by Joyce Graff
Medical dramas can play powerful roles in increasing awareness of rare diseases, but it’s critical that the diseases are portrayed accurately. Mischaracterizations by the popular media of medical conditions, particularly rare ones, can be very demeaning to people afflicted with those conditions, and can affect how people look at them in the workplace or at school.
The TV show, Grey’s Anatomy recently broadcast the first of three episodes portraying a patient with von Hippel-Lindau (VHL) who has been diagnosed with a “dangerous adrenal tumor,” a pheochromocytoma, or “pheo.” So far, the script is looking good. Several other medical shows have included VHL in story lines, but their depiction of VHL has in most cases been quite a stretch. As an activist for VHL research, treatment and support, I worry that inaccurate fictional depictions of VHL and other rare diseases will cause people embarrassment or distress.
In real life, just getting a diagnosis of a pheo is extremely difficult. Most doctors were told in medical school that pheos were so rare that they will never see one. As a result, physicians usually forget to think of them. Pheos are in fact not that rare. VHL is one of five known risk factors for pheos, all of which account for only one-third of all pheos — the rest occur in the general population, with no family history to warn them. It is estimated that more than half of pheos are diagnosed on autopsy if at all.
We need to raise the visibility of pheos among doctors as well as among patients and families so that they will at least consider it and work through the diagnostics to rule it out. But the ultra-dramatic stories don’t help, because most people don’t experience the dramatic episodes depicted. The show, House, MD, depicted a patient with a pheo who had murdered people during rages. Most people with pheos don’t experience violent outbursts, they are more likely to have panic attacks, splitting headaches, heavy sweating, and cardio-vascular irregularities caused by the over-working of the heart by the pheo. How would you feel after such a show if you knew a colleague at work had VHL?
So far there have been four scripts involving VHL, three of which have had a pheo. In real life, only about 20% of people with VHL will have a pheo. The most common aspects of VHL are tumors of the eye, brain, spinal cord, and kidney. Yet one mythical patient on a medical show had a pheo in her lung, lung cysts, and cysts on the liver, all of which are very rare in VHL. People with VHL called me to ask “What kind of VHL is this?”
All case studies show only one patient’s experience, and an episode of a medical show is one time-slice of a case study. Nonetheless, the depiction of the condition should have more basis in reality.
We are very aware of the power of the media in raising awareness of rare diseases. It was two episodes of the medical show, Quincy, that spurred adoption of the Orphan Disease Act in 1983, which has increased the development of drugs for rare diseases. That power is best utilized when the depictions of rare diseases bear relationship to reality. They can empower people with these rare diseases to get an accurate diagnosis, to find the best possible options for care, and most of all, to find hope.
Joyce Graff is Executive Director of the VHL Family Alliance, the only international organization focused on von Hippel-Lindau disease.
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