Nutrition and Supplements

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1 Nutrition and Supplements Parent Project Muscular Dystrophy s 2017 Connect Conference Saturday July 1 3:50pm Zoe Davidson PhD AdvAPD Monash University, Melbourne, Australia Murdoch Childrens Research Institute, Melbourne Australia

2 TODAY 1) for nutrition care during childhood 2) Supplements

3 [1] Davidson et al. Eur J Clin Nutr Aug;68(8): [2] 2007 Australian NaEonal Children s NutriEon and Physical AcEvity Survey

4 What does appropriate growth look like?

5 Measure height and weight every 6 months Plot the height and weight on your child s growth chart (doctor, dietitian)

6 Evidence for weight management Only 1 case study of dietary intervention: 1 boy with DMD and 1 boy with BMD Weight loss was possible: increase in mobility and self esteem no negative effects on lean mass To achieve weight loss food energy reduced to 600 kcals/day Edwards RH et al. Dev Med Child Neurol. 1984;26(3):

7 Considerations for weight management Primary goal = STABILISE weight Weight loss may or may not be required if some potential for height growth Weight loss should be carefully monitored Need to consider on individual basis

8 Considerations for weight management prevention of excess weight gain is preferable to severe restriction in the already obese Provide anticipatory guidance for boys at key periods Follow up with regular monitoring of growth Griffiths & Edwards. Arch Dis Child. 1988;63(10):

9 Eat More ü Fruit ü Vegetables ü Lean meat ü Low fat dairy ü Wholegrain cereals ü Low energy snacks Eat Less x Sugary drinks: softdrink, juice, cordial x x High fat foods: fast foods, deep fried food, bakery snacks Snacks: lollies, chips, chocolate 9

10 Know your dietitian Monitor weight/height regularly

11 Key times to connect with a dietitian ü Soon after diagnosis ü On starting steroids ü Yearly ü If flagged on growth monitoring

12 DMD and bones Boys with DMD are at increased risk of decreased bone mineral density Two factors: Steroid therapy Decreased muscle mass Optimising nutrition can assist with maintaining bone mass Calcium and Vitamin D

13 Ensure adequate intake of calcium 2-3 serves dairy/dairy equivalents each day Vitamin D supplementation Monitor vitamin D levels

14 SUPPLEMENTS

15 Creatine monohydrate Creatine is an amino acid derivative found in muscles Stores energy for immediate use Commonly used in athletes Cochrane systematic review (2013): High quality evidence from RCTs shows that short- and mediumterm creatine treatment increases muscle strength in muscular dystrophies. There is also evidence that creatine improves functional performance in muscular dystrophy. Creatine is well tolerated in these people. Kley RA et al. Cochrane Database Syst Rev. 2013; Jun 5; (6):CD

16 Creatine monohydrate 5g/day, 3 g/day, 100mg/kg/day, 10g/day (adults) Significant increase in maximum voluntary contraction weighted mean difference of 8.47% (95% CI: 3.55, 13.38) Increase in lean body mass weighted mean difference 0.63kg (95% CI: 0.02, 1.25 ) Increased bone mineral density by 3% Kley RA et al. Cochrane Database Syst Rev. 2013; Jun 5; (6):CD

17 Using creatine monohydrate White powder, no taste Creatine monohydrate (1g/g) Mix with milk or small amount of juice Breakfast time

18 Glutamine Inhibit or slow down whole body protein breakdown Randomised control trials x 2 Escolar et al. (2005) 0.6g/kg/day unable rule out an effect of glutamine supplementation Mok et al. (2009) 0.5g/kg/day No additional benefit of glutamine on muscle mass or function in ambulatory DMD boys Improvements when used in conjunction with steroids Escolar D et al. Ann Neurol. 2005;58(1): Mok E, et a. PLoS ONE 2009;4(5):e5448.

19 Coenzyme Q10 (CoQ10) Potent antioxidant prevent oxidative damage Spurney et al. (2011) CINRG pilot trial of coenzyme Q10 in boys with DMD 12/16 boys completed Dosed to achieve blood levels of 2.5 µg/ml (90 510mg/day; average ~30mg/kg/day) 9/12 boys showed an increase in strength; overall 8.5% increase. No change in cardiac parameters Salehi et al. (2017): Double blind RCT 3-5mg/kg/day for 6 months No significant effect on echocardiographic parameters (myocardial performance index) Spurney CF et al. Muscle Nerve. 2011;44(2): Salehi et al. Electron Physician. 2017; 9(3):

20 Amino Acids Carnitine - Escobar-Cecillo et al. (2013) Double blind RCT 50mg/kg twice daily for 12 months No effect on functional outcomes Leucine - Mendell et al. (1984) Double blind RCT 0.2g/kg/day for 12 months No therapeutic effect Escobar-Cecillo et al. Current Topics in NutraceuEcal Research. 2013;11(3): Mendell et al. Muscle Nerve. 1984;7(7):

21 Supplements in the mouse model L-Arginine (amino acid) Green tea extract (antioxidant) Protandim (antioxidant) HMB in combination supplement Fish oil Chaubourt E et al. Neurobiol Dis. 1999;6: Voisin V et al. Neurobiol Dis. 2005;20: Buetler TM et al. Am J Clin Nutr. 2002;75(4): Dorchies OM, et al. Am J Physiol Cell Physiol. 2006;290(2): Qureshi MM et al.j Diet Suppl June 1; 7(2): Payne ET, et al. Muscle Nerve. 2006;33(1): Mauricio et al. Clin Nutr Aug;32(4):636-42

22 Considerations when using supplements Seek advice from Dietitian/Doctor Consider safety Can be expensive Are you getting what you paid for? careful buying over the internet Objective outcomes

23 Thank you

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