Baillie’s credentials were impeccable. He wrote the first British work on pathology as a distinct discipline in 1793 (Morbid Anatomy of Some of the Most Important Parts of the Human Body). He was the son-in-law of Thomas Denman (1733–1815), author of a classic textbook on obstetrics. His uncle was the anatomist John Hunter (1728–1793), regarded as the father of experimental pathology. Baillie’s treatment was orthodox, and better yet, relatively effective. Using gentle manipulation and a special boot, he was able to straighten young Byron’s foot so that he could walk easily, although with a slight limp.
As a result, Byron did manage to overcome his disability and to excel at cricket while at the prestigious British boarding school, Harrow (someone else ran for him), to become a champion swimmer, and according to British poet Roden Berkeley Wriothesley Noel (1834–1894) in his Life of Byron, to “enter a room quickly, running rather than walking, and stopt himself by planting his left foot on the ground and resting on it.” Nevertheless, he seems never to have overcome his shame at being born less than perfect. On the contrary, he appears to have sought out risky ventures to prove himself heroic, ending with a trip to Greece that proved fatal.
Nobody doubts that Byron was born with a problem, but accounts disagree as to exactly what was wrong. Was one foot clubbed or were both? The left or the right? Did Baillie heal only a deformed foot or was it an entire leg? Inexplicably, the debate continued throughout Byron’s life, and when he died in 1824, of “marsh fever” in Greece where he had gone to support the campaign for independence from the Turks, the picture was further complicated by conflicting postmortem reports.
Byron’s doctor, Julius Michael Millingen (1800–1878), had attended the poet prior to his death; after, he reported that “the only blemish of his body, which might otherwise have vied with that of Apollo himself, was the congenital malformation of his left foot and leg.” Byron’s intimate Edward John Trelawny (1792–1881) made a hurried trip to Greece to examine the body and noted that “both his feet were clubbed and his legs withered to the knee—the form and features of an Apollo with the feet and legs of a sylvan satyr.” Twenty years later, Trelawny changed his mind: “I uncovered the Pilgrim’s feet,” he wrote, and discovered “the contraction of the back sinews which the doctors call tendon Achilles that prevented his heels touching on the ground and compelled him to walk on the fore part of his feet.” Seventy-nine years after that, at a meeting of the Royal Society of Medicine in London in March 1923, Sir Hector Clare Cameron (1843–1928), Dean of Faculties at Glasgow University, displayed models purportedly used to make Byron’s boots. They showed two perfect feet. The only possible explanation is that Dr. Cameron, Knight Templar, Commander of the British Empire and holder of a Regius professorship conferred by Queen Victoria, had been snookered.
Meanwhile, Hippocrates’ basic stretch-and-bind method with a small change here and there—a boot instead of bindings, a slightly different massage regimen—remained the best known, safest, and most effective approach to clubfoot. However, the notion that if one could cut the constricting tendons and muscles one might release the foot quickly and effectively was beginning to bubble to the surface.
In the first half of the nineteenth century, surgery was a cure worse than the disease. Contemporary statistics suggest that as few as 50 percent of all surgical patients made it out of the hospital alive. The reasons were obvious. Cutting into a living body and keeping that body alive and healthy afterward, creates three challenges: to control bleeding, to subdue pain, and to prevent infection. Ambroise Pare had solved the first in 1552 by teaching surgeons how to tie blood vessels. In 1846, Boston dentist William Morton (1819–1868) demonstrated the anesthetic effectiveness of inhaled ether. Infection remained the final challenge.
In 1816 French surgeon Jacques Mathieu Delpech (1777–1832), Director of the Hôtel-Dieu Saint-Eloi in Montpellier, performed the first modern clubfoot surgery, cutting the Achilles tendon to release the twisted foot. Attempting to reduce the risk of infection by making smaller incisions, Delpech used what he called a “blind” technique on two patients, using a curved knife to make two small cuts to divide the tendon holding the foot in its fixed, clubbed position. Unfortunately, even these smaller surgical wounds tended to become infected, and Delpech abandoned the procedure.
