Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients

Abstract Continuous subcutaneous (SubQ) treprostinil is an effective therapy for pediatric patients diagnosed with pulmonary hypertension (PH). To date, the clinical characteristics and factors associated with failure to tolerate this therapy have not been described. The purpose was to describe pati...

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Main Authors: Julia McSweeney, Elizabeth Colglazier, Jasmine Becerra, Brienne Leary, Kathleen Miller‐Reed, Stephen Walker, Katy Tillman, Melissa Magness, Michelle Ogawa, Whitney Bannon, Tisha Kivett, Emma O. Jackson, Anne Davis, Cathy Shepard, Susan Richards, Elise Whalen, Shannon Engstrand, Zachary DiPasquale, Jean A. Connor
Format: Article
Language:English
Published: Wiley 2023-04-01
Series:Pulmonary Circulation
Subjects:
Online Access:https://doi.org/10.1002/pul2.12224
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author Julia McSweeney
Elizabeth Colglazier
Jasmine Becerra
Brienne Leary
Kathleen Miller‐Reed
Stephen Walker
Katy Tillman
Melissa Magness
Michelle Ogawa
Whitney Bannon
Tisha Kivett
Emma O. Jackson
Anne Davis
Cathy Shepard
Susan Richards
Elise Whalen
Shannon Engstrand
Zachary DiPasquale
Jean A. Connor
author_facet Julia McSweeney
Elizabeth Colglazier
Jasmine Becerra
Brienne Leary
Kathleen Miller‐Reed
Stephen Walker
Katy Tillman
Melissa Magness
Michelle Ogawa
Whitney Bannon
Tisha Kivett
Emma O. Jackson
Anne Davis
Cathy Shepard
Susan Richards
Elise Whalen
Shannon Engstrand
Zachary DiPasquale
Jean A. Connor
author_sort Julia McSweeney
collection DOAJ
description Abstract Continuous subcutaneous (SubQ) treprostinil is an effective therapy for pediatric patients diagnosed with pulmonary hypertension (PH). To date, the clinical characteristics and factors associated with failure to tolerate this therapy have not been described. The purpose was to describe patient‐reported factors contributing to SubQ treprostinil intolerance in pediatric patients with PH. A retrospective descriptive study was performed at 11 participating sites in the United States and Canada for patients younger than 21 years of age diagnosed with PH who failed treatment to tolerate SubQ treprostinil between January 1, 2009, and December 31, 2019. All data were summarized using descriptive statistics. Forty‐one patients met the inclusion criteria. The average age at SQ treprostinil initiation, and length of treatment, was 8.6 years and 22.6 months, respectively. The average maximum dose, concentration, and rate were 95.8 ng/kg/min, 6.06 mg/mL, and 0.040 mL/h, respectively. The reasons for failure to tolerate SubQ treprostinil included intractable site pain (73.2%), frequent site changes (56.1%), severe site reactions (53.7%), infections (26.8%), and noncompliance/depression/anxiety (17.1%). Thirty‐nine (95.1%) patients transitioned to a prostacyclin therapy with 23 patients transitioning to intravenous prostacyclin, 5 to inhaled prostacyclin, 5 to oral prostacyclin, and 7 to a prostacyclin receptor agonist. A subset of pediatric PH patients failed to tolerate SubQ treprostinil infusions despite advances in SubQ site maintenance and pain management strategies. Intractable site pain, frequent SubQ site changes, and severe localized skin reactions were the most common reasons for failure.
