lOMoARcPSD|17706574
CYSTIC FIBROSIS
Pathophysiology
Cystic brosis is an inherited condition which caused damage to the
respiratory, digestive and other body systems. It is due to mutated CFTR
proteins, a chloride channel used to regulate salt and water balance; resulting
in increased sodium and water resorption, which causes thickened mucous
secretions (Sankari & Sharma, 2024). These secretions cause blockages along
linings and tissue.
Researchers have found that this disease affects one in 3500 of Northern
EPIDEMIOLOGY AND European descent and 1 in 15,000 Black patients (Sankari & Sharma, 2024). It
INCIDENCE is an autosomal recessive disorder, occurring primarily in children, in which
the defective gene must be inherited from both parents (Cystic Fibrosis
Foundation, 2025).
CLINICAL Salty tasting skin, persistent cough, wheezing, shortness of breath, poor
PRESENTATION growth or weight, frequent and greasy bulky stools, clubbing, nasal polyps
(Cystic Fibrosis Foundation, 2025).
NURSING Sweat chloride test will be performed; chest radiography to identify
ASSESSMENT AND abnormalities; monitoring progression of disease with pulmonary function
INTERVENTIONS tests. Nursing can also provide education on the various medical interventions
listed below:
MEDICAL Pulmonary rehab and chest physical therapy such as breathing technique to
MANAGEMENT loosen mucus; learning airway clearing techniques; Medications to target
genetic dysfunction i.e. cystic brosis transmembrane conductance regulator
PHYSICAL, (CFTR) modulators (Mayo Clinic, 2025).
PSYCHOLOGICAL AND Cystic Fibrosis (CF) has no cure, which can be dif cult for any child or parent
EMOTIONAL IMPACT to understand. Another major aspect of CF is maintaining a diet regiment to
ON INDIVIDUAL AND accommodate for digestion complications. Patients will be advised to take
FAMILY pancreatic enzymes with every meal and snack, to increase caloric intake,
consume extra salt and ber, and to maintain hydration (Mayo Clinic, 2025).
It’s important to uplift families and educate them on how far CF treatment has
EMOTIONAL AND come.
PSYCHOSOCIAL Cystic Fibrosis Foundation: https://www.cff.org/intro-cf/about-cystic- brosis
RESOURCES FOR Cystic Fibrosis support group for families: https://clairesplacefoundation.org/
INDIVIDUAL AND family-resource-support
FAMILY Everyday CF: https://www.everyday-cf.com/
Downloaded by Nicholas Marks ()
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CYSTIC FIBROSIS
Pathophysiology
Cystic brosis is an inherited condition which caused damage to the
respiratory, digestive and other body systems. It is due to mutated CFTR
proteins, a chloride channel used to regulate salt and water balance; resulting
in increased sodium and water resorption, which causes thickened mucous
secretions (Sankari & Sharma, 2024). These secretions cause blockages along
linings and tissue.
Researchers have found that this disease affects one in 3500 of Northern
EPIDEMIOLOGY AND European descent and 1 in 15,000 Black patients (Sankari & Sharma, 2024). It
INCIDENCE is an autosomal recessive disorder, occurring primarily in children, in which
the defective gene must be inherited from both parents (Cystic Fibrosis
Foundation, 2025).
CLINICAL Salty tasting skin, persistent cough, wheezing, shortness of breath, poor
PRESENTATION growth or weight, frequent and greasy bulky stools, clubbing, nasal polyps
(Cystic Fibrosis Foundation, 2025).
NURSING Sweat chloride test will be performed; chest radiography to identify
ASSESSMENT AND abnormalities; monitoring progression of disease with pulmonary function
INTERVENTIONS tests. Nursing can also provide education on the various medical interventions
listed below:
MEDICAL Pulmonary rehab and chest physical therapy such as breathing technique to
MANAGEMENT loosen mucus; learning airway clearing techniques; Medications to target
genetic dysfunction i.e. cystic brosis transmembrane conductance regulator
PHYSICAL, (CFTR) modulators (Mayo Clinic, 2025).
PSYCHOLOGICAL AND Cystic Fibrosis (CF) has no cure, which can be dif cult for any child or parent
EMOTIONAL IMPACT to understand. Another major aspect of CF is maintaining a diet regiment to
ON INDIVIDUAL AND accommodate for digestion complications. Patients will be advised to take
FAMILY pancreatic enzymes with every meal and snack, to increase caloric intake,
consume extra salt and ber, and to maintain hydration (Mayo Clinic, 2025).
It’s important to uplift families and educate them on how far CF treatment has
EMOTIONAL AND come.
PSYCHOSOCIAL Cystic Fibrosis Foundation: https://www.cff.org/intro-cf/about-cystic- brosis
RESOURCES FOR Cystic Fibrosis support group for families: https://clairesplacefoundation.org/
INDIVIDUAL AND family-resource-support
FAMILY Everyday CF: https://www.everyday-cf.com/
Downloaded by Nicholas Marks ()
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