But the experiment obviously made an indelible impression on French novelist Gustave Flaubert (1821–1880), son of the chief surgeon at the Hotel-Dieu hospital in Rouen. In Madame Bovary, Flaubert describes in grisly detail the experience of Hippolyte Tautain, the crippled stableman at the Hotel du Lion d’Or in Yonville, the hometown of Emma’s cloddish husband, Dr. Charles Bovary.
Charles, Flaubert wrote, had read about a new way to treat clubfoot— it sounds exactly like Delpech’s surgery—and decided it would be “a fine thing for Yonville to show how up to date it was by going in for the operative treatment of strephopody.” Bullying Hippolyte into the surgery, which turned out to be surprisingly painless, Charles sat back, waiting for applause. Instead, he fell into disaster. The surgical wound became infected; the infection spread. A prominent physician from a neighboring town was called in to amputate Hippolyte’s leg, leaving Emma “disgusted with herself for imagining for a single moment that [Charles] could ever do anything well” and poor Charles to feel “that some fatal and incomprehensible influence, he knew not what, was at work around him.”
How could he have known?
Nine years before Madame Bovary made her entrance onto the French literary stage in 1856,2 Ignaz Semmelweis (1818–1865) made washing one’s hands before examining or treating a patient the rule for students at the maternity clinic at the Vienna General Hospital in Austria, dramatically reducing the incidence of death from puerperal (“childbed”) fever. Semmelweis must have been operating on intuition because it was not until 1865, nine years after Flaubert’s novel appeared, that Louis Pasteur (1822–1895) theorized that decay—infection—was caused by microorganisms carried through the air to land on any available surface, including human skin, mucous membrane … or a surgical wound.
Two years later, in 1867, British surgeon Joseph Lister (1785–1869) made the connection between Pasteur’s microorganisms and surgical infection. His solution was to bring carbolic acid (phenol) into the operating theater at Glasgow Royal Infirmary. He used the acid to wash his hands and his instruments, saturate the bandages on patients’ wounds, and even sprayed it into the air to kill floating germs. This regimen eventually lowered the overall rate of death due to infection after surgery in his hospital from a standard 45 to 50 percent to 15 percent, an astoundingly low figure for the time. The Europeans, particularly the Germans, accepted Lister’s antiseptics fairly quickly, but not until 1877, when Lister was named a Professor of Surgery at King’s College Hospital in London, did the British medical community fully embrace his advance in surgical patient care.
Of course, hospitals and operating theaters were—and still are—hazardous places, but thanks to Semmelwies, Pasteur, and Lister, the chances of a patient’s getting out alive were much improved. So when Delpech put down his scalpel, others were eager to pick up the knife.
Chief among them was Georg Friedrich Louis Stromeyer (or Strohmeyer, 1804–1878), a German pioneer in orthopedic surgery and a founder of Charité - Universitätsmedizin Berlin, the medical school for both Humboldt University and the Free University of Berlin. Stromeyer believed that congenital clubfoot was due to a “deficiency” in the internal structure of the malleolus, the bony bump on each side of the ankle; however, he operated mostly on patients whose clubfoot was due to paralysis. His procedure was essentially Delpech’s: He inserted a small knife through the skin and underlying tissue to divide the Achilles tendon and free the foot.
One of Stromeyer’s patients was the British orthopedic surgeon William John Little (1810–1894), whose foot had been twisted by polio when he was two. Little survived his illness and went on to found the Royal Orthopaedic Hospital in London. He was the first to describe a condit
ion, Little’s disease, now known as cerebral palsy. As for his clubfoot, once Stromeyer repaired it in 1836, Little was so grateful that he named his son Louis Stromeyer Little. He also took Stromeyer’s procedure back to England, where it was adapted and used to free tendons in other parts of the body as well. In addition, Little, like Hippocrates, emphasized the virtue of manipulating and stretching the tissues around the foot. Unfortunately, like Scarpa, he designed his own punishing device, iron braces extending from the pelvis to the foot.
