Carole thought of her colleagues at Children’s. She could have used their support now. She might be surrounded by family, but the truth was, none of them understood what a diagnosis of spina bifida really meant. She alone had no comforting ignorance to hide behind as she accepted being this child’s mother.
My mother.
For whom, on that day, life itself was all that mattered.
*2 Warkany’s work eventually earned him the sobriquet “the Father of Teratology.” From Warkany’s obituary in The New York Times, June 25, 1992: “Dr. Warkany’s discoveries helped create the medical specialty of teratology, which includes the study of the fetus as well as efforts to cure and prevent the prenatal ailments responsible for much of the infant death rate. He contended that congenital ills should be treated medically just like postnatal ailments….Dr. Warkany…was the founding president of the Teratology Society and helped develop the field as a certified specialty.”
Carole was part of Warkany’s team when he “loaned” her to Albert Sabin for the last frantic days of the race to invent the polio vaccine. At the time, Jonas Salk was Head of Pediatric Research at Children’s Hospital. Sabin and Salk had spent years in neck-and-neck competition to invent the oral form of the polio vaccine. Reportedly, she was there, on March 26, 1953, when the announcement came over the radio that Jonas Salk had perfected an effective, injectable formula. According to her, Sabin screamed invective and hurled a full beaker of chemicals the length of the lab, barely missing the frantically ducking heads of his staff. And yet Sabin’s vaccine would become the more widely used, being both cheaper and easier to administer.
*3 “Yocheved” is the female version of “Yosef.”
Cauda Equina
2005
CHAPTER 2
Cauda Equina
The medical effects of spina bifida myelomeningocele (SBMM, or MM) are different for each person, but roughly, they are as follows:
Each spinal vertebra has a specific nerve branch (a dermatome). Wherever a lesion occurs—that is, a hole in the spinal column—the nerves nearest the breach are damaged. Muscles, bones, and organs rely on enervation to grow and function. This means they may malfunction or never develop at all. When organs don’t receive normal enervation, bowels and bladders may become spastic, nonfunctional, or incontinent. Kidneys are particularly at risk.
Given that neural tube failures happen in the fetal stage, spina bifida can affect almost any form of mammal (for instance, I have a friend who has a cat with SB; it’s paralyzed and needs help voiding its bowels). When I was born, techniques to close the lesions had only just been invented; it appears as if the first successful surgeries occurred somewhere in the mid-1950s. Before this period, the mortality rate was upwards of 85 percent. Doctors are only now learning how to treat spina bifida as we grow and age. My own adulthood has only been possible for the last fifty or so years.
Even with a successful closure, the spinal canal may become narrowed and constricted. This can force cerebrospinal fluid up into the head, a condition known as hydrocephalus (so-called “water on the brain”); the danger is that fluid pressure will squeeze away the space for the developing infant brain. A shunt must be inserted to drain the excess fluid, otherwise hydrocephalus may result in cognitive, communicative, and learning disabilities; severe cases may lead to death. I was unusual in that I did not have hydrocephalus—also fortunate, as shunts did not come into use until several years after I was born.
My version of spina bifida is properly called lipomyelomeningocele (LMM), meaning that a lipoma—a fatty tumor—was adhered to the cord at the lesion site. People with any degree of myelomeningocele may develop “tethered cord,” which happens when the spinal cord becomes ensnared by scar tissue at the site of the lesion. Tension on the cord pulls the spine downward, like the string on a bow. This may cause or increase any scoliosis, but the most serious danger is that tethered cord can be fatal should it pull the brain stem out of position.
* * *
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Before Josef Warkany established the field of teratology, anomalies were explained via a swampy mess of mythology and superstition, as people searched for the comfort of an explanation. Myths gathered details, characters, causes and effects. Mythical monsters can be viewed as explanations for fetal conditions, and vice versa; the names given to many impairments speak to this entanglement of science and superstition. When a child is born with legs fused together, it’s called Mermaid Syndrome (or sirenomelia). A baby that lacks a nose and possesses but one eye in the middle of his face is a Cyclops (the medical condition is known as cyclopia or holoprosencephaly). Infants covered with fur-like hair have Werewolf Syndrome (hypertrichosis). Babies born with vestigial tails or “horns” or with albinism have been seen as animals and ghosts.
“Monstrous” children were blamed on mothers via an ancient concept known as maternal imprinting. Maternal imprinting dates back to the first century C.E., the time of Pliny the Elder, and persisted well into the nineteenth century. A fetus could be “imprinted” by something its mother saw. Women were warned against looking at disturbing sights if they were pregnant or trying to conceive. Visual trauma, it was believed, could ripple through the womb, as the boundary between outside and inside was thin and indefensible. “Disturbing” sights included those that lay outside the sanctioned domestic routine of a well-contained woman. A well-contained woman did not attend carnivals, where she might see the antics of a performing monkey and give birth to a baby covered in hair, or a frolicking seal that might cause her child to be born with shortened arms. Maternal imprinting not only condemned a mother for the nature of her infant, it was a tool for denying women their freedom of movement.
We pride ourselves on being more sophisticated (few pregnant women would avert their eyes from a Pekingese in fear for their child’s profile), yet fear of and distaste for the disabled is alive and well. I was born just after a surgical breakthrough, yet 1958 hardly constitutes a medical bright line, a turning point when the surgical ability to repair these lesions led to a comprehensive agreement that it should be done. Advances in spina bifida treatment have kept coming, but international debates (such as those in Belgium and the Netherlands) persist over whether to treat infants like me at all. The bioethicist Peter Singer used spina bifida as his central example as to why disabled children should be allowed to die. He opined that we will never have a reasonable “quality of life,” and that we consume far too many medical resources for too little societal payback. I can only be grateful that Peter Singer wasn’t stalking the halls of Cincinnati Children’s Hospital sixty years ago.