Pharmacists are essential for managing complex CF regimens, monitoring their effectiveness, and improving adherence.
Cystic fibrosis (CF) is a progressive genetic disease that causes pulmonary and digestive system issues and affects more than 30,000 children and adults in the United States and more than 70,000 individuals worldwide.1 CF symptoms include a persistent cough that produces thick mucus, wheezing, exercise intolerance, repeated lung infections, chronic sinusitis, intestinal blockage, and poor weight gain and growth.2
Multidisciplinary teams for CF management typically include specialists like pulmonologists, gastroenterologists, palliative care specialists, respiratory therapists, nurses, social workers, dietitians, psychologists, and pharmacists who can work to improve health outcomes and patient quality of life.3
Pharmacists on the Care Team
Patients with CF require a complex, life-long pharmacotherapy regimen that may include, on average, 10 medications,2 making pharmacists a vital member of the CF care team. Pharmacists can manage therapies that might include CF transmembrane conductance regulator protein modulators, inhaled antibiotics, bronchodilators, pancreatic enzymes, supplemental vitamins, inhaled mucolytics, and anti-inflammatories.2,3 CF is also associated with comorbidities, including diabetes, liver disease, depression, and anxiety—all of which add to a patient’s medication burden.2
In 2020, the Cystic Fibrosis Foundation published the most recent consensus guidelines for managing patients with advanced CF lung disease, the most common cause of death in this population.3 The 22 panelists, which included pediatric, adult, and transplant pulmonologists; a gastroenterologist; a palliative care specialist; a pharmacist; a respiratory therapist; a nurse coordinator; a social worker; a dietician; a methodologist; one parent; and 2 patients with CF— met to discuss pulmonary management, comorbid condition treatment, symptom management, and psychosocial issues.3
According to these guidelines, intermittent inhaled antibiotics (28 days on/off) are the standard of care for patients with CF and chronic air-infection.3 Pharmacists can also recommend drug dosages for a trial of continuous alternating inhaled antibiotics—generally alternating between 2 different drugs—appropriate for treatment based on bacterial patho- gens identified in respiratory culture, which is one of the recommendations based on evidence demonstrat- ing additional benefit in patients with advanced disease.3
Antimicrobial resistance has become a growing concern for patients with CF and pulmonary infection. Appropriate use of antibiotic therapy is critical in this population. Ototoxicity and nephrotoxicity related to antibiotics (eg, aminoglycosides) can be monitored by pharmacists.3 Tobramycin is the preferred aminoglycoside in patients with Pseudomonas aeruginosa infections.3 Patients with CF may also develop chronic kidney disease. Pharmacists are critical in making antibiotic renal dose adjustments to prevent toxicity.
Pharmacists can also monitor patients with advanced CF lung disease who require opioid therapy for pain management for adverse effects such as respiratory depression; provide education about drug administration, storage, and proper disposal; monitor bowel function for constipation prevention; and consult with pain and/or palliative care specialists.3 Patients with anxiety should be treated with a stepped-care model, using psychological interventions as first-line treatment and reserving short-term benzodiazepines for refractory symptoms—with close monitoring to prevent disease exacerbation and respiratory failure.3 Pharmacists should educate other health care providers to avoid coprescribing benzodiazepines and opioids, which interact and can lead to a drug overdose.