Dividing the Achilles tendon remained the surgery of choice until midcentury. In 1866, William Adams (1820–1900) was awarded the Royal College of Surgeons’ Jacksonian Prize for his essay on the causes and treatment of clubfoot. Adams was the first to examine muscle and bone tissue from a clubfoot under a microscope. His specimens, taken from stillborn children, showed the tissues in the deformed foot to be no different from those in a normal foot, so he opposed dividing the Achilles tendon, at least as a first step in alleviating clubfoot. Like Hippocrates, Pare, and Baillie, Adams believed that what twisted the foot was the force of the surrounding muscles and tendons. He dismissed the Scarpa shoe, but he too had his own mechanical device: a straight splint of sheet metal running the length of the outer leg.
In other words, everything old was new again. Until the end of the nineteenth century when suddenly there really was something new: The X-ray, which like so many scientific marvels, was discovered by accident. On November 5, 1895, German physicist Wilhelm Conrad Roentgen (1845–1923) noticed that “rays” (i.e., electron beams) generated by the cathode inside a vacuum tube were not pushed aside by magnetic fields, which meant they could “see” through all kinds of matter. One week later, Dr. Roentgen took an X-ray picture of Mrs. Roentgen’s hand: Her wedding ring and her bones were clearly visible along with the pale shadow of her skin.
The new technology fascinated most people, but annoyed others who foresaw a world in which X-rays would let people look through walls and invade privacy, rather like today’s airport security devices. Doctors had no such qualms. For them the salient point was that X-rays made it possible to look into the body without cutting and see exactly how bones including those in a clubfoot were deformed or displaced.
Inevitably, this pushed the surgeons treating clubfoot to ever more radical procedures. It became the fashion to divide and lengthen not just the Achilles tendon, but every single tissue around the foot and ankle, or one might remove supposedly deformed bones from the foot. The popularity of both operations faded as it became obvious that one left the patient with a foot that was straight but too stiffened by excessive scar tissue to move freely, and the other gave the patient a foot that was also straight but might never be strong enough to support his or her weight. No wonder that even a medical student like Philip Carey, the long-suffering anti-hero of Somerset Maugham’s Of Human Bondage (1915), did not seek treatment. Carey’s clubfoot, often described by critics as a literary substitute either for Maugham’s stutter or for his homosexuality, both of which made him acutely uncomfortable, perfectly captured the idea of a twisted foot as a disabling, but not fatal disorder.
Enter Ignacio Ponseti (1914–2009). Born on the Spanish island of Minorca, Ponseti started his professional life as an orthopedist on the battlefields of the Spanish Civil War, setting fractures and ferrying injured Loyalists across the Pyrenees to France. In 1939, he took himself over the mountains. Two years later he emigrated to Mexico, and from there, a referral from an orthopedist friend sent him to the United States to study and practice orthopedics at the University of Iowa Hospitals and Clinics. Once settled in the American Midwest, the Spanish surgeon discovered large numbers of children whose clubfeet were being treated, but not cured, so he drew up his own stretch-and-bind regimen known as—what else?—the Ponseti method.
Ponseti’s method entails manipulating and stretching the tissues to move the foot into a more natural position, beginning as soon as possible, a moment the current Global Help Organization-sponsored Ponseti manual puts at seven to ten days after birth. The regimen begins with the physician’s immobilizing the child’s foot in a plaster cast for seven days to maintain the correction, then removing the cast, stretching the muscles and tendons again, and replacing the cast. The procedure is repeated each week for a period of three to four months, a regimen that requires as many as twenty different casts per foot. At the end of this part of the treatment, the child is fit with shoes attached to a metal bar to be worn twenty-four hours a day for about six months, and at night for up to two years. Afterward, perhaps three in ten children are said to require a “minor” procedure to lengthen a tendon; fewer than one in one hundred treated with the Ponseti method need major correction surgery.
