Introduction

Cystic fibrosis (CF) is an autosomal recessive disorder affecting the cystic fibrosis transmembrane conductance regulator (CFTR) gene.1 Patients with CF have a mutated gene, resulting in defective CFTR proteins causing thick mucus in multiple body systems.2 CF mainly affects the lungs and the digestive systems in the body.2 At present there is no cure for CF, so the aims of treatment are to delay disease progression with lifelong pharmacological and non-pharmacological interventions.3 CF pharmacists are involved in medicines optimisation, antimicrobial stewardship and medication counselling to improve treatment adherence.

Learning objectives

After successful completion of this CPD activity, pharmacists should be able to:

  • Define cystic fibrosis
  • Describe the pathophysiology of cystic fibrosis
  • Discuss the management of patients with cystic fibrosis
  • Identify the role of the pharmacist in managing a patient with cystic fibrosis.

Competency standards (2016) addressed: 1.1, 1.3, 1.4, 2.1, 2.3, 2.4, 3.2

Extended learning activity

After additional reading and research from recommended resources, pharmacists should be able to:

  • Identify drug-drug interactions and modify treatment regimens when managing people with cystic fibrosis
  • Assess the pharmacokinetic and pharmacodynamic properties of drugs used in cystic fibrosis when formulating treatment options
  • Discuss the drug treatment used for managing cystic fibrosis, including the management of adverse drug reactions.

Competency standards (2016) addressed: 1.1, 1.3, 1.4, 1.5, 2.2, 2.4, 3.1, 5.3

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