Rhianna Cenci, Sodexo Dietetic Intern

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/m 2 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: 10-25 th percentile Weight: 10-25 th percentile BMI: 15.2 25 th 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. 6 th ed. Philadelphia: Lippincott Williams & Wilkins. Print. Thank You! Questions?