The introduction of sonography (ultrasound) in the 1950s promised diagnosis as early as eighteen weeks into a pregnancy, making it possible to prepare parents in advance if the image indicated that the child had a clubfoot. As for modern surgery, in 2000, researchers at the Department of Orthopaedic Surgery and Department of Pediatrics at the University of Iowa wrote that “a few reports indicate that surgery [for clubfoot] is almost invariably followed by deep scarring, which appears to be particularly severe in infants. In addition, the average failure rate of clubfoot surgery is 25% [range 13% to 50%] and many complications can occur including wound problems, persistent forefoot supination, loss of reduction and recurrence, overcorrection of the hindfoot, dorsal subluxation of the navicular, and loss of normal motion of the ankle and subtalar joints.”
How much better it would be to identify the cause of clubfoot, and thus be able to prevent it.
Entering the genome
When searching for answers as to how to reduce the risk of a birth defect abnormality such as clubfoot, you can start by asking, “What do the people with this problem have in common?”
Centuries ago the answer was simple: Giving birth to a child with a congenital abnormality was proof of your being out of favor with the gods. Then the blame shifted to human beings, specifically the woman careless enough to think the wrong thoughts or glance the wrong way while pregnant. Simply looking at a rabbit while pregnant might lead to the birth of a baby with a “hare” (cleft) lip, or seeing a one-armed person, a baby with a missing arm. Mary Jane Merrick, mother of John Merrick who was incorrectly diagnosed as suffering from elephantiasis (hence the label “the elephant man”), was said to have been frightened by an elephant at a local fairground.
This theory, known as maternal impression, has not died an easy death. As recently as 2003, Canadian biochemist Ian Pretyman Stevenson (1918–2007), one-time head of the Division of Perceptual (i.e., paranormal) Studies at the University of Virginia, was still trying to prove that a mother’s thoughts—or, shades of Bridey Murphy3—even her “past lives” could influence fetal development. To this day, a pregnant Parisian, hoping for a boy, may visit the Louvre to stare at paintings of handsome men, most commonly those of the noble persuasion.
When the maternal impression theory did not work out, the search for an explanation turned to more verifiable causes in or out of the womb, such as the physical environment inside the uterus. Perhaps the fetus had been positioned so that its developing foot was bent forward or back under its body. Or crowding in the womb might have twisted the foot out of its natural position or restricted the fetus’ movements so that its muscles did not develop the strength required to hold a foot straight. Some studies do show a higher incidence of clubfoot among multiples than among single infants; in the 1960s, researchers at Johns Hopkins proved that paralyzing a chick embryo in the egg prevented its legs from developing normally.
A more demonstrable prenatal culprit is amniotic band syndrome, a condition in which the amnion (the inner layer of the amniotic sac) separates and tears into strings of tissue that may wind around parts of the fetus’ body. According to the Fetal Treatment Center at the University of California, San Francisco, this syndrome occurs in as many as one in every 1,200 or as few as one in 15,000 live births. Wha
t happens when that is the case depends on where the strings land and how tightly they bind. In the most severe case, amniotic bands wrapped around the head or umbilical cord might be fatal to the fetus. Bands around the face or neck might cause cleft lip and/or palate. Those around fingers or toes might lead to syndactyly (webbing), ordinarily a genetic defect. Strings wrapped tightly enough to cut the blood supply might actually amputate a digit. And a band around the foot might cause a clubfoot.
Late in the nineteenth century, the environment outside the body became suspect. In 1877, Gabriel Madeleine Camille Dareste (1822–1899), professor of zoology at the University of Lille and the first director of the laboratory of teratology at the Ecole des Haute-Etudes (School for advanced Studies in the Social Sciences) in Paris, was awarded the grand prize in physiology by the French Academie des Sciences for his research into how birth defects occur. Exposing chicken embryos to extreme heat, Dareste was able to trigger skeletal and organ abnormalities in the chick developing inside the egg. A century later, his observations on the effects of heat on fetal chickens were extrapolated to human beings, specifically to the effects of temperature on the male reproductive system (the warmer the testes, the lower the production of sperm).
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