On completion of the chapter, the reader will be able to:
Recognize the mutation most commonly identified in this population is ΔF508.
Define the basic genetic defect that causes cystic fibrosis (CF).
Discuss the differences between classic and nonclassic clinical presentations of CF.
Interpret the use of the sweat chloride tests, and what values are considered abnormal.
List the various goals that pertain to each organ system.
Create an airway clearance therapy routine and discuss its components.
Explain the importance of antiinflammatory therapies utilized in CF.
List the various pathogens and their treatments that commonly colonize the airways of the CF patient.
Discuss the pharmacokinetic differences that apply to CF patients.
Identify the potential issues that older CF patients may encounter.
Explain the rationale behind the use of insulin for the treatment of cystic fibrosis-related diabetes (CFRD).
Formulate appropriate counseling regarding antibiotic therapy for the pregnant CF patient.
Describe the various controversial therapies for CF patients.
Critique new therapies being developed for the treatment of CF.
Discuss social and quality of life issues that impact the life of a CF patient.
“Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die.” This European adage accurately describes the fate of an individual diagnosed with cystic fibrosis during ancient times.1
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 disorder in the Caucasian population, with an incidence of 1 in 2000 to 4000 live births and a prevalence of 30,000 affected individuals in the United States.2–7
Currently with care, affected individuals have an expected life span of 36 years of age. Multiple organ systems are affected in CF individuals, especially, the lungs, the digestive system, and the reproductive organs. Mortality is most commonly due to chronic organ damage or resistant pulmonary infections.8
The pharmacist plays an essential role in the development and management of a pharmacotherapeutic care plan for the CF patient.
Cystic fibrosis occurs in approximately 1 in 3,500 newborns. In the 1970s, patients only survived into their teen years. By 2006, progress in care had extended survival to 36 years. Institution of care at a young age impacts long-term survival; hence, timing of diagnosis and recognition of signs and symptoms are crucial.2–7
Although CF occurs in all ethnicities, other ethnicities besides the Caucasian population display lower frequencies: ...