

[The prepared statement of Mr. Pote follows:]

PREPARED STATEMENT OF HAROLD POTE

INTRODUCTION

Mr. Chairman, and Members of the Subcommittee, on behalf of the Spina Bifida

Association of America (SBAA) and the Spina Bifida Foundation, thank you for the

opportunity to come before you today to discuss issues related to Spina Bifida, the

nation’s most common, permanently disabling birth defect.

I am honored to be here today to discuss with you the importance of preventing

Spina Bifida and improving the lives of the more than 70,000 individuals and their

families who are affected by this condition. I respectfully request that my testimony

be included in the Congressional Record.

While in my day job I currently serve as the head of the Regional Banking Group

at JP MorganChase, I also serve as the President of the Spina Bifida Foundation

Board, a cause that is near and dear to my heart. SBAA is the national voluntary

health agency working on behalf of people with Spina Bifida and their families

through education, advocacy, research and service. The Association was founded in

1973 to address the needs of the Spina Bifida community and today serves as the

representative of 60 chapters serving more than 100 communities nationwide.

My personal interest and passion which drives my involvement with these organizations stems from the fact that my family is one of the 70,000 affected by Spina

Bifida. My nephew Gregory Pote—who is here today in the audience—was born with

Spina Bifida 16 years ago. One of my proudest moments as Greg’s uncle was the

morning that our family awoke before the crack of dawn and gathered together on

the side of a street in Philadelphia to watch Greg carry the Olympic torch earlier

this year. To date, Greg has already had more than 20 surgeries. Double digit numbers for surgeries unfortunately are not unusual for children living with this condition. I am here today to outline what Congress can do to prevent Spina Bifida and

help improve the quality of life for Greg and the other individuals and their families

that live with this birth defect.

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BACKGROUND

Spina Bifida is a neural tube defect (NTD) and occurs when the spinal cord fails

to close properly during the early stages of pregnancy. This happens within the first

few weeks of pregnancy and most often before the mother knows that she is pregnant. Over the course of the pregnancy—as the fetus grows—the spinal cord is exposed to the amniotic fluid which increasingly becomes toxic. It is believed that the

exposure of the spinal cord to the toxic amniotic fluid erodes the spine and results

in Spina Bifida. There are varying forms of Spina Bifida occurring from mild—with

little or no noticeable disability—to severe—with limited movement and function.

Unfortunately, the most severe form of Spina Bifida occurs in 96 percent of children

born with this birth defect.

The result of this neural tube defect is that most children with it suffer from a

host of physical, psychological, and educational challenges—including paralysis, developmental delay, numerous surgeries, and living with a shunt in their skulls

which seeks to ameliorate their condition by helping to relieve cranial pressure associated with spinal fluid that does not flow properly. It is wonderful to report that

after decades of poor prognoses and short life expectancy, children with Spina Bifida

are now living long enough to become adults with Spina Bifida. These gains in longevity principally are due to breakthroughs in research, combined with improvements in health care and treatment. However, with this extended life expectancy,

our Nation and people with Spina Bifida now face new challenges—education, job

training, independent living, health care for secondary conditions, aging concerns,

among others.

PREVENTION

While the exact cause of Spina Bifida is unknown, over the last decade, medical

research has confirmed a link between a woman’s folate level before pregnancy and

the occurrence of Spina Bifida. Sixty million women are at risk of having a child

born with Spina Bifida and each year approximately 4,000 pregnancies in this country are affected by Spina Bifida, resulting in 2,500 births. Recent studies have

shown that if all women of childbearing age were to consume 0.4 mg of folic acid

prior to becoming pregnant and throughout the first trimester of pregnancy, the incidence of Spina Bifida could be reduced by up to 75 percent. There are few public

health challenges that our Nation can tackle and conquer by three-fourths in such

a straightforward fashion. However, we must still be concerned with addressing the

25 percent of Spina Bifida cases that cannot be prevented by folic acid consumption

as well as ensuring that all women of child-bearing age consume adequate amounts

of folic acid.

Progress has been made in convincing women of the importance of consuming folic

acid supplements and maintaining diets rich in folic acid. Since 1968, the Centers

for Disease Control and Prevention (CDC) has led the Nation in monitoring birth

defects and developmental disabilities, linking these health outcomes with maternal

and/or environmental factors that increase risk, and identifying effective means of

reducing such risks. While the CDC has a long-standing history of addressing birth

defects and developmental disabilities, in 2000—as you know—the National Center

on Birth Defects and Developmental Disabilities (NCBDDD) was established at the

agency as created by the Children’s Health Act of 2000. NCBDDD’s mission is to

improve the health of children by preventing birth defects and developmental disabilities. Mr. Chairman we thank you and other members of the subcommittee and

the full Health, Education, Labor, and Pensions (HELP) Committee for your assistance in creating the NCBDDD.

Former CDC Director Jeff Koplan stated last year that the Center’s folic acid prevention campaign has reduced neural tube defect births by 20 percent. This public

health success should be celebrated, but it is only half of the equation as approximately 2,500 babies are born each year with Spina Bifida. This public education

campaign must be enhanced and broadened to reach segments of the population

that have yet to heed this call to ensure that as many cases of Spina Bifida can

be prevented as possible.

However, even if we are successful in preventing the majority of Spina Bifida

cases in the future, our Nation must still take steps to ensure that the thousands

of individuals living with Spina Bifida can live full and healthy lives. To ensure the

highest quality of life possible, prevention interventions and treatment therapies

must be identified, developed, and delivered to those in need. As I mentioned earlier, major medical advances have permitted babies born with Spina Bifida to have

a normal life expectancy and live independent and fulfilling lives. Despite these

gains, individuals and families affected by Spina Bifida face many challenges—physical, emotional, and financial.

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CHALLENGES OF SPINA BIFIDA

Secondary conditions associated with Spina Bifida include full or partial paralysis,

neurological disorders, bladder and bowel control difficulties, learning disabilities,

depression, latex allergy, obesity, skin breakdown and social and sexual issues.

Spina Bifida children with learning disabilities may have difficulty with paying attention, expressing or understanding language, and grasping reading and math.

Early intervention with children who experience learning problems can help considerably to prepare them for school. With appropriate, affordable, and high-quality

medical, physical, and emotional care, most people born with Spina Bifida likely will

have a normal or near normal life expectancy. Ensuring access to these services is

essential to improving the quality of life for those born with this birth defect.

Julie Yindra, an active volunteer with the Spina Bifida Association of America

from North Carolina is with us in the audience today. As an adult with Spina

Bifida, Julie believes strongly that the key to being able to be independent, self-sufficient and productive is education. Because of that, Julie is close to completing a

PhD in Higher Education and working at Greensboro College to increase access for

students with disabilities.

It is important to note that the lifetime costs associated with a typical case of

Spina Bifida—including medical care, special education, therapy services, and loss

of earnings—are as much as $1,000,000. The total societal cost of Spina Bifida exceeds $750 million per year with just the Social Security Administration payments

to individuals with Spina Bifida exceeding $82 million per year. Moreover, tens of

millions of dollars are spent on medical care covered by the Medicaid and Medicare

Programs. I believe strongly that our Nation must do more to help reduce the emotional, financial, and physical toll of Spina Bifida on the individuals and families

affected.

IN-UTERO SURGERY TO ADDRESS SPINA BIFIDA

SBAA seeks to support individuals and families affected by Spina Bifida, maximize the prevention of Spina Bifida, and ensure that as many Spina Bifida pregnancies go to term as possible. When families recently diagnosed with a Spina

Bifida pregnancy contact SBAA, the organization puts them in touch with another

family who has a child with the condition so they can learn of the joys and challenges of having a child with the birth defect. Unfortunately, traditionally when

families have faced a Spina Bifida diagnosis they have had two difficult options. The

first is to continue the pregnancy with the expectation of multiple surgeries for the

child after birth, uncertain life expectancy, and many physical and developmental

challenges and complications. The second, unfortunately, is to voluntarily terminate

the pregnancy. Fortunately, now there may be a third option.

Since the late 1990’s, doctors at three U.S. hospitals—Children’s Hospital of

Philadelphia, Vanderbilt University Medical Center in Nashville, and the University

of California at San Francisco—have been operating before birth on fetuses diagnosed with Spina Bifida. Last year, the University of North Carolina, became the

fourth hospital in the Nation to perform these operations. By closing the lesion early

in pregnancy, these doctors believe they can minimize the damage created by fluid

leaking from the spine, as well as by the spinal cord’s contact with amniotic fluid.

Surgeons have found that closing the hole in the spine in this fashion before birth

may correct breathing problems in 15 percent of the children receiving the procedure and may reduce the need for a shunt to drain brain fluid buildup by between

33 percent and 50 percent. Given the potential for this surgery in the secondary prevention of conditions associated with Spina Bifida, we must do a better job of studying this procedure, educating health care providers and families about this surgery,

and making information about it available to more families facing a Spina Bifida

pregnancy. It is our hope that with improved quality-of-life for individuals and families affected for Spina Bifida available through this surgery as well as interventions

through a National Spina Bifida Program, the stigma and fear associated with a

Spina Bifida birth will decrease significantly. In turn, the number of Spina Bifida

pregnancies continued to term likely will increase.

