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Primary Pulmonary Hypertension: Drugs
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The orphan drug Flolan (epoprostenol sodium for injection or prostacycline) has been approved as a standard long-term treatment of individuals with severe primary pulmonary hypertension. This drug is used in individuals who do not respond to other types of therapy. This drug is administered by intravenous infusion through a permanent ambulatory in-dwelling central venous catheter. Since this drug requires continuous infusion, it must not be withdrawn suddenly (including sudden reduction of dosage). Flolan, which is a version of a natural hormone called prostacyclin that dilates constricted blood vessels, is manufactured by GlaxoSmithKline..
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Signs and symptoms of primary pulmonary hypertension include shortness of breath, fatigue, angina pectoris, and fainting. There is evidence that PPH is related to genetic factors, and women are more likely than men to be afflicted. Treatment for PPH varies and may include therapies, prescription drugs, and lifestyle changes. However, primary pulmonary hypertension must be diagnosed and treated on an individual basis by an experienced physician.
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Remodulin, a drug used in the treatment of Primary Pulmonary Hypertension... produced side effects including: headache, jaw pain, vomiting, nausea and diarrhea. The most serious side effect of this particular drug is pain associated with the insertion and presence of the metal tube inserted under the skin. Moreover, a separate prescription for pain relief has to be prescribed to ease the pain.
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Between 1967 and 1973, a 10-fold increase in unexplained pulmonary hypertension was reported in central Europe. The rise was subsequently traced to aminorex fumarate, an amphetamine-like drug introduced in Europe in 1965 to control appetite. Only about 1 in 1,000 people who took the drug developed PPH. When they stopped taking the drug, some improved considerably; in others, the disease kept getting worse. Once aminorex was removed from the market, the incidence of primary pulmonary hypertension went down to normal levels.
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The orphan drug bosentan (Tracleer) has been approved by the U.S. Food and Drug Administration (FDA) for treatment of pulmonary hypertension. The drug improves the exercise ability of individuals with primary pulmonary hypertension allowing them to exert themselves physically without shortness of breath. It should be carefully monitored while in use. Tracleer is manufactured by Actelion Pharmaceuticals US, Inc., of San Francisco, California. For information, contact the Tracleer Access Program Monday through Friday from 8 a.m. to 8 p.m. at 1-866-228-3546.
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Collagen vascular disease,4 portal hypertension,5 human immunodeficiency virus (HIV) infection6 and anorectic agents7 may produce a clinical picture similar to that of primary pulmonary hypertension. The use of appetite-suppressant drugs for more than three months is associated with a greater than 30 times increased risk of developing pulmonary hypertension.8 In the United States, the anorexic agents fenfluramine and dexfenfluramine were recalled in September 1997, only 18 months after they were released. The WHO considers other appetite suppressants, such as amphetamines, to have a "very likely" causative role in pulmonary hypertension (Table 3).3
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