Abstract

Cystic fibrosis (CF) is one of the most common life-threatening, genetic conditions. People with CF follow complex, time-consuming treatment regimens to manage their chronic condition. Due to the complexity of the disease, multidisciplinary care from CF Foundation (CFF)-accredited centers is recommended for people with CF. These centers include several types of healthcare professionals specializing in CF; however, pharmacists are not required members. The purpose of this study was to identify the outpatient care needs of people living with CF that pharmacists could address to improve their quality of care. Healthcare members from a CFF accredited center and pharmacists were recruited to participate in semi-structured, audio-recorded interviews. Prevalent codes were identified and data analysis was conducted, guided by the systems engineering initiative for patient safety (SEIPS) model. The objective was to understand the medication and pharmacy-related needs of patients with CF and care team perspectives on pharmacists providing support for these patients. From the themes that emerged, pharmacists can provide support for people living with CF (medication burden, medication access, medication education) and the CF care team (drug monitoring and adherence, prior authorizations and insurance coverage, refill history). Pharmacists are well-positioned to address these difficulties to improve quality of care for people living with cystic fibrosis.

Highlights

  • Cystic fibrosis (CF) is one of the most prevalent chronic and fatal genetic diseases, affecting approximately 70,000 people worldwide and 30,000 in the United States alone [1,2]

  • The systems engineering initiative for patient safety (SEIPS) 2.0 Model was applied to guide our understanding of medication management and interactions between pharmacists and other members of CF healthcare team [14]

  • This study identified specific opportunities for pharmacists to assist with medication challenges experienced by people living with CF, including poor medication adherence, medication counseling, and limited medication access because of barriers such as prior authorizations or insurance coverage

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Summary

Introduction

Cystic fibrosis (CF) is one of the most prevalent chronic and fatal genetic diseases, affecting approximately 70,000 people worldwide and 30,000 in the United States alone [1,2]. CF is a progressive, multisystem disease that primarily affects the respiratory and digestive systems as well as the pancreas, liver, and reproductive system [1]. CF is an incurable autosomal recessive disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) [3]. Mutations in the CFTR cause thick secretions of mucus to line several organs in the body such as the lungs, pancreas, digestive system, and reproductive system [4]. The thick mucus production puts patients with CF at risk of developing bacterial infections in the lungs [1]. Advances in treatment and knowledge of CF have extended the median predicted survival age to 47.7 years for individuals born in 2016 (compared to age 42.7 years for those born between 2012 and 2016), patients manage complex, time-consuming, and lifelong treatment regimens [1,5]

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