A life worth living: myelomeningocele.

Do handicapped persons’ lives have value?

Even asking that question is offensive, at least to me, and I just asked it!

It seems to me that much of the focus of programs of antenatal diagnosis is based on the premise that being ‘handicapped’ is worse than being dead. So we search for anomalies and terminate many pregnancies based on the information that we then give to the parents; information which is strongly influenced by our own prejudices. I am convinced, and there is some supportive evidence, that the prejudices of physicians influence the choices that parents make. We know that most women terminate pregnancy after a diagnosis of trisomy 13 or 18, for example; but when couples are told that the disorder is lethal, and that the rare short term survivor is a ‘vegetable’, is it surprising that so many abort?

Our prejudices as health care providers are not shared by the rest of the population, in particular they are not shared by impaired members of the population. Quality of life studies in children with various impairments have uniformly shown much higher quality of life scores when children are asked themselves about their own life, compared to what health care providers estimate.

Let’s take the example of meningomyelocele. A very thoughtful review article just published in Pediatrics describes how attitudes have changed and continue to change (and need to change further) Pruitt LJ: Living With Spina Bifida: A Historical Perspective. Pediatrics 2012. I am sure that around the world different programs are at different stages of that process. In certain hospitals parents are erroneously told that ‘the quality of life is miserable if they survive’ (a direct quote from a medical dossier) ; whereas in others active treatment is never even considered to be optional except for the rarest, most severe cases. Unfortunately we often see pregnant women referred to our hospital who have already received extremely prejudicial misinformation. Who have been mis-informed about the intellectual disability of children with meningomyelocele, and who have been mis-informed of the discomfort, pain and repeated surgeries that the children need if they are actively treated. Not surprisingly many choose to terminate the pregnancy.

Lets put this straight.

Intellectual ability: infants in the control group of the MoMs study had a mean Bayley 2 MDI of about 87. This is a group of infants who had a fetal diagnosis of meningomyelocele with an upper level between T1 and S1 (almost all were lumbar) who also had hind-brain herniation, they were therefore among the group most severely affected. It is already well known that children with meningomyelocele often have multiple disorders of cerebral development. As well as the abnormality of closure of the neural tube, which is the primary problem, they have ‘abnormalities’ in other parts of brain development. If you do an MRI you will find other disorders of migration, and of cerebral structure. However. Even though the brains are structurally demonstrably different to infants who do not have a neural tube defect, they actually function quite well. So even if you select a group of children with an anomaly which is severe enough to be considered for antenatal surgery, the overall intellectual outcome is quite acceptable, and the large majority of the infants are close to the average of the general population. There are specific difficulties that the children face, but profound intellectual impairment is very rare.

Quality of Life: the health related quality of life of children (and adults) with meningomyelocele is reduced, but not disastrously lower, compared to those without the disorder. Subscales which are most affected by physical limitations are the most severely affected, which is hardly surprising. Other scales much less so. Interestingly there is not much difference between the QoL scores of children who are ambulant and those who are not. A recent Scandinavian study (Bartonek Å, Saraste H, Danielsson A: Health-related quality of life and ambulation in children with myelomeningocele in a swedish population. Acta Paediatrica 2012:no-no) asked parents to complete a QoL questionnaire for their 6 to 17 year old children. This study showed health related QoL scores which were lower than the scores from studies which asked the children themselves; such as the one mentioned below.  Other data note that quality of life of adolescents with spina bifida is much more related to their social interactions, such as by how many good friends they have, than it is by their impairments. (Muller-Godeffroy E, Michael T, Poster M, Seidel U, Schwarke D, Thyen U: Self-reported health-related quality of life in children and adolescents with myelomeningocele. Dev Med Child Neurol 2008, 50(6):456-461) which is probably the same as adolescents everywhere! The scores obtained from this study, which questioned the adolescents themselves, were somewhat lower than a healthy reference group, but not very much lower, and some scores were not statistically significantly different from the reference group.  

Pain: Both pre-op and post-op, children with spina bifida have very little pain. ( Ottenhoff MJ, Dammers R, Kompanje EJO, Tibboel D, de Jong THR: Discomfort and Pain in Newborns With Myelomeningocele: A Prospective Evaluation. Pediatrics 2012.) As the disorder interrupts some or all neural transmission below the level of the lesion, there is little discomfort associated with the actual abnormality.

When this diagnosis is made parents deserve accurate information. They deserve support and help. They deserve to hear the positives and not just the negatives. Annie Janvier hasn’t yet got round to doing her internet survey with parents of children with myelomeningocele (I don’t know if she will, she has about 20 projects on the go at present), but I would bet the responses after early antenatal diagnosis would not be much different to what she found with trisomy 13 and 18. I would wager that most parents who have an antenatal diagnosis before 20 weeks are repeatedly offered termination, and that those who decide against termination feel that they are judged by the medical community.

In contrast, I think that in most centers, once the child is born, active treatment options are usually offered with a positive attitude, and that multidisciplinary support is readily available in most developed countries. In this situation, at least, I think attitudes have changed somewhat. Sixty years ago, babies were often left to die, and parents were told that the infants would never have a life of quality. As the story told by that link proves, such predictions were often wrong. Fortunately, many parents chose not to believe them.

(In response to a comment from a reader, which you can see below, I edited this post on the 20th of September to replace some uses of the term ‘handicap’ with the term ‘impairment’- I however left the term in the introductory part as many physicians use that term, and I wanted the first paragraph or so to catch their attention.)

