In
the 19th century, Joseph Merrick was exhibited in a freak show. In
1979, his story became a Broadway play, and then a movie in 1980. Now
it’s going back to Broadway, with Bradley Cooper in the title role.
Merrick was known as “The Elephant Man” because of his multiple
deformities, but underneath he just wanted to be treated like anyone
else. He died in 1890, and scientists have tried ever since to pinpoint
what caused his disabilities.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.
Russell Saunders gives us the short version of
Merrick’s story at The Daily Beast. You can also read an
extensive biography at Wikipedia.
In
the 19th century, Joseph Merrick was exhibited in a freak show. In
1979, his story became a Broadway play, and then a movie in 1980. Now
it’s going back to Broadway, with Bradley Cooper in the title role.
Merrick was known as “The Elephant Man” because of his multiple
deformities, but underneath he just wanted to be treated like anyone
else. He died in 1890, and scientists have tried ever since to pinpoint
what caused his disabilities.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.
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