by Peter Ford
Time did nothing to fade the glow of Joseph Merrick’s bewitchment during the weeks that followed. He would talk of the pantomime continually, and relive each ephemeral moment. As with the faculty of make-believe in childhood, so, said Treves, every aspect of the show was real to him.
… the palace was the home of kings, the princess was of royal blood, the fairies were as undoubted as the children in the street, while the dishes at the banquet were of unquestionable gold. He did not like to discuss it as a play, but rather as a vision of some actual world.
The life of the pantomime story thus for Joseph went on living, even though he was no longer there to witness it. ‘I wonder what the prince did after we left?’ he would ask Treves among a host of questions. Or, ‘Do you think that poor man is still in the dungeon?’
There may well have been other visits to the theatre for Joseph, though no confirmation exists for those beyond an incidental remark contained in a later letter to The Times that Carr Gomm wrote after Joseph’s death. But it seems unlikely that anything he saw subsequently could have matched the intensity of his first experience of living theatre.
CHAPTER 10
What Was the Matter with Joseph Merrick?
The question of the diagnosis of Joseph Merrick’s puzzling condition was last left in the hands of the dermatologist Dr Henry Radcliffe Crocker (see Chapter Three), when, in 1885, he made his tentative yet positive suggestions to the meeting of the Pathological Society of London. Joseph was probably never aware of the fact that, three years further on, in 1888, he came to rest in a firm niche in medical history with the publication of Radcliffe Crocker’s magnum opus, a two-volume work entitled Diseases of the Skin: a Review of 15,000 Cases of Skin Disorder. It was destined to be a classic among medical textbooks, and the second volume contained a section on the rare group of skin diseases known as the fibromas. It was here that he mentioned the Elephant Man.
An extraordinary case of this kind was brought to the Pathological Society by Treves. I had an opportunity of examining the patient there, and at a show where he was exhibited as an ‘elephant man’.
Like Tuckett, Bland-Sutton and Treves, Crocker had therefore made his pilgrimage to the freakshop in the Whitechapel Road. His technical description continued:
The bulk of the disease was on the right side; there was enormous hypertrophy of the skin of the whole right arm, measuring twelve inches round the wrist and five inches round one of the fingers, a lax mass of pendulous skin, etc., depending from the right pectoral region. The right side of the face was enormously thickened, and in addition there were huge unsymmetrical exostoses on the forehead and the occiput. There were also tumours affecting the right side of the gums and palate; on both legs, but chiefly the right, and over nearly the whole of the back and buttocks; the skin was immensely thickened, with irregular lobulated masses of confluent tumours, presenting the ordinary molluscous characters. The left arm and hand were small and well formed. The man was twenty-five years old, of stunted growth, and had a right talipes equinus, but was fairly intelligent. The disease was not perceived at birth, but began to develop when five years old, and had gradually increased since; it was, of course, ascribed to maternal fright during pregnancy.
Before saying what was the matter with Joseph Merrick, it is easier to say what it most positively was not. To begin with it was not the clinical condition usually labelled elephantiasis. The show name of ‘The Elephant Man’ that his managers chose has sometimes had the unfortunate effect of leading writers into a misdiagnosis and assuming elephantiasis was responsible. It is therefore the disease cited for Joseph Merrick in several apparently authoritative sources, but elephantiasis is a complaint caused by a parasitic hair-like worm that invades the body’s lymphatic channels. It occurs only in tropical or sub-tropical regions, is transmitted to man through mosquito bites, and has entirely different features from those characteristic of Joseph’s example.
Joseph himself was never in any doubt over the cause of his great misfortune. His distorted body must, he considered, be attributed directly to the frightening experience his mother suffered when making her way through the crowded streets at the time of Leicester’s Humberstonegate Fair. As Bergen Evans comments in The Natural History of Nonsense:
Once conception has been accomplished a further set of delusions obtain. Chief of these is the belief that certain impressions made on the mother during her pregnancy will affect the child. Of late years it has been held that pleasant impressions will have a beneficial effect and that the expectant mother should therefore keep herself cheerful, listen to good music, and frequent art galleries.
Beliefs in the imprinting of maternal impressions on unborn children are ever-present in folklore, from earliest times and in all cultures. The ancient Greeks advised pregnant women to gaze on statues of Castor and Pollux and other objects of great beauty so their children might be born fair and graceful. Similarly motivated, pregnant ladies in nineteenth-century Paris spent many hours in the Louvre, perambulating gently through the galleries. But as Mr Evans indicates, seeking for benign effects is only one aspect. Traditionally the avoidance of undesirable or even malevolent consequences needed to be taken into account at every step by women made vulnerable by pregnancy. Dr Károly Viski records in Hungarian Peasant Customs:
The young woman must not hide a leaf in her bosom, because a similar mark will show on the child’s breast; fruit should not fall on her, and if it does, she must not make a sudden move to catch it, as that will result in the mark of that fruit on the child. It is unwise to throw meat in the direction of the woman, especially liver, because in that case the child will get freckles.
