Management of prostacyclin side effects in adult patients with pulmonary arterial hypertension

Abstract

Therapies that target the prostacyclin pathway are considered effective, yet are complex to dose and may cause dose-limiting side effects for patients with pulmonary arterial hypertension (PAH). Careful side effect management and the ability to discern side effects from worsening disease are essential in order for patients to continue, and benefit from, prostacyclin therapy. This manuscript was developed through a collaborative effort of allied health providers with extensive experience in managing patients with PAH who are treated with medications that target the prostacyclin pathway. This article provides an overview of individual prostacyclin pathway therapies approved in the United States, side effects most commonly associated with these therapies, and practical suggestions for side effect management. Most patients will experience significant side effects on prostacyclin therapy. Creating a proactive and careful side effect management program will increase the likelihood that patients are able to stay on therapy and receive the benefits afforded by prostacyclin therapy.

Keywords: adverse events; prostacyclin therapy; pulmonary arterial hypertension; pulmonary hypertension.

Fig. 1.

Balancing PAH symptoms versus prostacyclin pathway therapy side effects. PAH, pulmonary arterial hypertension.

Fig. 2.

Photos of rash (courtesy of Robin Hohsfield, RN, BSN, University of Colorado Health, Denver, CO, USA; informed consent was received from the patient to include picture in publication).

Fig. 3.

Photo of subcutaneous site reaction (courtesy of Kathy McCloy, NP, University of California, Los Angeles, CA, USA; informed consent was received from the patient to include picture in publication).