ESPEN Congress Madrid 2018

Transcription

1 ESPEN Congress Madrid 2018 Nutritional Management In Cystic Fibrosis Nutritional Requirements Of Cystic Fibrosis Patients M. Garriga (ES)

2 Nutritional requirements of cystic fibrosis patients María Garriga, Ph.D. CF Dietitian September 3, 2018

3 Conflict of interests Financial aid for attendance at courses / congresses: Fresenius Kabi, Nestlé Health Science, Mylan

4 Learning objectives Application of nutritional requirements for cystic fibrosis patients in the clinical practice To know the factors that influence nutrient requirements To improve knowledge to optimize the adherence to nutritional treatment

5 The basis of treatment in CF Prevention and treatment CFTR modulator Maintain a good NUTRITIONAL state and treat exocrine pancreatic insufficiency Detect and treat associated diseases: Diabetes (CFRD) and liver disease (CFLD) Adherence to treatment Routine monitoring in specialized units Figure adapted from: M Cohen-Cymberknoh. Hadassah-Hebrew University Medical Center, Jerusalem, Israel Yen EH. J Pediatr 2013;162(3):530

6 Nutritional Status and CF Pulmonary insufficiency is the main cause of death in cystic fibrosis (CF) Nutritional status has a strong positive association with pulmonary function and survival Corey M, et al. J Clin Epidemiol 1988;41(6):583 Kerem E, et al. Eur Respir J 2014;43(1):125

7 Nutritional Status and CF n = 3142 Greater body weight at age 4 is associated with greater BMI, height, weight, better pulmonary function, and better survival through to the age of 18 WAP: weight-for-age percentile Jen Y et al. J Pediatr 2013;162:530

8 years CF Guidelines > 6 years and adults

9

10 Garriga M, et al. Rev Esp Nutr Hum Diet. 2017; 21:74 Nutrition Assessment Nutrition Intervention Monitoring and follow up

11 Goals for nutritional status in CF BMI Percentile BMI Cystic Fibrosis Foundation patient data registry. Bethesda, MD: Cystic Fibrosis Foundation; 2016 Castellani C. et al. J Cyst Fibros 2018;17(2):153

12 Energy targets 120 to 150% of energy needs 110 to 200% of energy needs Personalise Energy needs depend on degree of malabsorption severity of lung disease level of chronic inflammation mutations Sinaasappel M, et al. J Cyst Fibros 2002; 1:51 Stallings VA, et al. J Am Diet Assoc 2008;108:832 ECFSPR Annual Report Orenti A, et al, 2018 Collins. Pediatric Respiratory Reviews :4

13 Macronutrient targets 50-55% % 30-35% 20% 15% Carbohydrates Protein Fat 35-40% non-cf population CF population Turk D, et al. 2016;35(3):557 Matel JL et al. JPEN.2012; 36(1 Suppl):60S

14 Precise PERT adjustment Final scenario What am I eating? PERT dose What should I eat? Education and tips Food record Nutritional evaluation Reach nutritional goals Close nutrition follow-up Research: generation of tools and knowledge Educative games Food and symptoms record Nutritional recommendations Alerts & messages Food & nutrients databases Scientifically valid method to adjust PERT & algorithm Innovative approach for self-management and social welfare of Cystic Fibrosis patients in Europe: development, validation and implementation of a telematics tool (MyCyFAPP) PERT: pancreatic enzyme replacement therapy

15 Macronutrient targets n= 207 Vertical dotted lines represent the recommended intake range or value vertical line represents the mean value of all the centers Calvo-Lerma J. et al. J Cystic Fibros. 2017;16: 510

16 Nutritional disorders Cystic fibrosis-related diabetes (CFRD) energy adequacy carbohydrate intake individualized limited use of artificial sweeteners Bone diseases (osteopenia and osteoporosis) achieve normal weight and growth in children and an ideal body weight in adults optimized dietary intake of calcium, promote the intake of foods rich in calcium (milk, yogurt, dairy products, water with a high content of mineral calcium) vitamin D Dyslipidemia avoid dietary saturated fats and trans fatty acids increase soluble fiber intake Moran A, et al. Diabetes Care 2010;33:2697 Moran A, et al. Diabetes Care. 2009;32:1626 Castellani C. et al. J Cyst Fibros 2018;17(2):153 Rhodes B, et al. J Cyst Fibros 2010;9: CYSTIC FIBROSIS FOUNDATION PATIENT REGISTRY HIGHLIGHTS

17 Minerals and vitamins Higher than normal requirements for salt, calcium, iron and zinc, as a consequence of the increased sweating / intestinal malabsorption / chronic inflammation Fat-soluble vitamin deficiency Turk D, et al. Clinical Nutrition 2016;35(3):557

18 Salt There is a risk for sodium loss hot environmental conditions, fever, rapid breathing, diarrhea Sodium deficiency can be a particular problem for infants sodium content breast milk standard infant formula first baby foods Inform the patient and family to PREVENT dehydration In ¼ teaspoon salt, about 25 mmol or 575 mg of sodium is delivered Turk D, et al. Clinical Nutrition. 2016;35(3):557

19 In infants, add salt to In clinical practice infant formula dilutes in liquid (water or apple sauce) In older children and adults add salt to meals to include salty foods in meals, bread with salt, salty biscuits Borowitz D, et al. J Pediatr 2009;155(6 Suppl):S73 Turk D, et al. Clinical Nutrition 2016;35(3):557

20 The goal of evaluation and treatment is to correct suboptimal levels and achieve optimal biochemical values of these vitamins Fat soluble vitamin levels should be measured: after initiation of vitamin supplementation at least annually and at 3-6 months after a dose change Turk D, et al. Clinical Nutrition 2016;35(3):557

