The Role of Diet in Wilson’s Disease

1. The Role of Diet in Wilson’s Disease Judy Fitzhugh RD, LDN Kovler Organ Transplant Center Northwestern Memorial Hospital

2. The Role of Diet in the Management of Wilson Disease • Wilson Disease cannot be managed solely with diet • The role of diet remains controversial • Many physicians recommend low copper diets during the initial stages of treatment (such as one year) • It is important to not excessively restrict your diet for your entire life (quality of life issue)

3. Dietary Copper Consumption • The recommended oral daily copper intake is 0.9mg/ day1 • The average daily Western diet typically provides 2-5mg/ day of copper1 • Most dietary copper is absorbed and excreted via secretion into bile 1Roberts EA and Schilsky ML. AASLD Practice Guidelines, 2008

4. Foods with High Copper Concentrations: Shellfish

5. Foods with High Copper Concentrations: Chocolate

6. Foods with High Copper Concentrations: Mushrooms

7. Foods with High Copper Concentrations: Beef Liver

8. Foods with High Copper Concentrations: Nuts

9. Other Foods of Concern • Avocado • Dried Beans • Whole Grains • Nectarines & Dried Fruit • Brewer’s Yeast • Vegetable Juice

10. The Water Supply • Municipal water is generally safe • Well water or water brought through copper pipes can be measured for copper content • Water purifying systems may be effective in reducing water with a high copper content

11. Cookware • Use common sense, no copper containers or copper cookware

12. Role of Dietician Consultation • Consultation with a dietician is advisable for practicing vegetarians1 • Consultation should be considered: • during the initial year of therapy • at any time if patient quality of life issues arise 1Roberts EA and Schilsky ML. AASLD Practice Guidelines, 2008

13. Copper Deficiency • Deficiency may cause; • Neutropenia • Impaired bone calcification • And hypochromic anemia not responsive to iron supplements.

14. Maintenance Therapy with Zinc • 50 mg elemental zinc 3 times a day • Take 30 minutes before meals • 220 mg of zinc sulfate • May cause gastric irritation • If not tolerated, zinc gluconate • Zinc is not to be given at the same time as penicillamine or trientine • Allow 5 hours between doses

15. Summary: The role of diet in Wilson Disease • Wilson Disease cannot be managed solely with diet • Dietary copper restriction should be considered during the initial phase of therapy • Wilson Disease patients can enjoy a relatively normal diet throughout their life • Dietician consultation is advised for vegetarians and should be considered in all patients