Miglustat (Zavesca) and Diet

1. Miglustat (Zavesca) and Diet Helena Champion Dietitian Cambridge University NHS Trust

2. What we will cover • What the side effects of Miglustat can be. • How we digest and absorb food • How Miglustat interferes with carbohydrate absorption • Review modified diet trial • Consider how to modify diet to minimise the risk of side effects

3. Miglustat and its side effects • Associated with unwanted side effects; • Wind • Bloating • Abdominal cramps • Watery diarrhoea • Nausea • Poor appetite • Weight loss

4. How do we process our food • Stomach mixes up the food to ‘chyme’ • Small intestine; food digested and nutrients absorbed • Large intestine; absorbs water and salts. • Bacterial fermentation takes place and faeces are formed

5. Carbohydrates • Come in all shapes and sizes • Single sugars • Monosaccharide, glucose, fructose • Double sugars • Disaccharides, sucrose (sugar), lactose • Long chains of sugars • Starches

6. Absorption of Carbohydrates • Carbohydrates are absorbed on specific transporters/ enzymes in the lining of the small intestine • For glucose • For fructose • For sucrose • For lactose • For starches

7. Small intestine

8. Unwanted Action of Miglustat • Inhibits the action of disaccharide enzymes in the boarder cells of the small intestine • Results in poor absorption of sucrose and lactose • These then pass into the large bowel. • They retain water – watery stools • They are fermented – wind and bloating • The bowel ‘speeds up’ – frequent trips to the toilet!

9. Study at Addenbrooke’s and Royal Manchester Children’s Hospital • Reviewed 29 patients who were taking miglustat • Reviewed weight over 6 month period • Reviewed adnominal symptoms reported

10. Different diets in study • 3 diets were tried • Standard diet with milk based supplements • Milk Free diet • Low disaccharide

11. Results – weight change

12. Results – GI symptoms • In Group A, frequent loose stools were controlled with loperamide • Group B had mild to moderate diarrhoea. • Group C had occasional loose stools. Controlled with individual episode of loperamide

13. Conclusion • Analysis of the data from 29 patients receiving therapy with miglustat shows that with dietary modification, weight gain during treatment can be maintained in line with weight gain potential and episodes of GI disturbances significantly reduced. A diet low in disaccharides was most effective especially when started before treatment commenced.

14. What Dietary Advise do we advise • Avoid foods with added sucrose • Sweets • Chocolate • Cakes • Fruit juices • Smoothies • Sugar coated cereals • Sweetened yoghurts, milk shakes • Mangoes and banana

15. Helpful tip • You can use honey, glucose or fructose to add on cereals, bake cakes and biscuits or add to milk puddings.

16. Unrestricted foods • Meats and fish • Cheese and eggs • Nuts and seeds • Butters, oil and margarines • Tea, coffee and low calorie fruit squashes and fizzy drinks. Unsweetened apple juice. • Leafy vegetables, salad, peas, sweet corn, broccoli, cauliflower. Avocado • Blackberries, cherries, grapes, melon, pears, plums, strawberries, raisins and sultanas. • Non sweet breakfast cereals, rice, pasta, bread, crackers, potato • Glucose, fructose, honey

17. Foods to be cautious with • Limit the portion size of foods containing lactose and sucrose • Milk, plain yoghurt • Low calorie ice cream • Apples, oranges, apricots, nectarines, peaches, pineapple • Carrots, beetroot, sweet potato, parsnips

18. Portion sizes • Milk 120ml at a time • Fruit - 120g i.e a small piece • plain yoghurt - 125g a small pot • Large portions of starch rich foods: • Pasta, potatoes, rice, bread

19. Carbohydrates in food

20. Carbohydrates in Foods

21. Conclusion • By selecting appropriate foods, weight can be maintained , and GI disturbance can be controlled to avoid discomfort and those unexpected incidents.