Weight Control and Exercise

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1 Weight Control and Exercise STEPHANIE HACKER, MS, RD, LDN, CNSC METABOLIC DIETITIAN NEWBORN SCREEN PROGRAM MANAGER DIVISION OF CLINICAL AND TRANSLATIONAL GENETICS

2 Disclosures Member of the BioMarin Speaker s Bureau Served on Advisory Boards for BioMarin

3 Fitness and Me

4 Overview General Health Weight Control Balance, Loss, and Gain Components: Metabolism, Diet, and Exercise Effects of excess weight Weight Loss and Maintenance PKU Exercise Benefits Types Components Nutritional considerations PKU

5 Healthy Living Balanced Diet Exercise Sleep Stress Avoidance Healthy Relationships

6 Weight Control Maintaining a healthy weight helps to control/prevent: Cholesterol Heart Disease Blood pressure Blood sugar Diabetes Arthritis Some cancers Components Energy is never created or destroyed" Output Resting Metabolism Physical Activity Thermic Effect of Food Input Food Intake

7 Excess Weight 70.2% of U.S. Adults are considered Overweight/Obese 32.5% are overweight 37.7% are obese Definition: BMI=kg/m 2 Overweight Adults: kg/m 2 Adolescents: 85 th - 95 th BMI-for-age Obesity Adults: >30 kg/m 2 Adolescents: >95 th BMI-for-age Waist Size Men >40 inches Women >35 inches Effects: type 2 diabetes high blood pressure heart disease and strokes certain types of cancer sleep apnea osteoarthritis fatty liver disease kidney disease depression/mood disturbances fatigue pregnancy problems high blood sugar during pregnancy increased risk for cesarean delivery

8 Weight Loss and Maintenance Weight Loss Energy In is less then Energy Out Increased Energy Expenditure Exercise Increases in everyday activities Decreased Energy Intake Alterations in diet Increased Protein and Fiber More vegetables and fruit Decreased Sugar Portion Control Extreme energy/calorie deprivation results in weight regain Hydration 5-10% of body weight loss has positive effect on health Weight Maintenance Weight changes of <3% of body weight

9 Weight Control and PKU Rates of Overweight/Obesity PKU patients, particularly females, may have increased rates of overweight/obesity compared to the general population. Mixed results in other studies Burrage (2012)

10 Rocha (2013) Weight Control and PKU Diet composition High energy intake Decreased satiety Metabolic foods Higher carbohydrate intake Many low Phe foods are high in carbohydrates Low fiber content Sugary drink consumption Specialized low protein foods High carbohydrate content Large portions consumed due to free nature Preference to sweet taste Limited fruit and vegetable intake Lower fiber Intake and increased reliance and Specialized low protein foods Simplified Low Protein Diet may be beneficial, but free fruits and veggie does not always correlate to increased intake. Medical formula Traditionally high in calories and carbohydrates ~ Calories per 10g Protein Equivalent 60g protein requirement means about 750 Calories from formula Lower Calorie formulas available GMP formulas have been shown to result in increased satiety compared to Amino Acid Formula

11 Weight Control and PKU Food Behavior Controlling, Rewarding and Restrictive Food Behavior is a factor associated with unhealthy weight gain and overweight Consideration for liberalization of Phe Restricted Diet (new treatments or self-relaxation of diet) Eating together as a family reduces risk of overweight/obesity Many PKU patients are fed alone Many PKU patients are reluctant to try new foods Limited intake of fruit and vegetables that were not introduced at an early age Diet Adherence Non-adherence related to increased weight in multiple studies Physical Activity Limited studies on PKU and activity Poor executive functioning, organizational skills, and depression may lead to lower motivation to participate in physical activity. Alterations in Metabolism Consumption of PKU type meal (metabolic formula and specialized low protein foods) resulted in lower thermic effect of food and postprandial fat oxidation compared to control diet Rocha (2013), Robertson (2013) and Alfheeaid (2018)

12 Exercise

13 Benefits of Exercise Weight Control Disease Prevention Strengthens Bones and Muscles Improves mood, concentration, and attention Boosts Energy Better Sleep Fun and Social

14

15 Types of Exercise Endurance Also known as Cardiovascular or Aerobic Activity Walking, Jogging, Dancing, Swimming, Biking, etc. Muscle Strengthening Also known as Resistance Training or Carrying a Load Pushups, Squats, Lunges, Planks, etc. Balance Prevents falls Shifting weight and single leg balance Flexibility Prevents injuries Stretching, yoga

