PAH patients face life-threatening risks from medication errors

Pulmonary arterial hypertension (PAH) patients using infused drugs have suffered serious injury or in some cases died from medication errors in hospitals and other inpatient settings, according to a peer-reviewed study by Accredo Health Group and several university hospitals that surveyed doctors, nurses and pharmacists.

The study, which was presented at the American College of Chest Physicians' annual meeting and will be published in the medical journal CHEST, examined medication errors for patients infused with epoprostenol (Flolan®) and treprostinil (Remodulin®) and provided guidelines to help reduce these risks.

Pulmonary arterial hypertension is a debilitating, life-threatening condition without a cure that can lead to heart failure and lung transplants. Epoprostenol and treprostinil inhibit the blood from clotting and also widen blood vessels to ease blood pressure in the lungs and reduce strain on the heart. The medications belong to a group called prostacyclins, which need to be continuously infused into the body for pulmonary hypertension patients to survive. The drugs are dosed in extremely small quantities and giving a patient too much or too little can lead to patient injury or death.

"Prostacyclins are the most potent drugs used to treat PAH and have a very narrow therapeutic window, making the proper use of medications paramount to keep patients safe," said Mark A. Tankersley, Ph.D., director of health outcomes solutions at Accredo Health Group, Inc. and one of the authors of the study. "These patients are in an extremely vulnerable condition - normally in the advanced stages of this debilitating condition. Our survey exposes the severity of the problem but also presents some possible solutions to address it."

An electronic survey of 97 clinicians at treating facilities found that 68 percent reported errors associated with administering prostacyclins and 28 respondents reported serious errors including nine that resulted in death. A separate telephone survey found that 17 of 18 nurses at pulmonary arterial hypertension centers reported serious medication errors, including three deaths. The results showed that medication errors included providing the wrong drug to the patient, improper dosing, incorrectly flushing the patient's catheter line and accidental stoppage of the infusion pump.

Researchers made the following recommendations: clearer record-keeping, clinician training, double-checking dosage factors (concentration, patient weight, pump rate and time), color coding cassettes for different drugs, marking line connections for drugs, proper medication storage, ensuring that the infusion pumps are working correctly, and requiring two nurses to sign-off on administering the medication.

PAH affects about 20,000 Americans and presently does not have a cure. One form of pulmonary arterial hypertension has no known cause. For other patients, it results from congenital heart disease, HIV infection, thyroid disease, and the use of certain diet medications or street drugs. The condition is often misdiagnosed in its early stages, but as the condition worsens it can lead to fainting, lightheadedness during physical activity, swelling in the legs and ankles, and bluish toned lips and skin (cyanotic).