When Art Imitates Medicine

One day, during my first year of residency, my son came home from school and proudly showed me his homework: a review of The Elephant Man – David Lynch’s biographical black-and-white film from 1980 starring Anthony Hopkins and John Hurt.

Most of you will know that the movie is based on the true story of Joseph Merrick (born August 5, 1862) – an English boy with severe deformities who became famous for his role in a traveling circus. A kindhearted surgeon, Frederick Treves, took him in at the famous Royal London Hospital and visited him every day; the two became close friends until Merrick’s unexpected death at the age of 27. An autopsy performed by Treves concluded that Merrick’s death was caused by his large, heavy head dislocating his cervical spine during sleep.

After reading my son’s review of this astonishing story, I immediately bought the DVD and watched the movie. I was deeply touched – and not surprised that Lynch’s film, a critical and commercial success in its time, had earned eight Academy Award nominations. (As a side note, after receiving widespread criticism for failing to honor the film’s makeup effects, the Academy was prompted to create an award for Best Makeup the following year).

With the film fresh in my mind, I felt compelled to search for literature regarding this fascinating case. Among other things, I discovered that, in 1986, J.A. Tibbles and M.M. Cohen, Jr. (1)  hypothesized that Merrick suffered from Proteus syndrome – a rare disorder characterized by the overgrowth of tissues (2).

Then, one day when I was on frozen section service, a breast specimen arrived from the operating room. The pathologists’ assistant who received it did not understand something on the requisition form and called for help. I looked for myself and saw “Proteus syndrome” written on the form – the same condition from which Merrick may have suffered. Caught up in a whirlwind of chance, I processed it immediately. I saw that the breast section presented multiple cystic changes and no concrete or solid nodules. The intraoperative diagnosis was just “atypical proliferation of mammary glands.” Later, under the microscope, this 28-year-old patient was found to have multiple foci of papillary duct carcinoma in situ on one side of her breast (with the sample sent to Paul P. Rosen, an authority on breast pathology at Cornell University, for a second opinion). In the literature, I found about 30 different benign and malignant neoplasms reported in around 200 cases from 200 families identified worldwide. This new specimen was the first reported breast neoplasm in a young woman with Proteus syndrome, a discovery that prompted me to present the case at the sixth International Conference on Breast Pathology.

If you pay attention when studying and practicing medicine, you’ll find that there are often echoes and clues to be found in the humanities, literature, and sociology. You may find that you can learn not only from classes and textbooks, but also from people and things you might never expect. After all, I learned about the pathology of Proteus syndrome – a rare and unique disease – not from a textbook, but from my son’s movie review! In life and work, knowledge and stories are everywhere – you just need to keep your eyes open.

Joseph Merrick’s skeleton remains on display at the Royal London Hospital long after his autopsy and the treatment of his bones. You may not know, though, that the late “King of Pop,” Michael Jackson, offered to pay US$1 million for this piece of medical history in 1987 – a deal rejected by the hospital museum on ethical grounds despite the desperate need for research funds (3).

While writing this article, I watched the movie a second time and again found myself with tears in my eyes – not unlike the pivotal scene in which Hopkins’ Frederick Treves first meets Merrick. I highly recommend this movie to all my pathological residents, fellows, and colleagues.

^{_{Credit: The Elephant Man (1980), directed by David Lynch. Cinematography by Freddie Francis.}}