THE NEED FOR A NATIONAL SPINA BIFIDA PROGRAM

SBAA and the Spina Bifida Foundation are working hard to ensure that our Nation is taking all the steps possible to prevent Spina Bifida and diminish suffering

for those currently living with it. To that end, we currently advocate that the CDC

receive $3 million in fiscal year (FY) 2003 to develop and implement a National

Spina Bifida Program to promote quality-of-life programs which support people with

Spina Bifida so they can live fulfilling and productive lives.

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A national program works on two critical levels—to reduce and prevent Spina

Bifida incidence and morbidity and to improve quality-of-life for those living with

Spina Bifida. A National Spina Bifida Program would ensure that what is known

by scientists is practiced and experience by the 70,000 individuals and families affected by Spina Bifida. For example, such an initiative would help individuals with

Spina Bifida and their families learn how to treat and prevent the secondary health

problems which range from learning disabilities and depression to severe allergies

and skin problems that make life difficult for these individuals. All of these problems can be treated or prevented, but only if those affected by Spina Bifida are properly educated and taught what they need to do to keep themselves healthy.

A National Spina Bifida Program offers young families hope and help through a

plan to educate the public of the importance of taking a vitamin with folic acid and

the maintenance of a healthy diet that includes foods fortified with folic acid. As

SBAA works collaboratively with CDC and the March of Dimes to increase awareness of the benefits of folic acid for those at risk of having a baby with neural tube

defects (those who have Spina Bifida themselves or those who have already conceived a baby with Spina Bifida) these activities need to be expanded to reach the

broader population in need of these public health education, health promotion, and

disease prevention messages. Such efforts should include reaching out to at-risk

populations (e.g. Hispanic-Latino communities), health professionals, and community outreach in this critical public health education and awareness effort.

Second, a National Spina Bifida program offers benefits to those who suffer from

Spina Bifida and their families by working to improve the outlook for a life challenged by this complicated birth defect, identifying valuable therapies from in utero

throughout the lifespan and making them available and accessible to those in need.

These secondary prevention activities represent a tangible quality-of-life difference

to the 70,000 citizens in the U.S. with Spina Bifida. Focusing on living well with

Spina Bifida, the initiatives as part of the secondary prevention program should be

designed to create and implement strategies to improve the quality-of-life. These

quality-of-life efforts would center on reaching the general population with Spina

Bifida, advancing treatment of Spina Bifida and its related conditions, and working

with adolescents living with Spina Bifida to address their specific academic, psychosocial, and vocational needs. In addition, we envision that a National Spina Bifida

Program would create and implement a comprehensive program to assist teens with

Spina Bifida in the development of life skills for independence, self-reliance, and

success in the world.

Initiating a National Spina Bifida Program will help ensure that our Nation

mounts a comprehensive effort to prevent and reduce suffering from Spina Bifida.

SBAA and the Spina Bifida Foundation stand ready to work with you and your colleagues to reduce suffering and improve the quality of life for the 70,000 individuals

and their families who live day in and day out with this birth defect. Again, I thank

you for your calling this important hearing and for your support and leadership for

establishing a National Spina Bifida Program. I would be happy to answer any

questions you and your colleagues may have. Thank you.

Senator DODD. Dr. Green, thank you for being here.

Dr. GREEN. Thank you for the opportunity to speak this morning,

Mr. Chairman, Senator Reed.

I am Dr. Nancy Green, medical director of the March of Dimes

Birth Defects Foundation, and I am pleased to have the opportunity to speak this morning on behalf of our 3 million volunteers

and 1,600 staff of the March of Dimes. I want to thank others for

their comments about the March of Dimes, including our work not

only on prevention but also treatment of neural tube defects and

other important and serious birth defects.

As you know, the March of Dimes works to improve the health

of mothers, infants, and children by preventing birth defects and

infant mortality.

The Foundation is a unique partnership of scientists, clinicians,

parents, volunteers, business and community leaders throughout

the Nation. We also have a chapter structure in all 50 States as

well as the District of Columbia and Puerto Rico.

The statistics that were discussing this morning on birth defects

are very troubling, obviously, to all of us. It is a serious health

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problem facing our Nation. We take a two-pronged approach to this

problem. One is research to identify causes and improve prevention

tools and treatment tools, and access to health care for women and

infants so they can best benefit from existing and emerging medical

knowledge.

So I want to support your efforts and those of several Federal

agencies to prevent birth defects and to improve the quality of life

for those born with birth defects. In addition, I want to use my

time to urge Congress to reauthorize the Birth Defects Prevention

Act of 1998 as a way of ensuring that this important work continues.

The March of Dimes defines birth defects as an abnormality of

structure, function, or body metabolism presenting at birth or in

early childhood that results in physical or mental disability, or is

fatal.

The entire research budget of the March of Dimes supports basic

and clinical research, the ultimate aim of which is to assure that

every baby born is healthy. The research funded by the Foundation

addresses basic biologic processes that underlie development and

also examines clinical aspects of pregnancy.

Research currently funded includes studies of developmental biology and genetics and those important processes leading to

healthy birth outcomes. When these steps go awry, normal birth is

abrogated, with consequences of miscarriage, stillbirth, or live

births of infants with birth defects.

So all research that is supported by the March of Dimes deals

with prevention of birth defects. In 2001, the Foundation’s research

budget was $39.7 million. In the history of our grant program,

awards have been made to more than 1,200 universities including

Yale, Brown, Harvard, Columbia, Stanford, University of California, and other fine institutions throughout the Nation. The average

award in 2001 was $72,000.

As a private foundation, the March of Dimes can respond relatively quickly to new directions or promises in research to accelerate the pace of scientific discovery. Previous March of Dimes research included identification of fetal alcohol syndrome, which we

heard about this morning, thus establishing the association between birth defects and alcohol use. Fetal alcohol syndrome is the

most common preventable birth defect in the United States, affecting about 4,000 infants every year.

More recently, the Foundation has been funding professional and

public educational outreach efforts to reach women and teach them

about healthy behaviors during pregnancy to help prevent serious

birth defects such as fetal alcohol syndrome.

The March of Dimes has also funded research on fetal surgery,

as you heard about just now, for successful interventions for congenital diaphragmatic hernia, as well as early but promising studies on spina bifida.

In addition, the March of Dimes has funded research on Fragile

X, including Steve Warren, who identified the Fragile X gene. This

is the most common form of inherited mental retardation and has

generated three important outcomes for Fragile X. One, it has increased the understanding in the medical community that some

forms of mental retardation, including some common forms of menVerDate 11-MAY-2000 10:48 Mar 06, 2003 Jkt 000000 PO 00000 Frm 00027 Fmt 6633 Sfmt 6601 S:\DOCS\81046.TXT SLABOR3 PsN: SLABOR3
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tal retardation, are inherited. This leads to the ability to refer affected families for genetic counseling. Second, the work on Fragile

X has launched a major research effort to identify additional genes

and disorders causing mental retardation. And third, the work on

Fragile X has resulted in early screening possibilities for this and

related disorders and to begin to determine the impact of early

interventions from that early screening for affected children to determine whether their intellectual development can in fact be augmented.

So we are very proud of our research program, and our volunteers and staff vigorously support that program.

In addition, we support Federally-funded research related to

birth defects. As you know, the March of Dimes was a vigorous advocate for two recent legislative efforts on birth defects prevention—the Birth Defect Prevention Act of 1998 and the Children’s

Health Act of 2000, through which the Center that Dr. Cordero

represents so ably was created.

In addition, the March of Dimes has been reaching out to Congress for approval on these important measures. As you know, in

1998, Congress passed the Birth Defects Prevention Act with broad

bipartisan support. This has allowed for, as I mentioned, creation

of this National Center on Birth Defects and Developmental Disabilities.

As you know, the Prevention Act of 1998 authorized allocations

through fiscal year 2002. We understand that Senator Bond will be

introducing the Prevention Act of 2002, and we urge you to support

this throughout Congress to maintain Federal commitment to preventing birth defects and ensuring the quality of life for those living with birth defects. And we are privileged to be working closely

with the Center on their efforts in this regard.

To help find the causes of birth defects, Dr. Cordero mentioned

the funding of the Centers for Birth Defects Research and Prevention. Four months ago, the March of Dimes testified before the

House Appropriations Committee and submitted a written statement to the Senate Appropriations Committee requesting an increase in the budget of these centers so they can expand and intensify their research pertaining to genetic and environmental causes

of birth defects.

These seven regional centers and the eighth site at CDC are participating in the National Birth Defects Prevention Study, the largest such study ever conducted. I think this research holds a lot of

great promise for finding etiologies of birth defects and appropriate

prevention.

The Birth Defects Center also supports the development, implementation, and expansion of State birth defects tracking systems,

programs to prevent birth defects, and activities to improve access

to health services for children with birth defects. March of Dimes

chapters around the country work closely with these State-based

birth defects surveillance programs.

