The Hermansky-Pudlak Syndrome Network (HPS Network) is supporting the Pulmonary Fibrosis Research Enhancement Act, re-introduced this Congressional session in the Senate by Senators Chris Coons (D-DE) and Mike Crapo (R-ID). In the U.S. House of Representatives, Rep. Erik Paulsen (R-MN) and Rep. Tammy Baldwin (D-WI) have introduced the bill. (The bill numbers are S.1350 and H.R. 2505)
The legislation calls for a national registry of pulmonary fibrosis patients to assist research. As Hermansky-Pudlak Syndrome causes pulmonary fibrosis 100 percent of the time in types 1 and 4, it is the Network’s hope that those with HPS will be included in these efforts.
The bill also calls on the National Institutes of Health (NIH) and the Centers for Disease Control (CDC) to intensify and expand efforts to research pulmonary fibrosis.
The legislation also calls for the creation of a national pulmonary fibrosis education and awareness plan which would strive to improve public awareness of pulmonary fibrosis and accelerate patient and medical education strategies about pulmonary fibrosis. This is another key area in which the HPS Network is keen to participate. “It is relatively simple and inexpensive to look for albinism in pulmonary fibrosis patients. You can look for nystagmus in the eyes, and if you see it, test further for HPS,” says Heather Kirkwood, Vice President of the HPS Network, “Identifying pulmonary fibrosis patients who may have HPS could save or prolong lives.”
HPS is a rare form of albinism that causes a bleeding disorder, as well as a Crohn’s-like digestive problem and pulmonary fibrosis in some gene types. “We’ve had a number of HPS lung transplants, but it’s important for physicians to be aware of the bleeding aspects of HPS so they can be treated. Open lung biopsies to diagnose pulmonary fibrosis should also be avoided in patients with HPS.”
Other groups in the pulmonary fibrosis community have championed this legislation since 2007 including the Coalition for Pulmonary Fibrosis and the Pulmonary Fibrosis Foundation.
The HPS Network hopes that by raising the profile of pulmonary fibrosis research, the entire pulmonary fibrosis community, including those with HPS, will benefit from better diagnosis, better treatments, and someday, a cure.