The Wilson's Disease Diet: What Not to Eat
If you are here, it is likely that you have Wilson's Disease or you think you might have it. Wilson's Disease is a rare genetic disorder characterized by excessive accumulation of copper in the body's tissues. This excess copper can lead to severe neurological and psychiatric symptoms, liver disease, and even death if not managed properly. The condition arises from mutations in the ATP7B gene, which plays a critical role in regulating copper levels in the body by facilitating its removal. Under normal circumstances, copper absorbed from the diet is transported to the liver, where it binds to a protein called ceruloplasmin. This binding is crucial because ceruloplasmin serves as the primary copper-carrying protein in the blood, helping to transport copper to various parts of the body where it is needed, and more importantly, to remove excess copper by excreting it into bile.
In individuals with Wilson's Disease, either the production of ceruloplasmin is deficient, or the protein's ability to bind copper is compromised, leading to significantly reduced levels of this essential protein or its function. As a result, copper is not properly excreted into the bile and instead begins to accumulate within the liver. Over time, the liver's capacity to store copper is exceeded, and the excess copper spills over into the bloodstream and is deposited in other organs, including the brain, kidneys, and eyes. At this point, a Free Copper test will likely show elevated results of 200+ mcg/L. This widespread deposition of copper can cause a range of debilitating and potentially life-threatening symptoms. Thus, managing dietary copper intake becomes crucial for those affected, as even normal dietary amounts can exacerbate the condition significantly.
Wilson’s Disease can present itself in various forms, often described as a spectrum, not only because of the wide range of symptoms but also due to the different genetic mutations that can affect copper metabolism. Interestingly, not all individuals with Wilson's Disease have low levels of ceruloplasmin. Some may have a mutation that reduces ceruloplasmin’s affinity for copper rather than its quantity, which complicates diagnosis and management. Chronic constipation can significantly worsen the condition, regardless of its severity. Since copper is excreted through bile and then eliminated in stool, infrequent bowel movements lead to reabsorption of copper into the bloodstream. This phenomenon was a key factor in the manifestation of my symptoms at the age of 33, highlighting the importance of regular gastrointestinal health in managing the disease.
Top 15 Foods to Avoid If You Have Wilson's Disease
Liver and other organ meats - Extremely high in copper, one serving can exceed the daily copper intake limit.
Shellfish - Particularly oysters and lobster, which contain significant amounts of copper.
Nuts and seeds - Especially walnuts, almonds, and sunflower seeds.
Mushrooms - Known for their higher copper content.
Chocolate - Dark chocolate is particularly rich in copper.
Dried fruits - Such as prunes, raisins, and dried apricots.
Avocados - While nutritious, they are also high in copper.
Soy products - Including tofu and soy milk.
Leafy greens - Such as spinach and kale.
Beans and lentils - These are healthy but have high copper levels.
Whole grains - Whole wheat, barley, and rye can contribute to excess copper intake.
Potatoes - Particularly when eaten with the skin on.
Some shellfish - Crab and lobster can contain high levels of copper.
Cocoa and coffee - Both contain notable amounts of copper.
Tap water - In some areas, copper pipes can increase water’s copper content.
As individuals with Wilson's Disease need to manage their copper intake carefully, understanding food labels and the copper content of foods can become a part of daily life. It’s also beneficial to work with a nutritionist who can help tailor a diet that fits within the low-copper guidelines while still providing all the necessary nutrients for good health. Furthermore, integrating regular physical activity and ensuring a diet rich in other minerals and vitamins that can help the body regulate copper absorption, such as zinc, may also be advised by healthcare providers.
In addition to dietary management, regular medical check-ups are crucial for monitoring copper levels and liver function. Medications that help remove excess copper from the body, such as chelating agents, are often prescribed to help manage the disease. It’s important for patients and families to understand the potential triggers of copper accumulation and the lifestyle adjustments necessary to maintain health. Awareness and education about Wilson's Disease can empower patients to lead healthier lives and can facilitate early detection and treatment, significantly improving the prognosis.