EXPRESS: Pediatric Subcutaneous Treprostinil Site Maintenance and Pain Control Strategies from the Pediatric Pulmonary Hypertension Network (PPHNet)
Pulmonary arterial hypertension (PAH) is a chronic, progressive and life-threatening disease in children with diverse causes of PAH. The most severe cases of PAH require aggressive treatments with systemic administration of continuous prostacyclin therapy, including treprostinil and epoprostenol. The successful use of continuous subcutaneous (SubQ) treprostinil therapy eliminates the need for an indwelling central venous catheter and its associated risks. However, pain at the SubQ infusion site, an expected side effect of this therapy, is often a deterrent to its widespread use. Effective SubQ treprostinil site maintenance and pain management is essential to achieve success with this therapy, but strategies surrounding site maintenance and pain control vary significantly between pediatric pulmonary hypertension (PH) treatment centers. In an attempt to standardize practice, a survey on the use of SubQ treprostinil and site maintenance and pain management strategies, as well as its perceived effectiveness, was disseminated to 13 pediatric PH centers of the Pediatric Pulmonary Hypertension Network (PPHNet). Responses to the survey were collected and analyzed and were developed into a set of formalized strategies to facilitate knowledge sharing and standardization of practice.