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Cystic Fibrosis: A Clinical Nutrition Case STudy

Cystic Fibrosis: A Clinical Nutrition Case STudy. Wendy Anderson February 1, 2013. Outline. What is Cystic Fibrosis? Overview of Patient Nutrition Assessment of Patient Nutrition Diagnosis Nutrition Intervention Nutrition Monitoring/Evaluation Medical Nutrition Therapy for

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Cystic Fibrosis: A Clinical Nutrition Case STudy

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  1. Cystic Fibrosis: A Clinical Nutrition Case STudy Wendy Anderson February 1, 2013

  2. Outline • What is Cystic Fibrosis? • Overview of Patient • Nutrition Assessment of Patient • Nutrition Diagnosis • Nutrition Intervention • Nutrition Monitoring/Evaluation • Medical Nutrition Therapy for Cystic Fibrosis • Other Possible Treatments

  3. Cystic Fibrosis • Most common life-limiting, autosomal recessive disease in the United States • Defect on chromosome 7 in the area responsible for coding the Cystic Fibrosis TransmembraneRegulator (CFTR) protein • Abnormal transport of ions and water across cells in many organs of the body • Thick, tenacious mucus and organ damage, especially in the respiratory, gastrointestinal, endocrine, and reproductive symptoms

  4. Cystic Fibrosis Stats • Incidence of CF is approximately 1:3,500 live births • 30,000 people who have CF in the United States, half being older than 18 years of age • CF was first identified in the 1930s, life expectancy was less than 5 years of age • Current median predicted survival of 38.3 years • Mutation of the single gene that encodes for CFTR identified in 1989 Cystic Fibrosis Foundation. Patient Registry: Annual Data Report 2010. Bethesda, MD; 2011

  5. Cystic Fibrosis: Life Expectancy

  6. What’s the Chance?

  7. CFTR: The Gene Associated with CF • Type of protein classified as an ABC (ATP-Binding Cassette) transporter • CFTR transports Cl- Ions across membranes in lungs, liver, pancreas, digestive and reproductive tracts and skin • Made up of 1480 amino acids • Made up of 5 domains • MSD1 and MSD2 (Form Cl- Channel) • NBD1 and NBD2 (Bind and Hydrolyze ATP) • R (Regulates movement of Cl- ions across membrane)

  8. CFTR: The Gene Associated with CF

  9. 5 Classes of CFTR Mutations: classified by effect on CFTR protein Pancreatic Insufficient Pancreatic Sufficient

  10. Delta F508 Mutation • 70% of mutations result from deletion of three base pairs of CFTR’s nucleotide sequence • Loss of AA Phenylalanine located in position 508 in protein: delta F508 • Normal CFTR: Protein synthesized/transported to endoplasmic reticulum and Golgi apparatus • CFTR protein with delta F508 reaches endoplasmic reticulum, recognized that protein is folded incorrectly…marked for degredation…delta F508 never reaches cell membrane.

  11. Delta F508 Mutation

  12. Diagnosis of CF • Sweat Chloride test performed • Measures level of Chloride/Sodium in sweat using an electric current • Pilocarpine is a chemical used to stimulate sweat glands • Sweat collected on gauge for 30 minutes, weighed • Positive (>60 mEq/L Cl- in sweat) • CFTR mutation analysis in patients suspected of having CF with normal/borderline sweat chloride value

  13. Symptoms of CF • Vary depending on severity of disease • Screening of newborns performed in all 50 states • Respiratory symptoms • Persistent cough, production of thick mucus • SOB, wheezing • Decreased ability to exercise • Repeated lung infections • Digestive symptoms • Fowl-smelling greasy stools • Poor weight gain and growth • Intestinal blockage (meconium ileus) • Severe constipation

  14. Organs Affected by CF

  15. Respiratory Implications • Lungs become colonized with bacteria • Bronchiectasis develops, making infection more difficult to treat • Manifestations • Wheezing • SOB • Persistent cough and excessive mucus • Repeated cases of pneumonia • Digital clubbing

  16. GI/Nutritional Implications • Intestinal • DIOS ( Distal Intestinal Obstruction Syndrome) • Blockage of intestines by thickened stool • Pancreatic • Pancreatic insufficiency and recurrent pancreatitis • CFRD • Hepatic • Chronic hepatic disease • Focal biliary cirrhosis/multilobular cirrhosis • Nutritional • Failure to thrive or gain weight (PEM) • Steattorrhea • Abdominal discomfort, gas • Osteoporosis

  17. Distal Intestinal Obstruction Syndrome (DIOS) • Complete intestinal obstruction • Abdominal radiography useful to differentiate between Constipation and DIOS • DIOS: Fecal mass in the ileocecum with or without fluid levels in the small intestine • Constipation: Distribution of fecal material throughout the colon • Intensive laxative treatment (oral laxatives and/or enema or polyethylene glycol lavage) • Adequate hydration important!!

