However, even being labeled “human” was not enough to protect all infants. Sometimes even healthy children were seen as threatening. Egyptians considered twins evil children of the gods; twins of opposite gender were thought to have committed incest in the womb. Well into the Middle Ages, a visible disabling birth defect such as a missing limb was reason enough to eliminate the theoretically useless child, and disabled adults or those afflicted with disfiguring diseases, most commonly leprosy and small pox, were regarded as sinners and scapegoats for natural disasters and catastrophes ranging from the periodic visitations of the plague to earthquakes and drought.
The legal and social disqualification of the disabled was reinforced by art. As the often flat, one-dimensional Medieval portraits gave way to the richer paintings of the Renaissance, artists such as Titian, Donatello, Tintoretto, Botticelli, Caravaggio, and Raphael sought to revive the Greek and Roman ideals of perfectly proportioned human beauty so clearly depicted in Leonardo’s Vitruvian Man, resulting in yet another reason to reject those who did not measure up—with the exception of those with a clubfoot, which from the beginning seemed to be a deformity with a difference.
First impressions, catalogues, and curiosities
If your leg and foot were one long L-shape bone, you would never need a knee replacement or an ankle brace. Of course, you would have to lift the entire leg to move, so your ability to turn quickly would be severely compromised, and if you fell and broke the bone, you would have to replace the entire leg, thigh to toe. Luckily, as American architect Louis Sullivan once observed, form follows function (what Sullivan really said was “form ever follows function,” but removing one word made the point faster and neater, so it stuck).
Our anatomy agrees. We have joints at the knee, at the ankle, and in the foot itself that make it easy to move forward, backward, and sideways. The downside, of course, is that we risk knee and ankle injuries and arthritis, as well as clubfoot.
The Greeks called clubfoot strephenopodia from the words streph meaning twist and the obvious pod. The name was perfectly descriptive. Congenital clubfoot is a dislocation either of the tibiotalar joint that connects the tibia (the larger and stronger of the two bones in the lower leg) to the ankle (talus) or the talocalcaneonavicular joint that connects the ankle bone to the heel (calcaneus) and to the navicular bone, one of the small tarsal bones on the top of the foot.
The basic medical name for congenital clubfoot is talipes (talus plus pes). A foot twisted down from the ankle is called talipes equinus, from the Latin equus meaning horse. In talipes varus (Latin for bent), the foot is turned toward the other foot. In the less common talipes valgus (Latin for twisted outward), the foot is turned away from the other foot. If the twisting is severe, an untreated child may walk either on his toes or on the outer edge of the foot. Either way, the deformed foot does look something like a horse’s hoof. Talipes calcaneus is a second form of clubfoot. Here the foot is bent up, with the toes pointing toward the knee and the heel towards the floor. Normally, a person walks by putting the toe or the heel, then the rest of the foot, down on the floor. In talipes calcaneus, only the heel hits the floor. In either case, if the twisted foot is not treated fairly quickly, its muscles and tendons begin to contract to hold the twisted foot in its unnatural position.
Given the fact that birth defects were often regarded as a sign of worthlessness or worse, a person with a congenital clubfoot might well have tried to blame the problem on an injury. For example, Charles-Maurice de Talleyrand-Périgord (1754–1838) claimed his clubfoot was caused by his having fallen off a chest of drawers as an infant. The accident, he insisted, had crushed his foot which then hardened into a round mass. Medically, this is highly unlikely. Besides, biographers note that Talleyrand’s mother disliked him as soon as he was born, suggesting that she was reacting to the sight of a congenital abnormality unwelcome in her eighteenth century aristocratic family. It might also explain why the family packed the boy off as quickly as possible, first to boarding school and then—because he was unfit for military service—to a seminary to train to be a priest, and eventually to become a bishop in keeping with his family’s status. Talleyrand resented this mightily; the exile is said to have turned him hard and merciless, just the attributes he required to serve as Grand Chamberlain to Napoleon, to avoid the guillotine by siding with the revolutionaries during the Revolution, and generally to come out on top in virtually every political situation.
