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.orgwww.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)