Finally, the ultimate goal of tracking and research is the development and implementation of effective programs to prevent birth defects and developmental disabilities. One such example of this success has been in the national folic acid campaign, led by the March

of Dimes and, as Dr. Cordero mentioned already this morning, with

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the CDC and many partner organizations including, of course, the

Spina Bifida Association. As you heard, we know that this campaign is getting results both through outreach to professionals and

to the public, women of childbearing age, as you know so well, as

well as grain fortification. So we are cautiously optimistic that this

trend will continue and that there will continue to be a decrease

in the number of children born with spina bifida and related neural

tube defects, and we continue our work on folic acid.

In addition to our support of the National Center, the March of

Dimes works collaboratively with other Federal agencies. For instance, we have been a very outspoken advocate for the doubling

of the NIH research budget. We also work closely with HRSA and

CMMS on initiatives to treat children with birth defects and other

special health care needs.

Improving access to newborn screening is one of the Foundation’s

highest advocacy priorities here in Washington as well as in every

State capital. This year, the March of Dimes asked the Appropriations Committee to allocate $25 million for implementation of the

newborn screening program authorized in Title XXVI of the Children’s Health Act of 2000. If adequately funded, this program

would make it possible for States to develop, evaluate, and acquire

innovative testing technologies and improve the screening, counseling and special services for those at risk, these newborns and children.

In conclusion, on behalf of the March of Dimes, I want to thank

you, Mr. Chairman, Senator Reed, for holding this hearing today.

The March of Dimes volunteers and staff around the country are

ready to work with you and other members of this committee to

support public policies and provide the resources necessary to prevent birth defects and ensure that those who are born with birth

defects enjoy the best life possible.

Thank you for your attention.

Senator DODD. Thank you, Dr. Green, very much.

[The prepared statement of Nancy Green, M.D. follows:]

PREPARED STATEMENT OF NANCY GREEN, M.D.

INTRODUCTION

Good morning, Mr. Chairman. I am Dr. Nancy Green, medical director of the

March of Dimes Birth Defects Foundation. I am pleased to have the opportunity to

testify this morning on behalf of the over 3 million volunteers and 1600 staff of the

March of Dimes, and share with you the Foundation’s views on ‘‘Birth Defects:

Strategies for Prevention and Ensuring Quality of Life.’’

As you know, the March of Dimes is a national voluntary health agency founded

in 1938 by President Franklin D. Roosevelt to find a scientific prevention of the

threat of polio to the public. Today, the Foundation works to improve the health of

mothers, infants and children by preventing birth defects and infant mortality

through research, community services, education and advocacy. The March of Dimes

is a unique partnership of scientists, clinicians, parents, members of the business

community, and other volunteers in every state, the District of Columbia and Puerto

Rico.

The statistics on birth defects are very disturbing and illustrate a serious health

problem facing our nation. Of the four million babies born each year in the United

States, approximately 150,000, or 34 percent of all live births, have at least one serious birth defect. Severe birth defects often require lifelong medical treatment. Because many conditions cannot be fully corrected, birth defects are a major cause of

childhood and adult disability. Birth defects are also the leading cause of infant

mortality. The March of Dimes believes a two-pronged approach to prevention and

treatment of birth defects is required: research to identify causes and improve preVerDate 11-MAY-2000 10:48 Mar 06, 2003 Jkt 000000 PO 00000 Frm 00029 Fmt 6633 Sfmt 6621 S:\DOCS\81046.TXT SLABOR3 PsN: SLABOR3
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vention tools, and access to health care so women and infants can benefit from existing medical knowledge. I am grateful for the invitation to come before this subcommittee to share the March of Dimes agenda for prevention and treatment. I am

here today to support your efforts and those of several Federal agencies to prevent

birth defects and to improve the quality of life for those born with birth defects. In

addition, I want to use my time with you to urge Congress to reauthorize the Birth

Defects Prevention Act of 1998 as a way of ensuring that this important work continues.

BACKGROUND ON BIRTH DEFECTS

The March of Dimes defines birth defects as an abnormality of structure, function

or body metabolism (inborn error of body chemistry) presenting at birth or early

childhood that results in physical or mental disability, or is fatal. There are more

than 4,000 known birth defects. Both genetic and environmental factors can cause

birth defects. In some cases, a child inherits a genetic disease when one parent (who

has the disease) passes along a single faulty gene; examples of birth defects caused

by dominant inheritance include Marfan syndrome and achondroplasia (a form of

dwarfism). More frequently, a child inherits a genetic disease when both parents

(who are healthy) pass along the same faulty gene; this is called recessive inheritance. Examples include Tay-Sachs disease and cystic fibrosis. Abnormalities in the

number or structure of chromosomes can cause numerous birth defects. Down syndrome, in which a baby is born with an extra chromosome 21, is one of the most

common chromosomal abnormalities.

Birth defects also result from environmental factors such as drug or alcohol abuse,

infections (such as rubella) or exposure to certain medications (such as the acne

drug Accutane) or other chemicals. Often, birth defects appear to be caused by a

combination of one of more genes and environmental factors, such as cleft lip/palate,

clubfoot and some heart defects.

Birth defects generally are grouped into three major categories: structural/metabolic, congenital infections, and other conditions. When a baby has a structural birth

defect, some part of the body is missing or is malformed. Heart defects are the most

common type of structural birth defects, affecting one baby in 125. Spina bifida affects one in 2,000 babies. Metabolic disorders are not visible, but can be harmful

or even fatal, and affect one in 3,500 babies. PKU (phenylketonuria) is an example

of a metabolic disorder, in which affected babies cannot process a part of protein,

which builds up in blood and results in brain damage. Fortunately, this disorder is

routinely detected with newborn screening tests, so affected babies can be placed on

a special diet that prevents mental retardation.

Rubella (German measles) is probably the best known congenital infection that

can cause birth defects. If a pregnant woman is infected in the first trimester, her

baby has a one-in-four chance of being born with one or more symptoms of congenital rubella syndrome (deafness, mental retardation, heart defects, blindness). Fortunately, because of aggressive immunization programs, this syndrome has been

eradicated in the United States and many other countries.

Other causes of birth defects include substance abuse during pregnancy. For example, fetal alcohol syndrome (FAS), which affects one baby in 1,000 (about 4,000

babies per year in the United States) and results in mental and physical birth defects, is common in babies whose mothers are heavy drinkers of alcohol during pregnancy. FAS is the most common preventable birth defect in the United States. Babies of mothers who use cocaine early in pregnancy are also at increased risk of

birth defects. Studies suggest that these babies are five times more likely to be born

with urinary tract defects than babies of women who do not use cocaine while pregnant.

MARCH OF DIMES RESEARCH ON BIRTH DEFECTS

The entire research budget of the March of Dimes supports basic and clinical research, the ultimate aim of which is assurance that every baby is born healthy. Research supported by the Foundation addresses basic biological processes that underlie development and also examines clinical aspects of pregnancy. Research currently

being conducted includes studies of developmental biology and genetics aimed at explaining fundamental steps that lead to healthy birth outcomes. When these steps

go awry, normal birth is abrogated with the consequence of a miscarriage, still

birth, or live birth of an infant with a birth defect. If we improve our understanding

of these normal steps and their occasional aberration, we will be in a better position

to devise means of prevention and intervention. Thus, all research that is supported

by the March of Dimes deals with the prevention of birth defects.

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In 2001, the Foundation’s research budget totaled $39.7 million. In the history of

our grant program awards have been made to more than 1,200 institutions, including Yale, University of Connecticut, Washington University, Harvard, Columbia

University, Cornell, University of California—Los Angeles, Stanford, University of

California—San Francisco, and Johns Hopkins University. The average award in

2001 was $72,000 per year; the median $73,000; the range $48,000–97,000. Typically grants are awarded for 3 years. As a private foundation, the March of Dimes

is able to act quickly to fund creative new approaches, accelerating the pace of scientific discovery.

Previous March of Dimes research achievements include identification of fetal alcohol syndrome (FAS), thus establishing the association between the birth defects

and alcohol use. More recently, findings from research supported by the Foundation

have been used to develop professional and public educational messages that aim

to change unhealthy behaviors of pregnant women. The March of Dimes has also

supported research to refine fetal surgery techniques, a successful intervention for

congenital diaphragmatic hernia and spina bifida. In addition, the March of Dimes

supported research on Fragile X, the most common form of inherited mental retardation, which has generated three important outcomes. First, it has increased understanding in the medical community that some forms of mental retardation are

genetic in origin, leading to referral of affected families for genetic counseling. Second, it has launched a major research effort to investigate other heritable and potentially preventable forms of mental retardation. And, third, it has resulted in

early screening for disorders such as Fragile X and in clinical studies to ascertain

the impact of early interventions for affected children to determine whether their

intellectual development can be augmented.

FEDERALLY SPONSORED BIRTH DEFECTS RESEARCH AND SERVICES

Although we at the March of Dimes are justifiably proud of the Foundation’s research program, the volunteers and staff of the March of Dimes strongly support

federally funded research related to birth defects and programs that provide services

to those living with birth defects. For example, the March of Dimes was a vigorous

advocate for two recent legislative initiatives that focus on birth defects prevention:

the Birth Defects Prevention Act of 1998 (P.L. 105–168) and the Children’s Health

Act of 2000 (P.L. 106–310) through which the CDC National Center on Birth Defects

and Developmental Disabilities was created. In addition to Chairman Dodd, Senators Kennedy and Bond who are members of this subcommittee were instrumental

in obtaining Congressional approval of these important measures.

