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What is the median survival age for cystic fibrosis patients born between 2018-2022 in the US?
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56 years
What is considered an abnormal sweat chloride level in cystic fibrosis diagnosis?
Chloride levels >60 mmol/L
What pancreatic condition affects 2% of children and 50% of adults with cystic fibrosis?
Diabetes Mellitus
How does CFTR dysfunction affect sweat glands?
Chloride and sodium remain in sweat causing salty sweat and increasing the risk of dehydration
What nutritional support is recommended for cystic fibrosis patients?
50% excess calories, multivitamins, and enzyme replacement therapy for pancreatic insufficiency
What are some key respiratory care strategies for managing cystic fibrosis?
Vaccines, airway clearance methods, CFTR modulators, antibiotics, bronchodilators, mucolytics, and careful use of NSAIDs
Which diagnostic imaging is recommended for cystic fibrosis patients and how frequently?
CT every 1-2 years or during exacerbations
What is the inheritance pattern of cystic fibrosis?
Autosomal recessive
Which chromosome is the CFTR gene located on?
Chromosome 7
What is the role of the CFTR protein in normal epithelial cells?
Forms a cyclic AMP chloride channel that allows movement of chloride, bicarbonate, sodium, and water across epithelial membranes
What triggers suspicion for cystic fibrosis in a clinical setting?
Positive newborn screening, affected sibling, clinical manifestations, or dysfunctional CFTR genes
Name two common lung infections in cystic fibrosis patients.
Staphylococcus aureus (early life), Pseudomonas aeruginosa (60% adults)
What are the pulmonary consequences of cystic fibrosis?
Exacerbations, bronchiectasis, worsening lung function, hypoxemia, pulmonary hypertension, right-sided heart failure
What pulmonary function tests are recommended for cystic fibrosis patients older than 5 years, and how frequently should they be done?
Spirometry measuring FVC, FEV1, FEV1/FVC ratio, recommended 4 times per year
What percentage of cystic fibrosis cases are due to the F508 deletion variant?
85%
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