In East Anglian Folklore, Enid Porter records how the eating of strawberries was and is believed in that area of Britain to cause a strawberry-coloured birthmark on a baby’s body. In her Cambridgeshire Customs and Folklore, she cites the case of a Cambridge man born with his hands deformed, which his mother put down to a large, strange dog having leaped up shortly before his birth and pressed its paws to her stomach. Mentally retarded children born in East Anglia during the Second World War were often thought to be a consequence of mothers being frightened by bombing raids. In the Cambridgeshire fens, pregnant women traditionally tried to avoid the sight of a chimney sweep in case their babies were born black-skinned.
International folklore is rich in such material, and even in the most sophisticated urban environment the folklorist would not need to search far to find current examples. The conviction that a fear of spiders can be transmitted to an unborn child remains remarkably common, as does the belief that spilling a cup of tea or coffee over the mother’s stomach may cause birthmarks. That modern medical science and Bergen Evans define such notions as nonsense matters little to those who believe in them. They may even be helpful to some mothers in modifying the emotional guilt unfortunately often present where a blemished or malformed baby comes into the world. In any case, it is only relatively recently that medical science has rejected these beliefs. Before that, over the centuries since Hippocrates, maternal impression was an integral part of a physician’s advice to mothers-to-be.
In the sixteenth century a great French doctor of the Renaissance period, Ambroise Paré, listed maternal impression among the thirteen causes for the births of abnormal children. Two hundred years later in London, by contrast, William Hunter, who helped to raise obstetrics to a recognized branch of medicine, approached the belief more questioningly. He carefully cross-examined the expectant mothers who came under his care for details of emotional shocks suffered since conceiving. In no example did he come across a malformation or abnormality attributable to psychic injury. He did notice, though, that whenever a small foetal abnormality occurred, the mother would have little difficulty in searching her memory to find an incident to explain it.
But the rational William Hunter, in remaining sceptical of the theory of maternal impression, was well ahead of his time. The belief only lost ground during the nineteenth century a
s medicine began to find a more securely scientific and empirical base. It came to be pointed out that perfectly normal babies are often born to mothers who have been through appalling emotional crises; that, in any case, few mothers could hope to live through a gestation period of nine months without encountering some of the shocks normal to the business of living; and yet that most babies are born unmarked.
As research came to confirm, malformations of the foetus are generally present before the end of the third month in pregnancy while most events described by mothers happen during the later stages. Furthermore, no means could be discovered by which a nervous trauma might be transferred to the personality of the unborn child, for no nerve connections exist between mother and foetus, nor even an intermingling of bloodstreams.
More than at any other time in its history, the medical profession is aware today of the hazards that may damage a baby in the womb. It seems no more than common sense to advise an expectant mother to avoid certain drugs (including alcohol and tobacco) as well as serious emotional stress, and that any maternal infection should be referred to her doctor without delay. Thus, where medical science is concerned, the ancient theory of maternal impression, with its essentially magical linking of cause and effect, may safely be left to the attentions of folklorists.
At the time when Joseph Merrick was first examined, there were considerable problems of classification, and several factors made the situation even more perplexing. The first and most important source of confusion was the simple fact that the disorder from which Joseph has most generally been said to suffer was not then completely described or labelled. A second difficulty lay in its nature. It involved a condition capable of manifesting itself in many different ways, it being unlikely that any two cases, selected at random, would bear the slightest resemblance to one another in appearance or clinical history. A third factor lay in the extreme rarity of the disorder in its more striking forms.
From the vantage point of a century later, when we have a far wider if still incomplete understanding, the problem of diagnosis in the 1880s can be placed more precisely in context. It was rather as if Treves and his contemporaries were trying to comprehend the picture of a disease that had been broken up into a jigsaw puzzle, the pieces of which were widely scattered. No individual doctor was likely to find himself in possession of more than a couple of pieces of the jigsaw at any one time; he would be even less likely to know about other pieces of the puzzle, or to realize that the two or so pieces he held or knew about were related, let alone were fragments of a far larger as yet amorphous picture. That Treves, with the prompting of Radcliffe Crocker, succeeded in linking three major pieces of the puzzle and connecting the condition with the central nervous system was a credit to the high standard of British medicine in 1885. During most of the twentieth century, it has been broadly accepted that Joseph should be classified as a sufferer from neurofibromatosis, otherwise known as multiple neurofibromatosis or von Recklinghausen’s disease. This diagnosis became controversial during the 1980s, though it retains its importance in the history of the medical understanding of Joseph Merrick’s condition as new research leads into new areas.
Friedrich Daniel von Recklinghausen (1833–1910) was, as professor of pathology at the then newly founded University of Strasbourg, among Germany’s most distinguished pathologists. He was a keen observer of disease processes, and was the first to describe several different conditions with which his name is linked. In 1882, two years before Joseph was exhibited before the Pathological Society of London, von Recklinghausen gave the first description of a hitherto unrecognized disorder. It was characterized by the presence of types of tumour called neurofibromas that were linked with the nerve trunks as well as by soft, lumpy tumours of the skin and areas of cutaneous pigmentation. He noted no other abnormality and suggested no cause for its origin.
