Penicillamine challenge

I'm about to do the Penicillamine challenge the day after tomorrow but the instructions weren't clear. It says to take the pills 12 hours apart. Do I take them the day before?


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i guess you should take one pill every 12 hours - until the pen test.

but since i never done that challange - and i've started taking it every 8hours and passed quickly to every 6h (4 pills a day)... maybe you should call your doctor.

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I don't think he was too familiar with it so I wanted to double check with somebody who had taken it.

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If your doctor doesn't have a clue - i suggest that him or you to try to find a specialist - you must be active on your heath and learn as much as possibel of Wilson Disease - and in here we all learn everyday from eachothers.

Please note that with WD you must taking some chelating meds for the rest of your life... not drinking alchool... and try to have the most healthy life as possible.

the D-Pen should be taking with water one hour before or after of your meals :)

Anyway what symptomns did you had?

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I had some bad liver tests and some of the symptoms of Wilson's but I'm not nearly as bad off as a full blown Wilson's patient. I did have some severe mood swings a mild heart attack anxiety, insomnia and would often be confused and sick where I couldn't work. I went on the Wilson's disease diet and am doing much better. Right now I'm still spacey and my ears are ringing and have some chronic health issues but I'm not doing too bad. I did a 24 hour urine copper test and the results were almost zero and the other tests were near the Wilson's threshold but not into the ranges. I told the doctor I've been on a low copper diet for a long time and maybe we could try the penicillamine challenge and see what happens. I think with a little chelator now and then I'll be a hundred percent good health. I don't know we'll see.

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hi , there is no such thing as full blown WD , you have it or you dont from case i was 47 when i found out i had WD . I didnt have KF rings or neuro problems. but i did have 3rd stage liver disease. i bet 3 years from death if i wasnt treated. some kids have had 3rd stage or cirrhosis at the age of 5. Now a person like me how why at 47 ? I havent been able to find that answer.Only thing Ive been told is strong liver that held all copper and let very little in to my blood. it wasnt i had a low copper diet , looking back I ate alot of things high in copper and alot of it .
the big question is can ATP7B mutations occur spontaneously. I have read it can , but have never been able to find a case of it. It almost seems that is what could of happened to me .
Take care all

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Thanks Jim,

You said there is no such thing as full blown WD but then later you say there are mutations. I think some people have a mutation where copper excretion is only impaired and others where they don't excrete it at all. How sick you become is going to be influenced by which mutation you have and how much copper you have been eating.

I avoided high copper foods and don't have the more severe form of Wilson's so my health is much better than most people with Wilson's. Actually I've never been formally diagnosed. That's why I'm taking the Pen Challenge.

Does anybody know when to start the Pen challenge. Should I start the pills the same day as the 24hour urinalysis or the day before?

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I have the instructions but they struck me as odd. I get up the morning of the 24 hour analysis pee in the toilet and take two pills. Then collect the rest of the urine in a bottle not forgetting to take two more pills 12 hours later. What struck me as odd that the pills would start releasing copper that fast so I thought maybe the doctor made a mistake and then I couldn't find anything on the internet. I guess I shouldn't have doubted him and that was correct. I've just been through so much and I didn't want to screw this up.

Thanks for everyones responses I'll post again in a week or two if I'm positive or not if anyone's interested.

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I have never heard of the penn challenge. even a person with out WD will get higher copper urain excretion if they take D-penn. ive never read this to be a test for WD. What kind of test have you done ? what kind of doc are you seeing .
What i meant is you have WD or you dont which means 2 inherited abnormal ATP7B genes
. How WD actes in each of us is different. they have now found over 300 mutations.What i said is spontaneous mutations meaning good genes that have gone bad I have been searching for 3 years and can not find one case report spontaneous mutation other then in rats they do testing on .
but back to your d- penn challenge i would not test for wd that way . -diagnosis

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I read more about your post . and then a reply that talks about COPPER TOXICITY SYNDROME
by Lawrence Wilson, MD if you go about 2/3 down the page you find this penn challenge. here is a link

it says this. Urine challenge testing. With this procedure, one first gives a dose of penicillamine and then collects the urine for 24 hours. However, this still will miss much copper that is stored deep within body organs and tissues. Chelating agents primarily remove minerals from the blood and arterial walls. ( this is not true my dywt. of copper was 996 i take syprine to remove the copper deep in my liver . here is the next thing i see that is wrong acording to studies )
(this is so wrong its not funny . here is what it says next) . A liver biopsy for copper can be very accurate. However, it is costly, invasive and in my experience unnecessary. However, it is used rarely to assess Wilson’s disease (a rare inherited copper storage disease).

