3/19/2013 * Cysti tic c Fibrosi osis s • Classify age specific nutrition related guidelines for Cystic Fibrosis patients. and Nutriti ition n • Understand supplementation of fat soluble vitamins: A, D, E and K. • Identify signs and symptoms of Pancreatic Insufficiency in patients with Cystic Fibrosis. • Calculate pancreatic enzymes and make By: Alliene Farley MS RD LD recommendations for Pancreatic Insufficient West Virginia University Hospital patients with Cystic Fibrosis. Mountain State Cystic Fibrosis Center • Recognize comorbidities related to Cystic fibrosis. * * • About 1,000 new cases of cystic fibrosis are diagnosed each • Cystic fibrosis (CF) year. • Chronic disease that affects the lungs and • More than 70% of patients are diagnosed by the age of two. digestive systems of about 30,000 • At least 45% of the CF patient population is age 18 or older. children and adults in the United States • (70,000 worldwide) The predicted median age of survival for a person with CF is in the • A defective gene inherited from both late 30s. • In the 1950s, few children with cystic fibrosis lived to attend elementary parents which causes the body to produce unusually thick, sticky mucus. school . • Clogs the lungs and leads to life- threatening lung infections. • Obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. * * • Sweat Test • People with CF can have a • Gold Standard variety of symptoms, including: • very salty-tasting skin • Newborn Screening • persistent coughing • Improve growth • frequent lung infections • Help keep lungs healthy • Reduce hospital stays & • wheezing or shortness of breath • Add years to life • poor growth/weight gain in spite of a good appetite & • Genetic Carrier Testing • frequent greasy, bulky stools or difficulty in bowel movements 1
3/19/2013 * * • Monitor Absorption of Nutrients • Energy and Protein • Identify Nutritional Status • Calorie Requirements: • Provide Diet Education 1.2 – 2 times the DRI for age. • Assess Nutritional Needs • Protein Requirements: • Provide Enteral and Parenteral 1.5 – 2 times the DRI for age. Nutrition Recommendations • Energy needs will be • Assist in Recommendations of influenced by: Pancreatic Enzymes • Severity of lung disease & • Degree of malabsorption * * • Patients with CF do NOT absorb nutrients properly. • At Diagnosis • Increased need for fat soluble • Infants vitamins: • Check vitamin levels 2 – 4 months • Vitamins A, D3, E & K after starting supplemental vitamins • CF Vitamins: • Children/Adolescents & Adults • AquaDEK – Liquid, Chewable & • Check levels at diagnosis Gel Capsule • SourceCF – Liquid, Chewable & • After Diagnosis • Vitamin A, D 25-OH, and E levels Gel Capsule • Vitamax – Liquid & Chewable should be checked annually. • Increased need for minerals • Vitamin K can be assessed using PIVKA II Or indirectly by using • Calcium, Iron , Sodium Chloride & Prothrombin Time. Zinc • Possible K deficiency: easy bruising, difficulty with blood clotting . * * Age Liquid Chewable Soft-Gel • Poor weight gain 0-12 months 1 ml (0.5 ml BID) despite a good appetite. 1-3 years old 2 ml (1 ml BID) • Frequent, loose 4-8 years old 1 tablet and/or large bowel 9-18 years 2 Capsules (1 Capsule movements. old BID) • Foul-smelling bowel movements. >18 years old 2 Capsules (1 Capsule BID) • Mucus or oil in the bowel movement. • Excessive gas and/or • Additional supplementation: stomach pain & • Vitamin A: 8,000 – 10,000 IU once daily • • Distention or bloating. Vitamin D: 10,000 – 50,000 IU weekly • Vitamins with meals and enzymes! 2
3/19/2013 * * • Identifying PS or PI: • Pancreatic Enzyme Replacements • Test 72 hour fecal • Lipase, Amylase & Protease • >200 Normal • Capsule Form • 100 – 200 Moderate to Mild • Inside each capsule are many small “beads” Exocrine PI • <100 Severe Exocrine PI that contain digestive enzymes. • Each bead is covered with a special • Pancreatic Insufficient Patients “coating.” • Prescribed Pancreatic • This coating allows the beads to dissolve in the small intestine. Enzymes • The main functions of enzymes are: • Pancreatic Sufficient Patients • To digest carbohydrate, protein and fat • Do not take enzymes • To help with weight gain & • To promote nutrient absorption . * * Enzyme Strengths (USP Units of Lipase) • Enzymes should be taken: Creon 24,12,6 &3 24000,12000,6000 & 3000 • Before meals and snacks. • Before, during and after nightly tube feeding. Zenpep 25,20,15,10,5 & 3 25000, 20000, 