The ever-widening chasm between doctors who respect life and those who believe they have the right to decide who should live and who should die is perhaps nowhere more clearly shown than in their attitudes towards the child with spina bifida.
Thirty years ago, a baby born with spina bifida had a miniscule chance of living beyond a few months. Today, thanks to the heroic efforts of surgeons like Dr. Everett Koop, babies treated – preferably at birth – can become ambulatory, intellectually able, and competent to succeed in a competitive world.
Whether they have the chance to become so depends in a large measure on the medical philosophy of the attendant physician. Is his philosophy “How should we treat?” or is it “Should we treat?”, in other words “Should we be bothered to treat?”
Spina bifida is a relatively common congenital condition, in North America it occurs twice in every 1000 births. Although the causes of spina bifida are not known, certain facts are now clear. We know that in the third week of gestation a primitive neural plate begins to close to form a neural tube – the forerunner of the spinal cord.
Dr. Jerome LeJeune comments that “the closing continues in both directions, exactly like a zip system going both up and down.” If the tube does not close at the lower end, or if in some cases it closes and then ruptures open, spina bifida, or meningomyelocede, results.
Why the neural tube fails to close is still not clear, but recent studies are providing some possible answers. Medical statistics show a clear geographical pattern in the incidence of spina bifida. It is known that there are very few cases in southern Italy, and that there is a progressive increase as one goes north to northern France. A similar distribution has been noted in Britain: the south of England has few cases, and again the incidence increases as one goes further north. Thus, it seems likely that in some way climate is a factor. In addition to this geographic pattern, statistics show a socio-economic distribution, with more cases in the lower income groups, and fewer in the wealthy.
One of the first hints that one day spina bifida might be prevented came in 1968, in England, with the publication of a paper in the British Medical Journal. The article by Hibbard and Hibbard entitled “Folate Metabolism and Reproduction” revealed that mothers of babies with a neural-tube defect had a low level of folic acid in their blood.
The Hibbards’ research led R. W. Smithells, of Leeds, England, to investigate further. Statistics show that when a mother has one child with a neural tube defect, the risk that the next child will be affected rises dramatically. In the group that he was studying the risk of the first child being affected is 0.2 per cent, but if that child has a defect the average risk for the next baby rises to five per cent – that is 25 times greater.
Smithells decided to test the folic-acid discovery of the Hibbards, and to treat mothers, who had already had one baby with spina bifida, by giving them a special diet containing folic acid and other vitamins, beginning before conception. He established two groups: the first group of 178 mothers were on a special diet; the second, or control group, of 260 women were not treated. Of the latter group of 260 pregnancies, 13 babies had neural defects, exactly the five per cent expected. Only one baby of the 178 mothers who had revised the folic –acid diet was affected, or 0.56 per cent.
Smithells’ study throws light on the geographic and socio-economic distribution of spina bifida. Folic acid is found on the leaf of fresh vegetables, and the further north one goes, the shorter the growing season, the fewer the vegetables, especially in winter, and the greater the price. Poorer mothers cannot afford many fresh vegetables. It is of interest to note that in 1981 a further study of a team (Lawrence et al.) entitled “Double-blind Randomised Controlled Trial of Folate Treatment before Conception to Prevent Recurrence of Neural-Tube Defects,” published in the British Medical Journal, confirmed in part Smithells’ findings.
There is now a distinct hope that spina bifida can be prevented. Research at Guys Hospital in London is underway and a Research Trust is helping to underwrite the costs.
Medical practitioners with a different philosophy are also determined to stamp out spina bifida, but their efforts are directed to identifying the pre-born child who is affected, and then killing it. They are aided in their “search and destroy” campaign by the discovery that when the neural tube does not close, alpha-fetoprotein leaks into the amniotic sac, and then into the blood of the mother. It is now possible to measure the alpha-protein level of the mother’s blood and if a defective baby seems indicated, then the amniotic fluid can be tested.
If the baby is found to be less than perfect the remedy of these doctors is to kill the child. Extensive (and costly) screening processes have already begun in some areas to test as many as 50 per cent of pregnant women, and abort any child who does not register a perfect score. It is tragic that equal sums of money are not available for research into ways of preventing spina bifida.
What happens to the baby who is born with spina bifida depends largely on the luck of the draw: where the child is born; the attitude of the doctor; the extent of the handicaps; the parents. For many babies, their fate is “non-treatment.”
One type of non-treatment involves feeding (on demand only) and sedation. According to Dr. Everett Koop (Currently Surgeon General of the United States, and one of the world’s leading pediatric surgeons) the “technique is to oversedate them with choral hydrate Phenobarbital. They become so floppy they cannot take their feedings and die of dehydration and starvation.” The mortality rate is 90 to 100 per cent within the first year of life. This, in fact, is passive euthanasia. Dr. Koops adds “I an certain the day will come when the euthanasia force will say “Why are you concerned about euthanasia? We have had euthanasia for infants for a long time and there has been no outcry.”
For Dr. Everett Koop, a man who has devoted his life to the surgical rehabilitation of children born with congenital defects, this type of infanticide is made even more heinous because it is being practiced by pediatricians, and being ignored by the law.
For months now he, together with the Reagan administration, the National Right to Life, the Christian Action Council, groups supporting the handicapped, and most national medical groups have been working, and fighting, in the United States for a bill – the Baby Doe Amendment – to protect the handicapped newly-born. They have faced strong opposition from the American Medical Association. When the U.S. Senate approved the bill on July 1984, Dr. Willke, the President of the National Right to Life, commented: “The AMA apparently thinks there should be a 10-day return privelege on new-born babies. Fortunately the Senate has recognized that new-born babies have constitutional rights of their own, and has empathetically rejected the “pro-choice” approach advocated by the AMA.”
