Cystic fibrosis (CF) is a chronic genetic disease that affects more than 30,000 children
and adults in the United States, and approximately 70,000 people worldwide. This disease
affects people of every racial and ethnic group. In people with CF, mutations in the cystic
fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to become
dysfunctional. When the protein is not working correctly, it is unable to help move chloride, a
component of salt, to the cell surface. Without the chloride to attract water to the cell surface, the
mucus in various organs becomes thick and sticky. This causes repeated lung infections and
gradually reduces breathing ability in affected patients. Cystic fibrosis is a chronic, lifelong
disease that requires treatment that changes with the patient's needs as they age. CF patients
frequently have recurring symptoms that impact their physical, social, and emotional health.
This disease affects the lungs, pancreas, upper airways, liver, intestine, and reproductive organs,
according to Naehrig et al. (2017). Obstruction, inflammation, infection (in the lungs and upper
airways), tissue reorganization, and loss of function are the outcomes. Other disease-modifying
genes affect the severity of CF. Early diagnosis and treatment improve quality of life for cystic
fibrosis patients. Modern medicine has extended the lives of CF patients. But much of the self-
directed care that improves health and quality of life must be done at home. Unfortunately, non-
adherence to treatment is the leading cause of treatment failure and death in cystic fibrosis
patients.
Cystic fibrosis is a disease that is difficult to manage due to its complexity. Individuals'
symptoms and severity can vary significantly. Numerous factors have an effect on an
individual's health and disease progression. If the screening test is positive, the patient should be
evaluated by a multidisciplinary team composed of physicians, physiotherapists, dieticians,
psychologists,
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