  18. CF-related liver disease • ~ 24% CF adults have hepatomegaly or abnormal LFTs • Ultrasound of right upper quadrant • Gallstones, common bile duct stones, nodules of liver (suggest cirrhosis), steatosis (retention of lipids within cells) • Cholestasis • Flow of bile from liver is slowed/blocked • Ursodeoxycholic acid (UDCA) • Improves bile flow • Stimulate bicarbonate secretion into bile

  19. Overview of patient

  20. Patient Background • IM is a 27 year old Caucasian Female admitted on 12/6/12 • Diagnosed at 8 months (Sweat test: 113 mEq/L Cl-) • First JHH CF clinic visit was in November, 2010 • Current Medical History • CF; unknown genotype • Complicated by Burkholderiacepacia • Pancreatic insufficiency • Past Medical History • Gerd • Sinus problems • Hx of asthma • Anxiety/ADD

  21. Social History • Previously lived in San Diego, CA (lost job in TV production in wardrobe during writer’s strike) • Moved home to live with parents in Southern MD, 2010 • Has a boyfriend • Works as a waitress in Annapolis, MD • Does not smoke • ETOH 3-4x/month • Occasional illicits (marijuana) • Twin brother in excellent health

  22. Hospital Course • Seen in JHH CF Clinic on 12/3/12 • Admitted on 12/6/12 with one week history of worsening of pulmonary symptoms • Last admission was in August 2012 • Diagnosis: CF exacerbation • CXR on 12/6/12 revealed improved minimal mucous plugging in right lower lobe since 8/13/12 • Automatic nutrition consult for CF patients (within 48 hours)

  23. Nutrition Assessment

  24. Why WAS Nutrition consulted? • Monitor energy and protein intake • Address pancreatic enzyme and vitamin regimen • Provide education to promote achieving and maintaining a healthy body weight

  25. Nutritional Assessment • Height: 63” • CBW: 49 kg • UBW: 50 kg • %UBW: 98% • Weight History: 8/2012 (50.8 kg); 3/2012 (49.2 kg); 6/2011 (52.7 kg) • BMI: 19.1 • %FEV1 upon admission: 44% • %FEV1 baseline: ~80%

  26. Interview • IM was extremely tired • Experiencing nausea and decreased appetite • No breakfast; last meal was nachos and cheese she shared with her mother • Endorsed AquADEKSand CREON 24 • **Reports taking AquADEKS after enzymes • Not taking Vitamin D… “I forget” • Prepares her own food and is a huge snacker • Prior snacks preferences on previous admissions: • Ensure CS, pretzels, yogurt, Sierra Mist, potato chips, orange, Oreos, fruit cocktail, etc.

  27. Common Nutrition Diagnosis/PES Statement • Increased energy expenditure (NI 1.2) • Inadequate oral intake (NI 2.1) • Unintentional weight loss NC 3.2) • Altered GI function (NC 1.4) • PES Statement • “Inadequate oral intake (NI 2.1) related to increased energy expenditure as evidenced by pancreatic insufficiency, poor lung function and a BMI < 22 (ideal for female with CF to promote optimal lung function)”.

  28. Nutrition Assessment • Goals • Increase PO intake • Maintain weight • Preserve lean body mass • Intervention/Monitoring • Supplements and snacks (increased calorie and fat intake), monitor labs, weights • Follow up in 5-7 days • Recommendations: • Continue with regular diet and provide snacks per patient preference • Ensure Clinical Strength TID • Order Cholecalciferol 50,000 IU q7 (IM not taking at home)

  29. Home Medications

  30. What labs do we check in CF Patients? • Vitamins A, D 25-OH, and E checked annually • Vitamin K • Indirectly assessed by evaluating prothrombin time • CBC • Hemoglobin/Hematocrit levels • Zinc • Liver enzymes checked annually • Casual glucose levels annually (monitor for CFRD) • Sputum sample

  31. IM’s Lab Values

  32. Pulmonary Function Tests…What is FEV1%??? • FEV1 definition • Volume of air that can forcibly be blown out in one second, after full inspiration • Measure lung functionality and need for and response to antibiotic therapy • IMs FEV1 History • 11/18/10 (64%) • 10/12/11 (80%) • 8/27/12 (64%) • 12/3/12 (44%)

  33. Nutritional status & pulmonary Function in Adult CF Patients • Journal of Physiology and Pharmacology, 2008 • 39 CF patients (21 females and 18 males) • Mean age 23.9 + 3.7 years • BMI used to single out groups: • Normal weight (n=28) w/ BMI > 18.5 kg/m2 • Malnourished (n=11) w/ BMI < 18.5 kg/m2 • Pulmonary function abnormal if FEV1 < 80% • Statistical analysis revealed significant differences btw malnourished and not malnourished pts concerning FEV1% • Poor clinical outcome associated with significant loss of body weight (Malnutrition , or risk of malnutrition remains a frequent complication of CF)

  34. Medical NutritionTherapy for CF

  35. Nutrition Goals in CF • Control adequate maldigestion and malabsorption • Provide adequate nutrients • Promote optimal growth • Maintain weight for height • Support pulmonary function • Prevent nutritional deficiencies • PERT (Pancreatic Enzyme Replacement Therapy) • Fat-soluble vitamins

  36. What Nutritional Indicators should be evaluate? • Decreased Oral Intake • Diarrhea, steatorrhea, or changes in stool • Diet quality (macro and micronutrient quality) • Abdominal pain • Weight loss or lack of weight gain • Inadequate growth • Reduced skeletal muscle mass • ** Patients with weight loss = HIGH nutritional risk!

