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.