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CYSTIC FIBROSIS

CYSTIC FIBROSIS. Tyra Bradbury, MPH,RD,CSP Neonatal/Pediatric Dietitian. Outline . Arizona Newborn Screening Definition Diagnosis Nutrition Estimated calories and protein needs Salt Enzymes Types and dosing CF related issues. Outline. Resources Families Websites CF centers

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CYSTIC FIBROSIS

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  1. CYSTIC FIBROSIS Tyra Bradbury, MPH,RD,CSP Neonatal/Pediatric Dietitian

  2. Outline • Arizona Newborn Screening • Definition • Diagnosis • Nutrition • Estimated calories and protein needs • Salt • Enzymes • Types and dosing • CF related issues

  3. Outline • Resources • Families • Websites • CF centers • Healthcare providers • Websites • CF centers • Explanation of the project currently being worked on • Community/rural education • Nutrition practice guidelines

  4. Arizona Newborn Screening • All babies born in Arizona • Blood spot screening – 28 disorders • Hearing screen • CF screening in Arizona began in November of 2007 • 46 mutations www.AZNewborn.com

  5. Cystic Fibrosis - Definition • Genetic disease affecting the mucus glands in the liver, lungs, pancreas and intestines • Defective gene produces a protein that causes thick mucus to accumulate • Affects 1 in every 3000 Caucasian babies born • 30,000 people in the US (70,000 worldwide) www.cff.org

  6. Cystic Fibrosis - Diagnosis • Newborn screen • Sweat test • Measures the amount of salt in the sweat • Positive test - >60 mEq/L • Negative - <40 mEq/L • Genetic testing • Used to confirm diagnosis • Also used to test for carriers • More than 1000 mutations

  7. Cystic Fibrosis - Nutrition • Calories • 1.2-2 times the DRI for age • Goal is sustained weight gain • Tips: • Infants – higher calorie breast milk/formulas • Children and adults • Butter or margarine added to foods • Add oils, cheese, heavy cream • Whole milk • High calorie snacks – supplements, flavored syrups on ice cream Cystic Fibrosis Nutrition 101 (2009)

  8. Cystic Fibrosis - Nutrition • Protein • 1.5-2 times the DRI for age • Fat • 35-40% of calories • Hyperlipidemia generally not a risk Cystic Fibrosis Nutrition 101 (2009)

  9. Cystic Fibrosis - Nutrition • Vitamins • Fat soluble vitamin needs increased • Require water miscible forms • Serum levels assessed annually • Sodium • Salt lost through sweat • Infants with CF • 2-4 mEq/day = 1/8-1/4 teaspoon daily added to breast milk/formula Cystic Fibrosis Nutrition 101 (2009)

  10. Cystic Fibrosis – Enzyme Replacement • 85-90% pancreatic insufficient • Usually in capsule form with small beads inside (enteric coating) • Swallow capsules whole or beads mixed in acidic foods • Take before (and sometimes during) meal, not after • Never take generic enzymes Cystic Fibrosis Nutrition 101 (2009)

  11. Cystic Fibrosis – Enzyme Dosing • Infants • 1000 Units lipase/kg/meal • 2000-4000 Units lipase/120ml breast milk or formula • Children • 1000 Units lipase/kg/meal • 500 Units lipase/kg/snack • Older Children and Adults • Start with 500 Units lipase/kg/meal • Maximum dose • 2500 Units lipase/kg/meal • 10,000 Units lipase/kg/day Cystic Fibrosis Nutrition 101 (2009)

  12. Cystic Fibrosis - Enzymes • Creon (second smallest microsphere) • Creon 5 = 5000 units lipase/capsule • Creon 10 = 10,000 units lipase/capsule • Creon 20 = 20,000 units lipase/capsule • Pancrease MT • 4, 10, 16 and 20 (number x 1000 = units lipase/capsule) • Ultrase • 4500 units/capsule • Ultrase MT • 12, 18 and 20 (number x 1000 = units lipase/capsule)

  13. Cystic Fibrosis - Enzymes • What if patient is at max dose and still symptomatic? • Acid blockers • Switch brand of enzyme • ? non-adherence (with snacks/drinks/etc) • What else causing loose stools? • Refer to GI Cystic Fibrosis Nutrition 101 (2009)

  14. Cystic Fibrosis – Related Issues • Meconium ileus • Ileum completely obstructed • No stool at 24-48hrs old, emesis, abdominal distention • Enemas vs surgery • CFRD • Usually diagnosed after 18 years old (using OGTT) • Insulin treatment common • Do not follow usual dietary advice Cystic Fibrosis Nutrition 101 (2009)

  15. Cystic Fibrosis – Related Issues • Bone disease • Low body weight, vitamin D deficiency, delayed puberty, lung disease • Tube feedings • ~50% of needs • 8-10 hours overnight • Intact formulas • Meal enzyme dose before and after Cystic Fibrosis Nutrition 101 (2009)

  16. Cystic Fibrosis – Related Issues • Pregnancy • Lung function may suffer • Vitamin A is teratogenic at high doses (>8000 IU/day) • Transplant • Good nutrition status key before transplant • CFRD common secondary to steroids • Pancreatic disease persists Cystic Fibrosis Nutrition 101 (2009)

  17. Cystic Fibrosis - Resources • Cystic Fibrosis Foundation • www.cff.org • Cystic Fibrosis Nutrition 101 • Amanda Leonard, MPH, RD, CDE – The Johns Hopkins Children’s Center • Terri Schindler, MS, RD – Rainbow Babies and Children’s Hospital • University of Arizona • Pediatric Pulmonary Center (PPC)

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