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Cystic Fibrosis

Cystic Fibrosis. Kelsey Leach, RD. What is Cystic Fibrosis (CF)?. A recessive genetic disease Effects the exocrine glands Abnormality in the CF transmembrane conductase (CFTR) Decrease in Cl ¯ secretion –> which causes the production of mucus –> which effects some organs.

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Cystic Fibrosis

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  1. Cystic Fibrosis Kelsey Leach, RD

  2. What is Cystic Fibrosis (CF)? • A recessive genetic disease • Effects the exocrine glands • Abnormality in the CF transmembrane conductase (CFTR) • Decrease in Cl¯ secretion –>which causes the production of mucus –> which effects some organs

  3. Incidence/Stats of CF • Mostly in Caucasians • 1 in 2500 – 3500 live births • 1 in 20 are carriers • Girls tend to die at a younger age from CF than boys. • Life expectancy now reaching to 30 years

  4. Screening for CF • Sweat test • Measures Cl¯ levels. >60mmol/L = CF • For these characteristics: • 1. Chronic sinopulmonary disease • 2. Gastrointestinal and nutritional abnormalities • 3. Salt loss syndromes • 4. Obstructive azospermia in males • Sibling has the disease

  5. “The child will soon die whose brow tastes salty when kissed” Almanac of Children’s Songs and Games from Switzerland 1857

  6. Pulmonary Chronic cough Repeated infections Inc work of breathing Clubbing of fingers Bronchospasm Cyanosis Chronic pneumonia Nasal polyps Gastrointestinal FTT Steatorrhea Hypoalbuminemia Rectal prolapse Frequent, foul stools Abdominal cramps Voracious appetite Anemia Vit deficiency Manifestations of CF

  7. Lungs • Mucus dec. breathing and causes obstruction. Also a great breeding ground for bacteria • Lots of antibiotics to dec. bacterial growth • Chronic use of antibiotics dec. gut flora, supplement Vit K • P. aeruginosa assoc. w/ anorexia, inc met rate, and E req • Increase work for breathing, increase E needs • Chronic cough • Chest physiotherapy

  8. Pancreas • Blocked ducts –> dec in enzyme release • Need enzyme therapy (for most CF people) • Take when eat anything • Because of malabsorption problems need supplements of Vit/Min, especially fat soluble vit. • May lead to diabetes

  9. Enzyme therapy • Take w/in 1 hr before meal, not after • For those who can’t swallow pill, can mix w/ low acid food (i.e., applesauce) • <4 yr = 2000-4000 units/ 120mL BM or formula OR 1000 u/kg/meal • >4 yr = 500 u/kg/meal • No >2500 u/kg/meal because may cause fibrosing colonopathy

  10. Other organs/Problems • Easily overheat and dehydrate • Digital Clubbing • As disease progresses: • Liver failure- from clogged ducts • Heart failure- effects of chronic pulmonary hypertension • Sterility in all males and some females

  11. CF that affects nutritional status • Low albumin • Loss of Na and Cl • Pancreatic enzyme therapy • Vit/ Min supplementation • Energy deficit • Due to Pulm function

  12. Energy needs • Children with (CF) are at nutritional risk due to impaired absorption and increased energy needs. • 120%-150% of the RDA for energy • They have inc. REE • Diet usually high in fat (40%) • “Typical” American diet is high in protein, therefore CF person usually gets enough pro

  13. Goal of Nutrition therapy • Overcome the energy deficit and promote normal growth and development for CF patients

  14. Infants • Breastfeeding or formula • BM lower in protein, but many benefits. • One study showed BF CF babies weighed more • Special formulas for gut resection/fat malabsorption = Alimentum and Pregestimil • May be malnourished • Have high energy needs, highest growth rate • Need enzyme therapy, fluoride supplementation, and vit. suppl

  15. Infants Cont • May need salt suppl, esp. BF infants • Start solids at appropriate age • Problems with GER can dec intake • Digestive problems with those who have bowel resection • Pancreatic Insufficiency • 50% at birth • 80-90% by 1 year

  16. Toddlers • Variable intake • Warn parents of lower intake • Have scheduled meals and snacks • Continue vit/min suppl • Avoid excessive snacking (grazing) • Monitor growth velocity

  17. School Age • More responsibility of diet/ and treatment • May still need some help, esp. younger children, but they should become more involved in taking enzymes/ vitamins • Involve school in need to take enzymes with meals • Some peer pressure + self-management = dec compliance

  18. Adolescents • More independence • Need more kcal for increased growth/activity • Those with poor lung function have harder time with maintaining energy needs. • Females more likely to develop under nutrition and growth failure b/c lean body mass. • Peer pressure/influence may be a problem.

  19. Adults • Maintain energy needs • Weight loss associated with decreasing lung function • Still need enzyme therapy and vit/min suppl • Usually solely responsible for disease management.

  20. Pregnancy/BF with CF • Can be accomplished • Need adequate weight gain. • Prenatal vitamins on top of usual vit/min suppl • BF possible, inc E needs • Monitor nutr in preg and lactation

  21. Early diagnosis • Farrell et al. Pediatrics 2001; 107:1-13 • Children who were diagnosed early with CF, 13 weeks of age compared to 107 weeks at initial diagnosis, had better growth than children diagnosed later (p<0.001). • Length (p<0.001), weight (p=0.027), and head circumference (p=0.003) were significantly higher in the early diagnosed group compared to the later diagnosed group.

  22. Transplants • Luca A et al. Ann Thorac Surg 2002; 74:13-8 • 64 people with CF- 22 HLTx and 42 BLTx • 1.6% died in operation • Survival rate 1 yr 93.2%, 3 yr 77.7%, 5 yr 61.8%, and 10 yr 48.1% • No sig between HLTx and BLTx in survival rate • Long term survivors have good pulm function and quality of life; survival is limited by infections and bronchoilitis obliterans.

  23. Growth • Stettler N et al. AJCN 2000; 72(2): 407-13 • 3- yr study • Growth of preadolescent children with CF and compared it to the growth of children without CF • The study was inclusive of all children above the 3rd percentile for growth. • Rate of the height of the boys with CF was slower than the control group (p=0.004). There were also significant differences in fat-free mass for the boys. Skinfold thickness was different by p=0.008 and total body water was significantly lower by p=0.02 in the boys with CF.

  24. Growth Cont • Routine nutr screening and assess important • Most are malnourished at dx • Growth an issue due to high E needs and inc malabsorption • May need calorie boosters or suppl

  25. References • Article: • Ramsey BW, Farrell PM, Pencharz P, and the Consensus Committee. Nutritional assessment and management in cystic fibrosis: a consensus report. Am J Clin Nutr 1992; 55:108-16. • Books: • Alex: The Life of a Child • Handbook of Pediatric Nutrition

  26. Questions? Thank you

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