Rhianna Cenci, Sodexo Dietetic Intern

Objectives

 Overview of CF and Treatments

 CF Medical Nutrition Therapy

 CF Case Study

Cystic Fibrosis (CF) Overview

  Inherited chronic disease  Produces unusually thick and sticky mucus

secretions  Affects mainly lungs and pancreas  Affects gastrointestinal (GI) tract, bile ducts

and liver, sweat glands, and other exocrine glands

 Nicknamed “65 roses”

CF Symptoms   Meconium ileus

  Persistent coughing with wheezing and phlegm   “Salty” skin

  High levels of NaCl in saliva, tears, sweat

  Recurrent lung infections   Shortness of breath   Poor growth and weight gain

  Greasy, bulky stools   Clubbed fingers

CF Statistics

 More than 70% of cases diagnosed by age 2   1,000 new cases diagnosed each year  Affects 30,000 Americans  Affects 70,000 people worldwide  Affects 1 in 3,200 Caucasians  More than 45% of the CF population is age

18 or older  Predicted median age of survival for a

person living with CF is in the early 40s

CF Genetics

 Autosomal recessive disease   2-5% of all Caucasians carry the CF gene  Carriers of the CF gene:

  1 in 29 Caucasian Americans

  1 in 46 Hispanic Americans   1 in 65 African Americans

  1 in 90 Asian Americans

 Genetic carrier testing

CF Genetics C c

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CF Genetics

 Gene on chromosome 7  Cystic fibrosis transmembrane regulator

(CFTR) protein   1,800 mutations of the CFTR gene have

been found  G542X, G551D, Delta F508, R117H

CF Diagnosis

 Newborn screening (NBS) at birth

  Tests for up to 50 diseases

  Immunoreactive trypsinogen (IRT)

○  High in babies with CF, stressful delivery, and premature birth

 Need a diagnostic test if results are positive

CF Diagnosis  Sweat test

  “Gold standard”  Measures amount of

chloride in sweat  Diagnosis: High amounts

of salt (NaCl) ○  Chloride >60 mEq/L

  Inconclusive results need more testing  Repeat sweat test, gene

analysis, sputum cultures

CF Complications

 Gastroesophageal reflux disease (GERD)   25-100% of CF patients  Coughing can make it worse  Medications to treat

 Pancreatic insufficiency (PI)   85-90% of CF patients   Pancreatic enzyme replacement therapy (PERT)

 Pancreatitis

CF Complications

 CF-related diabetes (CFRD)   5-15% of patients will develop CFRD   40% of CF patients have some glucose

intolerance   Associated with decline in nutritional status

and overall health  Decreased bone density   Infertility in males  Decreased bile flow, cirrhosis of liver

CF Treatments  Pancreatic enzyme replacement therapy

(PERT)   Lipase, protease, amylase  Dosage based on age and individual response  Recommended: ○  <4 yrs: 1,000 lipase units/kg/meal ○  4+ yrs: 500 lipase units/kg/meal

  Should not exceed: ○  2,500 lipase units/kg/meal ○  10,000 lipase units/kg/day

CF Treatments

 Airway clearance techniques (ACTs)  Manual chest physiotherapy (CPT)  High frequency chest compression (HFCC)   Active-cycle breathing techniques (ACBT) ○  Huff coughing

  Acapella

 Nebulizer, inhalers, medications

CF Treatments

CF Treatments

  Lung transplantation   For end-stage lung disease  Risk of rejection  Weight criteria for adults ○  80-130% of IBW or BMI 18.5-30 kg/m2

 Most aggressive therapy

 CF Foundation-accredited care centers

CF Medical Nutrition Therapy

 Calories  Recommended Dietary Allowances (RDAs)  WHO equations for REE   Schofield equations for REE

  Adjust with activity/stress factor   110-200% or 120-150% of normal caloric needs,

based on different sources

  Infants: 200 kcal/kg, Children: 150 kcal/kg

CF Medical Nutrition Therapy Age Gender WHO Schofield 0-3y M 60.9W* - 54 0.17W* + 15.17H* – 617.6

F 62.0W - 51 16.25W + 10.232H – 413.5 3-10y M 22.7W + 495 19.6W + 1.303H + 414.9

F 22.5W + 499 16.97W + 1.618H + 371.2 10-18y M 17.5W + 651 16.25W + 1.372H + 515.5

F 12.2W + 746 8.365W + 4.65H + 200.0

*W is weight in kilograms, H is height in centimeters

Recommended Dietary Allowances (RDAs) Infants 0-6 months 108 kcal/kg 6-12 months 98 kcal/kg Children 1-3 years 102 kcal/kg 4-6 years 90 kcal/kg 7-10 years 70 kcal/kg Adolescent Male 11-14 years 55 kcal/kg or 17 kcal/cm height 15-18 years 45 kcal/kg or 17 kcal/cm height Adolescent Female 11-14 years 47 kcal/kg or 14 kcal/cm height 15-18 years 40 kcal/kg or 13.5 kcal/cm height

