On December 8, the House Energy and Commerce Subcommittee on Health held a hearing to examine federal research efforts on pulmonary hypertension and chronic pain.
Chair Nathan Deal (R-GA) focused his opening remarks on the planned reorganization of the National Institutes of Health (NIH), stating that pulmonary hypertension, chronic pain, and other rare conditions “highlight the need for further advancements in scientific research.” He announced his intention to introduce legislation that would “overhaul” the NIH by expanding the NIH director’s authority to set research priorities.
Acting as ranking member, Rep. Frank Pallone (D-NJ) said that pulmonary hypertension is a “serious disease” with a poor prognosis often leading to heart failure. He expressed his support for the Pulmonary Hypertension Research Act (H.R. 3005), which would authorize $50 million annually through FY2010 to expand research activities on the disease at the NIH. The bill states that “in the United States it has been estimated that 300 new cases of PPH [primary pulmonary hypertension] 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.” Reps. Kevin Brady (R-TX) and Tom Lantos (D-CA) sponsored H.R. 3005 and participated in the hearing, although they are not members of the House Energy and Commerce Committee.
Dr. Mark Gladwin, Vascular Medicine Branch chief at the National Heart, Lung, and Blood Institute at the NIH, explained that pulmonary hypertension “is a disabling condition caused by a narrowing of the small arteries that carry blood through the lungs, resulting in damage to the heart. As the arteries tighten, the heart must work harder to pump blood through them. Pulmonary hypertension can manifest itself as rapid heart rate, dizziness, shortness of breath, chest pain, fatigue, and fainting symptoms so general that the disease is often misdiagnosed until the overworked heart muscle has become too weak to pump enough blood through the lungs and the patient is unable to perform even the simplest daily activity.” Dr. Gladwin said that five drugs had recently received approval from the Food and Drug Administration, and they have been successful in relieving symptoms and improving patient quality of life; however, he noted that the drugs “do not and cannot cure the disease because they act only as the first critical mechanism of pulmonary hypertension. Researchers now believe that the devastating blood pressure increase in pulmonary vessels also is caused by abnormal, almost cancerous (though not metastatic, i.e., not spreading to other tissues), proliferation of the smooth muscle cells of the pulmonary artery that crowds the blood vessel and eventually chokes off blood flow. Scientists are building on advances in treatments for patients who have cancer or coronary heart disease as they search for compounds that can interfere with the cancer-like growths and thereby not only prevent disease progression but also cure the disease by reversing vessel obstruction.”
Summarizing NIH research on pulmonary hypertension, Dr. Gladwin said that in FY2005, “our research portfolio included more than 90 research and training projects on pulmonary hypertension that address the problem from multiple perspectives. In FY2005, we also requested grant applications for 3 or 4 pulmonary vascular disease research centers. These centers will fuse basic research, studies of pre-clinical animal models, and human clinical trials to expedite development of the next generation of therapeutics.” He also noted that the Vascular Medicine Branch has launched a “bench-to-bedside” initiative to develop new therapies for pulmonary hypertension; test whether certain therapies work for patients who have pulmonary hypertension in conjunction with sickle cell anemia; identify “pre-disease” in high-risk patients, such as those who have scleroderma, HIV/AIDS, or sickle cell disease; and develop trials using chemotherapeutic medications.
Vice President for Advocacy at the Pulmonary Hypertension Association Carl Hicks said that when his daughter, Meaghan, was diagnosed with pulmonary hypertension, he was told that she only had less than a year to live. He added, “Since that time she has fought a valiant and protracted fight, and due to the hellishness of this disease, we have very nearly lost her 3 times, twice in the past two months. To remain alive now, she must take over 12 different pills daily, as well as flolan, an IV drug delivered by pump directly to her heart through her chest wall 24 hours a day. She’ll never know the joy of motherhood or even marriage. Were she to marry, she would lose my insurance benefits that are keeping her alive.”
The subcommittee also heard testimony from Rep. Lantos’ granddaughter, Charity Sunshine Tilleman-Dick, a singer and actress who was diagnosed with primary pulmonary hypertension in March 2004. “It seems simple enough to isolate PH patients’ experience to the medical drama, but we have to go on living our very real lives. With those I don’t know well, I deal with the social awkwardness of the reality of not being able to keep up, only going somewhere with elevator access, not going out to eat, and people thinking I’m clutchy for my never putting down my purse…Patients are overlooked for promotions, and I have been overlooked for castings because directors or employers often have valid concerns about medical concerns interfering with productivity or the final production. We hope to live as normal a life as is possible, but in reality, our lives are being cut tragically short, every day. I am in relatively good health, but a chest x-ray taken Tuesday indicates that even with the very invasive treatments I am undergoing, my heart continues to get larger.”
