The Elephant Man Disease - Joseph Merrick's Story
by Dr.Sal MD on
JOSEPH MERRICK, known by his stage name "The Elephant Man", is one of the most famous patients in history, not just on account of his exaggerated appearance while he was alive, but also because of the division of opinion amongst the medical establishment after his death over just what disease he had suffered from.
Joseph Merrick was born on August 5th 1862 - a year after the start of American Civil War - in Leicestershire, Great Britain. His mother is recorded as slightly crippled but his brother was normal. Joseph Merrick's development was apparently normal to age two when small growths began to be noticed on his face - the first harbingers of a frightful disorder which would consume his body and transform him into the Elephant Man.
By age seventeen Joseph Merrick had joined the British labor force working at a menial capacity at a workhouse which he left at twenty-one for greater pecuniary opportunity with the freak shows of 1883. It was here that show promoters styled him as the "Elephant Man" and created fantastic stories of his origins to attract public viewing attendance. Three years later in 1886 Joseph Merrick was discovered by a doctor, Sir Frederick Treves, and through his agency gave up life as a sideshow.
Joseph Merrick was admitted by Dr.Treves to the Royal London Hospital where he took residence. He died at twenty-seven during sleep either by dislocation of his cervical spine from the weight of his head, or from obstructive sleep apnea - he was known to sleep sitting upright to avoid suffocation.
Mr. Merrick's head measured a whopping 36 inches - roughly one-third larger than an average man's and a great deal additional weight for his neck to support, possibly interfering in his balance and ability to sit or stand for periods without pain and desire to rest his strap muscles. The Elephant Man's deformity favored the right side of Joseph's body e.g. enlarging his right hand to a flipper like state. We do not have access to audio recordings of him speaking but it is reasonable from looking at photographs to believe that he must have spoken with a severe dysarthritic splutter.
Internal structures of Mr. Merrick's - The Elephant Man - were no less spared. Toward the end of his life severe arthritis forced him to limp and use a walking stick. In addition, the scoliosis (curvature) of his spine revealed in surviving photographs probably would have reduced his lung capacity predisposing him to shortness of breath and chest infections (aspiration pneumonia being another possible contender for his cause of death).
The psychological cost to Mr. Joseph Merrick, taunted as The Elephant Man, are not hard to imagine in a society less educated or tolerant than today. Study of his surviving writings prove him to have been of at least normal intelligence and able to appreciate the horror of his condition.
Joseph penned this refrain: "Tis true my form is something odd, But blaming me is blaming God; Could I create myself anew I would not fail in pleasing you. If I could reach from pole to pole Or grasp the ocean with a span, I would be measured by the soul; The mind's the standard of the man."
There is a great divide amongst The Elephant Man medical investigators as to just what disease Joseph Merrick had actually suffered from. An early theory was that Joseph Merrick had elephantiasis - a disease of blocked sewage vessels in the body - lymphatics - that leads to tissue swelling, but this is not currently in favor. Neurofibromatosis had been a very strong contender for a number of decades until 1976 when a very rare condition called Proteus syndrome was put forward. Proteus is so rare that less than one hundred cases to date have ever been documented, but it agrees with the 'fossil' evidence left by Mr. Joseph Merrick. It describes overgrowth of soft tissues and bone, sometimes only on one side of the body (hemihypertrophy). Classical neurofibromatosis on the other hand, is a tumorous growth of nerve Schwann sheaths (insulated covering over nerves like the plastic wrapping around copper wiring), and does not readily lend itself to explain The Elephant Man's Bones.
The Royal London Hospital which used to keep Mr. Joseph Merrick's bones had allowed medical sleuths in the past to take casts, photographs, caliper measurements, and X-rays of The Elephant Man's Bones. We may never conclusively know what he suffered from since efforts in years past to preserve his bones for us, may have irreparably damaged DNA that forensic scientists could have used for incontrovertible proof. Indeed, some claim that perhaps Joseph Merrick had a one-of-a-kind unique disease... 'Merrick's Disease'.
I had read years ago in a gossip rag that pop singer Michael Jackson wanted to buy The Elephant Man's bones. But I think this unlikely as I visited the Royal Hospital where Joseph spent his last days and asked to see them. I was told by staff there that his remains had been buried for some time.
For me Joseph's mystery is part of an even larger dialogue, not the "what?" was wrong with Joseph Merrick, but the existential "why?". Why does a fair-minded, omnipotent deity permit such unwarranted misery on one unwitting person transforming them into the Elephant Man. Fittingly Charles Darwin's published 'On the Origin of Species' in 1859, three years before Joseph was born, a work that seems to better explain his affliction and our own personal struggles to our sensibilities as the work of random chance.