Nora Burdis, Tijana Milinic, Lauren E. Bartlett, Louisa Goss, Erin Tallarico, Abigail Boyle, Abigail Thaxton, Grant A. Turner, Luke J. Benvenuto, Albert Faro, Christopher H. Goss, Siddhartha G. Kapnadak, Kathleen J. Ramos
University of Washington and Seattle Children’s Research Institute. Cystic Fibrosis Foundation. University of California at Los Angeles. Columbia University.
United States
Pediatric Pulmonology
Pediatr Pulmonol 2024;
DOI: 10.1002/ppul.27273
Abstract
Rationale: In 2015, a survey of cystic fibrosis (CF) physicians showed significant gaps in lung transplant (LTx) referral knowledge. Subsequently, LTx referral guidelines for people with CF were published, and elexacaftor/tezacaftor/ivacaftor (ETI) became available for >80% of people in the United States (US). We sought to assess physicians’ LTx referral knowledge and self-reported referral practices.
Methods: CF center directors in the US were surveyed about LTx. Questions addressed transplant referral indications, contraindications, testing, and the impact of ETI on referral timing. Thematic analysis was used to assess responses to open-ended questions.
Results: There were 110/309 (36%) responses. Respondents identified several referral indications, including rapid decline in FEV1 (93%), recurrent hemoptysis (80%), hypoxemia (79%), and pulmonary hypertension (75%). Over 70% of respondents reported using oximetry, echocardiogram, and blood gas to assess disease severity. Respondents were more likely to find early LTx discussions useful for patients not on modulators versus on modulators (87% vs. 63%, p < .005). Most respondents (66%) reported delaying LTx referral for some patients with FEV1 30%-40% who met criteria, while 26% had delayed referral for patients with FEV1 < 30%. Uncertainty regarding optimal LTx referral timing for patients on ETI was a prominent theme of the qualitative analysis.
Conclusions: While physician knowledge about LTx referral indications appears improved since the CF referral guidelines were published, uncertainty about referral timing is pervasive, and the guidelines will need to be updated as more data become available about the long-term effectiveness of ETI in advanced lung disease.
Category
Lung Transplantation for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No