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“Simmer Gently for 40 Years: Evolving Impact of RD Research, Education, and Practice on CF Care” Nancy H. Wooldridge, MS, RD Co-Director and Nutrition Faculty, Pediatric Pulmonary Center Pediatric Nutrition Coordinator, CF Center University of Alabama at Birmingham firstname.lastname@example.org IMPACT OF RDs EDUCATION RESEARCH CLINICAL PRACTICE Learning Objectives Upon completion of this educational activity, participants will be able to: • list 3 components of the nutrition management of cystic fibrosis. • explain the significance of optimal nutritional status in CF care. • give 3 examples of successful strategies employed in by CF dietitians in research, education, and clinical practice. CYSTIC FIBROSIS (CF) Discovery of CF Gene Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) CF Gene Mutation • Malfunction of the chloride channels in cells • Increased sodium reabsorption • Decreased chloride secretion • Dehydrates mucus • Increased viscosity of secretions • Losses of sodium and chloride in the sweat ∆F508 Mutations Homozygous ∆F508 50.2 % Heterozygous ∆F508 37.7 % No ∆F508 or both 12.1 % unidentified Cystic Fibrosis Foundation Patient Registry, 2002 GENETICS Autosomal recessive trait • Both parents are carriers • Each pregnancy has a: – 1 in 4 chance that the child will have CF – 1 in 2 chance that the child will be a carrier of the mutation – 1 in 4 chance that the child will not inherit the mutation Lungs – viscid secretion Bronchial & Secondary Obstruction Infection Bronchiectasis Recurrent Atelectasis Pneumonia or Lung Abscess Progressive Fibrosis & Emphysema Pulmonary Manifestations • Chronic cough • Bronchial obstruction • Secondary bacterial infection • Nasal polyps • Chronic pneumonia Pulmonary Manifestations • Increased respiratory rate • Cyanosis • Digital clubbing • Bronchospasm • Chronic sinusitis Gastrointestinal Disease Pancreatic Insufficiency Liver Disease CF related diabetes mellitus Gastrointestinal Manifestations • Meconium Ileus • Failure to thrive • Steatorrhea • Bulky stools • Intussusception of the small and large bowel Gastrointestinal Manifestations • Rectal prolapse • Hypoproteinemia • Anemia • Vitamin deficiencies • Gallbladder disease DIAGNOSIS DIAGNOSIS • Elevated levels of chloride on sweat test (2 or more times) • AND one or more characteristic of CF • OR family history of CF • OR positive newborn screening test result Newborn Screening Median Predicted Survival Age, 1986-2008 Median Survival Age (years) 40 36 32 28 24 '86 '88 '90 '92 '94 '96 '98 '00 '02 '04 '06 '08 Year CF Foundation, Patient Data Registry, 2008 Danny Bessette – 2003! Treatment of Pulmonary Disease • Chest clearance • Antibiotics • Anti-inflammatory medications • Infection control Treatment of Gastrointestinal Disease • Adequate nutrition • Pancreatic enzyme replacement therapy • Vitamin supplementation Increased sweat electrolytes • Supplemental salt • High salt foods Pulmonary Complications • Atelectasis • Bronchiectasis • Pneumothorax • Hemoptysis • Cor pulmonale • Respiratory failure Gastrointestinal Complications • Meconium ileus • Distal intestinal obstructive syndrome (DIOS) • Fibrosing colonopathy • Rectal prolapse • Biliary disease • Pancreatitis Other Medical Complications • CF Related Diabetes • Bone Disease Cystic Fibrosis Foundation, Patient Registry, 2007 Growth and Pulmonary Function • Better growth associated with longer survival (Corey, et al., 1988). • Continuous weight gain associated with better FEV1 (Peterson, et al., 2003). • Increased stature of CF patients associated with longer survival (Beker, et al., 2001). Malnutrition and Lung Function • Malnutrition at any age has negative impact on lung function. • Malnutrition associated with a more rapid yearly decline in FEV1. Steinkamp et al., Thorax, 2002 Growth and Lung Health • 1990s data from Epidemiologic Study of Cystic Fibrosis (ESCF) –Better growth at age 3 → better pulmonary functions at age 6 –Improved growth from age 3 to age 6 → better pulmonary functions at age 6 Konstan, et al. J Pediatr 2003 FEV1 Percent Predicted vs. BMI Percentile in Children 6 to 20 Years 100 90 FEV1 Percent Predicted Males Females 80 Goal 50th Percentile 70 60 <5 10 20 30 40 50 60 70 80 90 BMI Percentile FEV1 percent is positively correlated with BMI percentile for patients 6 to 20 (correlation > 0.95, p < 0.0001). CFF Patient Data Registry, 2008 Pulmonary Factors Affecting Nutritional Status • Increased work of breathing • Cough/emesis cycle • Chronic infection • Chronic antibiotic therapy GI Factors Affecting Nutritional