CYSTIC FIBROSIS Tyra Bradbury MPH RD CSP NeonatalPediatric

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 – Healthcare providers • Websites • CF centers • Explanation of the project currently being worked on – Community/rural education – Nutrition practice guidelines

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

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

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

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)

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)

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 m. Eq/day = 1/8 -1/4 teaspoon daily added to breast milk/formula Cystic Fibrosis Nutrition 101 (2009)

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)

Cystic Fibrosis – Enzyme Dosing • Infants – 1000 Units lipase/kg/meal – 2000 -4000 Units lipase/120 ml 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)

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)

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)

Cystic Fibrosis – Related Issues • Meconium ileus – Ileum completely obstructed – No stool at 24 -48 hrs 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)

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)

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)

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)