Pulmonary hypertension is a disease that causes the blood vessels in the lungs to have unusually high pressure. This high pressure in the pulmonary arteries results in the heart having difficulty pumping against the resistance of the blood pressure in the lungs. The resistance caused by the high pressure in the pulmonary vasculature eventually damages the right ventricle and it becomes less efficient. The patient will eventually suffer heart failure and die.
Pulmonary Hypertension is often the result of another disease that affects the body such as lung disease, heart disease or liver disease, as well as HIV infection or systemic connective tissue disease such as scleroderma. These conditions give rise to "Pulmonary Hypertension," or "Secondary Pulmonary Hypertension”, however if the cause cannot be pinpointed the disease is referred to as "Primary Pulmonary Hypertension."
Flolan or epoprostenol is a synthetic prostaglandin which helps the body open blood vessels. This synthetic substance contained in Flolan is called Prostacyclin. Flolan (Prostacyclin) is used to treat patients with primary pulmonary hypertension who do not respond to traditional treatment with calcium channel blockers (e.g. Nifedipine). Flolan imitates the natural prostaglandin produced by the body to keep blood vessels healthy by removing the build up of lipids and thereby lowering blood pressure. People respond to increased doses of Flolan which dilates 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 an FDA approved drug treatment for primary pulmonary hypertension.
Flolan is a lifetime therapy which requires uninterrupted infusion because of its short, 3 to 5 minute, effect. Flolan is administered intravenously 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. The pump is filled daily with the mixed Flolan solution. The dose is based on the amount of relief it provides the patient balanced with 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. Side effects can include jaw pain, headache, flushing, nausea, diarrhea, and vomiting. The drug requires special handling including a constant controlled temperature and protection from light.
Interruption of Flolan can be life-threatening, even a brief interruption can result in a sudden reoccurrence of symptoms. The indwelling central I.V. line, needs a high degree of maintenance and infections can be serious. 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.
Patients on Flolan are trained to care for their IV line and for the drug and pump. The patients are trained to troubleshoot both the line and the pump and so can cope with most problems that occur.
If the central line ceases to function they will require IV access to maintain the infusion.
Paramedics should immediately establish a peripheral I.V. to allow the patient to reinitiate their infusion through a patent line.
Because of the short half-life of Flolan, symptoms of pulmonary hypertension can occur within minutes. Most often this will be manifest only by some shortness of breath; however, in rare cases patients have had rapid circulatory collapse within minutes of cessation of the drug.