Status • Fat and protein losses in stools • Fat soluble vitamin losses • Essential fatty acid losses • Bile salts and bile acid losses GI Factors Affecting Nutritional Status • Gastroesophageal reflux/esophagitis • Distal intestinal obstruction syndrome (DIOS) • Fibrosing colonopathy Nutrition Assessment • Anthropometric Measurements • Biochemical Data, Medical Tests • Physical Examination Findings • Client History (including medications) • Food/Nutrition History American Dietetic Association, 2009 CFF Recommendation (Pediatrics) • For children and adolescents with CF, ages 2.0 to 19.9 years, utilize the BMI percentile method for weight- for-stature assessment. • Maintain BMI percentile ≥50th %. Stallings VA, et al., JADA, 2008 Biochemical Monitoring at Diagnosis • Hemoglobin, hematocrit • Serum albumin • Plasma or serum retinol • Alpha-tocopherol • 25-hydroxy Vitamin D • Prothrombin time or PIVKA-II • Serum sodium, if exposed to heat stress Borowitz et al, J of Pediatr Gastro and Nutr, 2002 Biochemical Monitoring Annually • Hemoglobin, hematocrit • Plasma or serum retinol • Alpha-tocopherol • 25-hydroxy Vitamin D • Serum albumin • Random blood glucose Borowitz et al, J of Pediatr Gastro and Nutr, 2002 Client History/Physical Examination Findings Pulmonary Status GI Status Other medical conditions Food/Nutrition History • Analysis of 24-hour dietary recall or a 3-day food record • Nutritional adequacy • Meeting estimated needs • Caloric distribution Nutrition Intervention in CF • Promote normal growth and development through: – High Calorie, high protein, unrestricted fat diet – Enzyme replacement therapy – Vitamin and mineral supplementation – Addressing behavioral issues – Referral to food and nutrition resources General Recommendations • Aim for 35% to 40% of kilocalories from fat. • Encourage snacks. • Include high calorie foods. • Discourage “grazing”. • Discourage/limit sweetened beverages. Estimating Calorie Needs • 12 year old girl with cystic fibrosis • Daily recommended intake = 2000 Calories • Multiply by 1.3 – 1.5 for cystic fibrosis = 2600 – 3000 Calories per day Sample Menu • Breakfast • Lunch – 2 (4” square) waffles – 1 slice pizza – 2 tsp. margarine – 1 medium serving fries – 1 oz. syrup – 1 cup salad – 2 slices bacon – 2 Tbsp. salad dressing – 8 oz. whole milk – 8 oz. whole milk – 1 banana • PM Snack • AM Snack – 1 whole sandwich – 4 oz. fruit cup (2 slices bread, 1 slice – 4 oz. juice drink lunch meat, 1 slice cheese, 1 Tbsp. mayo) – 4 oz. juice drink Sample Menu (continued) • Supper • Bedtime Snack – 3 oz grilled chicken – ¾ cup dry cereal – 2 oz. gravy – 8 oz whole milk – 1 cup cooked rice – ½ cup greens TOTAL CALORIES: – 1 roll 3000 – 1 tsp. margarine 47% carbohydrate – 8 oz. sweet tea 13% protein 40 % fat Poor Appetite Behavioral Interventions • Gradually increase calories by working on one meal at a time. • Take full advantage of offering snacks. • Parents can learn alternate ways of responding to child during meals. • Identify appropriate rewards for eating the expected amount of food. • Consider behavioral therapy. Cystic Fibrosis Foundation, 2001 Nutrition Intervention of CF • Nutritional Counseling • Oral Supplements • Enteral Feedings Nutrition Intervention of CF • Enzyme replacement therapy – Taken before each meal and snack – Not to exceed 2500 units lipase/kg body weight/meal – May need to add medicines to decrease gastric acidity to improve effectiveness Borowitz 1995; FitzSimmons 1997 Nutrition Intervention of CF • Vitamin and mineral supplementation – ADEK®, AquADEK™, SourceCF®, or Vitamax™ daily – Use of salt on foods and inclusion of high salt foods in diet – Calcium supplements if intake not meeting needs – Vitamin D supplements if levels low Nutrition Intervention in CF • Referral to Food/Nutrition Resources – Supplemental Food Program for Women, Infants, and Children (WIC) – State Children with Special Health Care Needs Program – Child Nutrition Programs – State Medicaid – Patient Assistance Programs • Asking about Alternative/Complementary Medicine Nutrition Goals • Provide energy intakes greater than standard for general population. • Maintain normal growth parameters and weight for length or BMI-for-age ≥50th%. • Provide intensive intervention, including nutrition counseling, behavioral intervention, and nutrition supplements to help meet goals. Median Predicted Survival Age, 1986-2008 Median Survival Age (years) 40 36 32 28 24 '86 '88 '90 '92 '94 '96 '98 '00 '02 '04 '06 '08 Year CF Foundation, Patient Data Registry, 2008 IMPACT OF RDs CF Foundation Pediatric Pulmonary Centers Pediatric Nutrition