Evaluation of the Need for Comprehensive Care for Patients with Cystic Fibrosis

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have revolutionized care for people with cystic fibrosis (pwCF) by improving quality of life and extending life spans. These factors support the need for pwCF to establish care with a Primary Care Provider (PCP). It is currently unclear how many adult pwCF routinely interact with a PCP.

This IRB approved, retrospective study included patients over 18 years old, seen in the adult Cystic fibrosis (CF) clinic, who were prescribed elexacaftor/tezacaftor/ivacaftor (ETI) between 8/31/2022 and 8/31/2023 at University of Iowa Health Care. The primary outcome of the study was to determine the percentage of pwCF with a PCP designated as a member of their care team in the electronic medical record (EMR). The secondary outcomes determined the percentage of pwCF who completed preventative health screenings for comorbid conditions and received appropriate cancer screenings and immunizations.

Of the 115 included patients, 60% (69/115) had a PCP identified in the EMR. PwCF have uncontrolled blood pressure regardless of whether they have a PCP identified in the EMR (76.5%) or not (78.3%). Rates of influenza (p=0.006) and pneumococcal (p=0.006) vaccinations were significantly lower for pwCF without a PCP.

There is a gap in healthcare for pwCF, especially for those who do not routinely interact with a PCP. CF clinicians may not be up to date on primary care management and PCPs may not be comfortable with treating patients with a high acuity chronic condition. PCPs should be better integrated into the CF care team to ensure pwCF are receiving comprehensive care.