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.

Sürekli İntravenöz Prostasiklin Tedavisi Alan Pulmoner Arteriyel Hipertansiyon Hastalarının Hemşirelik Bakımı ve Eğitimi

“…Hastalar yan etkiler konusunda bilgilendirilmelidir. [23-25, 27, 30] Tedavi [31] Epoprostenol tedavisi sürecinde santral katetere ilişkin komplikasyonlar yaşanabilmektedir. Bu komplikasyonlar, lokal kateter enfeksiyonları, kateter yolu enfeksiyonları, bakteriyemi veya sepsis, tromboz, kateter hattı bozukluğu (delik veya sızıntı), tıkanıklık, kateter hattı yer değiştirmesi, kateterin çıkması olabilmektedir.…”

Treatment and Palliation of Symptoms in Patients With Advanced Pulmonary Arterial Hypertension

“…Gabapentin is often successful for leg pain. 13 Naturally, opioids are avoided when possible but should be utilized on a case-by-case basis and certainly considered when aforementioned strategies do not achieve adequate pain control. In cases of refractory pain, potential metabolic contributors such as vitamin B 12 deficiency or thyroid dysfunction should be considered, and a referral to a pain management or palliative care specialist can be an option.…”

“…Loose stools are expected and can be controlled with antidiarrheal agents such as loperamide or diphenoxylate/atropine. 13 If diarrhea becomes severe, other pathology including Clostridium difficile should be excluded and diuretics may need to be limited to avoid hypovolemia.…”

“…The pain can be associated with erythema, induration, inflammation, site pain, bleeding, and occasionally cellulitis and abscess formation. 13 The site can vary depending on what a patient finds comfortable, including the abdomen, upper buttocks, lower flanks, outer thighs, and even the back of the upper arm. From patient reports, leaving the infusion at a "good" site could minimize site pain.…”

“…If site pain is particularly severe, systemic therapies such as gabapentin, tramadol, or tricyclic antidepressants can be administered within the first week of a site change. 13,16,17 Overall, the benefits of these medications in alleviating the troublesome and often very severe symptoms of PAH must be balanced with the common side effects. Despite the potential considerable side effects, patients receiving prostacyclin pathway therapies have improvement in their quality of life.…”