Practice Group, ADA 25th Annual Meeting of the CF Club • San Jose, California, April 1984 • Planning Committee: – Physicians – Nurses – Social Workers – PhDs – NO RDs!!! • Nutrition topics covered by MDs CF Foundation Activities •Member, Program Planning Committee, 2nd Annual North American Cystic Fibrosis Conference (NACFC), 1988 [First Registered Dietitian invited to serve on Program Committee] •Member, Program Planning Committee, 3rd Annual NACFC, 1989 •Member, Nutrition Grants Review Committee, 1989 •Member, 1st Consensus Panel on Donna H. Mueller, Diabetes Mellitus and Cystic Fibrosis, PhD, RD, FADA, LDN 1990 Associate Professor •Member, 1st Consensus Panel on Drexel University Nutrition and Cystic Fibrosis, 1990 Suzanne H. Michel, MPH, RD, LDN • Current Position: • Clinical Dietitian, Pediatric CF Center, The Children’s Hospital of Philadelphia • Previous CF Positions: • Nutritionist, Adult CF Program, Drexel School of Medicine • Department of Pediatrics, Hahnemann University Hospital Suzanne H. Michel, MPH, RD, LDN • Highlights of CF Foundation Activities – Member, Planning Committee, NACFC, 1990, 1991 – Member, Consensus Committee, Nutrition Assessment & Management in CF – Member, Clinical Practice Guidelines – Member, Working Group on Care of the Infant Diagnosed with CF through NBS Suzanne H. Michel, MPH, RD, LDN • CF Nutrition Source: Online nutrition and cystic fibrosis professional education series. Initiated 2002 and currently ongoing. • Educational Grant from CF Services Pharmacy for Patient Booklets, 2005 – “Color Your Calories” – “Grab ‘N Go, Meals and Snacks for People on the Run” Cystic Fibrosis Services Pharmacy Justine Spisak, BS, Nutrition Student Suzanne Michel, MPH, RD, LDN, Editor Donna H. Mueller, PhD, RD, FADA, LDN, Editor Elizabeth McFarlane, Format Approved by the CFF Education Committee CFF’s Golden Apple Award • Given in recognition of personal commitment to excellence in the advancement of professional education and patient care. • To date, Suzanne is the only RD to have received this award!!! Midwest CF Nutrition and Social Work Consortium • Grew out of an Ohio multidisciplinary consortium • Became an Ohio CF RDs consortium • Expanded to the Midwest in 2007 • Social workers joined in 2009 • Have met in Chicago (2007), Milwaukee (2008), Cincinnati (2009), Indianapolis (2010) Suzanne H. Michel, MPH, RD, LDN 2010 Recipient, Midwest RD Achievement Award Jean Tomezsko, MPH, PhD, RD, LDN • CF-related Clinical Experience: – Clinical Nutritionist, CF Center, Hahnemann University, 1991-1995 – Clinical Nutritionist, CF Center, St. Christopher’s Hospital for Children, 1995- 1999 – Clinical Nutritionist, CF Center at Children’s Hospital of Philadelphia (CHOP), 1999 – 2007 • Retired from CHOP, October 2007 Jean Tomezsko, MPH, PhD, RD, LDN • CF Foundation related activities: • Member, Program Planning Committee, NACFC, 2002 through 2008 • Chaired numerous Discussion Sessions and Workshops at NACFCs • Publications: • PhD Dissertation – Energy expenditure, body composition, and dietary intake of children with CF • Numerous publications with Dr. Virginia Stallings in peer-reviewed journals Judy Fulton, MPH, RD, LDN • Current Position: • Clinical Dietitian/Research Coordinator, Children’s Hospital of Pittsburgh of University of Pittsburgh Medical Center Judy Fulton, MPH, RD, LDN • CF Foundation related activities: • Selected Member, Pediatric Nutrition Consensus Conference, 2001-2002 • Selected Member, CFF Education Committee, 2006-present • Pediatric Nutrition Practice Group, ADA • CF/Pulmonary Nutrition Networking Coordinator • Member Support Co-Chair, Chair • Author of CF Chapter for Pediatric Diet Manual Amanda Leonard, MPH, RD, CDE, Terri Schindler, MS, RD Johns Hopkins Children’s Center Rainbow Babies & Baltimore, MD Children’s Hospital, Cleveland, OH Other CF Foundation Activities • RD presenters included in international Teleconferences • RD members of CF Education Committee • RD members of CFRD consensus guidelines Maternal and Child Health Bureau Pediatric Pulmonary Centers Pediatric Nutrition Practice Group of ADA • Practice group started in 1978 • Cystic Fibrosis included in Quality Assurance Manual published in early 1980s • CF/Pulmonary Nutrition Networking Group 1990s Mary Marcus, MS, RD, CSP Senior Clinical Nutritionist and Co-Director, PPC, University of Wisconsin – Madison 1st Pediatric Nutrition Practice Group Network Representative with the CFF IMPACT OF RDs EDUCATION RESEARCH CLINICAL PRACTICE Anna dePlanter Bowes Lecture April 27, 2010 Pittsburgh, PA
"Nutrition Assessment and Management in CF Pennsylvania"