Mutants (23 page)

D
ARU OR
T
ARON
. U
PPER
B
URMA C
.1937.

Who were they? The Burmese weighed up the evidence and decided that the Taron were probably identical to the Nungs of earlier reports, and therefore a race of genetically short people. How many more of them there were, and their precise origins, were questions left unanswered. The hypothesis that they were true pygmies appeared to be supported by the fact that they lived in close proximity to taller people whose diets seemed no worse than theirs. Yet there were disquieting aspects to the Taron. Of the ninety-six living in the two villages, nineteen were mentally defective. This is a high proportion, even allowing for the fact that they were inbred (pedigrees showed many first-cousins marriages). Several had severe motor-neuron disorders and were unable to walk. And the Taron themselves claimed that when they had come from China they had been of normal size; only in Burma had they become small. That is all we know of the Taron, and we are not likely to know more soon – foreigners have not been allowed into Upper Burma for decades. But it is possible that the Taron are not so much pygmies, or even dwarfs, but rather simply cretins.

It is not a pretty word, but it is the correct one. Cretins are people who are afflicted from birth by a mix of neurological and growth disorders. Traditionally, they have been classified into two types: ‘neurological’ cretins who are mentally defective, have severe motor-neuron problems and tend to be deaf-mute; and
‘myxedematous’ cretins who have severely stunted growth, dry skin, an absence of eyelashes and eyebrows and a delay in sexual maturity. A peculiarly vicious form of myxedematous cretinism, in which growth and sexual development simply stop at about age nine, is found in the Northern Congo. These Congo cretins may be in their twenties and still show no sign of breasts or pubic hair, menstruation or ejaculation, and they never grow taller than 100 centimetres (three feet three inches). This is an extreme. The Taron may have a milder form of the same disease.

Cretinism is a global scourge. In 1810 Napoleon Bonaparte ordered a survey of the inhabitants of the Swiss canton of Valais; his scientists found four thousand cretins among the canton’s seventy thousand inhabitants. The location is telling. As the Taron Valley lies in the foothills of the Himalayas, so Valais lies
at the base of the Alps. Swiss cretins have not been spotted since the 1940s, but a belt of cretinism still tracks most of the world’s other great mountain ranges: the Andes, the Atlas, the New Guinea highlands, the Himalayas. What these areas have in common is a lack of iodine in the soil. People and animals alike rely on their food for a ready supply of iodine, but in many parts of the world, especially at high altitude, glaciation and rainfall have leached most of the iodine out of the soil so that the very plants are deprived. Cretinism is caused by a diet that contains too little iodine. Globally, about one billion people are at risk of iodine deficiency; six million are cretins.

M
YXDEMATOUS CRETINS AGED ABOUT TWENTY, WITH NORMAL MAN
. C
ONGO
R
EPUBLIC
1970.

In the Gothic cathedral of Aosta, ten kilometres south-east of Mont Blanc, the choir stalls are decorated with portraits of cretins. They were carved to keep their fifteenth-century viewers mindful of the unpleasantness of Eternal Torment: a local version of the fabulous creatures and demonic creatures of misericords elsewhere. Many of the cretins have a curious feature: their necks are bulging and misshapen; one even has a bi-lobed sack of flesh hanging from his throat large enough to grasp with both hands. Just over a hundred years after Aosta Cathedral was built, Shakespeare would write in
The Tempest
: ‘When we were boys/Who would believe that there were mountaineers/Dew-lapp’d like bulls, whose throats had hanging at them/Wallets of flesh?’

The Aosta cretins and Shakespeare’s mountaineers were goitrous. Goitre is an external manifestation of an engorged thyroid, a butterfly-shaped organ located just above the clavicles. Like cretinism, it is a sure sign of iodine deficiency. When first discovered in 1611, the thyroid was thought to be a kind of
support for the throat, a cosmetic device to make it more shapely. In fact it is a gland that makes and secretes a hormone called thyroxine. The thyroid needs iodine to make this hormone, and should iodine become scarce, the thyroid attempts to restore order by the rather drastic device of growing larger. The result is at first a swollen neck, then a bulging neck, and finally, in elderly people who have lacked iodine all their lives, an enormous bag of tissue that spreads from beneath the chin onto the chest, and that contains vast numbers of thyroid tissue nodules, some of which are multiplying, others of which are dying, yet others of which are altogether spent. In England this is called ‘Derbyshire neck’.

A goitre is an ugly but useful thing to have, particularly for a pregnant woman. Thyroxine is yet
another
hormone, albeit not a protein, that promotes cell proliferation in the bones of foetuses and growing children. It also controls the number of cells that migrate down the growth plate to swell and die before forming bone. A foetus gets the thyroxine it needs from its mother; should it not get enough it is born cretinous. Lack of dietary iodine during childhood can also cause cretinism. And cretinism can also be, albeit rarely, a genetic disease. Many human mutations are known that disrupt the production of thyroxine, its storage, its transport around the body, or its ability to dock to its receptor.

