Brittany A. Wright

Background: The clinical impact of pharmacists and pharmacy technicians on the care of people with cystic fibrosis (pwCF) has been well documented within the literature, including medication access, reduction of hospitalizations, and consolidation of pharmaceutical delivery for pwCF with complex medication regimens. However, challenges related to access and affordability have become increasingly complex, increasing the administrative burden placed upon these members of the multidisciplinary CF care team. The goal of this study was to quantify the time pharmacists and pharmacy technicians at CF Foundation‐accredited care centers (CFACC) spent triaging and resolving errors, categorize the types of interventions required, and tabulate the financial burden for pwCF averted through these interventions. Methods: This study was a retrospective review of pharmacy team members' interactions following a change in limited distribution network of cystic fibrosis transmembrane conductance regulator modulators (CFTRm) over a 4‐month period of time. Each site received institutional review board exemption. Measures were characterized using descriptive statistics and were analyzed in SAS v9.4 (Cary, NC). Collected data were extrapolated to be nationally representative based on the 2023 CFF Patient Registry report. Results: Six CFACC and 1781 pwCF were included in this review. Of these pwCF, 216 required additional interventions following a required change in the dispensing pharmacy for their CFTRm. Collectively, 201.5 h were spent on resolution of issues during the study period with interventions successfully averting a financial burden to pwCF of $226,094.73. Extrapolations of time and financial burden were estimated to be 3700 h and over $4.2 million, respectively. Conclusions: Medication access and affordability continue to present significant barriers for pwCF. We aim to highlight the critical services provided by clinical pharmacy teams at CFACC as well as their integrated health‐system specialty pharmacies, and the vital role they play in improving outcomes and providing cost‐effective care for pwCF.

Introduction: Advancements in the care of people with cystic fibrosis (PwCF) have made discussions about reproductive health increasingly important. Rising rates of both intended and unintended pregnancies highlight the need for effective contraception counseling. Understanding current contraception use patterns and identifying priorities for contraception education amongst PwCF are therefore essential. Methods: A web‐based survey was developed in partnership with Community Voice, a virtual community of PwCF and their family members supported by the Cystic Fibrosis Foundation. The survey was distributed over a 5‐week period (June–July 2025) to participants from Community Voice and Elevate. Females with cystic fibrosis aged 18 years and older were eligible to participate. Results: Ninety‐seven PwCF completed the survey, representing 27 U.S. states and four Canadian provinces. Among individuals currently using contraception, the most common methods were intrauterine devices (34%), combined oral contraceptives (28.3%), and condoms/barrier methods (24.5%). Although 90% of PwCF were not actively trying to conceive, only 55% reported using a form of birth control. Knowledge‐based questions revealed persistent misconceptions about the impact of common antibiotics on contraceptive effectiveness. While most PwCF (68.1%) wanted contraception information from their CF care team, only 28.9% reported receiving such education. Side effects were a major factor in decision‐making: 78.9% selected contraceptive methods based on side‐effect profiles (valued more than efficacy), 68.6% reported experiencing side effects, and side effects were the leading reason for contraception discontinuation. Conclusion: CF care teams can play a critical role in initiating and supporting conversations about contraception. Team members, including pharmacists, can provide medication counseling and help PwCF select contraceptive methods that align with their individual goals and needs.

The advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulators has dramatically changed the landscape of the clinical care of people with cystic fibrosis (PwCF). In particular, highly effective modulator therapy (HEMT) availability for the majority of PwCF has led to changes in pharmacotherapy regimens and clinical outcomes but has also been associated with adverse effects and drug interactions. The role of CFTR modulator therapy perinatally continues to be of great interest, related to both the mother with CF as well as the infant in utero and after birth. As the median life expectancy for PwCF continues to increase, consideration must also be given for treatment of cardiovascular disease and other comorbidities as well as cancer screening and other preventative care measures.