what is know as the gold standerd for testing for Wilson disease he say is rarely used . sounds like your doc is testing from this site and not looking for WD .
take care Jim

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they generally use the penn challenge for children they suspect of Wilson's because the 24 hour urinalysis is not accurate for them because they often haven't lived long enough to be too copper toxic. Although I am not a child I think I'm not too copper toxic because I'm less susceptable and I'm on a low copper diet.

Any mutation of genes would happen at birth I think so I don't know where you are getting spontaneous mutations from. If you have Wilson's you are not supposed to drink alcohol. Some people are born with two copies of the Wilson's gene and some only have one or something like that. It gets confusing then the article said something about 300 mutations.

All I know for certain is is that my liver is kind of screwed up and every time I eat foods high in copper I get sick. I usually don't get too sick though unless I eat tons of it, just some insomnia and spaciness and the ringing in my ears turns from static to vacuum cleaner sound.

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People who have two copies of the Wilson disease gene have the disease. People who have only one copy of the gene are carriers. They can pass the gene on to their children, but they do not have the disease. Some carriers have low serum ceruloplasmin levels or other symptoms similar to WD patients, but much milder and they do not require treatment. Among WD patients in the US, they have identified over 300 mutations of the WD gene. These mutations may be the reason that some WD patients present with severe liver disease while others present with psychiatric or neurologic symptoms and why some get sick at age five while others don't have symptoms until they are much older, etc.

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York my friend

you and your doctor seem a little lost on it. We cannot avoid the copper all our life - almost everything that we eat has copper - we can avoid the high copper but we get it in other things. And if we cannot get it out of our body - we'll suffer damage that can lead us to death. My older brother died with 18 years with liver failure. I was 17 when i was needing of a liver transplant or i would die - thank god i got better and i didn't needed the transplant.

Your symptomns are much commum and can be WD. we're all a little different - i had leg cramps, nose bleeding, i was clumsy, slightly discoordinated, and very melancholic. Since this is a very complicated disease and if it's not checked properly and not-treated if can make terrible things and kill you... i suggest that you go deeped into this and check it... your doctor is going blind... without few ideas on that...

You should get a specialist or a doctor that have a clue to have 100% sure that you have it or not.
i don't know if i've scare you... but because of doctors who don't know there's a lot of people that are suffering... and they could be just like me - lucky to find a good doctor in time and having a normal life.

try a specialist please :)

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My husband has wd and did'nt find out until 50, both my kids a carriers and my son has low cer, and we put him on a copper free diet and his levels went up. So I believe carriers can get symptoms and not enoughresearch has been done on this. I wantted a pen-D chalange done on him but the dr would not do it. Good luck

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Dear Friends
I have had D- pen challenges three or four times in my life time so far. I volunteered to this test as my doc was doing research.
So the test were not to find out if I had WD or not but to find out how much copper comes out with the med. The test was performed under supervision in day clinic in the hosp.
I use to stop all WD meds 72 prior to test. I use to collect 24 hr urine a day before. take that to the clinic. where my blood was drawn for testing. .After that I use to be given 2caps of either D pen or Trientine HCl which ever the doctor wanted to carry test with. I would also be handed urine bottle to collect urine. After 4 hrs the nurse would draw another set of blood and change the urine bottle again. I was allowed to drink and eat in normal way. again after 4 hrs blood test and the the bottle would be taken away and I would be allowed home.
I would not know if this test should be done in similar way on each and every one . But I presume that it should be as it will show 2 things. Copper excretion before taking WD med and after. Also blood copper and ceruloplamin before taking med and after taking med. It will also tell if the med is working or not.
Any one taking the test should consult a WD specialist if in doubt. Because if the test is not conducted the way it should be then the treatment based on the test will not benefit the patient.

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Thank you for you replies. I should have the results in a couple of weeks. Me and my doctor are lost. I do know that a low copper diet is working for me and that I was on a slow glide path towards an early miserable death if I didn't make changes. There aren't any WD specialists in my area. I called and emailed a couple that were about five hours away but got no response.