Later in life, clubbing may follow an infection or an illness. Joseph Goebbels (1897–1945), the Nazi Reich Minister of Propaganda, is believed to have acquired his clubfoot as a complication of osteomyelitis, an infection inside a bone. Afterward, Goebbels walked with a leg brace and that, along with his puny 100 pound physique led to his being rejected when he sought to enlist in the German armed services during World War I. Sir Walter Scott (1771–1832) acquired his clubfoot after a bout with polio. Scott’s recollection of his illness appears in John Gibson Lockhardt’s seven volume Memoirs of Sir Walter Scott (1808):
“I showed every sign of health and strength until I was about eighteen months old,” Scott recalled. “One night, I have been often told, I showed great reluctance to be caught and put to bed, and after being chased about the room, was apprehended and consigned to my dormitory with some difficulty. It was the last time I was to show much personal agility. In the morning I was discovered to be affected with the fever which often accompanies the cutting of large teeth [teething]. It held me three days. On the fourth, when they went to bathe me as usual, they discovered that I had lost the power in my right leg…. when the efforts of regular physicians had been exhausted, without the slightest success…. the impatience of a child soon inclined me to struggle with my infirmity, and I began by degrees to stand, walk, and to run. Although the limb affected was much shrunk and contracted, my general health, which was of more importance, was much strengthened by being frequently in the open air, and, in a word, I who in a city had probably been condemned to helpless and hopeless decrepitude, was now a healthy, high-spirited, and, my lameness apart, a sturdy child.”
The first depictions of clubfeet appear to be in Egyptian tomb paintings, which is not surprising considering the consequences of the marital histories of the Egyptian royals. Pharaohs were believed to be children of the gods. Likewise, Roman emperors were considered celestial individuals temporarily on earth, and European royalty claimed appointment by divine intention. In each case, to keep the bloodlines godly, and the fortune in the family, the royals frequently married each other.
Unfortunately, what pleased the gods and the accountants did not necessarily result in healthy babies. In Europe’s ruling houses, multiple inter-marriages produced numerous genetic problems. Among the Hapsburgs of the House of Austria who claimed the Spanish throne in 1516, nine of eleven marriages during a 200-year reign were between blood relatives. One highly visible result was the Hapsburg jaw, a prominent mandible (lower jaw) sometimes pushed so far forward as to make it difficult if not impossible to bring the teeth together to chew solid food. There were also kidney abnormalities, mental retardation, impotence, and a sky-high rate of infant mortality. It is estimated that just half of the children born to the Spanish Hapsburgs made it alive to his or her first birthday; the end of the line came in less than two centuries. In the British House of Hanover, Queen Victoria’s recessive hemophilia gene, carried by at least six of her female descendants, is known to have struck nine of her male children, grandchildren, and great-grandchildren including history’s best-known hemophiliac, the ill-fated Alexei Nikolaevich, Tsarevich of Russia, son of Victoria’s granddaughter Alexandra.
Geneticists generally consider marriages between relatives closer than second cousins to be potentially problematic. The Europeans pretty much stuck to relationships no closer than first cousins, but even that was a problem because children of such unions inherit more than six percent of their genes as identical copies, one from each parent. The Egyptians, on the other hand, sanc
tioned matches between siblings, half siblings, or in a pinch, father and daughter or grandfather and granddaughter. (Mindful of this history, modern Egyptian law prohibits marriages among persons whose relationships is closer than that of first cousins.)
Tutankhamen (c. 1325–1342 BCE), whose parents were brother and sister, was born into the much intermarried 18th Dynasty, one of the three clans that ruled from 1570 to 1085 BCE during the period known as the New Kings. For nearly 100 years after his tomb was discovered and opened in 1922, it was assumed that King Tut had expired after being hit on the head-with a blunt instrument, a theory supported by the fact that there was a hole in the mummy’s head. But in the winter of 2010, when a multinational team of geneticists began testing the DNA of the mummies in Tut’s tomb, including two infants believed to be his daughters with his half sister Ankhesenatmun, the daughter of his stepmother Nefertiti, their tests showed not only that most of the people buried with Tut were related, but also that Tut had been a victim of haphazard consanguinity, suffering not one but at least two genetic strikes. Like his father Amenhotep IV, he had a cleft palate, and like his grandfather Amenhotep III, he had a clubbed foot.