BIRTH DEFECTS PREVENTION ACT REAUTHORIZATION

In 1998, Congress passed the Birth Defects Prevention Act with broad bipartisan

support. The act, first introduced by Senator Bond, authorized the Centers for Disease Control and Prevention to (1) collect, analyze, and make available data on birth

defects; (2) operate regional centers for applied epidemiological research on the prevention of birth defects; and (3) inform and educate the public about the prevention

of birth defects. CDC’s current birth defects prevention activities described below

are made possible by the Birth Defects Prevention Act and conducted under the auspices of the National Center on Birth Defects and Developmental Disabilities.

The Birth Defects Prevention Act authorized these prevention programs, as well

as the appropriations that support the National Center on Birth Defects and Developmental Disabilities, through fiscal year 2002. We understand that Senator Bond

will be introducing the ‘‘Birth Defects Prevention Act of 2002’’ shortly to revise and

extend the Birth Defects Prevention Act. Mr. Chairman, the March of Dimes urges

you and the members of the full HELP Committee to complete action on the Senator’s bill before the end of this Congress and thereby maintain the Federal commitment to preventing birth defects and ensuring the quality of life for those living

with birth defects.

NATIONAL CENTER ON BIRTH DEFECTS AND DEVELOPMENTAL DISABILITIES

Two years after passage of the Birth Defects Prevention Act, Congress took another major step toward preventing birth defects and improving the lives of those

born with a birth defect when it created the National Center on Birth Defects and

Developmental Disabilities (NCBDDD) as part of the Children’s Health Act of 2000.

The NCBDD was officially launched in April 2001. The mission of the Center is to

improve the health of children and adults by preventing the occurrence of birth defects and developmental disabilities; promoting optimal child development; and promoting health and wellness among children and adults who have a disability.

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The Center focuses on identifying the yet unknown causes of birth defects and developmental disabilities through research programs; preventing birth defects and

developmental disabilities through public as well as professional education; and promoting wellness for all Americans, including those with a disability. The March of

Dimes is privileged to work closely with the Center in carrying out activities to meet

these objectives.

To help find the causes of birth defects, NCBDDD currently funds regional ‘‘Centers for Birth Defects Research and Prevention’’ in Arkansas, California, Iowa, Massachusetts, New Jersey, New York, and Texas. Each center is funded at a level of

approximately $900,000 per year. Four months ago, the March of Dimes testified before the House Appropriations Committee and submitted a written statement to the

Senate Appropriations Committee requesting an increase of $6 million in the budgets for these centers so that they can expand and intensify their research pertaining

to genetic and environmental causes of birth defects.

These seven regional centers and the eighth site at the CDC are participating in

the National Birth Defects Prevention Study, the largest study on the causes of

birth defects ever conducted. Now, with information collected on more than 11,000

families, the data are being used in studies designed to help identify the causes of

birth defects. For example, work now underway focuses on the effectiveness of various methods for the primary prevention of birth defects, the mechanisms of inducing birth defects by various drugs, the environmental causes of birth defects, the

genetic factors that make individuals susceptible to birth defects, and the behavioral

causes of birth defects. Moreover other studies examine the costs associated with

birth defects. This exciting leading edge research holds great promise and merits an

increase in funding.

NCBDDD also supports the development, implementation, and expansion of State

birth defects tracking systems, programs to prevent birth defects, and activities to

improve access to health services for children with birth defects. March of Dimes

chapters around the country work closely with these State-based birth defects surveillance programs.

Finally, the ultimate goal of tracking and research is the development and implementation of effective programs to prevent birth defects and developmental disabilities. One example of a success in this area is the national folic acid education campaign, lead by the March of Dimes, the CDC and its partner organizations, such as

the Spina Bifida Association. This is a multi-year national education campaign designed to increase the number of women taking folic acid daily. Each year in the

United States, approximately 2,500 babies are born with neural tube defects

(NTDs). NTDs are severe birth defects of the brain and spinal cord, including

anencephaly and spina bifida. CDC estimates that the annual medical care and surgical costs for persons with spina bifida in the United States exceed $200 million,

and that up to 70 percent of NTDs could be prevented if all women of childbearing

age consumed 400 micrograms of folic acid daily, beginning before pregnancy. We

know that this campaign is getting results. In fact, a study published in the Journal

of the American Medical Association in 2001 showed that NTDs in newborns decreased 19 percent between 1995 and 1999 in the wake of this campaign. However,

we at the Foundation believe that many more fatal or disabling birth defects could

be prevented if more women of childbearing age were to take a multivitamin with

folic acid daily.

The Foundation also supports the vital work of the National Center on Health

Statistics which offers information essential for these research and programmatic

initiatives. Investment in the National Vital Statistics System, a major source of information on birth outcomes and birth defects, would allow CDC to modernize this

system using web-based technology that would provide for rapid compilation of data

as well as tools to improve the accuracy and completeness of information obtained

from physicians and hospitals. These improvements are needed to better detect

trends in birth outcomes and to support birth defects registries. Additional resources

would also support expansion of the National Survey of Family Growth to provide

essential information on factors that affect birth outcomes.

In addition to our support of the National Center on Birth Defects and Developmental Disabilities, the March of Dimes also works collaboratively with agencies

that fall within the jurisdiction of this committee. In particular, the Foundation has

been an outspoken advocate of doubling the research budget of the National Institutes of Health. With an increase in funding, NIH could expand its research into

the causes of birth defects, and also the causes of prematurity. Increased Federal

funding would also help accelerate the timetable for implementing a much needed

analysis of environmental influences on child health and development that is contemplated by the National Children’s Study authorized by this Committee in the

Children’s Health Act of 2000.

29

In addition to our work on behalf of a more robust Federal research agenda, the

March of Dimes also works closely with Health Resources and Services Administration and the Centers on Medicare and Medicaid Services on initiatives to treat children with birth defects and other special health care needs. Improved access to newborn screening is one of the Foundation’s highest advocacy priorities here in Washington and in every State capital. One of the great advances in preventive medicine

has been the introduction of newborn screening to identify certain genetic, metabolic, hormonal and/or functional conditions in newborns. As the Chairman knows,

such disorders, if left untreated, can cause death, disability, mental retardation and

other serious lifelong problems. This year the March of Dimes asked the Appropriations Committee to allocate $25 million for implementation of the heritable disorders (newborn screening) program authorized in Title XXVI of the Children’s Health Act of 2000. If adequately funded, this program would make it possible for

states to develop, evaluate, and acquire innovative testing technologies and improve

these screening, counseling, testing and special services for at risk newborns and

children. On June 14, 2002, a March of Dimes volunteer testified before this subcommittee about the importance of expanding the Federal role to assure that newborn screening programs in every State have access to the technical guidance and

financial resources they need to make it possible for every child born in the United

States to be screened using State of the art diagnostic and treatment tools.

CONCLUSION

On behalf of the March of Dimes, I want to thank you, Mr. Chairman, for holding

this hearing today. March of Dimes volunteers and staff around the country stand

ready to work with you and the other members of this committee to support public

policies and provide the resources necessary to prevent birth defects and ensure that

those who are born with birth defects enjoy the best possible quality of life.

Senator DODD. Mr. Liguori, thank you for being here, and thank

you, Jill, as well for coming, and Nicholas.

Mr. LIGUORI. Thank you, Mr. Chairman, Senator Reed. Thank

you very much for inviting me to testify before you here today. I

greatly appreciate this opportunity to help provide you with a parent’s perspective on spina bifida.

I respectfully request that my testimony be included in the Congressional Record.

Senator DODD. It will be.

Mr. LIGUORI. I am Fred Liguori from Granby, CT. I have been

a pilot for American Airlines for 16 years. I am also a lieutenant

colonel in the Massachusetts Air National Guard, where I have

flown the A–10 aircraft for the past 21 years. However, my most

important role is that of father to my 31⁄2-year-old son, Nicholas,

who was born with spina bifida.

As I am mindful of the time today, I will provide you with an

abbreviated version of my family’s experience with spina bifida.

However, I would like to draw your attention to my written testimony, as it contains a longer, more detailed account and information about this all too common birth defect.

When my wife Jill and I decided to start a family, it did not go

as expected. After losing two pregnancies, Jill became pregnant for

a third time. All went smoothly until an ultrasound exam during

the 18th week. As the doctor scanned the baby’s back, he stopped

and exclaimed, ‘‘Your baby has spina bifida.’’

I asked him what spina bifida was, and he explained that it was

an opening of the baby’s spine and informed us that babies born

with spina bifida may walk as children with the aid of braces and

crutches, but normally end up in a wheelchair as an adolescent.

They have no control of their bowel or bladder. They need to have

a shunt placed in their brain to control the hydrocephalus. And he

told us to expect some degree of mental retardation.

30

After telling us of our options, the doctor asked if we had any

questions. My questions were: Am I awake, or is this just a nightmare, and if I am awake, who just hit me in the head with that

baseball bat?

By the next day, we began to focus on the decision before us,

which we felt should be based on quality of life issues, and unfortunately, the medical professionals painted a very bleak picture. We

spent the next few days in the public library and medical libraries.

