Proteus Syndrome

Proteus syndrome is a rare congenital disorder that causes abnormal growth of skin, head and bones. There is partial gigantism of the limbs, skin overgrowth and tumours all over the body. It causes severe disfigurement with extreme social stigma. Mutiple organ systems of the body is affected. It is named after the greek God Proteus who could change his shape.

It was identified by Dr. Michael Cohen in 1979. Since then about 200 cases of proteus syndrome have been reported. Some minor forms of the disease have been identified and some remain undiagnosed.

One of the notable cases of proteus syndrome is that of Joseph Merrick, an Englishman who was known as "The Elephant Man" because of his physical appearance. Earlier, it was thought that he had aconditon known as neurofibromatosis, but it has now been confirmed that he had proteus syndrome.

Joseph Carey Merrick was an Englishman, who was known as "The Elephant Man" because of his severe disfigurement caused by proteus syndome. Because of his condition, he had the sympathy of Victorian era Britain.

Biography

Joseph Merrick was born in 1862 to Mary Jane Potterton and Joseph Rockley Merrick. He was the oldest. His deformity started appearing at the age of three. His mother died when he was 12. It is believed that his mother too had certain physical deformities. His father remarried and his stepmother did not care about little Joseph. He was forced to sell goods in the market. But he was unsuccessful at this and had to face a lot of harassment from the local kids. Fed up with his life, he left home.

Merrick was unemployed for the most of his life. On August 29, 1884, he became a sideshow performer where he earned some money. Here he met with physician Frederick Treves, who gave Merrick his business card, in case he was willing to undergo a physical examination. But in 1886, sideshows were banned in the United Kingdom and Merrick went to Belgium to find some work. But he was treated very badly and in the end the showman abandoned him after stealing his lifetime savings of £50 (worth approximately £3,900 in 2007 currency).

Merrick returned to London where the authorities found him in possession of the buisness card of Frederick Treves, and was taken to the London hospital where he worked. Merrick was given permanent residence in the London Hospital.

He captured the sympathy of the Victorian high society. Alexandra, then Princess of Wales and later Queen Consort, became interested in him. Merrick always wanted to go to the hospital for the blind and find a woman who would love him, and not be turned away due to his physical appearance. In his final years, he found pleasure in writing and visiting the countryside.

Merrick at the London hospital until his death at the age of 27 on April 11, 1890. He died from an accidental dislocation of his neck while sleeping, due to the inability to support his massive head. There is a small museum in the Royal London Hospital focused on his life.