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
C
c
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?
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