CF Medical Nutrition Therapy  Protein

  10-35% of calories, or 15-20% of calories   110-200% of normal protein needs

 Carbohydrates   45-65% of calories   Follow USDA MyPlate recommendations

  Fat   20-30% of calories, or 35-40% of calories   Encourage omega-3 (DHA, EPA) to reduce

inflammation

CF Medical Nutrition Therapy

  Fluids  Normal pediatric needs  Holiday-Segar method

Body Weight Fluid Need 0-10 kg 100cc/kg 11-20 kg 1000cc + 50cc/kg>10kg >20 kg 1500cc + 20cc/kg>20kg

CF Medical Nutrition Therapy

  Fat-soluble vitamins

  Vitamins A, D, E, K

 Replaced with 2x RDA

 Water-soluble multivitamin brands

 Water-soluble vitamins

 Deficiencies rare

 Multivitamin will meet needs

CF Medical Nutrition Therapy  Sodium chloride (NaCl)

 Need to supplement   Birth-6 months: 1/8 tsp salt daily   >6 months: 1/4 tsp salt daily  More for physically active, up to 4-6g/day

  Zinc  Replete 1 mg/kg/day for 6 months for

excessive losses in pancreatic insufficiency   Iron  Calcium

Case Study RW   RW   9 years old   Caucasian male   NKA   Failure to thrive (FTT)

as an infant   Diagnosed at 7 months

of age   Delta F508 mutation

Past medical history:   FTT   Asthma, chronic rhinitis   Pancreatic insufficiency   GERD with Nissen

fundoplication & several repairs

  Malnutrition   Adenotonsillectomy   Tympanostomy tubes   Previous PEG tube

placement & removal

Case Study RW   Complicated home life

  Many siblings (one diagnosed with CF)

  Parents divorced

  Recent depression and anxiety   Grades went down

  Loves video games

Case Study RW: Hospital Admission

  Admitted to hospital for 15 days

  Shortness of breath   Cough   Wheezing   Declining weight   Not taking nutritional

supplements   Will need PEG tube

  Height:   10-25th percentile

  Weight:   10-25th percentile

  BMI: 15.2   25th percentile

  %IBW: 93%

Case Study RW: Medications   Flonase (fluticasone)

  1 nasal spray 1x/day   Albuterol (Proventil)

  2 puffs every 4 hrs as needed

  2 inhalations 2x/day with vest therapy

  Claritin (loratadine)   10mg 1x/day

  MiraLax (polyethylene glycol)   17g 2x/day

  Singulair (montelukast)   4mg 1x/day

  Protonix (pantoprazole)   20mg 1x/day

  Zenpep (pancrelipase)   3 with meals, 2 with

snacks/supplements

  Vitamin D   5,000 IU 1x/day

  AquADEK vitamin   2 softgels 1x/day

Case Study RW: MNT  WHO equation

 Male 3-10y: 22.7W + 495  Result x 1.7 (stress/act. factor) x 120%-150%

needs = 2162-2703 calories/day  Schofield equation

 Male 3-10y: 12.6W + 1.303H + 414.9  Result x 1.7 (stress/act. factor) x 120%-150%

needs = 2182-2727 calories/day  RDA

  70 kcal/kg = 1743 kcal x 120%-150% needs = 2091-2615 calories/day

Case Study RW: MNT   Calories: 2150-2700 calories/day

  Protein: 38-50+ grams protein/day

  1.5-2.0 grams/kg body weight

  Carbohydrates: 45-65% of calories

  Fat: 30-35% of calories

  Fluids: 1600 mL

  1500cc + 20cc/kg>20kg = 1500 + 20(4.9)

Case Study RW: MNT   Fat-soluble vitamins

  AquADEK vitamin 2x/day   Vitamin D 5,000 IU/day

 Water-soluble vitamins  Daily MVI at home

 Sodium chloride   Adds 1/4 tsp salt to meals   Eats salty foods

Case Study RW: Discharge

 Elecare Junior nocturnal tube feed   7pm-7am for 12 hours at 100mL/hr   1200mL formula   30 mL flush before and after  No enzymes needed with elemental TF

 Concentrated formula 45 kcal/oz   915 mL (31 oz) + 41 scoops formula

  1833 calories, 56 grams protein

Resources   Cystic Fibrosis Foundation (CFF)   Cystic Fibrosis Research Inc. (CFRI)   A.S.P.E.N. Pediatric Nutrition Support Core Curriculum   World Health Organization   Escott-Stump, Sylvia. (2009). Nutrition and Diagnosis-

Related Care. 6th ed. Philadelphia: Lippincott Williams & Wilkins. Print.

Thank You! Questions?