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 mEq/L – Negative - <40 mEq/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 mEq/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/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)

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 24-48hrs 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)

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)