On April 11, 1890, Joseph Carey Merrick was found dead in his bed at the age of 27. Though the exact cause is unclear, it is believed that he died as a result of the weight of his head, due either to spinal dislocation or asphyxiation from pressure on his airway. Measured by doctors at the time as having a circumference of 36 inches, Merrick’s malformed head was one of many deformities that people came to gape at as he toured England as a human oddity.
Billed at the time as “Half-a-Man and Half-an-Elephant,” Merrick’s life entered the American popular consciousness when “The Elephant Man” debuted on Broadway in 1979. A movie based on the play was released the next year, starring John Hurt in the title role as Merrick and Anthony Hopkins as Frederick Treves, the kindly London doctor who cared for him after gawking at “freaks” fell out favor in Victorian England. This coming week, a revival of the play goes into previews, with Bradley Cooper in the starring role.
Describing by biographers as an avid writer of letters, little of his correspondence appears available to public view. One remaining letter thanks a friend for sending some grouse and a book, the former described as “splendid.” He often concluded with a poem by hymnist Isaac Watts called “False Greatness,” which begins with the lines:
‘Tis true my form is something odd,
But blaming me is blaming God
In addition to his cranial deformity, Merrick also suffered from grayish skin growths, a curved spine, and a severely enlarged right arm and hand. Though DNA studies on his skeleton have been undertaken to ascertain the exact cause of these abnormalities, the work has been stymied by the repeated bleaching the bones have undergone.
Various diagnoses have been theorized, starting with Merrick’s own folk belief that the problems were due to his mother being frightened by an elephant when she was pregnant with him. While that cause may be safely discarded, the correct one is hard to pin down.
I recall being taught during my medical education that a likely diagnosis was neurofibromatosis type 1. A disease typified by tumors arising from nerve tissue, those with neurofibromatosis can develop skin lesions, spinal curvatures and large heads. During my pediatric residency I was involved in a very sad case of a severely affected young man, who had a large cranial mass that gave him an appearance similar to Merrick’s. That they shared a diagnosis seemed quite plausible to me.
However, the current thinking is that Merrick may have suffered from Proteus syndrome. Named after a shape-shifting Greek sea god, this very rare (fewer than 500 known cases) syndrome causes unchecked growth in some parts of affected individuals’ bodies, leaving other areas normal.
This growth is due to spontaneous mutations in a specific gene, and the severity of the disease depends on when the mutation occurs during embryonic development. Those with the disease have some cells that are genetically normal and some with the mutation. This discordant growth certainly sounds consistent with Merrick’s condition.
