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KEY CONCEPTS

KEY CONCEPTS

  • image Good nutrition with appropriate pancreatic enzyme and vitamin supplementation are essential in the management of cystic fibrosis (CF).

  • image Airway clearance and anti-inflammatory therapies are key components to improve pulmonary health in CF patients.

  • image Antipseudomonal agents are the cornerstone of antibiotic therapy for chronic lung infections in the CF patient.

  • image Altered pharmacokinetics of CF patients can impact the dosing and clearance of pharmacologic therapy.

PRECLASS ACTIVITY

Preclass Engaged Learning Activity

Read Novak KJ. Chapter 33: Cystic Fibrosis: Blood, Sweat, Lungs and Gut Level II. In: Schwinghammer TL, Koehler JM, Borchert JS, Slain D, Park SK, eds. Pharmacotherapy Case Book, 10th Edition. New York, NY: McGraw-Hill Education, 2017.

This practice case gives a real-life example of a typical CF patient who might present to a clinic or hospital, the medications that are typically used, and monitoring that should be performed. This case will help develop the learner's understanding about the COLLECT, ASSESS, and PLAN steps in the patient care process.

INTRODUCTION

Cystic Fibrosis (CF) is a disease state resulting from a dysfunction in the cystic fibrosis transmembrane conductance regulator (CFTR). It is the most common life-limiting genetic disorder in Caucasians with a prevalence of 30,000 affected individuals in the United States. Historically, the saying “Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die1” would describe the ill fate of an individual affected with CF in the Middle Ages. With available treatments, affected individuals currently have an expected life span of approximately 40 years. Multiple organ systems are affected in individuals with CF, especially the lungs, the digestive system, and the reproductive organs. Mortality is most commonly due to respiratory/cardiorespiratory causes and complications of organ transplant.2

EPIDEMIOLOGY

CF occurs in approximately 1 in 2,500 to 3,500 Caucasian newborns. Although CF occurs in all ethnicities, the frequencies in other populations is much lower: 1 in 17,000 African Americans and 1 in 31,000 Asians.1 In the 1970s, patients only survived into their teen years. In 2013, progress in care had extended survival to 43 years. Initiating appropriate treatment at a young age impacts long-term survival. Hence, the timing of diagnosis and recognition of signs and symptoms are crucial.2–7

ETIOLOGY

CF is caused by a mutation of the CFTR gene. Extensive genetic studies have increased awareness regarding the large spectrum of mutations in the CF population. Over 2,000 mutations have been identified through the extensive collaboration between the CF Foundation and international researchers. The most common mutation identified in CF patients is ΔF508.4

CF is an autosomal recessive disease, in which one mutation present on each allele of the CFTR gene results in presentation of the disease. The presentation of a mutation on only one ...

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