Diet Analysis

1. Diet Analysis Holly Largent, Bethany Caudill, Jillian Pokorny April 23, 2013

2. Tate Chidsey Male 7 years old – April 29, 2005 Birth Height: 20 inches Birth Weight: 7 lbs. 15 oz. Breastfed until 5 mon. Current Height: 4 ft. Current Weight: 42 lbs. No allergies Cystic Fibrosis Related Metabolic Syndrome

3. What is Cystic Fibrosis? • Inherited chronic condition that affects the lungs and digestive system • According to the Cystic Fibrosis Foundation, CF is caused by a defective gene and its protein product cause the body to produce unusually thick, sticky mucus that: • clogs the lungs and leads to life-threatening lung infections; and • obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. • Cure? • Predicted median survival age is in the late 30s

4. Cystic Fibrosis conductance Transmembrane Regulator (CFTR) • This is the protein produced by the CF gene • Found in the cells that line organs such as the lungs and pancreas • It controls the movement of electrically charged particles in and out of the cells • Like sodium and chloride • With this defected protein, the salt balance is disturbed • Skin will be very salty • Because there is not enough salt and water on the outside of the cells, the thin layer of mucus becomes very thick and difficult to move • The salt channels in the airways, intestines, pancreas ad reproductive tract are all affected by the CF gene and its protein, CFTR

5. Symptoms of CF frequent coughing, at times with phlegm numerous lung infections wheezing or shortness of breath very salty-tasting skin poor growth/weight gain despite adequate nutrient intake regular greasy, bulky stools or difficulty in bowel movements.

6. DiagnosisCystic Fibrosis Related Metabolic Syndrome • Mother and sister suffer as well • Showed signs of digestive issues his whole life • Doctors explained it away until he fell off growth charts • Diagnosed with pancreatic insufficiency in February of last year • Officially diagnosed with Cystic Fibrosis Related Metabolic Syndrome in May, 2012 • Now believed to have Cystic Fibrosis Related Disease • Rare, very little information known

7. Cystic Fibrosis Related Metabolic Syndrome(CFRMS) • Not Cystic Fibrosis itself • Sweat test with a borderline result or genetic test showing one CF gene • indicator of CFRMS • Person with condition/disease will suffer in similar ways • Some have problems in the airways or sinuses, the intestines and pancreas, or the reproductive tract • Symptoms: • No weight gain • Loose stools, very bad gassiness or constipation that last more than 2 weeks • Very bad stomach aches • Coughing or wheezing that last more than 2 weeks

8. CFRMS Continued… • For Tate, he is sparred the severe lung consequences but is pancreatic insufficient: • Body correctly produces pancreatic enzymes, but do not reach the small intestine • The ducts in the pancreas become clogged by the mucus • Blocks the enzymes • Results in the inability to breakdown and absorb nutrients from foods eaten • Poor weight gain • Needs to take pancreatic enzymes before consuming anything with fat or protein

9. Functions of the Enzymes • •Digest carbohydrates, protein and fat • • Help with weight gain • • Promote nutrient absorption • Family bases amount of enzymes taken on: • Amount of fat or protein in each meal or snack • 1 enzyme per snack, 2 enzymes per meal • Given an extra enzyme at the end of rich meals (pizza, lasagna, dessert)

10. CFRMS Continued.. • Also takes: • An anti-acid in the morning • minimize effects stomach acid has on breaking down enzymes • ADEK vitamin • A Cystic Fibrosis vitamin that has the fat soluble vitamins already broken down for him, since fat is especially hard for his body to process

11. Typical Dietary Plan for a child with CFRMS • Make sure they are eating even when in pain or not hungry • Meals are high in calories (needs to consume more than an average child similar in age and size) • Need to consume more salt • Due to the way CF regulators regulate salt transfer • Typical diet includes: fast food, butter, heavy cream, desserts, etc. • Anything to increase the child’s desire to eat, while maintaining the calories needed

12. Health Implications of a Typical CFRMS Diet • Focused on immediate concern of keeping weight on • Not the long term heart and pancreatic health of the patient • Heart disease in people with CFRMS is increasing • Due to high fat, low density food choices • Many children are given Scandi-Shake (extremely high in calories and fat, also contains trans fat) • Confirmed by dietitians and doctors

13. Modifications to Tate’s Diet • Whole foods, high density • Mother makes most foods at home • Adds fat through more natural sources • Olive oil, nuts, flax seed, full dairy products (at times uses low fat dairy, supplements accordingly) • Receives Ensure products because of the enzymes he is on • High in fat, only used a few times per week to help give calories

14. Food Log Example: Total Calories: 2,191 • Breakfast: Oatmeal • Quick Cooking Oats – ½ cup • Chocolate Coconut Milk – ½ cup • 2% milk – ½ cup • Crunchy PB – 1 Tbsp • 1 small banana • Snack: Gala Apple – 1 small • Lunch: Quesadilla • Soft taco shell (2); shredded mexican cheese – ¼ cup • Tortilla chips (white corn) – 1 serving • Medium salsa – 2 tbsp • EnureEnlive – Apple flavored juice – 1 box • Snack: Yogurt Parfait • Plain non-fat greek yogurt – ½ cup • Strawberries (2 berries) & blackberries (4) • Honey oat granola – ¼ cup • Dinner: Chicken Sub • Whole wheat hot dog bun (1) • Italian chicken sausage (1) • Green peppers – ¼ cup • Tomato & basil sauce – ½ serving • Sweet potato fries – 1 serving • Broccoli – 1 cup • 2% milk – 1 cup

15. 3-Day Food LogMacronutrient Averages

16. 3-Day Food LogMicronutrient Average

17. Micronutrient Average Continued..

18. Dealing with the Disease • Specified eating times & no grazing • Only allowed 10 enzymes throughout the day • Snacks without enzyme must have less than: • 1-2g fat • 1-2g protein • Fruit is always allowed

19. Dealing with the Disease • Used to be a picky eater • Due to stomach aches • Now open to trying a variety of foods • Certain foods make him feel worse (typically processed foods) • Likes: pizza, spaghetti & meatballs, ice cream, broccoli & spinach salad • Dislikes: casseroles with veggies mixed in, most soups except chili

20. Dealing with the Disease • School: • Goes to school nurse before lunch to take an enzyme • Packs lunch every day • Peanut butter & jelly on whole wheat bread • yogurt parfaits, veggies with dip, and fruit • Mid-Morning snack at school: • Only snacks that require enzyme • Minimizes his visits to the nurse’s office • Minimizes exposure to sick children • Mother notified of special snacks via text or email • Cookbook once a month: • Making his own creation

21. Activity Level • Very active • Loves to ride his bike and run • Took 1st place in competitions at school • Played flag football & basketball this year • Recent restrictions to his participation in school sports by his doctor • Incidence of osteoporosis

22. Family Support Mother and sister suffer from CFMRS as well Financially well off Mother does not work Put much time & thought into their diets Has become very passionate about nutrition

23. Recent Victories to their Health • Put on a pound at last doctor’s visit • No longer needs to take an ADEK vitamin • Dietary intake is sufficient • Decreased pain

24. Great Strides Walk to raise money for the CF Foundation CF Foundation is making progress by leaps and bounds in their treatment options. The lives of those who suffer from CF are improving drastically. http://www.cff.org/great_strides/dsp_donationPage.cfm?registeringwalkid=8368&idUser=608585

25. Sources "About Cystic Fibrosis." CysticFibrosisFoundation.Org. Cystic Fibrosis Foundation, 08 May 2011. Web. 21 Apr 2013.