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spelling doaj.art-d2fc43a93d194f25a4bff2c0c00d1da72023-06-28T07:04:37ZengWileyPulmonary Circulation2045-89402023-04-01132n/an/a10.1002/pul2.12224Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patientsJulia McSweeney0Elizabeth Colglazier1Jasmine Becerra2Brienne Leary3Kathleen Miller‐Reed4Stephen Walker5Katy Tillman6Melissa Magness7Michelle Ogawa8Whitney Bannon9Tisha Kivett10Emma O. Jackson11Anne Davis12Cathy Shepard13Susan Richards14Elise Whalen15Shannon Engstrand16Zachary DiPasquale17Jean A. Connor18Department of Cardiology and Patient Care Operations Boston Children's Hospital Boston Massachusetts USADepartment of Nursing UCSF Benioff Children's Hospital Oakland California USADepartment of Pediatrics UCSF Benioff Children's Hospital Oakland California USADepartment of Cardiology and Patient Care Operations Boston Children's Hospital Boston Massachusetts USADepartment of Cardiology Children's Hospital of Colorado Aurora Colorado USADepartment of Cardiology Children's Hospital of Philadelphia Philadelphia Pennsylvania USADepartment of Cardiology Children's Hospital of Wisconsin Milwaukee Wisconsin USADepartment of Cardiology Cincinnati Children's Hospital Cincinnati Ohio USADepartment of Cardiology Lucille Packard Children's Hospital Palo Alto California USAPulmonary Department Riley Hospital for Children at Indiana University Health Indianapolis Indiana USAPulmonary Department Riley Hospital for Children at Indiana University Health Indianapolis Indiana USADepartment of Cardiology Seattle Children's Hospital Seattle Washington USADepartment of Cardiology Seattle Children's Hospital Seattle Washington USADepartment of Pediatric Cardiac Intensive Care Stollery Children's Hospital Edmonton Alberta CanadaDepartment of Cardiology Stollery Children's Hospital Alberta Edmonton CanadaDepartment of Pediatrics Texas Children's Hospital Houston Texas USADepartment of Cardiology Boston Children's Hospital Boston Massachusetts USADepartment of Cardiology Boston Children's Hospital Boston Massachusetts USADepartment of Cardiology and Patient Care Operations Boston Children's Hospital Boston Massachusetts USAAbstract Continuous subcutaneous (SubQ) treprostinil is an effective therapy for pediatric patients diagnosed with pulmonary hypertension (PH). To date, the clinical characteristics and factors associated with failure to tolerate this therapy have not been described. The purpose was to describe patient‐reported factors contributing to SubQ treprostinil intolerance in pediatric patients with PH. A retrospective descriptive study was performed at 11 participating sites in the United States and Canada for patients younger than 21 years of age diagnosed with PH who failed treatment to tolerate SubQ treprostinil between January 1, 2009, and December 31, 2019. All data were summarized using descriptive statistics. Forty‐one patients met the inclusion criteria. The average age at SQ treprostinil initiation, and length of treatment, was 8.6 years and 22.6 months, respectively. The average maximum dose, concentration, and rate were 95.8 ng/kg/min, 6.06 mg/mL, and 0.040 mL/h, respectively. The reasons for failure to tolerate SubQ treprostinil included intractable site pain (73.2%), frequent site changes (56.1%), severe site reactions (53.7%), infections (26.8%), and noncompliance/depression/anxiety (17.1%). Thirty‐nine (95.1%) patients transitioned to a prostacyclin therapy with 23 patients transitioning to intravenous prostacyclin, 5 to inhaled prostacyclin, 5 to oral prostacyclin, and 7 to a prostacyclin receptor agonist. A subset of pediatric PH patients failed to tolerate SubQ treprostinil infusions despite advances in SubQ site maintenance and pain management strategies. Intractable site pain, frequent SubQ site changes, and severe localized skin reactions were the most common reasons for failure.https://doi.org/10.1002/pul2.12224advanced practice nursingprostacyclinpulmonary hypertensiontreprostinil
spellingShingle Julia McSweeney
Elizabeth Colglazier
Jasmine Becerra
Brienne Leary
Kathleen Miller‐Reed
Stephen Walker
Katy Tillman
Melissa Magness
Michelle Ogawa
Whitney Bannon
Tisha Kivett
Emma O. Jackson
Anne Davis
Cathy Shepard
Susan Richards
Elise Whalen
Shannon Engstrand
Zachary DiPasquale
Jean A. Connor
Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients
Pulmonary Circulation
advanced practice nursing
prostacyclin
pulmonary hypertension
treprostinil
title Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients
title_full Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients
title_fullStr Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients
title_full_unstemmed Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients
title_short Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients
title_sort failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients
topic advanced practice nursing
prostacyclin
pulmonary hypertension
treprostinil
url https://doi.org/10.1002/pul2.12224
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