New Hope For Pulmonary Hypertension Patients. New bill would allow for up to $25,000,000 per year (2004-2008) for research and awareness.

This important legislation, introduced by Congressman Kevin Brady (R-TX), would authorize increased funding for pulmonary hypertension research and public awareness activities at the National Institutes of Health. H.R. 1316

Pulmonary hypertension is a rare disorder of the lung in which the pressure in the pulmonary arteries (the blood vessels in the lungs) rises above normal levels and may become life threatening. Symptoms of pulmonary hypertension include shortness of breath with minimal exertion, fatigue, chest pain, dizzy spells and fainting.

Recently, NIH supported researchers made a landmark discovery in the fight against PH. Two separate groups of scientists simultaneously identified a genetic mutation associated with the disease. This historic breakthrough opens up exciting new pathways of research focused on the development of improved therapies for PH patients. Consequently, it is critical that we increase our commitment to PH research at NIH as called for in H.R. 1316.

The Congress finds as follows:

(1) In order to take full advantage of the tremendous potential for finding a cure or effective treatment, the Federal investment in pulmonary hypertension must be expanded, and coordination among the national research institutes of the National Institutes of Health must be strengthened.

(2) Primary, or unexplained, pulmonary hypertension (`PPH') is a rare lung disorder which occurs for no apparent reason. It has been historically chronic and incurable with a poor survival rate.

(3) In the United States it has been estimated that 300 new cases of PPH are diagnosed each year, or about two persons per million population per year; the greatest number are reported in women between the ages of 21 and 40. While at one time the disease was thought to occur among young women almost exclusively; we now know, however, that men and women in all age ranges, from very young children to elderly people, can develop PPH. It also affects people of all racial and ethnic origins equally.

(4) The low prevalence of PPH makes learning more about the disease extremely difficult. Studies of PPH also have been difficult because a good animal model of the disease has not been available.

(5) In about 6 to 10 percent of cases, PPH is familial.

(6) In the more advanced stages of PPH, the patient is able to perform only minimal activity and has symptoms even when resting. The disease may worsen to the point where the patient is completely bedridden.

(7) PPH remains a diagnosis of exclusion and is rarely picked up in a routine medical examination. Even in its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs.

(8) In 1981, the National Heart, Lung, and Blood Institute established the first PPH-patient registry in the world. The registry followed 194 people with PPH over a period of at least 1 year and, in some cases, for as long as 7.5 years. Much of what we know about the illness today stems from this study.

(9) Because we still do not understand the cause or have a cure for PPH, basic research studies are focusing on the possible involvement of immunologic and genetic factors in the cause and progression of PPH, looking at agents that cause narrowing of the pulmonary blood vessels, and identifying factors that cause growth of smooth muscle and formation of scar tissue in the vessel walls.

(10) During the period January 1996 through December 1997 almost 6,000,000 Americans took anorexic drugs, which can cause PPH in some people. Thousands now have PPH and are in terminal stages or have already succumbed to the disease. It is anticipated that many more cases of PPH from diet drugs will be diagnosed within the coming years.

(11) Secondary pulmonary hypertension (`SPH') means the cause is known. Common causes of SPH are the breathing disorders emphysema and bronchitis. Other less frequent causes are the inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome or systemic lupus erythematosus (`SLE'). Other causes include congenital heart diseases that cause shunting of extra blood through the lungs like ventricular and atrial septal defects, chronic pulmonary thromboembolism, HIV infection, liver disease and certain diet drugs.

Please call your local house represenitive and ask him/her to co-sponsor H.R. 1316.