16 Components of Exercise Intensity How hard the activity is Moderate Can talk but not sing Vigorous Can say a few words Frequency How often activity is preformed Duration How long the activity is preformed

17 Exercise Recommendations Adults 150 minutes per week of moderate intensity aerobic activity OR 75 minutes of vigorous physical activity AND muscle strengthening activities on 2 or more days per week (involving all muscle groups) Older adults should focus on balance exercises to prevent falls More Physical Activity equates to increased health benefits (>250 min/week) Children 60 minutes of moderate or vigorous physical activity each day 3 days per week of vigorous activity. Muscle and Bone strengthening exercises 3 days per week Body weight exercises (Squats, Lunges, Push-ups, etc.), Impact exercises (running, jumping), and using applied resistance (weights, bands, etc.).

18 Incorporating Exercise Take the scenic route Walking Taking stairs Deskercise Plank challenge Take long walks Make family time, active time Play at the park with your kids Get fit with your friends Find an activity that makes you happy Sports Group exercise classes Home workouts Incorporation into your daily activities

19 Nutritional Considerations for Exercise Energy Both Resistance and Endurance exercise increases energy expenditure Energy Output>Energy Input = Weight Loss Undernutrition can limit exercise adaptations Protein Increased muscle protein synthesis with exercise Needs increase with exercise Recommended Dietary Allowance (RDA )=0.8g/kg (in adults) Protein Needs for PKU Patients: g/kg (in adults, % of RDA) Needs can be met with medical formula Needs for training g/kg (in adults) High protein intake with decreased energy intake can maintain muscle mass Timing of intake Intake should be distributed throughout the day g/kg (or 15-20g proteins) should be consumed 0-2 hours after activity for enhanced muscle protein synthesis Protein Quality High quality proteins contain all essential amino acids in appropriate concentrations Amino Acid vs. GMP Formulas Leucine initiates muscle protein synthesis

20 Nutritional Considerations for Exercise Carbohydrates Most efficient fuel for muscles during exercise Supports activity at a large range of intensities Stored as Glycogen in muscle and liver Fatigue occurs when depleted Including a nutrient dense carbohydrate at meals and snacks will help sustain stores 3-12 g/kg/d carbohydrates are needed to replenish glycogen stores Fruit, low protein yogurt, sweet potato, low protein rice/pasta Fluid Dehydration (2% loss of body weight due to fluid loss) causes: Increased perceived exertion Reduced work capacity Once thirsty you may already be dehydrated Water vs Sports Drinks Water is the beverage of choice for most recreational activities Sports drinks provide: Calories May help decrease protein breakdown in long events (or workouts) Electrolytes Improves hydration by enhancing absorption Flavor May promote consumption

21 Exercise and PKU Limited Studies and Knowledge *Some recommendations based off patient experiences Phe levels Most studies show that Exercise has no effect on Phe levels Current Literature has measured acute response (30min-9 hours) Muscle protein synthesis is optimized at 24 hours post exercise Muscle breakdown may cause an increase in Phe levels, due to release of Phe from muscles. Insufficient protein/energy intake Weight loss Increasing muscle mass could possibly lead to increased Phe tolerance due to increased Phe incorporation into skeletal muscle. Helms (2014)

22 Exercise and PKU Protein Quality and Timing Formula Amino acid (AA) based formulas are made out of individual amino acids and do NOT contain Phe May be oxidized and absorbed more rapidly than whole protein Some studies have shown lower protein utilization with free amino acids To replicate higher quality protein source, can take a Phe containing food with AA formula Complimentary protein Glycomacropeptide (GMP) contains an intact protein, along with free amino acids. More physiologically similar to proteins absorbed in the diet Contains Phe, which may lead to improved protein synthesis when taken after exercise Both formulas should be taken within 4 hours (ideally 2 hours) of activity the anabolic effect of exercise diminishes with increased time during recovery Performance Supplements Not recommended by any scientific body for regular use Limited regulation on ingredients or labeling Many may contain Phenylalanine or Aspartame Contact your dietitian if you are interested in taking a supplement

