Who was the ‘Elephant Man’ Joseph Merrick, what is Proteus syndrome and what’s the debate over Charlie Heaton?
Here's the lowdown on what you need to know about Joseph Merrick, the Elephant Man
AS the life of 'Elephant Man' is set to be depicted in a new adaptation in 2019, a debate has erupted over the casting of an 'able-bodied' actor for the lead role.
Here's what you need to know about Joseph Merrick, Proteus syndrome and why the decision to cast Charlie Heaton has been criticised.
Who was Joseph Merrick and why was he called the 'Elephant Man'?
Joseph Merrick was born on August 5, 1862 in Leicester and developed severe face and body deformities throughout the first few years of his life.
His skin appeared to be thick and lumpy with lump protruding from his forehead, and he developed enlarged lips, an enlarged hand and enlarged feet.
At the age of nine his mother died and his father soon remarried, but after he left school at 13, he struggled to find work.
After being rejected by his father and stepmother, Merrick entered a workhouse when he was 17 where he remained for four years before contacting showman Sam Torr and proposed Torr exhibit him at a freak show in 1884.
Merrick became known as the 'Elephant Man' as a result of his appearance and was billed as 'Half-a-Man and Half-an-Elephant' on touring showcases across the East Midlands.
He then moved to London where he was exhibited at a shop on Whitechapel Road which was rented by showman Tom Norman.
The shop was located across the road from the London Hospital and visited by surgeon Frederick Treves who invited Merrick to be examined and photographed.
Soon after Merrick's visits to the hospital, the shop was closed by the police, and Merrick was sent on tour in Europe.
But in Belgium, Merrick was robbed by his road manager and abandoned in Brussels.
He made his way back to London, but unable to communicate, police found Dr Treves' card on him and he took Merrick back to the hospital with him, where Merrick would stay for the rest of his life.
Joseph Merrick died on April 11, 1890 aged 27 and although his official cause of death was asphyxia, Dr Treves - who performed the autopsy - said Merrick died of a dislocated neck.
He reportedly believed that Merrick - who slept upright because of the weight of his head - had been attempting to sleep lying down.
While the exact cause of his deformities has long been unclear, in 1986 it emerged he may have had Proteus syndrome, a very rare congenital disorder.
What is Proteus syndrome?
According to , Proteus syndrome is a rare condition which causes overgrowth of the bones, skin,organs and other tissues.
The condition results from a mutation in the AKT1 gene, which is not inherited from a parent, but arises randomly in one cell during the early stages of development before birth.
Newborns with Proteus syndrome have few or no signs of the condition, as the overgrowth only becomes apparent between the ages of six and 18 months and continues to worsen.
Experts say the condition can also cause a variety of skin growths, particularly a thick, raised, and deeply grooved lesion known as a cerebriform connective tissue nevus which occurs on the soles of the feet.
Some people with Proteus syndrome have neurological abnormalities, including intellectual disability, seizures, and vision loss.
Other potential complications of Proteus syndrome include an increased risk of developing benign tumours and clots which can lodge in the lungs and cause a life-threatening complication called a pulmonary embolism, a common cause of death in people with Proteus syndrome.
MOST READ IN NEWS
What's the debate over Charlie Heaton being cast as the Elephant Man?
Earlier this month, it was announced Stranger Things actor Charlie Heaton has been cast as Joseph Merrick in a two-part BBC1 drama adaptation of his life in The Elephant Man, set to air next year.
Previous versions of The Elephant Man were portrayed by Sir John Hurt, David Bowie and Bradley Cooper, but the new series is thought to focus on Merrick's younger years up until his death aged 27.
However, a disability charity has criticised the decision to cast able-bodie Heaton in the series, blasting it as "disappointing".
According to , Scope's head of communications Phil Talbot said: "It's disappointing that a disabled actor has not been cast in the remake of The Elephant Man, as it's one of the most recognisable films to portray a disabled character.
"Disabled actors still often face huge barriers to break in to the business, not only are the roles few and far between, but castings and locations are often not accessible... There is a massive pool of disabled talent being overlooked. The creative industries should be embracing and celebrating difference and diversity, not ignoring it."
Upon landing the role, Heaton said: "This is such a special role and a challenge for any actor. Joseph has such an incredible story and I can’t wait to bring him to life.”
We pay for your stories! Do you have a story for The Sun Online news team? Email us at [email protected] or call 0207 782 4368 . You can WhatsApp us on 07810 791 502. We pay for videos too. Click here to upload yours.