There is also a class of mutations more vicious by far than those that simply cause thyroid malfunction. These mutations affect the pituitary. Among the hormones that the pituitary produces is one that controls the thyroid. This hormone, thyrotropin, regulates the way that the thyroid absorbs iodine, the
rate at which it manufactures thyroid hormone, and the way it grows and shrinks according to need. The pituitary is the thyroid’s check and its balance. Goitre is a witness to its workings. The pituitary monitors the level of thyroid hormone that circulates around the body and, should it perceive a want, begins producing thyrotropin, which then spurs the thyroid to greater efforts – in the extreme, spurs it to make a goitre. Children who have defective pituitaries are dwarfed for want of growth hormone and cretinous for want of thyroxine.

But the vast majority of the world’s cases of cretinism are caused by a simple lack of dietary iodine. The tragedy of six million cretins is that the cure and the prevention of the disease is known, and costs next to nothing: it is simply iodised salt. It was the legislated spread of iodised salt in the early twentieth century that eliminated European goitre and cretinism within a generation, so that today these diseases are little more than folk-memories. Indeed, iodine deficiencies are so utterly forgotten in the developed world that outside medical and scientific circles the term ‘cretin’ exists only as a casual term of abuse. What is more, ‘cretin’ survives where comparable epithets have been justly banished from decent conversation. The word simply has no constituency, no defenders. Are the Taron of Upper Burma cretins? Is their smallness part of the vast and glorious tapestry of human genetic diversity, or are they merely victims of a peculiar form of high-altitude poverty? Were we to hear that there are no longer tribes of little people in the vertiginous gorges of the upper Irrawaddy, should we cheer or lament?

IL COLTELLO

Nearly fifteen hundred years ago, while working on a remote Aegean island, Aristotle made an observation that was at once banal, beautiful and chilling. ‘All animals,’ he wrote, ‘if operated on when they are young, become bigger and better looking than their unmutilated fellows; if they be mutilated when full grown, they do not take on any increase of size…As a general rule, mutilated animals grow to a greater length than the unmutilated.’

By ‘mutilation’ Aristotle meant castration. Hence the banality of his observation that merely repeated facts as well known to any fourth-century Greek farmer as to any modern one. What makes the observation beautiful is that Aristotle thought to write it down. He has taken a barnyard commonplace, that gelded rams, stallions and cockerels are larger than intact animals, and made a scientific generalisation of it – one, moreover, that still stands. What makes these facts so chilling is that when he spoke of animals, Aristotle also meant men.

Boys who are castrated before puberty grow up to be tall, unusually so. It is a fact that is largely lost to us now, but that would have been everyday knowledge in fourth-century Athens, a city pullulating with slaves culled from all corners of the Mediterranean, among them many eunuchs. It would also have been known to any fashionable eighteenth-century Italian. The monarchs of the great opera theatres such as La Scala were not, as now, the tenors, but rather the castrati. Feted for the range, power and unearthly quality of their voices, some castrati became rich, famous and influential. Farinelli sang for Phillip V of Spain
and was given the title
Caballero
; Cafarelli became a duke and built a palazzo in Naples; Domenico Mustapha became a papal knight and Perpetual Director of the Pontifical Choir. Rossini, Monteverdi, Handel, Gluck, Mozart and Meyerbeer all wrote for them. When they sang, audiences cried ‘
Eviva il coltello!
’ – ‘Long live the knife!’– and swooned in the stalls.

C
ASTRATO
. S
ENESINO SINGING
H
ANDEL
’
S
F
LAVIO
, L
ONDON C
.1723.
ATTRIB
. W
ILLIAM
H
OGARTH
.

The Italian castrati seem never to have been measured, so we do not know exactly how tall they were. But a wealth of anecdotes and images suggests that they were taller than their contemporaries, and somewhat oddly shaped. An engraving attributed to Hogarth shows a castrato performing a piece by Handel. Mouth ajar in soaring
bel canto
, ungainly limbs akimbo, he towers above his audience. It is a caricature, and a cruel one; all the more so as the castrati suffered from much more than
physical inelegance. Beyond the direct consequences of the invariably brutal surgery and the bar to marriage and fatherhood, old age frequently brought severe kyphosis, the broken-back posture that is symptomatic of osteoporosis, otherwise mostly a disease of elderly women. Many castrati also developed large and pendulous breasts. True, they never went bald, and never got prostate cancer, but these were small compensations. In eighteenth-century Italy, some four thousand boys per year lost their testicles for the sake of their golden voices. Few can ever have found the rewards that might have justified the sacrifice.

Why were the castrati so tall? Italian castrati fell from fashion and were banned by Pope Pius X in 1920; the last Vatican castrato, Alessandro Moreschi, died in 1922. But elsewhere hundreds, if not thousands, of men who had been castrated as boys survived well into the twentieth century. These were the court eunuchs, and there were many of them. At its demise, the Chinese Imperial family, last of the Qings, employed upwards of two thousand eunuchs in the Forbidden City at Peking. The last Chinese court eunuch, Sun Yaoting, was buried only in 1996 – along with his testicles, which had been carefully preserved in a jar. About two hundred eunuchs lived at the Topkapi palace in Istanbul until 1924, when the Sultan whom they had served was sent into exile, and many more must have been scattered about the vast territories once controlled by the Sublime Porte. In the 1920s some of these Istanbul eunuchs were carefully examined by a group of German physicians. What they found was distinctly odd. These elderly men, the last in a chain of eunuchs
who had successively served Roman, Byzantine and Ottoman masters, had the bones of adolescents.