We read sections of textbooks and research papers. We contacted

physicians and other health care professionals around the country

who were involved with spina bifida children.

Finally, we contacted the Spina Bifida Association of America,

SBAA, and asked if we could talk to parents of children with spina

bifida. They put us in contact with several families within the

State chapter in Connecticut.

After speaking with a number of families, we noticed a big discrepancy between what the medical professionals told us to expect

and what the parents thought regarding quality of life of their affected children.

One of the families invited us to meet their 9-year-old daughter,

Jaclyn, who was born with spina bifida. We arrived at the house

and rang the bell. As the door opened, there was a beautiful, smiling little girl with pink crutches and braces up to her hips welcoming us into their home. In that instant, we knew what kind of quality of life Jaclyn had.

We spent the night talking to Jaclyn about her hobbies and her

friends, and we spoke with her parents, Janet and Anthony, about

her numerous surgeries and their battle with the school system.

We left relieved that our decision was made. Now we needed to get

as much information as possible before our son was born. On our

way out the door, Janet handed us a copy of ‘‘Insights into Spina

Bifida,’’ a newsletter published by SBAA.

While reading through ‘‘Insights,’’ we came upon an article that

concerned in utero surgery on unborn fetuses to reduce the effects

of spina bifida. The reasons for doing the surgery as early as possible made sense, and years of animal research yielded some positive results.

After very careful consideration of the risks versus the hoped for

rewards for both our unborn son and my wife, we elected to proceed with the fetal surgery at Children’s Hospital of Philadelphia,

one of the two hospitals performing the surgery at the time.

After the surgery, we were to remain in Philadelphia until the

birth of our son. The surgery was performed at 25 weeks gestation.

Jill and I spent the next week in the hospital and the next 3

months at the Ronald McDonald House in Philadelphia.

On January 14, 1999, the joy of our life, Nicholas Ferdinand

Liguori, was born. Nicholas spent 8 days in neonatal intensive

care. We then returned home to Connecticut. Almost immediately,

the doctor and therapist visits began. He has been evaluated by a

neurosurgeon, an orthopedic surgeon, a urologist, a cardiologist,

and a host of other health care professionals.

Today, Nicholas’ bladder and bowels do not function properly. He

requires two medications daily to allow his bladder to fill at low

pressure, to prevent bladder spasms, and to prevent urinary tract

31

infections. We catheterize Nicholas five times a day to completely

empty his bladder, and we are working on a bowel training program to hopefully achieve social continence.

At 31⁄2 years old, Nicholas has attended spina bifida clinics in

Connecticut, Pennsylvania, Springfield, MA, and Boston, MA,

where he is currently treated. He receives physical therapy, occupational therapy, speech therapy, and developmental therapy— each once per week.

Despite all this, Nicholas has what is considered a ‘‘mild’’ case

of spina bifida. He walks on his own without the aid of braces or

crutches. Due to the positive changes that occurred in his brain as

a result of the fetal surgery, he has not required a shunt. At 31⁄2

years old, Nicholas had his first surgery since birth this past July

5. The little girl sharing his hospital room also had spina bifida,

was the same age as Nicholas, and she was recovering from her

23rd surgery.

As parents of a child with spina bifida, we are very grateful to

Congress for its assistance and leadership regarding programs for

the prevention of spina bifida and improving the quality of life for

individuals with spina bifida. The establishment of a National

Spina Bifida Program at the Centers for Disease Control and Prevention would provide a crucial necessary next step to help reduce

the occurrence of spina bifida and to help those already affected.

A national program would help improve public awareness, which

I believe should be considered a top priority. More also needs to be

done to improve the quality of life for those living with spina bifida.

The number one concern of parents and individuals is that few improvements have been made in the area of bowel and bladder continence, which has devastating emotional and social consequences

for the individual.

A national program would also help with the establishment of

multidisciplinary care facilities. Spina bifida is a complex defect

that cannot be handled by ordinary health care professionals. With

clinics established, the health care providers within that facility

will become more experienced with the problems affecting spina

bifida patients and how those problems interrelate.

In addition, we must have educated professionals within the

school system. Educators need to understand the types of learning

disabilities associated with spina bifida and develop alternative

methods of learning. They also need to teach independent living to

those individuals who may not have a support network at home.

Our collective goal should be for the majority of individuals with

spina bifida to live long, healthy, independent, and productive lives.

Finally, research into the cause of and a cure for all neural tube

defects must be increased. Our job will not be complete until there

is no longer a need for a National Spina Bifida Program.

Again, on behalf of my son, my wife, and myself, thank you, Mr.

Chairman, for giving me the opportunity to tell our story to you

today. It has meant a great deal to us, and we stand ready to be

of any assistance to you and your colleagues as you consider issues

relating to this all too common birth defect.

Thank you.

Senator DODD. Great job.

[Applause.]

32

[The prepared statement of Mr. Liguori follows:]

PREPARED STATEMENT OF FRED LIGUORI

Mr. Chairman and Members of the Subcommittee, thank you very much for inviting me to testify before you today. I greatly appreciate this opportunity to help provide you with a parent’s perspective on Spina Bifida.

I am Fred Liguori from Granby, Connecticut. I have been a pilot for American

Airlines for 16 years. I also am a Lieutenant Colonel in the Massachusetts Air National Guard where I have flown the A–10 aircraft for the past twenty-one years.

However, one of my most important roles is that of father to my three-and-a-half

year old son, Nicholas, who was born with Spina Bifida.

When my wife, Jill, and I decided to start a family it did not go as expected. After

losing two pregnancies Jill became pregnant for a third time. We were excited but

apprehensive. Given our past history, Jill was scheduled for routine ultrasound

exams at six, twelve, and 18 weeks into the pregnancy. All went smoothly until the

eighteenth week. I remember looking at the screen as the technician scanned our

baby’s head thinking that it didn’t look right to me—but what did I know, I’m a pilot not a radiologist. The technician excused herself and returned with the doctor.

As the doctor scanned the baby’s back he stopped and exclaimed, ‘‘There it is!’’ He turned to us and said, ‘‘Your baby has Spina Bifida.’’ I asked him what Spina

Bifida was and he explained that it is an opening of the baby’s spine. I then asked, ‘‘What does that mean? After our baby is born you sew up his spine and we go

home, right?’’ ‘‘Not exactly’’, he said. ‘‘Babies born with Spina Bifida may walk as

children with the aid of braces and crutches but normally end up in a wheelchair

as an adolescent. They have no control of their bowel or bladder. They need to have

a shunt placed into the brain to control the hydrocephalus. In addition, you should

expect some degree of mental retardation. Your options are to continue the pregnancy, continue the pregnancy and give the baby up for special needs adoption, or

terminate the pregnancy. Do you have any questions?’’ My questions were: am I

awake or is this just a nightmare and, if I am awake, who just hit me in the head

with that baseball bat?

Next, the technician handed us a box of tissues and escorted us into a room across

the hail. The genetics counselor came in with charts and graphs of ‘‘x’s’’ and ‘‘y’s.’’

Jill and I sat with tears in our eyes just wanting to go home to try to absorb what

was happening.

By the next day we began to focus on the decision before us, which we felt should

be based on quality of life issues. We knew we had many questions that needed to

be answered. What exactly is Spina Bifida?

How does it affect the child? What is the quality of life for children with Spina

Bifida? Unfortunately, the medical professionals’ responses painted a very bleak picture.

So, we decided to take matters into our own hands and educate ourselves. We

spent the next few days in the public library and the medical libraries at UCONN,

Yale and UMASS. We read sections of textbooks pertaining to neural tube defects

and research papers in medical journals regarding the physical issues and cognitive

development of children with Spina Bifida. We contacted physicians and other

health care professionals around the country who were involved with Spina Bifida

children. Finally, we contacted the Spina Bifida Association of America (SBAA) and

asked if we could talk to parents of children with Spina Bifida. They put us in contact with several families within our State chapter in Connecticut.

After speaking with a number of families we noticed a big discrepancy between

what the medical professionals and the parents thought about Spina Bifida’s quality

of life. One of the families we spoke with invited us over to continue our discussion

and meet their 9-year-old daughter, Jaclyn, who had Spina Bifida. We arrived at

their house and rang the bell. As the door opened, there was a beautiful, smiling

little girl with pink crutches and braces to her hips welcoming us into their home.

In that instant we knew what kind of quality of life Jaclyn had. We spent the

night talking to Jaclyn about her hobbies and her friends. She was excited to show

us her room and her American Doll collection—including the one in the wheelchair.

We spoke with her parents, Janet and Anthony, about her numerous surgeries and

their battle with their school. We left relieved that our decision was made. Now we

needed to get as much information as possible before our son was born. On our way

out, Janet handed us a copy of ’Insights,’’ a newsletter published by SBAA.

A few days later, while reading through ‘Insights into Spina Bifida,’ we came upon

an article that was of great interest to us. It concerned in utero surgery on unborn

fetuses to reduce the effects of Spina Bifida. The reasons for doing the surgery as

early as possible made sense and years of animal research yielded some positive reVerDate 11-MAY-2000 10:48 Mar 06, 2003 Jkt 000000 PO 00000 Frm 00036 Fmt 6633 Sfmt 6621 S:\DOCS\81046.TXT SLABOR3 PsN: SLABOR3
33

sults. The article indicated that there were two centers in the country performing

fetal surgery for Spina Bifida, Children’s Hospital of Philadelphia (CHOP) and Vanderbilt University Hospital. We contacted the surgeons at both centers and traveled

to both locations for evaluation and additional information pertaining to the surgery.

