D-pen intolerance? Trientine in Russia?

Hi, I am from Russia and my son 5 years old is diagnosing with WD. He has high blood transaminases (more than 10 times of normal values), low copper and ceruleoplasmin. We started penicilamine, but we have been forced to interrupt it after 10 days because of fever heat and macular eruption all over the body. The doctor said yesterday that there is no other medicine. Regarding zinc, the doctor says that my son has high blood zinc already.
I’ve read that trientine is used for such disease but there is no this medicine in Russia (trientine is not registered in our country). I have not talk about this with our doctor yet, but I am very worried about my son.
I just would like to know, is there any way to get it? Maybe anyone could give me any advice? And what is the price of trientine?

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I hope this isn't what we in the USA have to look forward to soon!!!

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Hi Eruanne, the penicillamine is safe in low doses and can be combined with zinc if taking away from each other (2 hrs at least). According to this publication, a child weighs 20kg should be treated with 3x25 mg zinc along with 160-200 mg penicillamine:
http://www.ncbi.nlm.nih.gov/pubmed/23599735.

Zinc has an effect to decrease the copper absorption and pulls out the copper via making a negative balance in serum so no reason to refuse. You can order from here, these tablets are enough small in diameter for a 5 yrs old:
http://www.amazon.com/Chelated-Zinc-25mg-Gluconate-Natural/dp/B000U8CMFS/re f=sr_1_1?s=hpc&ie=UTF8&qid=1380377275&sr=1-1&keywords=Chelated+Zinc+25mg

A low copper diet (0,5mg/day for him) is also recommended, you can download the copper lists here:
https://drive.google.com/folderview?id=0B2OU9IDvWYEnWGMxRGlqeHBFMzg&usp=sha ring

A great site about treatment options: http://iawdxxicentury.sitecity.ru/phtml_0503182249.phtml

All the best for you!

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Have u got a liver biopsy,,yet? How about. A couple of 24 hr urines ? Very unusual story about your son? Getting such prompt diagnosis and Rx for Wilson's d. at 5?
There are 20 or 30 diseases of liver most are genetic in childhood ? They are all rarer than Wilson's disease.
There is an absence of 1 out of around 20 enzymes? Some disease are more endogenous to locations and races of humans? A liver transplant may cure ?
What city are you in?
Dr vestal

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When I was first diagnosed, in 1973, penicillamine was the only drug available to treat WD. I had a similar reaction to your son, and was put on prednisone for about six months along with low doses of penicillamine which were gradually increased to full dose. I took penicillamine for ten years after that with no problem. I have been treated with zinc since 1983.

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I too had the same reaction to Penicillimine. They told me to stay out of the sun since it reacts with Pennicillimine. I can't remember what they put me on but will never forget that rash and hospitalization for weeks because I was so sick. I hope you can get the Syprine. Try contacting Wilson's disease association, Mary Grapper, and hope she can help you. Yes, Buddynussa, I fear we are going down that path. Also, is your son taking Vit. B6? Eruanne, best of luck and health to you and your son. Please let us know how he is doing.

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Thank you all for the replies.

It is true that we have not the exact diagnosis yet. 24h urine contains only 50 mg of copper, but the doctor says, that the “the normal values” are established for adults people. So, it doesn’t exclude WD. High levels of ALT/AST is counterindication to liver biopsy. KF rings are also dubious. There is a thin yellow ring on one eye and a half-ring on another eye, and it is difficult to say if it’s a beginning of KF-rings or just a personality trait.
My son has been hospitalized during 2 weeks, and our doctor has said after all examinations that WD is the most probable.
I am sure that the next step of our doctor is to start Penicillime with prednidosol. After the stop of first reaction to penicillime, I’ve give to my son 63 mg of penicillime and some hours after that he had a high temperature and red face. That’s why I would like to escape the third attempt and try Trientine.
However, stories of Caterry and Cheesecake are of great inspiration for me.
I’m thankful to sententer for links, especially about pediatric doses. We have arrived to 375 mg/ day of penicillamine in 1 week. And we were not told to take B6.
I will write to WD also, but it takes time for me to write in English.
And it’s difficult to believe that US people could have such problems with drug!

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I am not optimistic about getting Syprine also called trientene or enuff for life long care in Russia? Unless you know a millionaire? Are you any kin to an Ashkenazi Jew? They are 1 well known people with a lot of hereditary liver enzyme deficiency?
hepatitis A also probable, then Hepatitis B and C?
I'm not very confident in your Dr there?
We will pray for you and your son. Dr vestal

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We are not millionaires and not jews, just medium russian family.
Regarding viral hepatitis A,B,C, even D, and Epstein-Barr virus, cytomegalovirus - that was the first thing to verify, and my son has not it.
I know that trientine is expensive, but I still don’t know the real price and dosage, so I can’t even evaluate if we are able to try with it. Mary Grapper gave me e-mail of Univar (that produces trientine) but I have not the response till now. I hope this Friday we can speak with our doctor.
Thanks for listening.

