Editor’s note: A new evidence-based guideline on pulmonary arterial hypertension cautions clinicians about the use of calcium channel blockers in unstable patients with PAH due to the potentially fatal effects associated with the drugs in patients with this condition. The guidelines, published in the journal Chest, are “Medical Therapy for Pulmonary Arterial Hypertension” and “Screening, Early Detection, and Diagnosis of Pulmonary Arterial Hypertension.” They can be downloaded from the Chest website at
www.chest.org.

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Infusion apparatus for epoprostenol administration consists of a portable infusion pump and single lumen central venous catheter (generally a Hickman catheter). Note that the catheter exit site is positioned where it is accesible to the patient for self-care.

In 1997, the fen-phen phenomenon hit the public media with as much fanfare as the latest celebrity legal battle. Physicians linked the fat-fighting combination therapy of phentermine and fenfluramine or dexfenfluramine to valvular regurgitation in about 30% of patients taking the drugs. Manufacturers withdrew fenfluramine and dexfenfluramine from the market after a request from the U.S. Food and Drug Administration. Although many patients were asymptomatic, some were left with a sad legacy from their battle with obesity — pulmonary artery hypertension.

PAH is characterized by progressive increases in pulmonary artery pressure, leading to right ventricular failure and death. (See “The pressure of pulmonary arterial hypertension” sidebar.) The disease can frustrate health care providers, but treatment options do exist. Epoprostenol sodium infusion therapy can reduce the incidence of mortality from PAH.1 It is FDA-approved for patients with symptomatic, primary PAH (defined by the World Health Organization as Class III and IV) or PAH associated with scleroderma.

But epoprostenol is a long-term, complex, and expensive treatment. Nurses must understand this therapy to help patients derive the most benefit
from it.

Decision time

Determining eligibility for epoprostenol goes beyond assessing for clinical indications. (See “Epoprostenol sodium” sidebar.) Because the drug’s half-life is only minutes, patients must receive a continuous infusion via a central line and an infusion pump. Thus, the patient’s mental and physical capacity for the complicated therapy must be evaluated. The nurse should interview both the patient and a significant other who will offer help and support. (See “Is Your Patient Ready for Epoprostenol Therapy?”)

Providers also should consider whether their facility’s resources are sufficient for the comprehensive and intensive management these patients require. Although epoprostenol therapy can be lifesaving when used appropriately, it also can complicate and sometimes worsen symptoms with catastrophic results — rebound pulmonary hypertension or death — if initiated, administered, or managed incorrectly. If sufficient resources are not available, patients should be referred to a specialized PAH clinic.

Before making the final decision to proceed with epoprostenol therapy, patients should see the delivery system, observe all procedures, and, ideally, meet another patient undergoing therapy. These steps can dramatically reduce anxiety associated with starting long-term IV therapy. The Pulmonary Hypertension Association [www.phassociation.org] can help locate patients and provide support and information.

Patients must have realistic perceptions about the drug. On the plus side, epoprostenol can improve the quality of and even extend the patient’s life. The downside: It’s not a cure, and it can have adverse effects. What’s more, it’s impossible to predict the magnitude or duration of the therapeutic response. Patients should understand that initial improvement in symptoms does not guarantee continued improvement or preclude eventual decline.

Start-up

The approach to education and drug initiation differs among large centers, depending on experience and resources, but some common practices exist. Experienced health care providers — ideally, the ones who will follow the patients long term — should teach them. At Mayo Clinic, patients receive preprinted, step-by-step directions in a manual that covers pump operation, drug reconstitution, and cassette and tubing change. Teaching should occur in intensive blocks over a period of days and close to the initiation of the drug.

Whether the epoprostenol infusion is started in an inpatient or outpatient setting, the nurse must observe the patient closely, check vital signs, and have immediate access to emergency equipment and care. The starting infusion rate is typically 2 to 3 ng/kg/min, delivered via a CADD Legacy pump. Vital signs are obtained before and at least every half-hour for two to three hours after drug initiation.