And We’re Not Having Hot Mush Today…

2 Jul

“We’re having cold mush!”  jk 😉  Are you getting tired of all the Broadway musical references?  Too bad!  I grew up in a theatrical family, so you’ll just have to deal with it! ;-p

Aannyywwaayy…

Wilson’s disease is a rare genetic disorder.  It follows an autosomal recessive pattern(for those who remember biology class).  A person must inherit a specific mutated gene from each parent for symptoms to exist.  It is a condition characterized by the inability to excrete copper properly.  Copper is a mineral that is abundant in many types of healthy foods.  Everybody requires a certain amount of copper in their diet, much like we require iron or calcium.  However, in Wilson’s disease, copper is allowed to build up to poisonous levels and can lead to organ damage.

The liver and/or neurological system tend to be the first areas of attack.  Kayser-Fleischer rings (Seen here: http://www.wilsonsdisease.org/wilson-disease/kayserfleischerrings.php) are often a symptom in those whose neurological systems have been affected.  However, over half of those with liver damage will not experience this tell-tale sign.

Treatment for Wilson’s disease generally includes drugs that help release copper from the organs & zinc tablets to interfere with dietary copper absorption.  A low copper diet is critical during the initial drug treatment and strongly encouraged thereafter.  Still, many are able to incorporate high copper foods  into their meals on an occasional basis once they have reached the maintenance phase.

People with Wilson’s disease ought to be wary of their water supply.  I checked out the 2011 water report for my city and discovered that the copper content clocked in at 1.3 ppm.  According to the Wilson Disease Association, the goal is to consume water with less than 0.1 ppm of copper.  Luckily, I already have a water filter that guarantees a 96-99.99% reduction in copper, so I am covered on that front.

Supplements that provide copper are obviously forbidden.  Since I am purposely creating a deficit through diet and do not have Wilson’s Disease, I will be supplementing with a copper-infused multi.

Finally, alcohol is discouraged as it is a hepatotoxin (fancy word for: incurs liver damage).

Off the Menu: (NOTE: ** indicates moderate allowed amounts-no more than 6 total servings from this category per day)

*Dairy: all dairy is permitted

*Grains: millet, barley, wheat germ, quinoa, bran breads & cereals, cereals with > than 0.2 mg copper per serving

**whole wheat bread (1 slice), whole wheat crackers (6), instant oatmeal (1/2 cup), cereals with 0.1-0.2 mg of copper per serving

*Vegetables: mushrooms, veggie juice, & fresh sweet potatoes

**potatoes(1/2 cup), pumpkin (3/4 cup), parsnips (2/3 cup), winter/summer squash (1/2 cup), green peas (1/2 cup), bean sprouts (1 cup), beets (1/2 cup), spinach (1/2 cup cooked/1 cup raw), tomato juice/other tomato products (1/2 cup), broccoli (1/2 cup), asparagus (1/2 cup), olives (2 medium)

*Fruits: nectarines, commercially dried fruits including raisins, dates, & prunes, avocados

**mango (1/2 cup), papaya (1/4 average), pear (1 medium), pineapple (1/2 cup)

*Meat, poultry, eggs & fish: lamb, pork, pheasant quail, duck, goose, squid (well there go my 4th of July plans! ;-)), salmon, organ meats (liver, heart, kidney, brain), shellfish (oysters, scallops, shrimp, lobster, clams, crab) & meat gelatin

**all fish except shellfish & salmon (3 oz), dark meat turkey & chicken (3 oz)

*Nuts/Seeds: all nuts/seeds

*Beans/Legumes: dried beans such as soy beans (soy milk, tempeh, tofu, etc.), lima beans, baked beans, garbanzo beans, pinto beans, dried peas, lentils, soy flour

**peanut butter (2 Tbsp)

*Food additives/misc: brewer’s yeast

**ketchup (2 Tbsp), dehydrated & canned soups (1 cup)

*Fun Stuff: chocolate!

**syrups (1 oz), licorice (1 oz), carbonated beverages (12 oz), alcohol

The diet for Wilson’s disease is turning my world upside down.  It rejects beans & other legumes along with nuts and seeds.  Jumpin’ Jellyfish!  These have been my staples for the past 6 months!  When I read the list of “allowed foods” for this diet, the pattern sounded familiar.  Unrestricted foods include many types of meat, dairy, and refined grains.  Aha!  It’s the Standard American Diet (SAD)!  I found this slightly amusing as the SAD tends to be deficient in a number of vital nutrients, and copper just happens to be one of them.

A study published in the American Journal of Clinical Nutrition  found that copper is less bioavailable on a vegetarian diet.  However, vegetarian diets tend to be higher in copper anyway, so the difference may not be statistically important enough in terms of copper control.  Still, it seems reasonable to conclude that it may be safer to choose a vegetarian item, like tofu, over organ meats for those occasional high copper indulgences.  Some are able to manage a vegetarian diet by medicating properly and avoiding certain higher-copper foods (such as mushrooms & nuts) as much as possible.

What I ate my first day with Wilson’s disease:

Breakfast: Leftover porridge (scottish oats) fried with a scrambled egg, topped with cinnamon & raspberry applesauce

Lunch: Turkey sammich on wheat/brown rice bread with romaine lettuce & a handful of potato chips+ strawberries

Supper: Chicken (breast) curry with swiss chard, collard greens, spring onions (green parts only), & jalapeno over jasmine cilantro rice + cherries (I ❤ summer!)

Sources:

http://digestive.niddk.nih.gov/ddiseases/pubs/wilson/#what

http://www.wilsonsdisease.org/wilson-disease/kayserfleischerrings.php

http://ghr.nlm.nih.gov/condition/wilson-disease

http://www.wilsonsdisease.org/wilson-disease/wilsondisease-diet.php

http://www.mpsutility.com/index.php/mps-water-operations/drinking-water-reports

http://pur-waterfilters.blogspot.com/

http://www.jacn.org/content/12/5/527.abstract

http://www.ajcn.org/content/74/6/803.full

http://gicare.com/diets/copper-restriction/

https://www.inspire.com/groups/wilson-disease-association/discussion/veegtarian-with-wilsons/

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