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The full price of the Univar type Trientin was 785 dollars per bottle last year. I assume monthly dose is around 90 pills for children and there are 100 capsules in one bottle. You should request an individual aid from your health service if possible.

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Sententer, thanks for a lot of useful information. Well, I think the health is the most precious thing so I hope we can find this sum for example for 1 or 2 years…. if this med will be ok for my son. And maybe we can go with the smaller dosage (my son weights 18 kg), maybe he has not WD at all. So, for the moment I would like just to try to buy 1 bottle (if the doctors allow us to try it). But I still have not the response from Univar.

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What does the doctor mean by "high blood zinc already"? I don't think that's a counter-indication for using zinc. Treatment with zinc is not about restoring normal levels, but about inducing a reaction that blocks the body from absorbing copper. And it's usually monitored by checking urine zinc.

Any chance of getting a second opinion?

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I mean, my son has 23 mkmol/l of serum zink while the normal values are 10-16. As for me, it’s very unusual for WD. Now the doctor convinced us to try one more time with low doses of penicilamine.
And yes, I ‘m seeking for a second opinion.

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I am 14 year old help me what life wd

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Hi babdi. Welcome to this site. How are you doing? What kind of symptoms are you experiencing? I remember being 14 with WD. Everybody is different with WD. Many people do quite well as long as they take their medication and stay on a low copper diet. Others have more extreme symptoms and I Hope you are not one of them. Please write and tell us how you are doing. We care about you.

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When I first started taking penicillamine, I had the same problem as your son. That was in 1987. The WD doctor started me on very low doses of penicillamine along with cortisone. He slowly raised my levels of penicillamine and I was then able to take it. Ask your doctor about this. It is called desensitization. (I am a registered nurse) Let me know how you make out. Good luck!!

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Hi Eruanne,

I looked at your posts again. It doesn't sound as though WD is clearly established for your son. Were the KF rings evaluated by an opthalmologist using a slit lamp? And what were the exact numbers for his blood serum and ceruloplasmin levels?

As Drokv says, there are several other kinds of liver disease that can cause high liver transaminases. In fact I've read that very high transaminases are less typical for WD than slightly elevated levels.

Penicillamine is a powerful drug that can have powerful side effects so it should not be taken unless for a good reason. I hope you are getting that second opinion!

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Hi Heron, yes, we don't have the exact diagnosis, although our doctor (gastroenterologist) is quite sure of WD. She told me that she would never give Penicillamine to a child without a reason.
KF rings was closely evaluated by opthalmologist using 2 different devices. And they are non yellow as I wrote before (I made a mistake), but "light brown". And our gastroenterologist also can see them (I really don't know how, I see nothing in his eyes!) His ceruloplasmin levels were 100 mg/l and 200 mg/l in different labs, and serum copper 4 mkmol/l. I've recieved yesterday 24h urine copper = 70 mkg. We go to Moscow for genetic test next week.
BreeRose, I appreciate your story, in fact, we will try this way.

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Hello everybody, I would like to update you on our situation.
Regarding Trientine, theoretically it is possible to buy it from Univar, but we must have the approval of our doctor. And we have not it because of the legal complications related to orphan product usage.
So, we restarted d-peniccilamine 1,5 months ago. The initial dose was 40 mg/day and our doctor elevated it very slowly, and we have arrived to 180 mg/day through 1 month (that corresponds to 10 mg/kg). My son had a little temperature reaction (sometimes till 37.5 C in the evening in the first 2 weeks), and he had red spots on the skin sometimes on 4-5th weeks (on the dose 120-180 mg/day). These spots appeared almost every day in 4-5th weeks and vanished in 2-3 hours.
The copper in 24-houres urine was increased from 70 mkg to 330 mkg. I know that it is not much, but my son is a little boy and he is only on 1/2 of recommended dosage. Liver enzymes are still hight, and the results are “jumping” up and down.
Now I am studying and translating to russian the tables of copper content of various foods given by sententer.
Thank you all for supporting.

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My youngest son was also diagnosed when he was 5 and my oldest when he was 9. My five year old was put on Pennacilamine as well and after a month of horrible side effects I pulled him off it and begged his doctor to try zinc. It has been a year and a half since we started zinc treatment and his liver enzyme numbers are just about normal. Don't get me wrong we have had our troubles with zinc upsetting his stomach but the side effects are much better than the Pennacilimaine. We also follow a pretty strict low copper diet.I remember that same scaredness and worry and it has not gone away but feels managable now! Please ask questions, lots of questions!

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Hi Eruanne, just follow the treatment and check your son's symptoms regularly because there is a worsening period at the first months. Regarding the diet, I suggest to avoid foods what contains soy derivatives because it has an effect to increase copper retention of the cells, especially in liver. I found that potato, corn do the same and I cut out these of my diet. Hope this will helps to alleviate the problems!

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