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

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)

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

Foods with High Copper Concentrations: Shellfish

Foods with High Copper Concentrations: Chocolate

Foods with High Copper Concentrations: Mushrooms

Foods with High Copper Concentrations: Beef Liver

Foods with High Copper Concentrations: Nuts

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

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

Cookware Use common sense, no copper containers or copper cookware

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

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

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

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