Whether Treves or Radcliffe Crocker had come across von Recklinghausen’s monograph at that stage of the story’s unfolding is impossible to say, but even if they had done, it seems unlikely they could have linked the Elephant Man with the cases being described in Strasbourg, the manifestations in Joseph’s example being so bizarre and gross. Indeed, it seems improbable that a diagnosis could have been confirmed even if Professor von Recklinghausen himself had been on hand to make a direct examination.
The name neurofibromatosis by which this disease is now known simply means that there is a tendency in patients who suffer from it towards the formation of one or more tumours of a particular type. The kind of tumour involved is known as a neurofibroma (one composed exclusively of a dense proliferation of nerve and fibrous tissue). Such tumours may occur singly or in great numbers within many different body tissues and can vary in size between that of a pin’s head and an orange. It is the extreme variability in site, size and number of tumours that accounts for the variations of symptoms in individual patients. Despite their random nature it is even so the tendency to form neurofibromas that remains the consistent abnormality to be found in every patient in whom the disorder is diagnosed.
Most commonly the neurofibromas form within the layers of skin, and even here their appearance may be highly variable. In the disorder’s mildest manifestations, there will be nothing to see but a degree of skin pigmentation, this taking the form of patches of skin colouring of a pale coffee shade. These may vary between mere dots and areas the width of a man’s hand. Occasionally the pigmentation may occur as diffuse shading on one or both flanks. It is unusual to find a case of the disease in which such cutaneous discolorations are not present in one form or another.
A more severe manifestation of neurofibromatosis in the skin comes about when the fibromas form themselves into soft swellings. These may or may not be pendulous and they can vary between tiny warty prominences and swellings as large as a clenched fist. They are often present in large numbers on the trunk, but also, not uncommonly, develop on the face. Treves described such apparent tumours in great profusion in the notes he made on Joseph Merrick’s disorder.
The most extreme skin changes of all occur when forms of diffuse tumour develop within the network of nerve fibres within the skin. These then become associated with a thickening of the skin and subcutaneous tissue, so that large folds of skin are formed; or there may be a diffuse enlargement of the subcutaneous tissues of a limb. Such a change as this most usually affects the flesh overlying the temple, cheek, upper lip or back of the neck.
It was dramatic illustrations of the disease of this kind that Dr Radcliffe Crocker and his mentor, Dr Tilbury Fox, were citing when they spoke of dermatolysis and pachydermatocoele (see earlier). In fact, this was the technical description that continued to be applied to Joseph Merrick’s case over many years, for it was not realized that such cases might be explained as extreme manifestations of the milder forms of the syndrome described by von Recklinghausen. By 1909 the link had been assumed. In that year Dr Parkes Weber, a physician to the German Hospital in London and something of a connoisseur of rare diseases, wrote in an article on von Recklinghausen’s disease for the British Journal of Dermatology: ‘The most famous example of the class was undoubtedly the famous Elephant Man whom many must have seen when he was at The London Hospital.’
In the fatty subcutaneous tissues of the bodies of the more sorely afflicted, the neurofibromas develop as firm nodules on the trunks of the peripheral nerves and may be felt as tender, bead-like swellings in the limbs and on the sides of the neck. Wherever nervous tissue occurs in the body, neurofibromas are capable of forming, and they commonly crop up on the roots of the great nerves and within the skull or the actual spinal canal.
On the face of it, it seems curious that solid structures like the bones should ever become involved. It is therefore not surprising if this connection was one of the later pieces of the jigsaw to fall into place. Only at about the turn of the century did associated bone changes come to be recognized, as when, in 1901, Jonathan Hutchinson presented the case of a woman who suffered from involvement and overgrowt
h of the frontal bone of the skull linked with bony tumours. And, in 1906, Dr Cooper reported similar findings in a young girl. Such skeletal distortions were usually found to underlie diffuse types of skin tumour apparently similar to those Joseph exhibited.
It was not until as late as 1930 that Dr Parkes Weber put forward the suggestion that the bone malformations occurred as a result of the involvement in neurofibroma formation of the periosteum (the fibrous membrane that shapes and forms the surface layers of a bone). Once again Joseph Merrick was cited, Dr Weber remarking in his paper:
The famous Elephant Man … whom I once saw, and who died at The London Hospital at the age of twenty-seven years on April 11th, 1890, had many deformities of nature of pachydermatocoeles as well as many bony thickenings and outgrowths … Irregular periosteal neurofibromatosis may well have played a part in his osseous deformities.
During the past sixty years, a number of other manifestations of neurofibromatosis have been recognized, and tumour manifestation has been described in practically every tissue and organ of the body. Changes have been found to take place in such varied sites as the intestines, internal glands, including the adrenals, the kidneys and the retina of the eye. It is quite possible that variants of the disease exist that are yet to be recognized. Meanwhile, varieties of neoplastic disorder have been categorized which bear close visual resemblances to the classic signs of neurofibromatosis but are clinically quite distinct.