After very careful consideration of the risks versus the hoped-for rewards for both

our unborn son and for my wife, we elected to proceed with the fetal surgery at

CHOP. After the surgery we would remain in Philadelphia until the birth of our

son. The surgery was performed October 30, 1998 at 25 weeks gestation. Jill and

I spent the next week in the hospital and the next 3 months at the Ronald McDonald House in Philadelphia. On January 14, 1999 the joy of our life, Nicholas Ferdinand Liguori, was born. One chapter was closed and another about to begin.

Nicholas spent 8 days in neonatal intensive care. We then returned home to Connecticut. Almost immediately, the doctor and therapist visits began. We met with

‘Birth To 3’ therapists who set a schedule for follow-up. At 2 months we attended

our first multi-disciplinary Spina Bifida clinic. Actual therapy began around 6

months.

As you might expect, Nicholas generated a lot of interest as a fetal surgery patient. He was evaluated by a neurosurgeon, an orthopedic surgeon, a urologist, a

cardiologist and a host of other health care professionals. Of primary concern was

the function of his kidneys and bladder. The urodynamic testing revealed high bladder pressures, bladder spasms, incomplete emptying of the bladder and urine reflux

into the kidneys. We were scheduled for a head and spine MRI that showed a tethered spinal cord (common in children with Spina Bifida) and was therefore inconclusive. The neurosurgeon wanted to perform surgery to ‘de-tether’, or release, the spinal cord. We wanted another opinion. After additional tests and consultations in

Philadelphia and Boston, it was decided that surgery was not required but urologic

intervention was necessary.

Today, Nicholas’ bladder and bowels do not function properly. He requires two

medications daily to allow his bladder to fill at low pressure, to prevent bladder

spasms and to prevent urinary tract infections. We catheterize Nicholas five times

each day to completely empty his bladder and we are working on a bowel-training

program to hopefully achieve social continence. At three-and-a-half years old Nicholas has attended Spina Bifida clinics in Connecticut, Pennsylvania, Springfield

Massachusetts, and Boston, where he is currently treated. He receives physical therapy, occupational therapy, speech therapy and developmental therapy—each once

per week.

Despite all of this, believe it or not, Nicholas has what is considered a ‘‘mild’’ case

of Spina Bifida. He walks on his own without the aid of braces or crutches. Due

to the positive changes that occurred in his brain as a result of the fetal surgery,

Nicholas has not required a shunt. At three-and-a-half years old Nicholas had his

first surgery since birth this past July 5th. The little girl sharing his room also had

Spina Bifida, was the same age, and was recovering from her 23rd surgery. Can you

imagine the physical, emotional, and financial toll that must have on such children

and their families?

With Spina Bifida, the physical ability you have today may be gone tomorrow. It

is an ever-changing defect, which rarely, if ever, improves. The main problems that

plague individuals with Spina Bifida fall into four main categories: neurological, orthopedic, urologic and developmental. Most children with Spina Bifida have a tethered spinal cord, meaning the cord is attached usually at the level of the spinal defect. This mainly occurs as a result of scarring from the original closure surgery.

As the child grows their spinal cord is stretched. This often requires surgery to detether, or release, the spinal cord and occasionally surgery to decompress the

brainstem where it enters the top of the spinal column. These are extremely critical

procedures with a high degree of risk. Left uncorrected, they can result in loss of

motor function, paralysis, and in the case of brainstem compression, even death.

Chronic bladder problems and urinary tract infections can result in loss of kidney

function. Furthermore, the problems associated with incomplete bowel and bladder

control can have a huge effect on an individual’s self-esteem and overall social interaction. Finally, shunt malfunctions and infections can result in increased developmental delays.

As parents of a child with Spina Bifida, we are very grateful to Congress for its

assistance and leadership regarding helping to reduce and prevent Spina Bifida and

improving the quality of life for individuals with Spina Bifida. The Early Childhood

Intervention program (ECI), in particular, has made a big difference in the early

lives of children with Spina Bifida. However, there is a lot more that needs to be

done with respect to prevention and quality of life issues.

34

A Federal allocation of $3 million in fiscal year 2003 for a National Spina Bifida

Program would provide a crucial necessary next step to help reduce the occurrence

of Spina Bifida and to help those already affected. Such a national program would

help improve public awareness—which I believe should be considered a top priority.

Most people I have spoken with during ‘‘awareness events’’ have never heard of

Spina Bifida. Once the public knows what Spina Bifida is and understands how it

affects the individual, prevention will evolve automatically.

More also needs to be done to improve the quality of life for those individuals and

families—such as mine—living with Spina Bifida. The No. 1 concern from parents

and individuals is that few improvements have been made in the area of bowel and

bladder continence. This is a problem that has devastating emotional and social consequences for the individual. Families often have to change plans because a facility

or area is not equipped to handle the needs of an individual with Spina Bifida. Children with Spina Bifida often are excluded from events and activities because a facility or even a friend’s home cannot accommodate them and their needs.

A National Spina Bifida Program also will help with the establishment of multidisciplinary care facilities. Spina Bifida is a complex defect that cannot be handled

by ordinary healthcare professionals. With clinics established, the healthcare providers within that facility will become more experienced with the problems affecting

Spina Bifida patients and how they interrelate. Through the National Center for

Birth Defects and Developmental Disabilities at the Centers for Disease Control and

Prevention, health care providers can become better educated about the full range

of issues and challenges that face individuals and families affected by Spina Bifida.

This in turn with help improve the quality of care they receive and subsequently

improve quality of life.

In addition, we must have educated professionals within the school systems. Educators need to understand the types of learning disabilities associated with Spina

Bifida and develop appropriate alternative methods of learning. They also need to

teach independent living to those individuals who may not have a support network

at home. Our collective goal should be for the majority of individuals with Spina

Bifida to live long, healthy, independent, and productive lives.

Lastly, research into the cause of—and a cure for—all neural tube defects must

be increased. Our job will not be complete until there is no longer a need for a National Spina Bifida Program.

Again, on behalf of my son, my wife, and myself, thank you Mr. Chairman for

giving me the opportunity to tell our story to you today. It has meant a great deal

to us and we stand ready to be of any assistance to you and your colleagues as you

consider issues relating to this all too common birth defect.

Thank you.

Senator DODD. Let me turn to my colleague, Jack Reed, who has

some other obligations, so I am going to have him go first with his

questions, and I will follow up with some. I thank all three of you.

Senator REED. Thank you, Mr. Chairman.

I concur that it was extraordinary testimony by Mr. Pote, Dr.

Green, and Mr. Liguori. Thank you so much. And let me once again

thank the chairman for holding this hearing, but not just this hearing—I cannot think of any of my colleagues who has been more

committed to helping the welfare and the health of children

throughout their career than Chris Dodd, so thank you, Mr. Chairman, for that and for all that you do.

Let me raise just two questions. First, we talk about the cost of

birth defects to the child and to the society, but it strikes me that

any child with an illness of this severity has huge costs to other

children in the family, to the family itself, costs that are very difficult to objectify, to put into numbers. But Mr. Pote and Mr.

Liguori, if you could just comment on the impacts and the costs in

regard to other siblings that we have to capture, because this too

is a burden that we have to address.

Mr. POTE. I will just offer a couple of personal observations and

then generalize it a bit. There are two significant impacts that we

see in our families, and we are very lucky in our family—my older

nephew, Tim, who is a very strong young man and has been a very

35

important part of helping his family deal with the challenges of

spina bifida. But that is not always the case. Many siblings grow

up feeling like they are not getting the attention from especially

their mother, because the other important impact here is that the

nature of spina bifida—and you heard Fred list the number of care

providers that their family has to deal with—one of our chapter

folks in Nashville told me one day that there are 15 doctors that

she regularly visits with her son. It is a full-time job for one member of the family—it is almost always the mother, as it turns out— and that has tremendous demoralizing impacts. Women have given

up their careers. They often feel guilty that they are not spending

time with their other kids, and the siblings often feel like they are

not getting their fair share. Those are all very important psychological issues.

Senator REED. Indeed. Thank you so much for your testimony.

Mr. Liguori, again, thank you for your testimony, and thank you

also for flying those A–10s. That helps a great deal.

Mr. LIGUORI. Thank you, Senator.

Senator REEDS. Could we have your comments, because you see

it as a parent—and by the way, I have the same tractor at home

for myself; it is a perfect size for me.

[Laughter.]

Go ahead.

Mr. LIGUORI. That is his favorite.

As far as how it affects other children, it is a little early for us

to tell. We do have another child who is 8 months old—he is not

here today, thank God.

[Laughter.]

He is not quite as mellow as Nicholas. But we do see already

that Nicholas does require a lot of attention just from the therapies

that he receives, and of course, now that he has transitioned from

birth to 3 into our local school system in Granby, which is doing

a phenomenal job, he does receive therapy just about every day. So

that means loading everybody into the car, taking him to the

school, and it does take a lot of time away from the second child.

