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NUTRITION IN PULMONARY DISEASE. CYSTIC FIBROSIS( CF ): - An autosomal recessive disorder. Affects every organ with epithelial surface ,including: respiratory tract, sweat and salivary glands, intestine, pancreas, liver, and reproductive tract. The mutation results in dysfunction in the exocrine glands and production of abnormal thick secretion that obstructs glands and ducts in various organs . The chromosomal defect will also affect the production of a protein carrier involved in the transport of chloride ions. This will result in complications, as: bronchitis, pneumonia, maldigestion, malabsorption, impaired glucose tolerance, and lactose intolerance. Pt’s with CF are at high risk on malnutrition. Factors affecting adequate nutrient intake : dyspenia, cough, cough-induced vomiting, gastrointestinal discomfort, anorixa during episode of infection. Pancreatic enzyme replacement therapy is used to correct maldigestion and malabsorption. NUTRITIONAL MANAGEMENT: Goal: - To control malabsorption and maldigestion. Provide adequate nutrients to promote optimal growth or to maintain weight for height and pulmonary function, along with enhancing the quality of life. Diet: - High-calorie and high-protein diet with liberal amount of fat and NaCl+. Description: - A High-calorie and high-protein diet consists of foods that are calorie and protein dense. Serve small frequent meals with snacks. In case of SOB, provide soft easy to eat foods and beverages. Encourage companionship at mealtime. Supplementation (oral/ tube feeding) is considered for those who are unable to meet orally. Elemental and non-elemental formula with enzymes are used for adults , and for formula-fed infants, a standard (20-27 kcal/oz) formula is given along with supplemental enzymes. Hydrolyzed formula with MCT oil may also be used. 1 Kcal: 2 Protein: - When energy needs are adequately met, protein requirements is 15-20 % of Kcal. OR RDA’s for same age, sex and height. Serve HBV proteins. 3 Fat: - 30-40 % of Kcal or more as tolerated. If fat intolerance is evident, MCT oil is beneficial. Vitamins and minerals: - Supplementation of fat-soluble vitamins. Na+ requirements increases due to high losses in sweat. Especially in periods of fever, hot weather. (infant :1/8-1/4 tsp/day, older children and adults : unrestricted salt) Ca+ and zinc supplements. CHRONIC OBSTRUCTIVE PULMONARY DISEASE (COPD ): - A condition characterized by presence of chronic bronchitis, emphysema, or both, leading to the development of airway obstruction. Smoking is the most important causative factor Depletion appears to be related to decrease food intake, air flow obstruction, CO2 retention, and decrease respiratory and limb muscle strength. NUTRITIONAL MANAGEMENT: The following guidelines are also indicated for chronic bronchitis, emphysema, fibrosis, adult respiratory distress syndrome (ARDS) and acute respiratory failure. Goal: - To preserve or restore lean body mass without affecting respiratory status and to promote gradual, safe weight loss. Correct fluid imbalance, and manage drug-nutrient interaction. To facilitate weaning from mechanical ventilator. Diet: - If weight is normal: regular diet with adequate fluid intake (2-3 L/day). Underweight/malnourished: High-calorie and high-protein diet with adequate fluid intake (2-3 L/day). Overweight: energy-restricted diet to promote gradual weight loss if appropriate. Description: - A soft easy to eat diet is recommended. Incase of abdominal discomfort and bloating, eliminate gas- forming foods. Serve small frequent meals and calorie dense snacks . Supplementation with 2kcal / ml formulas through (oral/ tube feeding) is considered for those who are unable to meet orally. 4 - Special care should be taken to reduce aspiration risk. Kcal: - Calculate using BEE /REE X activity factor X stress factor. Excess energy intake (>1.5 X BEE) is not recommended. Overfeeding critically ill pt’s can lead to respiratory and cardiac complications. Excess energy is more significant to the production of CO2 that Fat : CHO ratio. Observe for edema , and Adjust weight if % IBW >126%. Protein: - Should be assessed individually. 1- 1.5 gm / Kg dry weight or 15 – 20 % of Kcal. Fat: - 30-40 % of Kcal . CHO: - 40 -55 % of Kcal. Fluid and Electrolytes: - Some pt’s with Cor pulmonale and fluid retention may require fluid and sodium restriction and increased potassium intake. 5