From: cash@convex.com (Peter Cash)
Subject: Re: spina bifida information needed, please!
Date: Thu, 20 Aug 1992 04:30:05 GMT
Organization: The Instrumentality
X-Disclaimer: This message was written by a user at CONVEX Computer
              Corp. The opinions expressed are those of the user and
              not necessarily those of CONVEX.

In article <16108@pitt.UUCP> geb@dsl.pitt.edu (gordon e. banks) writes:
>In article <44622@ucbvax.BERKELEY.EDU> tlin@eeyore.Berkeley.EDU (Tony Lin) writes:
...
>>Her infant niece has spina bifida.  A number of complications have
>>developed and, even worse, more are developing.  We would like to
...
>Spina bifida is a defect of neural tube closure on the caudal
>end, usually (near the rump of the baby).  It is usually associated
>with other nervous system defects in the brain, such as Chiari
>malformation and hydrocephalus.  Most of these infants would die
>as neonates (and still do in most countries) but in this country
>we do them the dubious service of "saving" them so they can spend
>their entire lives in institutions.  

This last is absolutely false. You seem to have no understanding whatever
of what spina bifida is, or of its prognosis.

>Certainly there are mild cases
>where, although the child will be paraplegic, there is still some
>functioning brain, but in many cases, the brain problems result
>in profound retardation and the child is really not able to have
>any sort of a life at all.  

Spina bifida is a so called "midline defect" that results when the two
halves of the human embryo do not grow together properly during early fetal
development. Other such defects are cleft lip and palate. More
specifically, spina bifida is a neural tube defect that results when the
bony spinal column does not close completely during fetal devdelopment. [1]

There are three forms of spina bifida: _spina bifida occulta_,
_meningocele_, and _myelomeningocele_. [1] Only the last form involves the
nervous system, and may result in disabilities--or, if supportive treatment
is not given, death. The seriousness of myelomeningocele varies according
to what portion of the spinal cord is damaged, and the degree of damage to
the cord.

Spina bifida alone does not affect the cerebral cortex, and does not cause
mental retardation. Mental retardation is, however, one of the possible
indirect side effects of spina bifida, because this defect often causes
hydrocephalus ("water on the brain"). Hydrocephalus can be treated (usually
with a shunt to drain the excess fluid), and the damage may be minimized or
prevented entirely. Meningitis is another frequent corollary of spina
bifida. 

Assuming the patient survives, the most frequent medium to long term
disabilities that result from spina bifida are paralysis of the legs and
lack of bowel and bladder control.  Depending on the position of the defect
on the spinal column, paralysis may be more extensive.

I do not wish to trivialize myelomeningocele: it is always serious; it can
be horrible. Often, there will be a series of complications, and
concomittant traumatic medical procedures. However, the prognosis is
nowhere as unilaterally bleak as Dr. Banks suggests.

To give you a more encouraging picture, let me quote from a fullow-up
study by Curtis, Butler, and Emerson of 100 children with myelomeningocele
who were over 12 years old [2] :

In their study, 80 percent of patients were able to walk (28% of these used
crutches or braces to perambulate), and 20% used a wheelchair. Bowel
control was good in 65%, but only 35% had good urinary control.
Hydrocephalus was arrested in 38%, and about half the patients had
ventriculatrial shunts. An IQ of 80 or above was found in 89%. (An IQ of 85
to 115 is regarded as being in the "normal" range.)

Seventy-nine percent of the spina bifida patients in this study had
graduated from high school, and 27% were college graduates or were in a
four year college. At the time of the study, of those who were considered
"adults", 58% were employed. Fourteen percent of the males and 41% of the
females were married. "Normal" sex activity was claimed in 43% of the men
and 24% of the women. 

This study gives us some idea of what can be expected if the child with
spina bifida survives past the age of 12; Dr. Banks is therefore not
correct when he states that saving the lives of these children results only
in a life of misery and institutionalization. If the effort to save the
life of the child is successful, it is often worthwhile. 

In all fairness, I must quote the following passage also [2]:

	 Myelomeningocele presents an almost overwhelming series of
	disabilities.  Because of the need for repeat neurosurgical,
	urological, and orthopedic consultations and procedures, allowances
	for fairly frequent absences from school must be made.

	 Until bladdeer and bowel problems are under control (and this
	takes time), the teacher will have to put up with some odors not
	usually present in other children.

	 Many of the children will have normal or slightly below normal
	intellectual function, so that, despite absences from school,
	learning is feasible and can possibly be compressed.
	Elementary-school-age children should, for the most part, be
	prepared for sedentary vocations, trade schools, high school, and
	college.

>When I was in Britain, (where spinal
>bifida is more common than in the US, incidentally) they had a
>room of the nursery that they wheeled such cases into where they
>were given benign neglect.  It sounds cruel, 

It _is_ cruel. 

>but a visit to
>one of the human warehouses where these kids end up in the US may make
>you think it isn't so cruel after all.  

Really my good doctor, I had no idea that the University of Pittsburgh was
_that_ bad.

>In this country, we
>have toll free numbers so if the parents and the doctors don't
>opt to "have everything done" any busybody can call in and have
>the pro-lifers decend on the hospital.

Given your evident ignorance of this subject, I can only laud the
"busybodies". 

>In short, if I had such an infant, I would have the pediatric
>neurologist make an assessment of the prognosis for functioning
>cerebral cortex.  If it was dismal, I would
>steadfastly refuse to permit any life-prolonging operations
>or other than comfort measures.  

Do you perhaps have spina bifida confused with anencephaly?

Dr. Banks' gross ignorance is yet more proof that the average medical
professional in this country receives very little training in the
evaluation of mental and physical disabilities and defects. If you want to
know something about spina bifida, Down's syndrome, or cerebral palsy,
you'd best not ask a doctor.

As far as spina bifida is concerned, your best bet is probably the Spina
Bifida Association. Most large cities have a local chapter.

---------------------------------------------------------------------
REFERENCES:

[1] _Exceptional Children_, 4th Edition, Daniel P. Hallahan and James M.
    Kauffman. Prentice Hall, New Jersey, 1988.  P. 368

[2] _Physically Handicapped Children_, Eugene E. Bleck, MD and Donald A.
Nagel, MD, editors. Grune & Stratton, Inc, New York 1982. P. 358


-- 
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
             |      Die Welt ist alles, was Zerfall ist.     |
Peter Cash   |       (apologies to Ludwig Wittgenstein)      |cash@convex.com
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~