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Cystic Fibrosis - Coggle Diagram
Cystic Fibrosis
Organ effects
Lung. Low Cl secretion into airway lumen = dense surfactant, low mucociliary clearance, infection, inflammation
Pancreas. Low Cl secretion into pancreatic ducts, dehydration/ductal blockage, insufficient enzymes production = maldigestion, malabsorption, gallstones, diabetes (fibrosis = islet cell atrophy like T1, although presents like T2)
Intestine. Low bicarb/Cl secretion in gut, unbuffered gastric secretion. Possible obstruction
Liver. Low Cl secretion in bile ducts, bile acid retention, ductal obstruction = cirrhosis, portal hypertension, oesophageal varices
GI complications. high viscosity of intestinal mucous, altered motility, gastro-oesophageal reflux
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Disease nature
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Overview
Affects the cells that produce mucus, sweat and digestive juices.
Thick and stick fluids ... plug up tubes, ducts and passageways
Severity dx
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Moderate - cough, dyspnoea, FEV1 40-59% predicted
Severe - chronic cough, sputum production, dyspnoea w ADLs, FEV1<40% predicted
General Symptoms
Steatorrhea, abdominal cramps, weight loss, chronic cough, dry mouth, fatigue, low appetite, low concentration
Nutritional Mx
PERT
Aims to correct malabsorption. pH 5.5 for activation (therefore wont achieve 100% specificity due to bicarb/Cl insufficiency)
Clinical guide: 500-4000U lipase per g dietary fat. Max 10,000U.
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1 Creon (25,000) per 25g of fat
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Fat-soluble vitamin deficiencies (ADEK) supplementation PRN, regular r/v
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Energy imbalance
Increased energy expenditure - inflammation, increased work for lungs (mucous/inflamed airways), infection.
Maldigestion, Malabsorption - exocrine insufficiency, hyperacidity, intestinal mucous
Inadequate intake - poor appetite, disease related anorexia (TNF-a/cytokines), dyspnoea, early satiety, dry mouth, taste changes, behavioural factors
Liaisation
CF gastroenterologist - obstructions, constipation, GORD, liver disease
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