CYSTIC FIBROSIS

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CYSTIC FIBROSIS

• Tyra Bradbury, MPH,RD,CSP
• Neonatal/Pediatric Dietitian

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Outline

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

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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

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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
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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
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Cystic Fibrosis - Diagnosis

• Newborn screen
• Sweat test
• Measures the amount of salt in the sweat
• Positive test - gt60 mEq/L
• Negative - lt40 mEq/L
• Genetic testing
• Used to confirm diagnosis
• Also used to test for carriers
• More than 1000 mutations

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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)
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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)
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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)
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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)
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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)
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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)

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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)
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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)
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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)
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Cystic Fibrosis Related Issues

• Pregnancy
• Lung function may suffer
• Vitamin A is teratogenic at high doses (gt8000
  IU/day)
• Transplant
• Good nutrition status key before transplant
• CFRD common secondary to steroids
• Pancreatic disease persists

Cystic Fibrosis Nutrition 101 (2009)
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Cystic Fibrosis - Resources

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