Objectives: Despite cystic fibrosis transmembrane conductance regulator (CFTR) proteins being present throughout the entire body and organ systems, typical presentation of cystic fibrosis (CF) involves lung disease. We report a series of individuals that were referred to our CF clinic for evaluation following a diagnosis of chronic pancreatitis or acute recurrent pancreatitis. After evaluation, they were deemed to have CFTR dysfunction and started on highly effective modulator therapy (HEMT). Methods: A retrospective chart review was completed for all individuals referred to the University of Iowa Health Care Cystic Fibrosis Clinic from January 1, 2012, through December 31, 2023, for follow‐up evaluation of chronic or acute recurrent pancreatitis. Along with demographic data, we quantified emergency department encounters or hospitalizations due to pancreatitis pre‐ and post‐HEMT as well as individual tolerability of therapy. Results: Ten individuals met inclusion criteria. Four individuals were found to have sweat chlorides that confirmed a diagnosis of CF, and three individuals with sweat chloride values below the diagnostic threshold were further evaluated with nasal potential difference (NPD) testing and found to have results consistent with CFTR dysfunction. All 10 patients were initiated on HEMT. In the 2 years prior to HEMT initiation, there was an average of 0.33 pancreatic episodes per month (range: 0.00–1.38), compared with 0.09 pancreatic episodes per month (range: 0.00–0.50) after initiation. HEMT was generally well tolerated with one individual discontinuing due to liver function abnormalities. Conclusions: Our study highlights a panel of individuals that may have been overlooked for CFTR dysfunction due to a nonpulmonary primary presentation, such as pancreatitis. However, our use of additional diagnostic tools followed by HEMT utilization was able to describe individual‐reported improvements in symptoms and quality of life in individuals with CFTR dysfunction.

Introduction: 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. Methods: 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. Results: 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. Discussion: 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.

Background and Aims: People with cystic fibrosis (pwCF) often have vitamin D deficiency and require vitamin D supplementation. The primary objective of this study was to evaluate the impact of ultrahigh dose cholecalciferol (“stoss dosing”) in pwCF with variable adherence to maintenance cholecalciferol.Methods: Retrospective cohort study of pwCF who received initial stoss dose per protocol between January 2017 and November 2021 at University of Iowa Health Care adult and pediatric CF centers. Serum 25(OH)D concentrations were extracted from the EMR. The increase in serum 25(OH)D concentration associated with receiving stoss doses was evaluated using a longitudinal linear mixed effects regression model.Results: Fifty‐eight patients were included in the final analysis. The mean baseline 25(OH)D concentration before stoss therapy was 20.1 ng/mL (standard deviation [SD] = 5.9). The mean serum 25(OH)D concentration following stoss therapy increased to 27.9 ng/mL (SD = 7.5) and was measured on average 116 days after stoss administration. For patients with self‐reported non‐adherence to vitamin regimens, 16 out of 20 (80%) achieved a serum 25(OH)D concentration of at least 30 ng/mL after stoss therapy.Conclusions: Our findings suggest that stoss dosing was associated with increased serum 25(OH)D concentrations. This benefit also existed for individuals with self‐reported non‐adherence to maintenance cholecalciferol regimens with most patients reaching concentration goals.

Background: Food insecurity is increasing in prevalence throughout the general population and has been noted to substantially exist within the cystic fibrosis (CF) community as well. Nutritional grant programs are in place to provide additional support to people with cystic fibrosis (PwCF). This study was designed to determine the current usage rate of nutritional grant funding as well as clinical outcomes such as body mass index (BMI). We aim to describe our CF center's enrollment and usage of privately funded nutritional grants and explore potential correlations to nutrition clinical outcomes. Methods: A single center retrospective chart review of the electronic health records (EHR) to collect requested data points was completed for all PwCF seen at that center between the dates of March 1, 2017 and February 29, 2024. Results: Two hundred and nineteen individuals met inclusion criteria. Over the course of the study period, the total number of PwCF enrolled in supplemental nutritional grant program(s) and amount of grant utilization increased steadily from 9% enrolled with 17% utilization in 2017 to 27% enrolled with 44% utilization in 2022, respectively. The percentage of PwCF with a BMI below Cystic Fibrosis Foundation (CFF) recommended goals also decreased over the course of the study period from 39% in 2017 to 31% in 2022. Conclusions: We conclude that the results of our study support continued efforts to identify and remediate the presence of food insecurity within the CF community.