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
PAUSE on slide, make note of cohorts and genotype

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