Clearly, the Egyptians, like the Greeks and Romans, had no problems dispensing straightaway with infants born with birth defects. But their royals, after all, were god-like, so who knows? Maybe the populace just decided that if a deformity such as clubfoot showed up in more than one member of the semi-divine ruling royal family, the problem was simply part of the royal package. The idea is pure speculation, but if so, would have bought Tut’s grandfather and his father their own rule and given Tut himself time on the throne until he died, perhaps of malaria. Or because Tut’s mummy had a broken leg, along with the cleft palate and clubfoot, maybe one day, lame and walking with canes, he tripped and fell and then, absent sterile surgery and antibiotics, developed a galloping infection that carried him off at the tender age of 19, a romantically early death that whatever the cause ensured that he would forever be remembered as the “boy king.”
He was by no means the only one in the neighborhood. Tiberius Claudius Caesar Augustus Germanicus, a.k.a. Claudius I (c. 10 BCE–54 CE), the fourth emperor of Rome and the first to be born outside Italy (he came from Gaul in what is today Lyon, France), not only had a clubfoot, he stammered. Widely considered a weakling, once in power as Emperor, Claudius proved the common wisdom wrong. He ruled for 13 years from 41 to 54 CE, during which time he conquered and colonized Britain and streamlined the administration of Rome itself. Then, like so many of his generation, he left office under suspicious circumstances, probably having been poisoned by his wife Agrippina, whose son Nero—Claudius’ stepson—took his place.
Catalogues of human monsters, including monstrous infants, date as far back as the Babylonians. These early records were random collections of abnormalities, ranging from the fantastic (races of men with animal heads) to the realistic (Egyptian and Sri Lankan representations of achondroplastic dwarfs). The Etymologies, compiled by Isidore of Seville (c. 560–636) sometime during the years 615 to 630, was different. Its author, later canonized as St. Isidore, was born into an orthodox Catholic family in Cartagena, orphaned as a child, and raised by his brother Leander, whom he succeeded as Bishop of Seville in 600. Isidore was a prolific writer; The Etymologies, his magnum opus, was a 51-volume encyclopedia designed to explain all branches of human knowledge, including human diversity (Volume XI), based on the meaning of words and names.
Semantics aside, Isidore’s important innovation was to group “monstrosities,” including children born with birth defects, into twelve distinct categories:
Excessive growth (i.e., giants)
Insufficient growth (i.e., pygmies and dwarves)
Unusually enlarged body parts
Extra body parts (i.e., a sixth finger or toe)
Missing body parts (i.e., a missing bone in leg or arm)
Animal parts (i.e., webbed fingers or toes or excessive hairiness)
Delivery of a newborn animal rather than a human child
Misplaced organs or body parts (i.e., eyes too wide apart)
Unusual aging (progeria)
Composite creatures (i.e., the centaur or the mermaid)
Hermaphrodites [infants with apparently male (Hermes]) and female (Aphrodite) genitalia]
Monstrous races [i.e., entire nations of men such as “the Cynocephali … so called because they have dogs’ heads (cyno is Latin for dog, cephalo, for head) and their very barking betrays them as beasts rather than men. These are born in India.”]
The Etymologies remained the standard reference for centuries. Although as time passed, the approach slowly—very slowly—became less fantastic and more scientific. Isadore’s carefully constructed categories created a template for later categorizations of human abnormalities such as those defined by Fortunio Liceti (1577–1657) and Ambroise Pare (c.1510–1590).
Liceti appears to have been a natural overachiever. He graduated from the University of Bologna with doctorates in philosophy and medicine and is the author of two major medical treatises. In De monstruorum causis, natura et differentiis (1616) he addressed genetic anomalies; in De spontaneo viventium ortu (1618) he affirmed the spontaneous generation of living beings. Although both essays reflect the unscientific tradition of his day, he did have one foot halfway into the medical future, arguing consistently against the idea that birth defects were caused by divine wrath and searching, albeit in vain, for a physiological explanation.
