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.comwww.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.orgwww.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 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 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 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 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/dayCystic Fibrosis Nutrition 101 (2009)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 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 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 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 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)