As far as the health care costs, right now at American Airlines,

we have a $1 million lifetime cap on our health insurance. That is

something that I am very concerned about with Nicholas, and we

do everything we can to keep his health care costs as low as possible, because that can be a factor.

One big thing with spina bifida is that it is an ever-changing

birth defect, and just because he is doing so well today does not

mean that that is the case tomorrow, next week, or next year. That

can change.

So those are our biggest concerns. We will see how it is when he

gets a little older, how it affects our family. Like I say, right now,

he does require a lot of extra care, and certainly the cost is a factor

for us.

Senator REED. Thank you so much.

The other point that I think both you, Mr. Liguori, and Mr. Pote

made is that the progress and the response from families that are

well-educated and affluent and have access to health care is challenging, but just think of families who do not have a health care

plan, and maybe do not have English as their first language. It is

36

a huge challenge, and that is our responsibility; that is where we

have to step up to the plate, particularly helping those families.

Dr. Green, it is encouraging to hear about Nicholas’ surgery, and

I wonder if you might talk about the impact of in utero surgery in

terms of limiting the number of surgeries going forward and potentially alleviating many of the symptoms of spina bifida. Could you

comment on that?

Dr. GREEN. Sure. Fetal surgery is not all that new, and in fact

it has been very well-established for quite successful treatment of

other disorders that I mentioned—congenital diaphragmatic hernia,

urinary tract obstructions. So that while there are issues which I

think you heard around risks of pre-term birth, and so on, I think

it is a well-established option for a number of different birth defects.

The spina bifida story is still early, as I mentioned. There are

procedures being done in three centers, and as Mr. Pote correctly

mentioned, the data seem most encouraging about one of the major

difficulties with spina bifida being the need for a shunt for draining

spinal fluid from the head, and it actually goes into the abdomen.

I can tell you from taking care of lots of kids with those shunts

that they are often a problem and result in innumerable surgeries

and problems as well as potential association with learning problems and mental retardation if shunts are not adequately taken

care of.

So the early data is that the fetal surgery may help avoid the

need for a shunt and some of the complications associated with it.

But again, it is early, and these programs are being evaluated, so

at this point, it is at the clinical study stage.

Actually, there was a report in this week’s New England Journal

about three kids who had had fetal surgery for spina bifida, and

they developed another condition associated with the surgery that

they may or may not have had had they not had this fetal surgery,

which in two of the three cases was treatable with another surgery,

in one of the cases not.

So I think there is a lot to be learned and that we need to be

cautious and study this very carefully, both in terms of short-term

as well as long-term outcomes. The long-term outcomes, it is too

early to say, so we do not know.

Senator REED. The other point and my last question—because

the chairman has been very kind to let me go first—is there are

three centers doing this type of surgery, which underscores the reality that to get access to the sophisticated health care and the

counseling, you have got to be fortunate enough to live in one of

these cities. Probably if you are in a rural area, you would be unlikely to logistically be able to access these services. You have got

to be fortunate to live in a school system that is really—and Granby, I think, is—sensitive and is working with the families.

So we have a huge challenge to whatever we develop in terms

of medical responses and educational responses to make sure that

they are diffused fairly across the country. Otherwise, rural Americans and poor Americans—even if you are doing everything you

can, you do not have the resources available.

Does that make sense? I see affirmation.

Mr. Chairman, thank you.

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Senator DODD. Thank you, Jack, very much for your presence

here today and your commitment to this issue as well. It has been

tremendously helpful to have you with us here this morning.

I probably should have noted at the outset that one of the reasons that I have been involved in this issue is because I have been

pleased every year for I do not know how many years now—I am

trying to recall the number—I have been the honorary co-chairman

with Bob Dole, my wonderful friend and colleague, former Senator,

of the spina bifida dinner we have each year to raise dollars. I have

enjoyed those roasts that we have here in Washington, where I

have mostly been a ‘‘roaster’’—and by the way, that is not an invitation for me to become the ‘‘roastee,’’ having seen what they do to

people.

Mr. POTE. It has been duly noted.

[Laughter.]

Senator DODD. I am glad to be the honorary co-chair with Bob

Dole and those involved. Judy Woodruff and Al Hunt are great

friends, and they have done a wonderful job bringing us all together for that particular event.

Let me run down some questions for you—and this is an informal enough setting here so I want you to jump in and comment

on these matters if you feel so inclined.

Let me begin with you, Mr. Pote. You mentioned in your statement that prevention, intervention and treatment therapies must

be identified, and as you said in your statement, in order to identify these, it would require, in your view, either new medical breakthroughs, more research, or just funding.

Where should the emphasis be from our standpoint?

Mr. POTE. I will just mention two, and I will reinforce something

that Dr. Cordero mentioned. We will be cofunding and participating with CDC, NIH, and AHRQ in the coming year in a consensus

conference which is, as you know—and you probably know this better than I—designed to bring together health care professionals,

scientists and researchers, public health professionals, and members of our community to figure out what is it that we know about

best practices, and what do we not know, and then to establish the

agenda which hopefully will help us, with Dr. Cordero, figure out

what the priorities are, but then will also lead to the promulgation

of best practices with the participation of the academies and the

Agency for Health Care Research and Quality.

So from the point of view of our families, I would say that our

number one priority now is that the quality of care delivered to our

families around the country is very uneven, and we need to establish those standards.

The second issue—and this is also why it is very complicated— is that there is no such thing as a spina bifida secondary condition.

It is a urological or a neurological or a dermatological, and that

makes it difficult for us to specifically pinpoint what we need to

pursue next. That is why, as Dr. Green said, with the help of people like the March of Dimes and these other professional academics, we can focus on the two or three most important secondary conditions in each of those areas of treatment and make sure we have

identified the research priorities to bring the level of care up in

each of those specific areas.

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Senator DODD. That is great.

Tell me—and this seems sort of self-evident, but I would like you

to explain why you think this is important for an audience that

may not be familiar with this and wants to know more—I find the

idea of locating the National Spina Bifida Program in the National

Center to be a wise one. But tell me what you think that is going

to mean for spina bifida families. What does it really do for those

living with spina bifida? What sort of difference does that make?

Mr. POTE. I think that it helps raise awareness, first of all. The

fact that we are sitting here in 2002 talking about this issue says

something about the level of awareness, and we in the spina bifida

community accept our share of that responsibility, because we need

to make sure this is a higher priority item.

I think the most important outcome of that center will be that

we will be able to once and for all establish a clear national agenda

and will be able to focus the efforts of the legislative world, the

medical community, and the research community and make sure

we are dealing with the most important issues in a very coordinated and focused way.

Senator DODD. That is a good enough reason.

Dr. Green, do you have any comments on that?

Dr. GREEN. I will give you three statistics. Up to 70 percent of

neural tube defects can be prevented with folic acid. As Dr. Cordero

mentioned, there is a 31 percent decrease. So there is a gap between what could be prevented and what has been prevented. And

the third statistic is that only one-third of women of childbearing

age take folic acid during the preconception period.

So we want to get to 100 percent prevention, so we need more

research on what the other causes are that cannot be prevented by

folic acid; we need to reach out to the public through health care

providers directly to the public to get as much prevention as possible.

And I think Dr. Cordero’s center at CDC is well-poised to do that

and also to do the surveillance.

Senator DODD. Mr. Pote, in your written testimony, you mention

that ‘‘With appropriate, affordable, high-quality medical, physical,

and emotional care, most people born with spina bifida can lead

relatively normal lives.’’ And your testimony, Mr. Liguori, about the difference between

what the medical people told you and what you encountered with

the family in Connecticut that you met was certainly instructive.

It is also clear from your statement that ensuring access to this

care is obviously necessary if we are to improve the quality of life

for people.

But my question is this. How do you know how affordable it

must be to be truly accessible? This is a complicated question, but

I am trying to get some sense of this—and are there other obstacles

such as lack of trained specialists—you have mentioned that to

some degree already—which make this care inaccessible to those

with spina bifida.

I would like all three of you to comment on that if you can.

Mr. POTE. I guess the point that I would emphasize is that once

quality of care is accessible because the health community has been

trained to the same level of awareness and skill, I think the next

39

issue, then, is making it clear to folks who have a child with spina

bifida that there are places where they can go to get that kind of

specialized attention.

The third issue is how do they pay for it. And as you mentioned

earlier, Fred is fortunate enough to have a good, solid private insurance policy. The good news on those policies is that for the most

part, they do fund what they should be funding, and as you know,

that is not always the case. That is not to say that the families do

not have to fight to get there, but most of those policies do pay

where they should. The issue is the number of people who are not

covered by those kinds of policies and are therefore totally reliant

on the public safety net, and as you know here also, that does not

always result in the highest-quality care.

Senator DODD. You mentioned earlier, Mr. Liguori, that the

Granby school system has been pretty good. We have been trying

up here for the last number of years—I started about 10 years

ago—to have the Federal Government live up to its commitment on

special education costs. We have not gotten there yet, and when I

go back home, and I meet with my first selectmen and mayors in

the 169 cities and towns in Connecticut, one issue that is right at

the top of the list all the time for them is special education dollars.

And we are getting better at this—we are at about 12, 13 percent.

