Pulmonary fibrosis is associated with a number of specific forms of interstitial lung disease (ILD) and can lead to progressive decline in lung function, poor quality of life, and, ultimately, early death. Idiopathic pulmonary fibrosis (IPF), the most common fibrotic ILD, affects up to 1 in 200 elderly individuals and has a median survival that ranges from 3 to 5 years following initial diagnosis. IPF has not been shown to respond to immunomodulatory therapies, but recent trials with novel antifibrotic agents have demonstrated lessening of lung function decline over time. Pirfenidone has been shown to significantly slow decline in forced vital capacity (FVC) over time and prolong progression-free survival, which led to its licensing by the United States Food and Drug Administration (FDA) in 2014 for the treatment of patients with IPF. However, pirfenidone has been associated with significant side effects, and patients treated with pirfenidone must be carefully monitored. We review recent and ongoing clinical research and experience with pirfenidone as a pharmacologic therapy for patients with IPF, provide a suggested approach to incorporate pirfenidone into a treatment algorithm for patients with IPF, and examine the potential of pirfenidone as a treatment for non-IPF forms of ILD accompanied by progressive pulmonary fibrosis.

OBJECTIVE Cystic fibrosis (CF) is a genetic disease that requires complex, lifelong treatment regimens to maintain health and reduce disease progression. The aims of this study were 1) to gain the perspectives of multiple health professions to understand medication and well-being challenges of people living with CF; and 2) to apply the Systems Engineering Initiative for Patient Safety (SEIPS) model to further identify opportunities for pharmacists to support people with CF. METHODS Health care professionals were recruited from a Cystic Fibrosis Center in the Midwest, to participate in audio-recorded semistructured interviews. Topics examined during the interviews included medication education for patients as well as experiences with outpatient, specialty, and community pharmacists. The themes assessed during the pharmacist interviews included support for people living with CF, preferences in conducting medication education, and pharmacist-specific counseling. Interview transcripts were thematically analyzed into categories to determine major themes. Prevalent codes were categorized into 5 major themes guided by the SEIPS model. Interrater reliability was strong (kappa = 0.94). RESULTS Five major themes were identified: 1) patient tasks; 2) external environment; 3) organizational conditions; 4) patient medication education; and 5) pharmacists' roles and tasks. Professionals identified the importance of the pharmacist on the multidisciplinary CF care team to enhance patient-centered care for people living with CF. CONCLUSIONS This study highlights health care professionals' views on the unique skillset that pharmacists add to the care team, including a reduction in medication errors, improved adherence, and overall enhanced patient care.

Background: Cystic fibrosis (CF) is an autosomal recessive genetic disease requiring complex, lifelong medication regimens. Given the importance of medication in CF treatment, pharmacists are vital CF care team members in the care of people living with CF (PwCF). Objectives: This study aimed to (1) define patients' CF medication experiences and educational needs and (2) investigate the CF outpatient clinic and community pharmacist's role in addressing patient challenges. Methods: A work system approach informed by the Systems Engineering Initiative for Patient Safety (SEIPS) model was used to characterize knowledge and perception of CF medication regimens, educational modalities, and pharmacist interactions for PwCF. Semistructured interviews were conducted with adults living with CF at a CF center clinic. Data analyses identified relationships between the themes in the data and 4 SEIPS work system domains: tasks, tools and technology, person, and environment. Results: Thirty PwCF interviews highlighted 4 themes regarding health care experiences: (1) medication use experience, (2) medication education needs, (3) disease experience, and (4) pharmacist and pharmacy interactions. Patients reported complex medication regimens leading to challenges with medication adherence, although the benefit of treatment was recognized. Although a high level of disease-state knowledge was identified among the participants, PwCF desired to learn about CF medication benefits and adverse effects through credible sources using multiple modalities. Many reported a benefit of pharmacist involvement in their care. Conclusion: Pharmacists are well-positioned to support PwCF in adherence, medication regimen management, and medication education. Opportunities exist for growth in these supportive roles of a pharmacist in both community and outpatient clinic settings.