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The Role of Diet in Wilson’s Disease

The Role of Diet in Wilson’s Disease

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The Role of Diet in Wilson’s Disease

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  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