Joseph Carey Merrick was born on the 5th August 1862 in Leicester, England. Whilst I have started this write-up with his original birth name, it was his unfortunate destiny in later years to become better known as the 'Elephant Man'. One has only to see a picture of him to realise why. His severe body and facial disfigurement was due to an extremely rare and sporadic genetic condition known as the Proteus syndrome. To date, there have been less than one hundred recorded cases of the disease worldwide in medical literature.
Proteus Syndrome
Proteus syndrome is named for the Greek god Proteus (the sea god who could assume different shapes), because of the variable manifestation of the disease. It was first described in modern literature in 1979 by Cohen and Hayden as part of the spectrum of harmartomatous diseases, and was given its current name in 1983 by Wiedemann and colleagues in a paper where they discussed the variable presentation of the disease in four young boys. Since the disease is so inconsistent in its effects, it is hard to say what the distinct signs of the disease are. However, most patients exhibit asymmetrically enlarged skulls, hands and feet; pigmented naevi over the body surface and subcutaneous tumours that can develop and grow rapidly.
It was originally thought that Joseph was instead affected by another disorder, neurofibromatosis, of which there are two types. Accounts differ as to whether he was a sufferer of NF1 (von Recklinghausen's disease) or NF2, but the diagnosis explained the strange swellings and tumours that were all over his body. However, this theory has now been mostly disproved by both a detailed study by the US National Institute for Health in 1987, and by x-ray and CT examination of Mr. Merrick's remains in 1996 (Kiernan, 1996).
Early Life
Joseph Carey Merrick, the son of Mary Jane and Joseph Merrick, came from a working class background and grew up with his mother, brother and sister in various sites around Leicester. He lost his sister to bronchopneumonia when he was eleven, and when his mother died in 1873 he went to live with his father and stepmother.
He started to exhibit the symptoms of his disease by his second birthday. By adolescence, he was so affected as to have become moderately disabled by the condition. His right arm became a functionless club, and he found walking, especially on the cobbled streets of Victorian England, to be difficult. He also had a large number of cauliflower-like growths develop over his head and body. Despite his disfigurement, Joseph managed to gain a valuable education from the Syston Street Board School. Here he learned to read and write, an admirable feat in the Victorian age considering that he came from a poor background.
As can be imagined, growing up with such an obvious deformity was not easy. His father did not care for him, and his stepmother found him an embarrassment and inconvenience. Whilst living with his father he was made to earn his keep by hawking shoe-black in the streets of Leicester, where he'd be followed by gangs of children who would taunt him mercilessly. Eventually, his stepmother gave his father the ultimatum of him or me
, and in 1879, Joseph went to live in the Leicester Union Workhouse.
Welcome to the Freak Show
His disability made him unemployable for the most part, but he willingly gave himself to any job that could earn him his keep. However, despite being literate and an eloquent speaker, opportunities were few and far between. In 1884, he finally took up employment working as a sideshow freak. His manager was Tom Norman, who was depicted in David Lynch's film 'The Elephant Man' as being a cruel man who mistreated and regularly beat Joseph. In truth, Tom Norman took great care of Joseph and made sure that he was well looked after. After all, Joseph was Norman's livelihood.
Joseph picked up the show name of the Elephant Man because his skin was thickened and hung in pendulous folds, and because he had a tusk-like growth on his face. One promoter enhanced this by dreaming up the story that his mother had been trampled by an elephant whilst pregnant with him, resulting in his deformity.
A Life-changing Encounter
Joseph eventually moved down from Leicester to London to continue his trade. There he met Mr. Frederick Treves (later Sir Frederick Treves), a surgeon at the London Hospital. Treves was greatly interested in Joseph's medical condition, and befriended him in order to find out more about his life. Joseph allowed Treves the honour of presenting his case to two medical societies, before continuing on his way. It is because of Treves that Joseph is often incorrectly refered to as being called John. In his memoirs, written in 1922, Treves calls Joseph 'John' throughout. It is not known as to whether this is a trick of his memory, or whether he did call him John as a nickname. There was never any other evidence that Joseph was ever called John by any others.
