Bedlington-L

The Bedlington Terrier Discussion Group

Wilson's Disease

Subject: Wilson's Disease vs. Copper Toxicosis
Sender: chelseabluebedlingtons@juno.com (Linda M Freeman)
To: Bedlington-L@LME2.LME.Mankato.MSUS.EDU

Wilson's Disease and Copper Toxicosis in Bedlington Terriers are very similar. That is why CT was one of the first genetic diseases to be part of a DNA study. There had already been so much research done with the human disease, that researchers were hoping the DNA info would easily transfer. Unfortunately, nothing is ever that easy. However, the Bedlington Terrier came out a winner, as we do have a DNA test, and we do have a lot of research to study. (By the way, Wilson's Disease does not parallel CT as closely in other breeds of dogs.)

I personally began my quest to understand the disease in 1985. Here is an article that I wrote for the Fall 1990 issue of the BTCA Wellness Bulletin*. Some of you may find it interesting.

COPPER TOXICOSIS VERSUS WILSON'S DISEASE,
A LAYMAN'S COMPARISON

In June of 1989, while watching the Phil Donahue Show, I was surprised to see Dr. Herbert Scheinberg, President of the National Center for the Study of Wilson's Disease as a guest. Fascinated, I listened as Dr. Scheinberg describe the horrible effects of Wilson's Disease on young people. Dr. Scheinberg went on to say, "There are at least two of you in the audience here who can give it to your children because it's that common as a single gene."

Donahue went on to interview a young man, Brad Lipshaw, who was suffering from Wilson's Disease, and whose brother had died from it. His story was sad, yet amazing.

Some facts about Wilson's Disease are:

Wilson's Disease in humans is "the inability to maintain a near zero balance of copper."
The genetic disorder is found worldwide, about 1 in 30,000 are affected.
Normal (and affected) babies have a high copper concentration, but by one year babies will see a drop in copper levels to the normal adult range.
Symptoms of Wilson's Disease can be found anywhere between the ages of 6 to 30 years old. Only about half of the affected people develop symptomsduring adolescence.
About half of the Wilson's Disease patients develop liver problems, such as:
1. Acute hepatitis (Inflammation of the liver). This comes on suddenly and can be lethal. Symptoms include jaundice, malaise, ascites (abdominal fluids), elevated liver enzymes, and low albumin (a protein).
2. Cirrhosis (Abnormally formed and connected liver tissue).
3. Liver Enlargement (with no clinical symptoms).
Most commonly, a patient develops cirrhosis of the liver with no hepatitis, and these patients are usually diagnosed through neurological or psychiatric symptoms. Sadly, these symptoms can produce serious irreversible damage such as:
1. Kayser-Fleischer Rings (green or golden deposits in the cornea). These rings always indicate some level of brain damage by acute copper build-up. They are sometimes accompanied by sunflower cataracts and can be seen with a slit lamp.
2. Neurological Damage (Movement disorders). Symptoms are tremors, twitching, shaking, becoming rigid or spastic, drooling, difficulty swallowing, and disfigured or painful joints.
3. Psychiatric disorders: Symptoms include schizophrenia, manic depressive psychoses, neuroses, or bizarre behavior.
Other problems caused by Wilson's Disease are abnormal suppression or nonoccurence of menstruation, and miscarriages caused by excess copper in the uterus.
Death can occur from the effects of copper toxicosis in the central nervous system with little or no evidence of liver dysfunction.

References and permission granted for reproduction:

Donahue Transcripts, "Kid's Health" Journal Graphics, Inc. c. 1989 Multimedia Entertainment, Inc. pages 11 - 13.
Harrison's Principles of Internal Medicine, 11th edition, McGraw-Hill Book Co., c. 1987, pages 1636 - 1638
Personal Interview between author and Dr. Scheinberg.

It is interesting to compare these human symptoms to our canine companions. Some are very similar, some are not.

Hope this helps some of you,

Linda Freeman
chelseabluebedlingtons@juno.com
Vienna, VA

*This letter and information used with permission of Linda Freeman. 6/6/97

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Bedlington-L

The Bedlington Terrier Discussion Group

Wilson's Disease

COPPER TOXICOSIS VERSUS WILSON'S DISEASE,A LAYMAN'S COMPARISON

COPPER TOXICOSIS VERSUS WILSON'S DISEASE,
A LAYMAN'S COMPARISON