21 Vitamin D n= 377 > 30 ng/ml aureus

22 In clinical practice Patients with CF have to take many drugs What can we do to improve It is not easy to take intake 4 or 5 adherence? different capsules of vitamins every day children have trouble taking vitamins they forget to take them Try to give all the vitamins together in a single formulation, liquid or capsule, depending on age Remind the family / patient that vitamin supplements taken with a meal that contains fats and with PERT not to take on the same day they are going to be monitored Prescribing is not enough, we have to ensure good intake adherence by asking on each visit how and when vitamins are being taken Turk D, et al. Clinical Nutrition 2016;35(3):557

23 MyPlate vs MyCFPlate Fat Energy Protein Adapted from: Kimberly Stephenson, MS, RD, CSP. University of North Carolina at Chapel Hill

24 Feeding undernourished people with CF Diet modification Oral Nutritional Supplements (ONS) Tube feeding (Enteral nutrition) Parenteral Nutrition Nutrition support should be tailored to individual patient needs, taking into account age, nutritional and pancreatic status, home setting, religious and cultural dietary beliefs, and food preferences Turk D, et al. Clinical Nutrition. 2016;35(3):557

25 Diet modification Eating more quantity or more often Fortifying foods Consuming more calorie-dense foods How can we increase their energy intake? Olive oil Cheese (grated) Olives Eggs Nuts (whole, chopped or ground) Cream, butter Avocado Mayonnaise Turk D, et al. Clinical Nutrition 2016;35(3):557

26 Mushroom and asparagus soup 125 g mushrooms 100 g asparagus 125 ml broth 5 g cornstarch 75 kcal (205g) 0.36 kcal/g 1 g fat 125 g mushrooms 100 g asparagus 125 ml broth 5 g cornstarch + 1 cheese (18 g) + olive oil (10 ml) 215 kcal (233g) 0.92 kcal/g 15 g fat 125 g mushrooms 100 g asparagus 125 ml broth 5 g cornstarch 1 cheese (18 g) olive oil (10 ml) + pine nuts (10 g) + cured ham (10 g) 315 kcal (253g) 1.25 kcal/g 24 g fat

27 Oral Nutritional Supplements In clinical practice In clinical practice, short term use of individually prescribed supplements have been shown to increase energy intake and weight in undernourished patients The type and amount of the supplement must be evaluated individually patient's age / preference / nutritional requirements It is recommended to consume them in small sips after meals / before going to bed It is important to recommend that they should not replace the normal food intake, but should complement it Turk D, et al. Clinical Nutrition 2016;35(3):557 Steinkamp G, et al. J Pediatr Gastroenterol Nutr 2000;31:418 Rettammel AL, et al. J Am Diet Assoc 1995;95:454

28 Nougat with orange Cooking workshops Ingredients: 1 person -1 dietary supplement, orange flavor -60g of nougat To achieve positive mealtime experiences

29 Behavioral intervention Nutritional education with behavioral strategies has been shown to be more effective in improving energy intake and growth of children than nutrition education alone Powers SW, et al. JAMA Pediatr 2015;169(5):1 Stark LI, et al. Pediatr Pulmonol 2011;46(1):31 Turk D, et al. Clinical Nutrition 2016;35(3):557

30 Tube-feeding (enteral nutrition) Gastrostomy feeding Feeds are usually introduced gradually and administered as continuous infusions overnight / bolus feeds during the day In clinical practice To increase the likelihood of success explain feeding needs and choices to the patient talk about gastrostomy long before it is needed Enteral nutrition needs PERT PERT is usually given at the beginning and end of the feed Turk D, et al. Clinical Nutrition 2016;35(3):557

31 Specialized nutrition-related treatments We do not make CF-specific recommendations on dietary supplementation of certain fatty acids, or on treatments with antiosteoporotic agents, anti-inflammatory agents, anabolic therapies, or probiotics, as evidence is not sufficient to do so and the risk-to-benefit ration may be too high Turk D, et al. Clinical Nutrition 2016;35(3):557

32 Probiotics in CF Use of antibiotics Diets rich in fat PPIs Use of pancreatic enzyme Alterations in the intestinal microbiota / inflammation Probiotics are hypothesised to correct these alterations by restoring the gut microbial profile towards normal CFTR: Cystic fibrosis transmembrane conductance regulator; PPIs: Proton-pump inhibitor Hee Y et al. Nat Rev Gastroenterol Hepatol 2016; 13:175

33 p=0,003* puntuación 13,0 GIQLY p=0.003 p=0,003* 12,0 11,0 10,0 9,0 n=39 8,0 7,0 1 2 Placebo Probiótico GIQLY: Gastrointestinal Quality Of Life Index L. reuteri significantly decrease intestinal inflammation and increase digestive comfort

34 Educational tools/resources CF webs that have a nutrition section, E.g.:

35 Educational tools/resources Nutritional food blogs on CF

36 The era of CFTR modulators Substantial improvements in nutritional status Strong T, et al. The Journal of Clinical Investigation 1994;93: 347 McKone EF. Lancet Respir Med 2014;2(11):902

37 Emerging pathologies Personalised Nutrition Cystic fibrosis foundation patient registry highlights 2017 Slae M, et al. Lancet Oncol 2018;19(6):719

38 Take-home messages Nutrition is a basic pillar of the cystic fibrosis treatment The major challenge for the CF team is to attain normal growth in children and maintenance of adequate nutrition in adulthood Initiation of nutritional management should begin as early as possible after diagnosis, with a regular follow-up and both patient and family education Promoting adherence to the treatment at all times

39 Thank you for your attention and your time