Imuran questions

Thought I'd post here since I'm not sure many are dealing with this in the kids section. My 15 year old daughter has had ITP since April 2009. This May/June I had her tested with an immunologist/rheumatologist and he found high SSA and SSB antibodies and a few other issues to suggest Jordan has Sjogrens syndrome. He spoke with Jordan's hematologist because they are at a different facility, and they agreed to try Imuran before Rituximab. (We were going to do Rituximab in June when school was out.) She has now been on 100mg of Imuran/day for almost 8 weeks. Her liver enzymes have been tested twice and though the first time a few were slightly high, the second time they were in the normal range. Her platelets were at 45 and then 48 two weeks later. But the doctor called me two days ago and said a metabolite test proved she is having a poor response to it and another metabolite test showed he cannot raise the Imuran either due to toxicity problems. He wants to change to Planquinel and Dapsone. Her SSA antibodies and SSB and antiplatelet antibodies are all high. I told him she tried Dapsone last summer for 4 months and it did nothing for her. In fact, now I'm sure she got a fever from it when she first started it too. Isn't it too soon to quit Imuran?
She has been doing so well this last month. No other treatment and even made the school tennis team. Now, I don't even want to change things at the moment. I have a second opinion with another rheumatologist at UCSF where her hematologist is but this isn't until the end of this month. Am feeling kind of stumped now on what to do. Any comments would be greatly appreciated.
Karen
mom to Jordan

I'm sorry, I don't really have any advice on that point. I have tried Imuran, didn't do anything for me. I don't recall a lot of people posting here over they years talking about success with Imuran.

Best of luck to your daughter.

Hi Karen. I also got a fever from Dapsone. I thought it was a coincidence the first time it happened, so I stopped the drug and waited a week before trying it again. The same thing happened.

I don't know if it's too soon to stop Imuran, but I don't think it would hurt to try Plaquenil.

Hi Karen, I was on Imuran for around 3yrs starting at 9k 150mg tapering down to 50mg and kept great counts on 50mg (275k). Ive now been off it for around 3months and even though my count is starting to drop its ok.
I found i had joint pain-quite severe in the right hip even thought they never found anything wrong with it. I was hoping the pain would go since stopping the Imuran but its gone to the other also so i take it it wasn't the meds.
It takes at least 6weeks to kick in so 8weeks is stopping it a bit short i think. If she is happy with it and is feeling a benefit why stop it.
Julia

Thank you Gort, Sandi, and Julia for your replies. I think things were going so well for a while, that this phone call threw me for a loop. I probably will keep her on it at least until her next visit with the current Rhuematologist in almost two weeks. Meanwhile, they are trying to get her in sooner for the 2nd opinion.

I have a few more questions that others might know. Do all chronic ITP patients have antiplatelet antibodies? I didn't recall Jordan being tested in the beginning of all this and I would think most ITP patients would have them, but I thought I also recalled reading here at least in the kids section that some were tested but didn't have any. I am kind of mad at myself that I didn't insist earlier that Jordan see a Rhuematologist because I have always been concerned with her low WBC's. Hematologist kept saying not necessary. I looked back to a CBC she had in 2007 when she had swollen glands and her WBC was below normal then. Doctor thought she just had a virus and we just went about our business after that and didn't worry. But after this first year of ITP and constant CBC's, she is always around 4.0 or slightly below.
Also, a question for Julia, did you have those metabolite tests I am speaking of? Rheumatologist said that first metabolite test indicated she was having a poor response to Imuran I asked why he thought her platelets had momentarily stabilized then, and he wasn't sure. This is all so confusing. But I also didn't mention here that other treatment options mentioned to us other than the Plaquinel/dapsone combination, were Rituximab or Cellcept. The one thing I do know now however, is that splenectomy is not an option.

Karen
mom to Jordan

Karen:

Not all ITP patients have the antibodies. From what I've read (and could understand), there are two types of anti-platelet antibodies. There are also antibodies against megakaryocytes which is fairly new research, so it's possible that those who tested negative to anti-platelet antibodies have those antibodies or other antibodies that have not yet been discovered. I was tested for anti-platelet antibodies early in my diagnosis and was told that the presence of antibodies was high. Having that information didn't make any difference in treatment choices or responses.

Don't beat yourself up about the Rheumatologist. Slightly low white blood cells are usually not a cause for concern. Unless other symptoms present themselves, there isn't much you could have done anyway. It IS all very confusing.

The one good thing is that most of the treatments can treat most of the autoimmune disorders, so you just have to find the one that works the best for her particular situation. Does she have symptoms of Sjogrens or just the antibodies?

Hi Karen, I don't remember having Metabolite tests, i might have without knowing. Ive had full blood counts, liver blood tests as norm but had lots of other tests at first. My counts did raise slowly i.e. count goes from 9k to 14k, 17k so dont expect big jumps at once. Take it easy im sure everything will turn out fine in the end.
Julia

Yes, this is ALL so confusing, the whole antibody thing. Thanks Sandi about answering the antiplatelet antibody question. Jordan doesn't have dry eyes or dry mouth, (or I didn't think so), which are some of the classic signs of Sjogrens but she did have a bump under her tongue that the rheumatologist told me was classic Sjogrens. He told me it was a blocked salivary gland. Since then, three doctors have told her to drink more water, among other things they told us, and the bump eventually went away. Also, the rheumatologist says he believes the low platelets are related to the high antibodies she tested positive for. That was before he tested for antiplatelet antibodies I think. Also, my aunt has Sjogrens along with RA, so we weren't totally suprised by the diagnosis. I am glad though like you said, many of the treatments for one autoimmune can be used for others, I just hoped we would be able to find one quickly, I guess I'm not a patient person. With Jordan being on continuous birth control pills too, I worry about blood clot issues. I'd really like to let her have a period too, just seems more natural. I need to update the Ob-gyn on the Imuran issues.
Julia, thanks for your reply. I am hoping Jordan will show some positive results at her next blood test. Otherwise, I think the Rhuematologist will want to move on to something else. My husband and I don't think the Plaquinel would be a bad idea though, we'd like to see Imuran through a little longer. Thank you both also for the reassuring words, I hope you both are doing okay too!

Karen
mom to Jordan