Medication adherence challenges in CF can lead to increased health care costs, disease complications, depression, anxiety, and hospitalizations.2 Studies have shown that CF medication adherence can be as low as 50%, especially in pediatric populations.2 Because daily administration of CF medication therapy can take between 1 and 2.5 hours,4 Including a pharmacist who can monitor regimen effectiveness, simplifying pharmacotherapy dosing schedules and ensuring medication access can improve outcomes in both inpatient and outpatient settings.2,5,6
Barriers to effective treatment can include poor motivation, suboptimal dose delivery due to inadequate training on inhalation technique, difficulty coping with CF diagnosis, poor understanding of the treatment regimen, and comorbidities.4
The Role of Specialty Pharmacy
Specialty pharmacists can provide 1-on-1 patient counseling for patients and caregivers to improve adherence through hospital discharge education and follow-up.6 Additionally, collaborative practice agreements are an important tool for pharmacist involvement on multidisciplinary CF teams. These agreements give pharmacists the authority to change drug therapy without physician oversight to ensure there are no delays in access to care.2 Patients with CF typically use multiple payers, such as primary and secondary insurances, along with specialty and mail-order pharmacies, to access medications.2 Because not all pharmacies stock common CF medications, therapy delays can occur. Pharmacists can assist with prior authorizations and take care of insurance-related issues to ensure that patients can access their medications in a timely manner.2 Pharmacists can also provide support in navigating patient assistance programs to help with CF drug therapy coverage.2 Compass, a personalized service provided through the Cystic Fibrosis Foundation, can assist patients with insurance issues and offer financial assistance for medical care.7
More than 130 accredited CF care centers, located at teaching and community hospitals across the United States, provide comprehensive treatment programs.1 In addition, AllianceRx Walgreens Prime is an approved specialty and mail service pharmacy that provides drug therapy services for CF.8 The AllianceRx Walgreens Prime Connected Care CF patient management program was established in 2014 and utilizes a multidisciplinary team that includes pharmacists, pharmacy technicians, patient care advocates, and insurance specialists. This team provides a range of services, including drug utilization reviews, patient counseling, medication refill reminders, drug administration and storage education, adverse effect monitoring, and financial assistance,4 and pharmacists receive comprehensive CF training on pharmacotherapy and disease-state management.4
Jennifer Gershman, PharmD, CPh, is a drug information pharmacist and medical writer residing in South Florida.
- What is cystic fibrosis? Cystic Fibrosis Foundation. Accessed April 5, 2022. https://www.cff.org/intro-cf/about-cystic-fibrosis
- Abraham O, Li JS, Monangai KE, Feathers AM, Weiner D. The pharmacists’ role in supporting people living with cystic fibrosis. J Am Pharm Assoc (2003). 2018;58(3):246-249. doi:10.1016/j.japh.2018.01.006
- Kapnadak SG, Dimango E, Hadjiliadis D, et al. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros. 2020;19(3):344-354. doi:10.1016/j.jcf.2020.02.015
- Impact of Walgreens Connected Care Cystic Fibrosis program on adherence and quality of life for enrolled CF patients. Walgreens. Accessed April 5, 2022. https://www.walgreens.com/images/adaptive/si/pdf/WalgreensConnectedCareCysticFibrosisWhitePaper.pdf
- Warda N, Rotolo SM. Virtual medication tours with a pharmacist as part of a cystic fibrosis telehealth visit. J Am Pharm Assoc (2003). 2021;61(5):e119-e125. doi:10.1016/j.japh.2021.04.005
- Abraham O, Morris A. Opportunities for outpatient pharmacy services for patients with cystic fibrosis: perceptions of healthcare team members. Pharmacy (Basel). 2019;7(2):34. doi:10.3390/pharmacy7020034
- Get help with CF Foundation Compass. Cystic Fibrosis Foundation. Accessed April 5, 2022. https://www.cff.org/support/get-help-cf-foundation-compass
- Newly published research shows cystic fibrosis patients stay on therapy, thanks to AllianceRx Walgreens Prime. News release. AllianceRx Walgreens Prime. May 24, 2021. Accessed April 5, 2022. https://www.alliancerxwp.com/news/newly-published-research-shows-cystic-fibrosis-patients-stay-on-therap
- Mehta Z, Kamal KM, Miller R, Covvey JR, Giannetti V. Adherence to cystic fibrosis transmembrane conductance regulator (CFTR) modulators: analysis of a national specialty pharmacy database. J Drug Assess. 2021;10(1):62-67. doi:10.1080.21556660.2021.11912352