Angela, re: Josh and tx

Angela,
I'm wondering about Josh's treatment. Why don't they try something like NPlate for him? It doesn't touch the immune system at all...it increases production of platelets. My chd son has Multiple Sclerosis as well. I know all about the fear of messing with the heart. Josiah's chd is rare and difficult to treat, and he's had five heart surgeries so far. He has been stable and off heart meds for five years when the MS hit. They treat MS with several 'disease altering meds' that are supposedly heart safe, but after one year on one of those meds Josiah's heart was found to be functioning at only 25% and he's on digoxin and enalapril now for four years (and has new tachy and arrhytmias)! His heart defect mainly affects his right heart, but this muscle damage was to the left ventrical! I know it was the injections he was on (REBIF), but everyone says "no, it doesn't cause heart problems." I reported to the drug company and they are required to investigate but NEVER DID! My point to them is, "how many studies have been done on kids with a congenital heart defect taking this drug? They say none...I say, that's what I thought. The PC says that I'm right, the drug may have been the cause of the damage...no way to know. So, Josiah is not taking any MS drugs. I'd rather him have a bit of disability and alive than go on that drug and destroy his already compromised heart! I feel your pain!

Dawn god Im sorry I only just found this message!!! And I only found it by accident!!!! First let me say that they havn't suggested the NPlate because they told me originally after his BMB that it was a destruction problem not a production problem, well after reading the study on here I will be sure to let them know that I KNOW that there is no way to tell for sure!!! They hate my "Googling" stuff or being a part of a board...My theory as to why they jumped to the pred after the IVIG didn't work was because they treat rejection with pred, and my son had rejection a few times so they KNEW that he could handle the pred, it also at the same time prevents rejection BUT we have to be seriously careful when doing the wean, if his body gets too used to the pred, he could reject when off it. He is having most of the side effects of the pred though. This pneumonia is something that has run rampant through our house since October. Once he is OFF the pred, I would suggest the NPlate. He is still on the cellcept as well and I think will continue to be, he was on that as well all his life until two years ago with no side effects, another reason they probably jumped on that one!
Your poor son, and poor you. Its so hard trying to juggle so many different ailments and trying to find someone who understands the mental stress is even harder. Lucky my best friend has a child who was a diaphramatic hernia baby and was in the hospital with my son for 5 months as well and thats how we met, he has a world of issues too. Im afraid out hem team was fueling my panic a bit too, after reading the stuff on this board Im much more comfortable with the ITP. Suggesting NPlate to my hem team will result in harsh words Im sure, especially if its my suggestion not THEIRS! Such is life though they are all I have to deal with. Anyways, let me know how Josiah is holding up okay??? How old is he? All the best and if you need to vent ill give you my email addy! LOL

Angela:

Sorry to butt in here but I wanted to give you some confidence (if possible).

You are 100% right that the bone marrow biopsy cannot determine if production is hampered. I have been trying to find written information on that but cannot find one thing. All I have is Dr. Drew Provan's word that it is true. I specifically asked him on a UK message board and he answered.

I asked him after I came to that conclusion myself. I've been here since 1998 and have never seen one person say that their bone marrow biopsy proved that production was affected. EVERYONE says that they are told it's only a destruction problem. When articles began to come out about so many people having production problems, I began to question it - so where are all the ones who do have inhibited production? Also, why would so many people be treated with TPO's and respond when NO ONE has a production problem? None of it makes sense and I wonder why doctors don't question it more. It's making me want to research again. I think I will.

Also - I cannot believe that your doctors give you a hard time about researching. I can understand their hesitancy about a discussion board...it's not the most credible reference. But if you do present credible information, they should at least appreciate your efforts and possibly consider it or look into it. My doctors have usually been receptive to my research and have at least acknowledged it as legitimate after they looked it up. They have admitted to me on numerous occasions that it's impossible to know everything and have been open to my suggestions. I can't imagine how frustrating it would be to be shut down all the time.

I'll let you know if I come up with anything. This is becoming a quest.

This article is wishy washy, but indirectly states the point:

www.hematology.org/Training/Trainees/2768.aspx

Strangely my password was mysteriously changed. I had to reset it to get in here.
Tim's hemo thought at first Tim's problem was production, but he's never had a BMA. He took promacta for a short time and did not respond, so the hemonc thought it wasn't a production problem. But he started him on NPlate and he responded brilliantly to it. At first requiring the 5mcg per kilo, but now he's been on 1mc per kilo for a few months and he's hovering between 150-200k every time.

When it comes down to it, the hemonc resisted NPlate for a long time, but when he found NOTHING else worked he gave us the options of chemo (a cocktail of drugs to surpress his immune system) and Nplate, we took NPlate. So fare we are doing well, and I'm hoping he will soon find a remission or at least a partial one.

Josiah is well. He recently had an MS exacerbation (optic neuritis), but high dose steroids (1000 mg a day for five days and then a three week taper) brought him under control. He is said to have very mild MS...good news for him as MS in kids is usually quite the opposite. He's had it since he was 14, now he is 19.5. One thing I've learned is the balance between shutting up (so you don't get kicked out of the hospital) and speaking up. There's a time for both, just have to know when and how far to go as your kids advocate.

Luci:

I would think that, in theory, N-Plate would work whether a person had a production problem or not. It supposedly ramps up thrombopoietin which in turn increases platelet production. The problem with that theory though is that it doesn't work for some people at all. Go figure. Too many unknown variables with ITP. I'm glad it works for Tim.