We tried last year in the Elementary and Secondary Education Act

to get those dollars up, and the Senate was overwhelmingly in support of it, but the House was not, and we did not have a lot of support out of the administration on this. I am hopeful that this year,

that may change.

There are a lot of problems obviously with the fiscal issues associated with that. But I have also been concerned about how in communities, because of the costs associated with special education, in

smaller towns, the pressures that can be exerted because people

start to complain about the cost of taking care of a handful of children.

Tell me about the response in the community of Granby. Obviously, it has been a good one, but I would like people to hear about

how that happened. How do you educate people about the importance of Nicholas getting the kind of education that the Granby

school system is giving him?

Mr. LIGUORI. I have to go back to what Mr. Pote said. I think

it comes back to public awareness. Public awareness is the most

important thing in my mind, because I think that once the public

is aware of what spina bifida is and how it affects children, you

will see a change in prevention, you will see a change in fundraising—all that stuff will follow on, and it will also help to educate

people on what the defect is, the developmental disabilities of these

children, and the needs that they have in the school system.

We were with the birth to 3 program initially in Connecticut

when we got back, and we were quite happy with the care that

Nicholas was getting at that time—we did not know anything better, though—but he was continuing to fall behind developmentally.

Once he turned 3, and he stepped into the Granby school system,

they came up with a program for him that was significantly more

than what birth to 3 was giving him, and he has just started that—

40

he is only 31⁄2 years old—but we are very happy with the system

that he has, and we hope to see him improve quite a bit.

But to answer your question, I think it just comes back to raising

public awareness on the defect and how it affects children.

Senator DODD. I probably should have said this ahead of time,

for those people who are not from Connecticut or from the area—

Granby is a small town. It is not a large metropolis in the State

of Connecticut, like Hartford or Bridgeport. Granby is a small community. So education is important, and local support is based on

people being aware of the kind of assistance and support that Nicholas would need. And that made a difference in terms of the system

being willing to step up to the plate and see that he gets that kind

of help.

Mr. LIGUORI. Yes. And Senator, if I could make one more comment as well, as far as the health care professionals, like I said,

a lot of the information that we got from them conflicted with the

information that we got from the parents. And again, I think that

goes to education, because a lot of the information that we did get

from the professionals we were dealing with was—I do not want to

say it was incorrect—it was old information. And with early intervention and multidisciplinary care, the children today do much better than they did 20 years ago.

Senator DODD. Dr. Green, why don’t you pick up on that point?

I would love to see us do something in this reauthorization that did

a lot more in terms of providing that kind of support for health

care professionals to have a far better understanding of these

issues. What can we do that will achieve that? It seems to me that

that is a major question here, and we need to put more emphasis

on it.

Dr. GREEN. I think Dr. Cordero mentioned the program with the

American Academy of Pediatrics and his Center, and that is a

major mechanism for outreach to professionals, for pediatricians. It

would also seem that it is important to reach out to the obstetrics

side as well, perhaps to the American College of Ob/Gyn, since

many of these decisions are made prior to the delivery of the child.

In fact, the March of Dimes has been working with ACOG, the

College of Ob/Gyn, on educational efforts in this and other aspects

of health care for infants and children. That is not it—obviously,

outreach needs to be made to the nursing staff, and as you have

heard, there is a whole spectrum of health care needs and health

care providers involved in care for children with serious birth defects like spina bifida. So educational outreach really needs to be

directed at this spectrum so that you get coordinated care.

Senator DODD. Let me ask three other quick questions if I can.

One, can you go into a little more detail about what is being done

on the research side—we have talked about it—but what areas of

research are showing some real promise in the prevention or treatment in the area of birth defects?

Dr. GREEN. The question came up earlier about genomics, and

certainly identifying genes so there can be screening and prediction

of course—maybe some children are affected with specific birth defects because of some genes in their mothers or themselves. The genetic diversity may help to explain some of the spectrum of disVerDate 11-MAY-2000 10:48 Mar 06, 2003 Jkt 000000 PO 00000 Frm 00044 Fmt 6633 Sfmt 6601 S:\DOCS\81046.TXT SLABOR3 PsN: SLABOR3
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order, so that is important to know in terms of making decisions,

planning treatments and interventions.

So that, for instance, the 30 percent or so of women who are resistant to folic acid, so are at risk of having affected pregnancies,

despite folic acid may need ten times the dose of folic acid or more,

or other kinds of medical interventions for prevention.

So I think that gene identification is critical for prevention as

well as for prediction of clinical course and therefore allows families to plan. So that is one aspect.

And certainly fetal surgery, as we have heard, has been important in a careful approach to assessing impact on these birth defects.

And then, the other issue is we should remember that research

involves surveillance and keeping track, so for instance, that is how

we know that Hispanics are at greater risk of having children affected by neural tube defects, and that has then led to outreach to

providers and outreach to communities. So I think that surveillance is a very important aspect of this that the CDC helps to provide.

Senator DODD. The March of Dimes and the public educational

messages that they have developed over the years for newly pregnant parents—have they been targeted to them, or is it sort of a

general message?

Dr. GREEN. It is an interesting question, because if you think

about folic acid needing to be taken by women of childbearing age,

once you know that you are pregnant, if you start taking folic acid,

it may be too late. Then, you have to really reach women from the

ages of 16 to 45. That is very challenging.

If you think about other disorders like fetal alcohol syndrome,

which was brought up today, similarly, you have to reach women

early, although a lot of the public outreach on fetal alcohol and also

smoking that is associated with pre-term delivery and low birthweight of children, you can target more toward pregnant women.

It is obviously easier to reach more well-defined populations, and

pregnant women are known to be more receptive to educational

messages. They want healthy babies as well. So it depends on the

disorder and the cause.

Some disorders associated with, say, medications—the thalidomide story is old, but one of the more active stories is with

accutane, an acne medication. Again, you have to deal with the

young adult female population. So one size does not fit all for this.

Mr. POTE. Senator, I would just add in terms of some of the outreach that the March of Dimes is doing, that next time you are in

an airport, look for Daisy Fuentes talking about folic acid. And I

am sure that, like me, you are an avid watcher of MTV, and you

are very familiar with Daisy Fuentes, but she is exactly the kind

of spokesperson that we need—a young, attractive Hispanic woman

who is ‘‘cool,’’ and it is ‘‘cool’’ to take folic acid.

One of the things that we have said about food fortification is

that if we could ever find a way to fortify soft drinks with folic acid,

or if we could find a way to convince young women that—

Senator DODD. I was curious about that, whether we could incorporate it in food sources, working with the Department of AgriVerDate 11-MAY-2000 10:48 Mar 06, 2003 Jkt 000000 PO 00000 Frm 00045 Fmt 6633 Sfmt 6601 S:\DOCS\81046.TXT SLABOR3 PsN: SLABOR3
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culture and others, so that this could become as much of a conscious choice as just normal eating habits and patterns.

Mr. POTE. You find it today in breads and cereals, and if you look

on the back of most cereal boxes, I believe that what you will see

is 170 micrograms in many cases—

Senator DODD. Not the 400.

Mr. POTE [continuing]. Not the 400—and then, of course, the

question is when you look at the population that we are targeting,

are they really eating the recommended daily dosage of breads and

other grains than cereals.

Senator DODD. One thing you might think about doing—I just

spoke over the weekend at the national convention of La Raza in

Miami, and 2 weeks before that, I spoke at the national convention

of LULAC. I was in the Peace Corps, so I have a strong interest

in the culture and history. And I would think that through

Telemundo and these other groups, and the ‘‘novellas’’ which are

tremendously popular, obviously, in any language—these are the

soap operas and so forth—among younger women, these are tremendously popular—I would bet that if you talk to these folks,

with Dr. Cordero, about how you might incorporate it as part of the

commercial programming, as part of the stories, it would have a

tremendous impact.

With all due respect—and I am very grateful to CSPAN for covering a hearing like this—but the audience that we are going to

reach probably is not going to be the one that we would like to be

reaching. Maybe we should do this with simultaneous translation

for people who speak other languages. But nonetheless, there is a

greater likelihood, I think, through the commercial popular programming that we are able to get a better education through, and

I bet if we had some contacts with them, they would be more than

willing, particularly with the information you have shared with us

here today about the particularly high incidence in the HispanicLatino community. I would bet you would have a tremendous response to this, and I will be happy to join you in getting in touch

with them, if you would like to do that.

Mr. POTE. Thank you.

Senator DODD. I apologize—this could go on and on. I have been

so impressed with all of you. I am tremendously impressed with

you, Mr. Pote, for the work that you have done, a tremendous effort.

And Dr. Green, we are excited to have you with the March of

Dimes, with your background as a pediatrician.

It has been great to have Nicholas and Greg here with us. I cannot tell you how much I appreciate having these young people with

us today. It makes a big difference.

And Mr. Liguori, you and Jill are terrific parents.

Mr. LIGUORI. Thank you, Senator.

Senator DODD. Okay. We will leave the record open for some additional questions.

I thank all of you for coming, and the committee stands adjourned.

[Additional material follows.]

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ADDITIONAL MATERIAL

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[Whereupon, at 11:34 a.m., the subcommittee was adjourned.]

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