Rhianna Cenci, Sodexo Dietetic Intern

Transcription

1 Rhianna Cenci, Sodexo Dietetic Intern

2 Objectives Overview of CF and Treatments CF Medical Nutrition Therapy CF Case Study

3 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

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5 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

6 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

7 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

8 CF Genetics C c C c

9 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

10 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

11 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

12 CF Complications Gastroesophageal reflux disease (GERD) % 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

13 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

14 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

15 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

16 CF Treatments

17 CF Treatments Lung transplantation For end-stage lung disease Risk of rejection Weight criteria for adults % of IBW or BMI kg/m 2 Most aggressive therapy CF Foundation-accredited care centers

18 CF Medical Nutrition Therapy Calories Recommended Dietary Allowances (RDAs) WHO equations for REE Schofield equations for REE Adjust with activity/stress factor % or % of normal caloric needs, based on different sources Infants: 200 kcal/kg, Children: 150 kcal/kg

19 CF Medical Nutrition Therapy Age Gender WHO Schofield 0-3y M 60.9W* W* H* F 62.0W W H y M 22.7W W H F 22.5W W H y M 17.5W W H F 12.2W W H *W is weight in kilograms, H is height in centimeters

20 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 years 55 kcal/kg or 17 kcal/cm height years 45 kcal/kg or 17 kcal/cm height Adolescent Female years 47 kcal/kg or 14 kcal/cm height years 40 kcal/kg or 13.5 kcal/cm height

21 CF Medical Nutrition Therapy Protein 10-35% of calories, or 15-20% of calories % 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

22 CF Medical Nutrition Therapy Fluids Normal pediatric needs Holiday-Segar method Body Weight Fluid Need 0-10 kg 100cc/kg kg 1000cc + 50cc/kg>10kg >20 kg 1500cc + 20cc/kg>20kg

23 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

24 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

25 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

26 Case Study RW Complicated home life Many siblings (one diagnosed with CF) Parents divorced Recent depression and anxiety Grades went down Loves video games

27 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: th percentile Weight: th percentile BMI: th percentile %IBW: 93%

28 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

29 Case Study RW: MNT WHO equation Male 3-10y: 22.7W Result x 1.7 (stress/act. factor) x 120%-150% needs = calories/day Schofield equation Male 3-10y: 12.6W H Result x 1.7 (stress/act. factor) x 120%-150% needs = calories/day RDA 70 kcal/kg = 1743 kcal x 120%-150% needs = calories/day

30 Case Study RW: MNT Calories: calories/day Protein: grams protein/day grams/kg body weight Carbohydrates: 45-65% of calories Fat: 30-35% of calories Fluids: 1600 ml 1500cc + 20cc/kg>20kg = (4.9)

31 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

32 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

33 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?