Since there is no cure for PPH, the drug treatments listed below 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.
Get more info about PPH and Primary Pulmonary Hypertension.
New drugs including the following haved provided hope to patients living with PPH:
Flolan or epoprostenol is a form of a naturally occurring molecule in our bodies called prostaglandin. Doctors use prostaglandin to treat patients with Primary Pulmonary Hypertension (PPH). Flolan is helping many severely ill patients who do not respond to treatment with calcium channel blockers such as Nifedipine. Flolan imitates the natural prostacyclin produced by the body to keep blood vessels healthy by removing the build up of lipids, lowering blood pressure. People respond to increased doses of prostaglandin which dilate, or open up, blood vessels in the lungs and throughout the body. This Flolan-Prostacyclin also appears to prevent blood clots from forming. Flolan has been studied in clinical trials, and is the only FDA approved drug treatment for PPH.
Flolan is administered intravenously directly into the bloodstream through a surgically implanted catheter by a portable, battery-operated pump. The pump is worn attached to a belt around the waist or carried in a small shoulder pack. Since the drug lasts only 3-5 minutes it must constantly be infused: it is slowly and continuously pumped into the body through the permanent catheter placed in a vein in the neck or chest. The pump is filled daily with the mixed Flolan solution.
Flolan is a lifetime therapy which requires uninterrupted infusion because of its short effect-3 to 5 minute effect. The drug is very expensive, at around $100,000 or more per year and is used only after a positive diagnosis of PPH. Because of the complexity of administering Flolan, patients use special health care services to learn about administering the treatment. Companies that distribute Flolan offer home health care services including infusion and nursing.
There is no set dose, the dose that is used is based on the amount of relief it provides the patient of their PPH symptoms and the patient's ability to handle Flolan side effects. The dose will increase during a patient's therapy in order to remain effective; no maximum dose has been demonstrated. The drug requires special handling including a constant controlled temperature and protection from light.
Flolan therapy has been credited with raising the life expectancy of patients with PPH by 3 to 5 years or more.
UT-15 (Remodulin)- prostacyclin delivered under the skin (subcutaneous infusion)
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.
Bostenan (Tracleer)- Orally active endothelin receptor antagonist. Made by Actelion *
Bostenan was one of the first available oral forms of of Prostacyclin. It has been made available to patients at the University of Pittsburgh Medical Center as part of a clinical trial. Bostenan 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. Bostenan was designed to offset endothelin, lowers the endothelin levels, reversing its effects, resulting in lower artery pressure.
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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