Thanks to everyone for their responses. It's going to be really interesting to see what my results are going to be like. I took 24 hour urine test before and there was virtually no copper in it. The range was 2-50 and my result was below that. The strange thing was that I decided to go off my diet before the test and eat chocolate nuts mushrooms soy and other items that were high in copper. How bizarre is that? I had insomnia and was sick for a few days but it was nice to eat again some of those things.

I think they should do something pretty similar with me that they did to Kalapi.

I've seen several posts where people said they were diagnosed in their 40's and 50's. I'm thinking that I might have a similar genes.

I'll keep you all posted on how it turns out

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good luck
your urain copper output will be higher now with the d-penn.i bet you will be at the high side of normal or a little higher. people with WD have reading over 100mcg/24 h with no type of chelator. when taking a chelator before being decoppered the reading is 200-500mcg/24 h
you wrote this in your post and i have never seen this, could you show us where you read this, from what diagnosis summary? i ask because it is important we have correct info on here.
" they generally use the penn challenge for children they suspect of Wilson's because the 24 hour urinalysis is not accurate for them because they often haven't lived long enough to be too copper toxic". uidelines/Diagnosis%20and%20Treatment%20of%20Wilson%20Disease.pdf
have your doc go here to this site this is a 1 1/2 hour read for me and i can understand 95 percent of it. this is for doctors in the USA use as a guideline it is aproved by the goverment.
the above is a link to site on wilson disease

take care

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Hi york

send an email to

asking about a specialist on your area :)

And create a post - seeing if anyone knows a specialist in your area :)

i cannot help you much more on that matter since i'm from the other side of the Atlantic

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I found it in the in the practice gide lines this says take your d-pen when you get up then again 12 hours
penicillamine challenge
test in children found it valuable for the diagnosis of
WD in patients with active liver disease (sensitivity 92%)
but poor for excluding the diagnosis in asymptomatic

Urinary copper excretion with D-penicillamine administration
may be a useful diagnostic adjunctive test. This
test has only been standardized in a pediatric population57
in which 500 mg of D-penicillamine was administered
orally at the beginning and again 12 hours later during the
24-hour urine collection, irrespective of body weight.
Compared to a spectrum of other liver diseases including
autoimmune hepatitis, primary sclerosing cholangitis and
acute liver failure, a clear differentiation was found when
1600 g copper/24 hours (25 mol/24 hours) was
excreted. Recent reevaluation of the penicillamine challenge
test in children found it valuable for the diagnosis of
WD in patients with active liver disease (sensitivity 92%)
but poor for excluding the diagnosis in asymptomatic
siblings (sensitivity only 46%).85 Others have found the
predictive value of the 25 mol/24 hours cut-off to be
100%.86,87 This test has been used in adults, but many
of the reported results of this test in adults used different
dosages and timing for administration of D-penicillamine.
Measurement of the basal 24-hour urinary excretion of
copper forms part of the assessment to screen siblings for
WD, but it has not been validated as the sole test for
5. Basal 24-hour urinary excretion of copper should
be obtained in all patients in whom the diagnosis of
WD is being considered. The amount of copper excreted
in the 24-hour period is typically >100 g (1.6
mol) in symptomatic patients, but finding >40 g
(>0.6 mol or >600 nmol) may indicate WD and
requires further investigation (Class I, Level B).
6. Penicillamine challenge studies may be performed
for the purpose of obtaining further evidence for the
diagnosis of WD in symptomatic children if basal urinary
copper excretion is <100 g/24 hours (1.6
mol/24 hours). Values for the penicillamine challenge
test of >1600g copper/24 hours (>25mol/24 hours)
following the administration of 500 mg of D-penicillamine
at the beginning and again 12 hours later during
the 24-hour urine collection are found in patients with
Wilson disease. The predictive value of this test in adults
is unknown (Class I, Level B).

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ok thanks I finished the challenge and am taking 24 hours worth of urine to the lab. Have the results in a week or so.

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The results came back today for the 24 hour urine copper. It was 175. The range is 0-30. The doctor says it's not a definitive diagnosis. I need to have kayser-fleischer rings to be certain. I was already tested and don't have them. That's where we are. Any ideas on where I should go from here would be appreciated.

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