About keithbarrington

I am a neonatologist and clinical researcher at Sainte Justine University Health Center in Montréal
This entry was posted in Advocating for impaired children and tagged , . Bookmark the permalink.

7 Responses to A life worth living: myelomeningocele.

  1. williampeace says:

    Yes, the question do handicapped people’s lives have value is offensive. Yet I have often been asked this question–the assumption being as a man with a disability I have a unique insight. The problem with posing such a question is that I am put in a inherently inferior position where I must defend my existence. Not the best way to start a supposedly good natured discussion. Thus I see this question as pure folly. If you doubt me read Harriet McBryde Johnson’s memoir in which she recounts her debate with Peter Singer.
    Anecdotally I would make the following observation: when I was in college, 1978-1982, the most common students with a disability were not people such as myself that had a SCI. Rather it was paralyzed men and women who had spina bifida. Fast forward to the present–I have not met a person or taught a university student with spina bifida in well over a decade. I hate to use the word Eugenics as it ends all reasonable discussion but something is profoundly amiss.

  2. Alison Davis says:

    I would like to thank you for pointing out the equal humanity of newborns with myelomeningocele, and the fact that negative prognoses encourage eugenic abortion, whereas positive information does the opposite. I am 57 years old, and have spina bifida, hydrocephalus, osteoarthritis, osteoporosis and breathing problems. I also have kyphoscoliosis/lordosis the latter of which happened after two relatively recent spinal fractures. In view of all this, iti probably won’t surprise you to know that I experience severe pain which cannot be controlled. But … still life is worth living, and I want to live mine to the full until I die naturally. I agree entirely with Willilam Peace’s comment that we see very few people with SB around these days. The reason is obvious given that the abortion rate for SB is rising 85%. BTW I would advise you to use the term “disabled” rather than “handicapped.” Some disabled people get very upset when the former term is used.

    • Thank you for the comment. I used the term, which I usually try to avoid, because that is how many docs refer to people with disabilities, and my web site has been written primarily for physicians involved in neonatal care. (also in French, and I work in a French speaking hospital, the other terms are never used, and not even easy to translate, so ‘handicap’ and ‘handicapé’ are the terms that almost everyone uses). I will probably edit the posting to reflect this.

      Although the abortion rate is indeed very high, the use of prenatal folate has also had a lot to do with the reduction in spina bifida, and probably other problems as well, some congenital heart disease for example.

      • wjpeace says:

        I am glad you replied to Alison Davis’ comment. I had assumed you used the word handicap for the reasons mentioned. As an American I am struck by the fact many Canadians use the word handicap–do you think this is in part due to being a bilingual nation? I have also found the vast majority of Canadian physicians use the word handicap as well. No doubt there is a different cultural response to disability in our two nations.

  3. Ian Bromelow says:

    Thank you for writing this. Like the person in the article you refer to, when I was born 54 years ago with a high myelomeningocele (between my shoulder blades) my parents were told that I wouldn’t survive, and that if I did I would be a “vegetable”, unable to walk or talk. Fortunately, they operated on me at three days old, and thanks to the new antibiotics I survived the meningitis, septicaemia and pneumonia I contracted subsequently. As you can see, I can talk (in several languages) and can walk, but not as far as I used to be able to (I can’t do 20-mile hikes any more!). After obtaining a university degree and postgrad qualification I worked in government service for 30 years. I am the father of two wonderful daughters, and recently became a grandfather to the most beautiful little girl in the world (well, I think so, anyway).

    It horrifies me how many babies never get the chance to enjoy the life I’ve had based on an over-pessimistic view of the outcome of spina bifida.

  4. Carolyn Bostic says:

    As a woman with spina bifida (full-time wheelchair user) who is close to 50 yrs old, I applaud this article. My miseries have rarely ever been about the spina bifida or its consequences but normal everyday angst. My life has been darn good. I’ve bitched about working a 40 hour work week just like any American citizen but have had the insight to be extremely grateful for the benefits of group health insurance, pension plan, and a steady decent paycheck.

    I joke that once the needles go bye-bye, I’m fine (I’m petrified of sharp objects such as needles due to so many tests, but what intelligent woman wouldn’t be, in my shoes?)

  5. Glynda says:

    I find this a great article and I am shocked at the percentage of people who abort just because their unborn child has any kind of defect. My son was born with spina bifida occulta (witch just means there was no hole to the outside of his body.) He also had a a tethered spinal cord. He has hydrocephalus, had decompression surgery for a Chiari Malformation and has so far had 12 surgeries and many MRIs and other appointments. We were formally diagnosed when he was 7 months old when I noticed the hemangioma on his lower spine seemed to be growing. It has been a bumpy ride but he is now 3 1/2 and loves to do everything a normal 3 year old would do. To look at him you would never know he had been born with any birth defects, untill you see the 4 scars on his head, the long scar on his lower back and the 2 small scars on his front torso. He is in a teakwondo class 2 hours twice a week and loving it. He is happy almost all the time, even when we spent all those days in the hospital witch is now up to aprox 60-70 days. He was always smiling, hardly ever cried and even during his last surgery in 2011 made the nurses and doctors amazed with his attitude. I look forward to seeing how he grows and could not see my life with out him. Even after all the bad luck we had in the beginning. I did have problems with my pregnancy but there was never any indication of issues before the day we had our fist MRI and found the SBO and TSC at 7 months old. Thank you for this great article, and as a parent of a very happy boy living life to the fullest, even with all our issues, I hope more people will see this and the doctors will start changing the way they present the information and options. I could not see my life with out him. his story here.

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