A diagnostic lab finding is a sweat chloride level > 60 mmol/L, as these patients cannot reabsorb chloride through their sweat glands.
Meconium is a black, tarry stool passed by newborns in their first 24 hours of life. Meconium ileus is something commonly seen in CF patients, as GI secretions are limited and patients form dry mucus. Thus, patients who have not passed meconium in their first 24 hours of life have meconium ileus, which is often a diagnostic tip-off to CF.
N-acetylcysteine is a medication given to patients with cystic fibrosis, as it loosens mucus plugs. This drug works by cleaving disulfide bonds within mucus glycoproteins.
Due to mucus stasis, these patients deal with recurrent pulmonary infections, and are typically given antibiotic prophylactic medications to help prevent infections.
Pulmonary maintenance in these patients. This includes therapy of percussive vibration vests to break and loosen mucus, BiPAP, and hypertonic saline to loosen secretions.
As lung function and exercise tolerance decreases, lung transplant is necessary in patients. Due to the proclivity to develop infection, both lungs must be transplanted.
Due to malabsorption and pancreatic insufficiency, these patients must take Vitamin replacement, especially those which are fat-soluble (A, D, E, and K).
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