ESPEN Congress Nice From child to adult nutrition. Cystic fibrosis. A. Munck

Transcription

1 ESPEN Congress Nice 2010 From child to adult nutrition Cystic fibrosis A. Munck

2 Cystic fibrosis nutrition: from child to adulthood -Positive impact of optimizing nutrition -Monitoring nutritional status -Malnutrition in registries -Nutrition, PERT, Fat-soluble vitamins A.Munck CF paediatric Centre and GI unit University Hospital Robert Debre, Paris France

3 Positive impact of nutrition Boston and Toronto studies 1988: free diet in Toronto (n=500), fat restricted diet in Boston (534 -Improved nutritional status, pulmonary function in Canada -Median survival 30 years Canada/ 21 years US Corey M J Clin Epidemiol : free diet in Canada (n=3145) and US (n=20610) -Similar BMI, PFT, survival Lai HC Am J Clin Nutr 1999

4 Growth/nutrition at 3 years: strong predictor pulmonary function at 6years Konstan MW J Ped 2003 Cognitive skills index in NBS/control patients Control cohort lower HC z-score at diagnosis and decreased cognitive skills at 6y Koscik RL J Ped 2004, 2005 Nutrition is one of the cornerstone of good health in CF.

5 Monitoring nutritional status in CF care centres Patients height and weight should be carefully monitored at each clinical visit and BMI calculated (percentile or z-score in children, absolute BMI in adults)... toddlers, adolescents and during pregnancy <2 years W, H, HC Aim: W/L 50th percentile OR recovery of birth weight z-score until years: BMI percentile Aim: 50th percentile. Malnutrition:< 10th percentile Adults: absolute BMI Aim: female 22kg/m 2 male 23kg/m 2 Malnutrition: EU<18.5kg/m 2 US<19kg/m 2 Sinaaseppel M J cyst Fibrosis 2002; Borowitz D J Pediatr 2002; Lai HJ Curr Opin Pulm Med 2006

6 Malnutrition in registries N=25,651 in 2008 N= 5,141 in 2007 Registries are used also for implementation of quality improvement programmes Lai HJ AmJClin Nutr 2008, Stern M Eur respir J 2008

7 Dietary energy, protein, fat, sodium intake Nutritional management should be started as soon as possible after diagnosis Energy intake Recommendation. Improved weight status has been found at intakes ranging from 110% to 200% of energy needs for healthy population of similar age, sex and size. (B recommendation) Stallings VA J Am Diet Assoc 2008 Protein needs are unknown, usually at the RDI 1-2g/kg/d Fat intake 50% RDI<6 months 40-50% RDI 6 months Sodium supplementation recommended in breast fed babies, hot climates, in case of fever, sport activities. Urine analysis. Heterogeneity in daily practice supplementation

8 Feeding a baby with CF Widespread of NBS Programmes Can be fed the same way as non-cf babies Try to avoid parental anxiety Seek dietician's advice However most babies with CF do need extra calories (carbohydrate, fat density, high energy infant formula (1cal/ml))! rather than increasing milk volume GER Breast milk should be encouraged! low protein, low sodium content Sinaasappel M J Cyst Fibros 2002; CFF; UK; It. May protect against pulmonary infection, better FEV1 Colombo C Acta Pediatr 2007 Infant formula milk. No evidence to support hydrolysed protein formula (exception: MI gut surgery, growth failure, cholestasis, cow s milk intolerance)

9 Weaning in CF infants Solid food can be gradually introduced at 6 months, as for non CF babies Solid food 3 times + 500/600 ml follow on milk formula Toddlers, children Take into account patient s food preferences, cultural dietary beliefs. 3 regular meals and 2 snacks of high nutrient-dense calories Dietician +++ can give advice on food/pert/diary

10 Adolescents A crucial period which needs careful monitoring Increased energy requirements related to rapid physical growth (anabolism), puberty, possible clinical degradation (catabolism), emotional disturbances (anorexia).evaluation of treatment compliance Allow early nutritional intervention if necessary

11 Adults, diabetes, pregnancy Prevalence of malnutrition increases with age-related CF complications Pre diabetes and diabetes up to 30% in adults. Diet rich in energy and fat can be taken. Carbohydrates distribution may be modified with minimal changes in quantity. Adequate insulin therapy. Rolon AM Acta Paediatr 2001, White H J Cyst Fibros 2009 Pregnancy (mild disease and slow rate of pulmonary decline) Nutritional status before and during are major factors of newborn outcome Additional cal/day. Special attention to glucose monitoring, GER Edenborough FP J Cys Fibros 2008

12 Pancreatic Enzyme RT Should be started as soon as PI is diagnosed.based on a biological test (fecal elastase1) delayed enzyme therapy would have devastating effect on nutritional status Breast/formula milk UI lipase/120 ml Solid food dietician UI lipase/g fat Meal UI lipase/kg/meal Snack half dose Borowitz D JPGN 2002 Sinnasappel M 2002 PERT derive from porcine extract, enteric coated; capsules can be opened; granules, tablets must not be chewed/crushed Dosage gradually increased according to stools, weight gain, symptoms Fibrosing colonopathy Smyth RL Lancet 1994 MAX UI/kg/d Borowitz D JPGN 2002 Sinnasappel M 2002

13 E D Fat-soluble vitamins Need to be taken with PERT Serum levels should be checked at diagnosis and annually and doses adjusted Vit A (IU) Vit E (mg) Vit D (IU) Vit K (mg) 0-12 months years 4-8 years >8 years, adult ,000 10, week Serum surveillance Retinol deficit<20µg/dl αtocopherol /lipids 25 OH D3 30ng/ml winter Prothrombin Maqbool A Curr Opin Pulm Med 2008

14 Poor nutritional status related to poor intake Oral nutritional supplements Should not replace food (timing), large variety of flavors and textures Maybe of some use, not fully assessed by RCT Smyth R Cochrane database 2000 Dietary counselling in children positive impact in one RCT Stark LJ Proc Nutr Soc 200 Nocturnal enteral feeding NG tube/ Gastrostomy Usually well tolerated, close monitoring (GER, hyperglycemia, local stomia infection, formula intolerance) Polymeric/semi elemental formula, up to 70% of RDI calories

15 Most studies involve small cohorts with no control group (unethical) Conway SP Cochrane database 2008 There is a strong need for nutritional intervention at earlier stage of the pulmonary disease because enteral feeding cannot slow disease progression in severely compromised patients Milla CE Chest 1998, Steinkamp G Thorax 2002, Oliver MR Peditar Pulmonol 2004, Efrati O JPGN 2006 Appetite stimulants (ie; megastrol acetate, cyproheptadyne hypochloride) Nasr SZ Pediatr Pulmonol 2008 Anabolic hormones (ie; early insulin therapy, IGF1)

16 To conclude Maintaining adequate nutritional status in term of lung health and survival is a cornerstone of CF multidisciplinary approach Consensus based nutritional guideline provides invaluable tool in daily care management Neonatal screening programmes enabling very early followup care, prior to pulmonary involvement, offer a unique opportunity for adequate normal growth Enhancing compliance with better efficacy treatments and low pill burden is a challenge for the future

17 Clinical research with large scale, well designed trials into areas of nutrition is of utmost importance, focusing on: -better understanding of malabsorption mechanisms -assessing PERT efficacy with a simple, validated, efficient biological measurement -recommendations for PERT to support adequate growth -new PERT(...microbial derived products TryzitekTM, porcinederived with bicarbonate buffer Pancrecarb...) -optimal fat-soluble vitamin formulation and dosage -inflammation and fat diet modulation with long chain omega 3 long chain fatty acid

18 Thank you!