Since there is no cure for PPH, the treatments listed below including Flolan at best only relieve the symptoms. Relief from these symptoms however can raise quality of life and raise life expectancy by several years. The general treatments for PPH include: Calcium Channel blockers that can be used to lower pressure; Oxygen enables the patient to breath easier and lowers pressure; Coumadin which thins the blood and prevents clotting; Diurectics or "water pill" relives fluid that collects in the ankles and belly area, digoxin slows the heart rate to improve muscle force.
Drug treatments in addition to Flolan include these new drugs:
Bosentan (Tracleer)- Orally active endothelin receptor antagonist. Made by Actelion *
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Bosentan has been made available to patients at the University of Pittsburgh Medical Center as part of a clinical trial. Bosentan works by blocking the action of a hormone called endothelin. Endothelin exists in higher levels in people with PH is a hormone that is harmful to the lung and pulmonary arteries. The damaged lung and pulmonary arteries create the blood flow resistance that results in hypertension. Bosentan was designed to offset endothelin, lowers the endothelin levels, reversing its effects, resulting in lower artery pressure.
UT-15 (Remodulin)- prostacyclin delivered under the skin (subcutaneous infusion)
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In addition to Flolan, another drug called Remodulin (also known as UT-15) has been created to treat pulmonary hypertension. UT-15 is a synthetic, stable form of prostacyclin. It has been developed by United Therapeudics as an important new treatment for advanced pulmonary hypertension (PH) as well as late-stage peripheral vascular disease (PVD).
According to the company that makes UT-15 (Remodulin), United Therapeutics, the drug is currently in important Phase III clinical trials for advanced PH. Phase III trials are usually used for FDA approval to market the drug. UT-15 is apparently significantly longer lived in the human body than Flolan reducing the need for constant infusion, additionally Remodulin is stable at room temperature for up to five years, unlike Flolan. The drug's dilation action lasts from 4-6 hours versus the the short 2-3 minute action of Flolan. Because of its long life in the body it can be administered under the skin rather than into the bloodstream. The biggest benefit of UT-15 (Remodulin) is this under the skin deliver called subcutaneous infusion which works with a pager-sized MiniMed microinfusion device. UT-15 lowers the risk of sepsis infection and related hospitalization associated with the Flolan catheter which is a central I.V. line.
The side effects of UT-15 include jaw pain, headaches, nausea, diarrhea, flushing and localized pain at the delivery site under the skin. This pain has been reported as slight irritation to severe.
The reported results of UT-15 have been encouraging. Patients using the drug seem to experience improvement in their condition including: decreased fatigue, decreased shortness of breath, decreased pulmonary artery pressures as well as overall improvement in quality of life.
Beraprost - An Oral Formulation of Prostacyclin
Beraprost made by United Therapeutics is also in Phase III clinical trials. Beraprost is an oral formulation of prostacyclin, for the treatment of early-stage pulmonary hypertension (PH) as well as early-stage peripheral vascular disease (PVD)
Beraprost is a chemically stable oral form of prostacyclin. Like natural prostacyclin, beraprost dilates blood vessels, prevents platelet aggregation and prevents proliferation of smooth muscle cells surrounding blood vessels. The company believes beraprost may be an important treatment for early-state PVD and for early-stage PH. However, intermittent oral doses of beraprost do not seem to provide consistent levels of the drug in the blood necessary to treat the advanced stages of PH.
According to the company, Beraprost has proven to be safe and effective for the treatment of PVD in clinical studies conducted outside the United States and has been approved for treatment of PVD in Japan since 1994. It may soon be available for PH use in the United States.
Ilioprost- inhaled form of Prostacyclin
Ilioprost is used as an aerosolized form of treatment that is inhaled by patients. Ilioprost dilates the pulmonary blood vessels by coating itself through breathing on the alveoli of the lungs. It relieves pulmonary vascular resistance. Patients inhale 6-8 puffs every 2-3 hours. This therapy is used mainly in Europe. The studies have reported minor side effects such as coughing, headaches and jaw pain.
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