In 1885, sideshows became illegal in Britain, and so Joseph moved over the Channel to Belgium to continue earning his living. In 1886, he had an unfortunate incident with an Austrian showman, who robbed Joseph of all his savings and cast him out on the street. He made his way back to England, and was found by the police in Liverpool Street Station, London. He was destitute, starving, crumpled in a heap on the floor breathless and the subject of the taunts of both adults and children alike. Police found Treves's business card in his coat pocket, and he was taken to the London Hospital in Whitechapel to be placed under his care.
In those days, hospitals were unable to offer long term care to 'incurables' such as Joseph. However, this is precisely what Joseph needed; a permanent home where he could be given the care that he required. This was eventually arranged on the East Wing of the London Hospital, and was built with charitable donations from those who had heard about his case from Treves's presentations to the public.
Later Life
It was here that he lived out the rest of his days. However, while he had finally found a place where he was accepted and cared for, times were not easy. He was very secluded from the world as he was unable to leave the hospital grounds. This was mostly due to his disfigurement, but was also partly due to another aspect of the London Hospital's Victorian history - Jack the Ripper. In 1888, the serial killer was terrorising the streets of East London where the hospital is sited. Some of the public, knowing he had the appearance of a 'monster', came to the erroneous conclusion that he must be the killer. For a time, it would have been dangerous for him to leave the hospital for fear of being lynched.
He was able to receive guests though, and he entertained a variety of people who wished to visit him. He had trouble speaking clearly, and so often Treves would have to act as his interpreter. In spite of this, he was considered a polite and thoughtful host. Even with these visits, he lived a very isolated existence. He was experiencing more and more pain due to the advancement of his disease, and he was often to be found by his carers staring into space and tapping his pillow with his disabled right hand. In this time, he is famed for having built a model kit of a church that he received as a present from a correspondent in Germany. This achievement is to be greatly admired, taking into consideration that he only had the use of one hand.
He was always aware that his residence at the hospital could never be a long-term thing. There had already been the necessity of two large-scale, but successful, appeals by the hospital on his behalf to allow him to stay in his residence. It was assumed that he would not live long enough for the cost of his care to become a financial drain on the institution. However, this didn't stop Joseph worrying that he might be moved from his place of comfort, and of what might become of him then. He went so far as to request of Treves that if he was moved, he'd want to be placed in an asylum for the blind, where the other residents would be unable to stare at him.
He died on the 11th April 1890, aged 27 years. He was found lying dead on his back, as if asleep, with no signs of a struggle. It was thought that his death was caused by the weight of his head falling back and dislocating his neck. Joseph had often said to his carers that he wanted to be able to sleep like normal people do; he himself had to sleep upright with his knees drawn up and his head leaning forward to rest on them. Throughout all the written accounts of his death, there was never any mention of suicide. Treves did note that it was peculiar that he should have died in such a manner, since he knew how to deal with his large and cumbersome head. Maybe Joseph finally decided, despite the consequences, that he was going to lie down as other people do.
His skeleton is still property of the Royal London Hospital's (formally the London Hospital) Pathology Museum and is not accessible to the public. For those who are interested, there is a campaign being organised by Jeanette Sitton to allow Joseph's remains to be laid to rest. There is no evidence that he ever gave his consent to allow the Hospital to keep his remains, and so there is a more than valid ethical arguement that maybe it is time to remove his skeleton from display and give him a Christian burial. For those who would like to read more about this, Jeanette Sitton's website is here. I warn you now, it is very flowery and sentimental. I advise a sick bag to be on stand-by at all times whilst viewing.
References
- Cohen M M, Hayden P W, 1979,
A newly recognized hamartomatous syndrome
Birth Defects 1979; 15:291-6
- Joseph Carey Merrick Tribute Site
http://www.jsitton.pwp.blueyonder.co.uk/elephantman/elephant_man.htm
- Kiernan V, 1996,
Xrays expose secrets of the Elephant Man
, New Scientist 1996; 2059: 12
- Seward G R, 1990,
The Elephant Man: Part 1
, Br Dent J. 1990; 169(6):173-5
- Wiedemann H R, Burgio G R, Aldenhoff P, et al, 1983,
The Proteus syndrome.
Eur J Pediatr 1983; 140: 5-12