A second type of non-treatment provides the baby with proper nourishment and medication but withholds any type of corrective surgery. The mortality rate for this group is 60-80 per cent.
There are medical men who refuse to accept the fallacy.
There is no doubt that spina bifida carries with it, to a greater or lesser degree, many serious problems such as paralysis, hydrocephalus, lack of bladder and bowel control, and curvature of the spine. It is equally true that many of these conditions can be corrected or ameliorated by surgery. However, in many areas, even where adequate hospital facilities and surgical expertise are readily available, fewer than 25 per cent of the babies are “selected” for surgical therapy. The other 75 per cent are considered to be of poorer quality and are relegated to “non-treatment.”
Luckily for some children there are medical men and women who refuse to accept the fallacy and the potential of a baby, only a few hours old, can be adequately and correctly addressed. Amongst the many fine pediatricians and pediatric surgeons who believe that every baby deserves a chance are Doctors James T. Brown, and David G. McLone, of the Children’s Memorial Hospital in Chicago.
That hospital has a special clinic for spina bifida, and pediatricians, neurosurgeons, orthopedic surgeons, nurses, therapists, dieticians, social workers and others, are co-ordinated within a team to deal with each baby patient. As soon as an evaluation of the case has been completed (and preferably within 24 hours after birth) the surgeons close the back wound, to prevent infection and leakage of the cerebrospinal fluid. The neural tube is completely restructured and the wound closed, layer by anatomical layer. Where hydrocephalus is present and progressive, shunts to divert the fluid are inserted. Other problems are dealt with in their turn, under the direction of the team co-ordinator.
Some years ago this clinic undertook a sample study of the patients’ progress from their first admission, with a follow –up at intervals, for a period of six years. There was no thought of “selection.” Every tiny patient who arrived became a part of this sample group. Each baby was treated surgically as soon as possible after arrival, and all 84 percent of the back wounds were closed within 24 hours after birth. Shunts were inserted where hydrocephalus was present and progressive.
After five years good-to-excellent results were seen in 80 per cent of the children (the mortality rate was 9 per cent). The remaining 11 per cent, some with poor intelligence, were judged unlikely to become competitive. It is obvious that hospitals which choose to operate on only 25 per cent of spina bifida cases have much to learn.
One other interesting fact emerged from the psychological assessments made during the study. Contrary to popular belief it seems likely that hydrocephalus of itself does not limit the intelligence of the child. However, there are a number of close inter-relationships which do affect the child’s intellectual development, namely (a) between the Central Nervous System (CNS) and infectious from shunts, and (b) between infections from shunts and the speed with which the back wound is closed. If the back wound is not closed within 24 hours the danger of shunt infections become three times greater; shunt infections are the cause of infections of the CNS; and infections of the CNS lower the I.Q. and increase problems of perceptual-motor handicaps. Thus, surgery at the earliest possible time seems indicated.
Forty or fifty years ago the gulf between doctors whose professional philosophy was based on “the sanctity of life” and those who talk of “the quality of life” was less apparent. At that time there were fewer options, and therefore fewer decisions. Dr. Eugene F. Diamond, a leading American pediatrician and a member of the editorial board of Spina Bifida wrote:
“How simple it was to talk to parents of children with meningomyelocele in the 1940’s. Shunts and other surgical methods being unknown, there was little other alternative to committing such children to institutions. There shielded from the eyes of the public and most of the medical profession, the answers came automatically. Little heads blew up like watermelons and tiny increments of pressure finally built up to a point where they turned off vital centers.”
Those conditions of lack of medical options no longer exist and every baby has the right to be spared this fate.
This is a bitter irony, too, in noting that health authorities are seemingly prepared to spend vast sums of public money on a wide and expensive screening process to identify and abort pre-born babies with spina bifida, and yet essential research to prevent the condition has to beg for money. When one looks at the trifling sum that is needed (at the present rate of exchange $75,000 Canadian) and compares this with the possible benefits, it is “enough to make the angels weep.” The research on defects on the neural tube affects more than spina bifida. Dr. LeJeune explains that the neural tube closes in two directions. Failure to close at the lower end results in spina bifida. But if it fails to close at the other end the result is even more tragic for it is “eventually anecephaly, that is, an absence of brain.” It is possible that prevention is in sight – cost $75,000!!!
The last word
The opposing medical view discusses options from the standpoint of “a life worth living” and “the quality of life” ethic. Rarely, if ever, do they consult the handicapped, and yet these are the ones who can say whether life is precious in spite of pain and lack of full physical facilities. It is interesting to note that the National Right to Life News (August 2, 1984) had the following report concerning the Baby Doe Amendment.
“It had been had been rumoured that Sen. Edward Kennedy (D-Mass.) would offer an amendment incorporating the “quality of life” approach adopted by the AMA. Hours before the Child Abuse Act reach the Senate floor representatives of 90 disability-rights groups – many in wheelchairs – staged a demonstration in Kennedy’s Boston office.
“For whatever reason, no senator offered the AMA amendment when the Senate took up the bill.”
Perhaps that last word should be given to the Tammy of the Easter Seal Campaign for the Crippled Children in 1984. Susan Moffatt, a beautiful girl, was born with spina bifida. She is quoted as saying that when she was born the doctors told her parents that she would not live, and maybe it was better that she did not live, and is glad because she loves and enjoys life. Her joy is shared by all who know her.
Next month: What the new U.S. Baby Doe regulations actually say.