Pulmonary Fibrosis and the End of an Era for Jerry Lewis & the Muscular Dystrophy Association

MedicalToday
Labor Day may signify the "end of the summer", but this year's Labor Day will bring the end to an era which has lasted since 1966. For the first time since then, Jerry Lewis will not be hosting the perennial Muscular Dystrophy Telethon.  In a , the Muscular Dystrophy Association (MDA) announced:
"Jerry Lewis has completed his run as its national chairman. In May, Lewis and MDA announced his retirement as host of the MDA Labor Day Telethon."
MDA Chairman of the Board of Directors R. Rodney Howell, M.D., said, “Jerry Lewis is a world-class humanitarian and we’re forever grateful to him for his more than half century of generous service to MDA. We will not be replacing him as MDA national chairman, and he will not be appearing on the Telethon.”

Since its national debut in 1966, Jerry Lewis’ Labor Day MDA telethon has raised over $2 billion in the battle against muscular dystrophy.

Although no reason has been given for Lewis' departure, it is no secret that the 85-year-old comedian has been in poor health for several years. Aside from the years of severe back pain caused by a comedic pratfall in 1965, Lewis has battled prostate cancer, diabetes, and pulmonary fibrosis, and has had at least two heart attacks. Lewis was diagnosed with pulmonary fibrosis in May 2001 when he began to complain of shortness of breath. He has been treated with prednisone, which caused him to gain over 50 lbs. but has slowed the progression of his disease. A number of other celebrities have also suffered from pulmonary fibrosis, including actors Marlon Brando, James Doohan (“Scotty” from Star Trek), Gordon Jump (station manager "Arthur Carlson" in WRKP in Cincinnati), and singers Robert Goulet and Odetta.

On July 12, 2011, Senator Christopher Coons (D-Del.) introduced , the Pulmonary Fibrosis Research Enhancement Act. The measure would expand research, prevention, and awareness activities of the CDC and the NIH with respect to pulmonary fibrosis.

Part of the bill will establish the National Pulmonary Advisory Board, the membership of which would include a representative from the NIH and its National Institute of Environmental Health Sciences, among other agency and department representatives. This board would be responsible for making recommendations concerning the development of a National Pulmonary Fibrosis (PF) Registry.

Research regarding idiopathic pulmonary fibrosis (IPF) (was recently covered in a MedPageToday story. Joyce Lee, MD, and her colleagues from the University of California San Francisco published an online article in the . Their paper offered further evidence that pulmonary fibrosis may be related to gastroesophageal reflux, as patients with IPF who are treated for GERD had prolonged survival compared to those to did not take those medications. According to the study, patients who used GERD medications had a median survival time of about 65 months, compared with about 29 months for those who did not use the drugs (P<0.01) and a 53% reduced relative risk of death.

The authors hypothesize that:
"... gastroesophageal reflux may impact progression in idiopathic pulmonary fibrosis through microaspiration of gastric droplets causing either slowly progressive lung injury and fibrosis or by triggering acute exacerbation of [the lung disease] ... Suppressing the acidity of gastric contents may reduce the injury caused by microaspiration."
Further studies will be necessary to test this hypothesis.

 Did you know?
  1. That there are currently on pulmonary fibrosis, testing everything from thalidomide to inhaled carbon monoxide in treating PF and/or its symptoms?
  2. That the U.S. National Institutes of Health (NIH) spends annually for research on lung diseases (excluding lung cancer)?
Would you consider referring your patients to one of these clinical trials or involving them in other NIH research projects?