Wilson’s Disease FAQ

IMPORTANT POINTS ABOUT WILSON’S DISEASE:

Wilson’s Disease (also known as hepatolenticular degeneration) is an autosomal recessive inherited disease of impaired copper metabolism resulting in copper toxicity. It affects approximately 1 in 40,000 people (approx. 6000 people in the USA, 150,000 worldwide), causing movement/speech disorders, psychiatric manifestations and liver failure.

1) WILSON’S DISEASE IS FATAL, USUALLY BY AGE 50, IF UNDIAGNOSED OR UNTREATED. DELAYED TREATMENT CAN RESULT IN SEVERE, PERMANENT DISABILITY (SUCH AS BEING UNABLE TO TALK, WALK OR EAT). CHILDHOOD DEATHS ARE NOT UNCOMMON, USUALLY CAUSED BY SUDDEN LIVER FAILURE.

2) LIFETIME TREATMENT IS NECESSARY FOR ALL WILSON’S DISEASE PATIENTS. NONCOMPLIANCE CAN BE FATAL.

3) BROTHERS & SISTERS OF WILSON’S PATIENTS MUST BE TESTED EVEN IF THEY HAVE NO SYMPTOMS. THERE IS A 25% CHANCE THAT THEY HAVE WILSON’S TOO.

4) CHILDREN OF WILSON’S PATIENTS SHOULD ALSO BE TESTED FOR WILSON’S DISEASE BY AGE 5. THEY HAVE A 1 IN 200 CHANCE OF HAVING WILSON’S.

5) ZINC ACETATE (GALZIN) GIVEN AS A 50MG CAPSULE THREE TIMES A DAY IS AN EFFECTIVE AND SAFE TREATMENT FOR NEW WILSON’S PATIENTS THAT HAVE NO SYMPTOMS AND FOR MAINTENANCE TREATMENT OF ESTABLISHED WILSON’S PATIENTS THAT HAVE ALREADY HAD INITIAL THERAPY.

6) TRIENTINE IS THE SAFEST FDA APPROVED DRUG FOR THE INITIAL TREATMENT OF A WILSON’S PATIENT THAT HAS SYMPTOMS.

7) YOU SHOULD SEEK A NEUROLOGIST OR GASTROENTEROLOGIST FAMILIAR WITH THE BEST CURRENTLY RECOMMENDED MEANS OF ACCURATELY DIAGNOSING AND TREATING WILSON’S DISEASE. THE PRACTITIONERS LISTED BELOW CAN HELP POINT YOU IN THE RIGHT DIRECTION OR COULD ASSIST YOUR DOCTOR IN YOUR CARE.