15000, 10000, 5000 & 3000 • Older Children and Adults Ultresa 23,20, & 13 23000, 20700 & 13800 • Capsules should be taken with liquid and swallowed whole. Viokase 16 & 8 16000 & 8000 • Infants and Small Children Pancreaze 21,16,10 & 4 21000, 16800, 10500 & 4200 • Capsules may be opened and beads can be mixed with a soft Pertzye 16 & 8 acidic food. 16000 & 8000 • Applesauce * * • Dosing enzymes: • To calculate the enzyme dose per kg/meal: • Max - 2500 U Lipase/kg/meal • Multiply Units of lipase by meal dose • Max - 10,000 U Lipase/kg/day • Divide the total Units of lipase per meal by weight • Infants and young children: • Example: • Start with 1,000 U Lipase/kg/meal until max dose is reached. • Enzyme prescription – Creon 12 (12,000 U of lipase) with 3 capsules per meal • Adults: • Weight 15 kg • Start with 500 – 1,000 U Lipase/kg/meal until max • 12,000 X 3 = 36,000 U Lipase/meal dose is reached. • 36,000 divided by 15 kg = 2400 U Lipase/kg/meal 3
3/19/2013 * * • High-calorie diet, • To calculate the enzyme dose per kg/day: • Multiply Units of lipase by day dose including supplements • Divide the total Units of lipase per day by weight when needed. • Behavioral intervention • Example: to encourage good • Enzyme prescription – Creon 12 (12,000 U of eating habits in children. Lipase) with 4 capsules per meal and 2 capsules per • Keeping track of snack (3 meals and 2 snacks = 16 capsules per day) • Weight 20 kg nutritional indicators, • 12,000 X 16 = 192,000 U Lipase/meal such as body mass index. • 192,000 divided by 20 kg = 9,600 U Lipase/kg/meal • Appropriate doses of pancreatic enzymes. * * • Ages 0-12 months • Breast milk, iron-fortified formula • Ages 1- 4 • Enzymes prior to all feedings • Provide a normal, healthy diet with • Vitamin supplement a variety of high-calorie foods and • Salt supplementation calcium rich foods. • 1/8 tsp 0-6 months • Encourage regular, pleasant meals • 1/4 tsp 6 - 24 months and snacks. • Add solids at 4 to 6 months • Avoid “grazing” or constant • Referrals to community programs snacking. • WIC • Teach appropriate self-feeding • Children with Special Health Care Needs skills. • Continue vitamin, enzyme, and salt supplementation. * * • Ages 18 and Older • Ages 12-17 • Well-balanced diet to support optimal nutritional • Limit sweetened beverages. status. • Make recommendations for easy, • Sufficient calories to quick, high-calorie foods. maintain healthy body • Increase energy intake during weight. • Observe for Anorexic growth spurts. Behaviors. • Promote independence with • Assess for Age-Related vitamin, enzyme, and salt Complications of CF. administration. • Continue Vitamins/Enzymes & Add Supplements as Needed. 4
3/19/2013 * * • Cystic Fibrosis Related Red Yellow Green Diabetes (CFRD) Nutritional Nutritional Risk Nutritional Health Failure Infants <10% ht-wt 11-49% ht-wt > 50% ht-wt • Cystic Fibrosis Related (0-23 Liver Disease months) Children <10% BMI for Age 11-49% BMI for Age > 50% BMI for Age (2-20 yrs. • Cystic Fibrosis Related of age) Renal Disease Adults < 18 kg/m^2 BMI 18-22 kg/m^2 BMI Males 23 kg/m^2 BMI Males (20 yrs. 18-21 kg/m^2 BMI Females 22 kg/m^2 BMI Females of age and older) * • Cystic Fibrosis-Related Diabetes (CFRD) is a unique type of diabetes • It is NOT the same as Type I or Type II Diabetes • 35% of adults 20 to 29 years of age • 43% of adults over 30 years of age • Symptoms of CFRD • Increased thirst and increased urination. • Excessive fatigue, weight loss and unexplained decline in lung function. • Screening and Diagnosis • Screening begins at 10 years of age • Hemoglobin A1C NOT a good indicator for diagnosis • 2 hour OGTT > 200 mg/dl • FBG > 126 mg/dl on two or more occasions • Treatment of CFRD • Insulin • Keeping blood glucose levels at a normal or near-normal level * * • More than 10 percent of people with CF have liver disease, a • Maintenance of a “Normal” Nutritional State number that may increase as people with CF live longer lives. • Maintenance of a “Normal” Nutritional State • Preventing Deficiencies • Preventing Deficiencies • Increased Energy Needs • Protein and Fat Recommendations • Protein and Salt Recommendations • Depend on Severity of Disease • Depend on severity of the disease • Increased Energy Intake • Monitoring of Renal Function Labs • Fat-Soluble Vitamins • Monitor Every 6 to 12 Months • Counseling Related to Risks of Alcohol Use 5
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