  37. General Dietary Guidelines • 3 meals, 2-3 snacks per day • Pancreatic enzyme and vitamin supplementation • Unrestricted diet (including high fat foods) • Supplements • Encourage variety of whole grains, fruits and vegetables • Counseling to provide ideas for calorie boosters • Extra salt especially during hot weather/exercise/febrile • Adequate calcium, vitamin D, vitamin K

  38. Energy Requirements for CF Patients • Calorie and protein requirements are usually 1.2-2x the DRI for age • Factors to consider • Age • Gender • Physical Activity • Respiratory Infections • Severity of lung disease • Severity of malabsorption

  39. Estimated Needs: Energy/Protein • Not defined in literature at this time • Energy equation: JHH • (DBW x 35 x FEV1 factor) to (DBW x 40 x FEV1 factor) • FEV1 Factors: • FEV1 > 90% predicted = 1.0 FEV1 40-50% predicted = 1.5 • FEV1 70-90% predicted = 1.2 FEV1 33% predicted = 1.75 • FEV1 60-70% predicted = 1.3 FEV1 25% predicted = 2.0 • FEV1 50-60% predicted = 1.4 • Protein: • 18-20% of caloric needs or ~2.2g/kg

  40. IM’s Estimated Needs (JHH Formula) • Energy needs • IM’s IBW (BMI 22 for women, 23 for men) • 56.3 kg • Promotes optimal lung function • (DBW x 35 x FEV1 factor) to (DBW x 40 x FEV1 factor) • (56.3 kg x 35 x 1.5) to (56.3 x 40 x 1.5) • = 2955-3378 kcals/day • Protein needs • 2.2 x 56.3kg • = 124g/day

  41. Energy Requirements Calculation from the AND Nutrition Care Manual Step 1: Calculate BMR

  42. Energy Requirements Step 2: BMR x (Activity Coefficient + Disease Coefficient)

  43. Energy Requirements • Step 3: Calculate Daily Energy Expenditure, taking into account the degree of steatorrhea • For Pancreatic Sufficient Patients: (including pts with a coefficient of fat absorption [CFA] > 93%) • Daily energy requirement equals the daily energy expenditure • For Pancreatic Insufficient Patients: • Daily energy requirement equals the daily energy expenditure times (0.93/CFA) • If a stool fat collection is not available to determine fraction of fat intake, use approximate value of 0.85

  44. IM’s Energy Requirements (AND Formula) • BMR = 14.7 (49kg) + 746 • = 1216 kcals • Daily Energy Expenditure = 1216 kcals x (1.5 + 1.7) • = 3891 kcals • Total Daily Energy Requirement • = 3891 kcals (0.93/0.85) • = 4257 kcals • ** JHH formula = 3378 kcals

  45. Macronutrients • Protein: 15–20% calories • Levels are increased due to malabsorption • Fat: 35–40% calories • Carbohydrates: Varies

  46. CF Sample Meal Plan Approximate Nutritional Analysis: Calories: 3,094; Protein:129g (16% of calories); CHO:362g (46% of calories); Fat: 129g (37% of calories); Cholesterol: 750 mg; Sodium: 4,250 mg; Fiber 23g SOURCE: AND Nutrition Care Manual

  47. Fat Soluble Vitamin Supplementation • Vitamin A: 4,000 -10,000 IU • Vitamin D: 800 – 2,000 IU • Vitamin E: 150 – 300 IU • Vitamin K: No recommendation • IM takes AquADEKS x1 tablet BID • Also recommended: Cholecalciferol 50,000 IU q7 days; (IM has forgotten to take)

  48. Pancreatic Insufficiency • 85-90% of pts with CF • Definition: Elevated fecal fat excretion and presence of steatorrhea (fecal fat excretion > 7%) • Pancreatic enzyme supplements (enteric coating) • Prevents inactivation in acidic environment • Hyperacididty of upper GI tract in CF patients • Inadequate bicarbonate secretion by pancreas • H2 blockers and proton pump inhibitors (decrease gastric acid production)

  49. Pancreatic Enzyme Replacement Therapy (PERT) ** IM takes Creon 24: 4 tabs with meals and 3 tabs with snacks **24,000 USP units of lipase; 76,000 USP units of protease; 120,000 USP units of amylase capsules **CF Foundation recommends 500 to 2,500 units lipase per kilogram body weight per meal

  50. Cystic Fibrosis-Related Diabetes(CFRD) • > 15% patients that are > 35 years old • OGTT or fasting glucose >126 mg/dL when confirmed by second fasting blood glucose • Marker of advanced disease • Treatment • Insulin therapy • Oral diabetes agents not as effective as insulin • Check blood sugars TID • Check HbA1c on regular basis • Moderate aerobic exercise IM’s HbA1c was 5.8 (slightly increased) on 7/30/12 but does not have CFRD at this time

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