23 EVERYONE CAN and SHOULD BE ACTIVE! INFORM CLINIC/DIETITIAN OF ANY CHANGE IN EXERCISE OR ACTIVITY

24 Thank You! STEPHANIE HACKER

25 References Agarwal S. Cardiovascular benefits of exercise. Int J Gen Med. 2012;5:541-5 Alfeeaid H., Konstantinos G., Nastase A.M., et al (2018) Impact of phenylketonuria type meal on appetite, thermic effect of f eeding and postprandial fat oxidation. Clinical Nutrition. 37(3) The Association Between School -Based Physical Activity, Including Physical Education and Academic Performance. Centers of Disease Control. Accessed February 12, 2018 Breymeyer, K. L., Lampe, J. W., McGregor, B. A., & Neuhouser, M. L. (2016). Subjective Mood and Energy Levels of Healthy Weight and Overweight/Obese Healthy Adults on High-and Low- Glycemic Load Experimental Diets. Appetite, 107, Bruinenberg, V.M., Gordijn, M.C.M., MacDonald, A., et al. (2017) Sleep Disturbances in Phenylketonuria: An Explorative Study in Men and Mice. Front. Neurol. 8:167. Burrage LC, McConnell J, Haesler R, O'Riordan MA, Sutton VR, Kerr DS, et al. High prevalence of overweight and obesity in fem ales with phenylketonuria. Molecular Genetics and Metabolism 2012;107(1 2): Demirdas, S.; Coakley, K.E.; Bisschop, P.H.; Hollak, C.E.; Bosch, A.M.; Singh, R.H. (2015) Bone health in phenylketonuria: A systematic review and meta-analysis. Orphanet J. Rare Dis. 10, e46. Evans S, Daly A, Chahal S, et al,. (2018). The influence of parental food preference and neophobia on children with phenylketonuria (PKU). Molecular Genetics and Metabolism, 14, Exercise: 7 benefits of regular physical activity. Mayo Clinic. Accessed February 12, 2018 Glossary of Terms. Accessed February 12, 2018 Health Risks of Being Overweight. National Institute of Diabetes and Digestive and Kidney Diseases, U.S. Department of Health and Human Services, 1 Feb. 2015, Helm, J. (2014) The effects of an acute bout of moderate -intensity exercise on plasma amino acid concentrations in adolescent boys with phenylketonuria. Scholar Archive

26 References How much physical activity do adults need? Centers for Disease Control and Prevention. Accessed February How much physical activity do children need? Centers for Disease Control and Prevention. Accessed February 12, 2018 Jani R., Coakley k., Douglas T., Sing R. (2017). Protein intake and physical activity are associated with body composition in individuals with phenylalanine hydroxylase deficiency. Molecular Genetics and Metabolism. 121, Jahja, J., Huijbregts, S.C., de Sonneville, L.M., et al., (2017) Cognitive profile and mental health in adult phenylketonuria : A PKU-COBESO study, Neuropsychology, 31, 4, MacDougall, J.D., Gibala, M.J., Tarnopolsky, M.A., MacDonald, J.R., et al. The time course for elevated muscle protein synthesis following heavy resistance exercise. Canadian Journal of Applied Physiolohgy. 1995, 20, Mazzola P.N., Tiexeira B.N, Schirmbeck G.H., et al. (2015) Acute exercise in treated phenylketonuria patients: Physical activity and biochemical response, Molecular Genetics and Metabolism. Reports 5, Robertson L.V., McStravick, N., Ripley S., Weetch E. et al. (2013)Body mass index in adult patients with diet-treated phenylketonuria Journal of Human Nutrition and Dietetics, 26 (Suppl. 1), 1-6 Rocha, J.C., MacDonald, A., Trefx, J. (2013) Is Overweight an Issue in PKU? Molecular Genetics and Metabolism. 110 Suppl:S Stevens J., Truesdale K. P., McClain J. E., Cai J. The definition of weight maintenance. International Journal of Obesity. 2005;30(3): Thomas, D. T., Erdman, K. A., & Burke, L. M. (2016). Position of the Academy of Nutrition and Dietetics, Dietitians of Canada, and the American College of Sports Medicine: Nutrition and Athletic Performance. Journal of the Academy of Nutrition and Dietetics,116(3), Retrieved February 25, Types of Fitness. American Heart Association. Fitness_UCM_462352_Article.jsp#.WoOdWZM-fOR. Accessed February 12, 2018

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