Pare, the royal surgeon to the French kings Henry II, Francis II, Charles IX, and Henry III who ruled, one after the other, from 1547 to 1574, did the usual cataloguing. Intellectually, he was several steps ahead of his contemporaries in believing that birth defects were due to a “lack of discipline in the parents’ seed,” a crude, but intuitive suggestion that they were due to human malfunction rather than divine intent. On the other hand, his theories about the cause of clubfoot were classically naive. He thought the problem arose from the mother’s sitting cross-legged while pregnant or having held the baby too tight against her body while nursing. His advice on treatment was also classic: He endorsed the method of the master, Hippocrates.
The Egyptians may have been the first to draw a picture of a clubfoot, but Hippocrates was the first to produce a written description of the condition, sometime around 400 BCE. Given the medical surround in which he worked—no anesthetic, no antiseptics, no diagnostic tools save fortune telling—the treatment he proposed was pretty much on target.
He advised beginning with gentle manipulation of the foot, then securing it with bandages to stretch the soft tissues and allow the foot to fall into a natural position in which the sole would be flat on the floor. Once the foot was overcorrected, new coverings were used to reinforce the correction and keep the foot from slipping back into the abnormal position. It is an extraordinary compliment to his skills as a physician operating strictly on observation and intuition that 2,000 years later Ambroise Pare endorsed as his own most of Hippocrates’ treatment for clubfoot. Pare did introduce a boot of turpentine-softened leather with a wedge sole to hold the foot in place long enough to increase the chance of permanent straightening, but he understood that correcting the foot took time and patience. In “The defect called varus and valgus,” one chapter in Oeuvres (1585), the multi-volume summary of his own career and accomplishments, Pare explained that when treating a clubfoot “one must not make varus and valgus children walk until the joints are well strengthened, so that they do not dislocate. And when one wants to make the children walk, one should split open some high shoes, little half boots, and laced up at the front, or fastened by little hooks: they should be of hard leather so as always to keep the bones firmly on the joints, and so that they have to stay there. And the sole must be higher on the side where the malformation will be inclined to turn, in order to force it to turn back to the necessary side.”
This adaptation of Hippocrates’ simple but effective approach worked well enough to keep
it in vogue for centuries more—with occasional embellishments, not necessarily for the better.
Cruel and unusual cures
Italian anatomist and surgeon Antonio Scarpa (1752–1832) was a man whom one biography describes as authoritarian and “picky” with few friends and many enemies—rather like Tosca’s nemesis Scarpio without the music.
He was also a man of many interests. His first success came in 1772 with a treatise on the structure of the ear. He is credited with creating the specialty of orthopedic surgery. His 1803 essay Memoria Chirurgica sui Piedi Torti Congeniti dei Fanciulli, e sulla Maniera di Corregere questa Deformita (A Memoir on the Congenital Clubfeet of Children, and of the Mode of Correcting that Deformity) was the first accurate description of a clubfoot. However, unlike Hippocrates and Pare, Scarpa insisted on forcibly straightening the foot, then fitting the patient with a multistrap rigid boot known as Scarpa’s shoe to hold the foot in position. No one else was able to show that his method worked, so it was soon abandoned.1
Before the punishing regimens and devices prescribed by Scarpa and some of his contemporaries were discredited, they claimed a host of young victims including George Gordon, later George Gordon Noel, 6th Baron Byron, better known as the romantic poet Lord Byron (1788–1824). Byron was born “with lameness and a twisted foot.” As a child, he was treated first by a man named Levander, generally identified only as a “trussmaker for the local hospital,” whose therapy consisted in rubbing the boy’s foot with oils, roughly twisting it into normal position, and then locking the foot and leg into a “wooden machine” to hold it straight. When this did not work any better than Scarpa’s shoe, Levander was sent away, and Byron’s mother took her son to London to see the Scottish physician Matthew Baillie (1761–1823).
Leonardo's Foot Page 5