Calendar | For Your Patients | PHA Main Site | Contact Us | About Us | Not a registered user? Sign up here.

Resource Library

Case Report: Transition from oral treprostinil to selexipag in a pediatric patient with pulmonary arterial hypertension

S Melissa Magness

Jenna Faircloth

Susan Hoelle

Michelle Cash


Russel Hirsch

Reviews

  Sign in to add a review

0 comments
Leave a Comment

Conference: 2017 PH Professional Network Symposium

Release Date: 10.06.2017

Presentation Type: Abstracts

File Download: 2017 PHPN Abstract 1039

Download Adobe Acrobat

Abstract presented at the 2017 PH Professional Network Symposium held in Bethesda, MD on October 5-7, 2017

Purpose

The use and transition to selexipag in a female patient with WHO Group I pulmonary arterial hypertension (PAH) will be described.

Background

Selexipag is an agonist of the prostacyclin receptor, and has gained wider use in the adult PH population. Pediatric use is limited. The patient was an 18-year-old with a history of ventricular septal defect, initially banded, and with delayed complete repair at 8 years of age. She had documented progression of her PAH, with progressive therapy culminating in oral treprostinil as her third drug. However, debilitating symptoms of headache and facial flushing, despite dose adjustment prompted the transition. This report summarizes the development and implementation of a standardized approach to transition from oral treprostinil therapy to selexipag.

Methods

Consideration for this decision was prompted by ongoing side effects on oral treprostinil that remained unresolved after 6 months of attempts at symptom alleviation, and with growing concern for deterioration in medical compliance. Literature and industry also reported a lesser side effect profile with similar efficacy with selexipag in treating PAH. The model for the transition plan was developed within our multidisciplinary PH service and with input from our adult colleagues, since no data for transitioning between these two therapies exists. At the commencement of the transition, her oral treprostinil dose was 6mg QID (approximately equivalent to an infused prostacyclin dose of 45-50ng/kg/min). The calculated initial goal dose of selexipag was 800-1,000mcg, an equivalent to intra-venous treprostinil dose of 40-50ng/kg/min. Upon admission, her oral treprostinil dose was reduced to 6mg TID to ease the transition. Selexipag was started on the same day with a dose of 200mcg Q12 hours scheduled with a simultaneous decrease in oral treprostinil dose by 2mg. In the subsequent days, doses were adjusted with increases in selexipag by 200 mcg and decreases in oral treprostinil by 2mg with every dose. By day 3, oral treprostinil was discontinued, and the selexipag dose was 600mcg. On day 4, the final increase to 800mcg selexipag was completed shortly before discharge.

Results

During the hospital stay, the patient was prophylactically treated with scheduled ondansetron, with acetaminophen and ibuprofen available as needed for headache. She had no emesis or abdominal discomfort. Vital signs were monitored consistently during the admission and remained stable at patient baseline. Post discharge follow-up consisted of daily phone calls for the first week with self-reporting of only minor headaches. One week after discharge, she tolerated the final increase to 1000mcg of selexipag with no further side effects reported. Further follow up is pending.

Conclusion

We have shown that with multidisciplinary team planning, a transition from oral treprostinil to selexipag can be completed safely and with minimal side effects in the pediatric population. This model of comprehensive and collaborative pediatric medical care has resulted in improved patient outcomes and quality of life, which we hope will contribute to improved compliance.