ITP for 7 years

New to this site and wondering if any parent has been dealing with
this for a long period of time.......Josh was diagnosed at age 4
and is still coping at age 11. Had a spleenectomy 2 years ago that worked
for about a year ,but now we are back to IVIG every 3 weeks again.
Platelet count was 5,000 last week. I am worried about the long term
effects of the IVIG and doctor is talking about bone marrow transplant
due to his lack of immune system now. Any thoughts????

Welcome to the new site!

Yes - unfortunately, we have also been dealing with ITP for a long time. It doesn't seem like there are that many kids who have had this long-term, but maybe people just don't post much. My son, Brady, is 13 and was diagnosed with ITP when he was 5, so we're in the 8-year club. In the beginning, we treated fairly regularly, first with Prednisone, then WinRho, then tried Rituxan, then IVIG. About 3 years ago, we switched back to WinRho and during that time, Brady has only needed about 4 treatments. He received WinRho in Dec.'08 and didn't need treatment again until March '10. We don't know if puberty is helping or what, but his ITP has definitely changed over the years and become easier to manage. Hopefully, Josh will catch a break.

I also worry about the cumulative effect of all these drugs on their little bodies, but Brady has bleeding issues when he gets below 15k or so and we have to treat. We've thought about splenectomy many times over the years but have never taken the plunge. I"m sorry that Josh's didn't work out. It's too bad that he drops so low, because it may be beneficial to give him a longer break between treatments and see if his body can adjust. But we would have to treat Brady if he dropped down to 5k also.

I think there's just one adult on here who had a stem cell transplant because of many issues. Several kids are now trying NPlate with success- has your doctor mentioned that? Or what about Rituxan? It seems like there are several other options left for Josh to try. Maybe you could ask your doctor to spend some time discussiong other options at Josh's next visit.

Hang in there. After all these years it's easy to get worn down from the whole process and the worry, isn't it? It's always nice to know that there are others in similar circumstances. There's still hope that Josh could go into remission!

Beth - mom to Brady (age 12, diagnosed 1/18/02)

Wow, a bone marrow transplant is pretty drastic! How is Josh feeling? I'm sorry to hear that the splenectomy didn't work for him. We had someone on the old site that was in the same boat as Josh (also a boy who was diagnosed around the same time) that had a failed splenectomy and alot of bleeding issues. I wish she was on here to tell you about her son's story. I know the last time she posted on the old site, her son was having some stable counts with Nplate, but they weren't high. It just kept him from having the bleeding symptoms and he was able to do more things.

My daughter is in her 4th year of having ITP. We suspect it's been about 6 or 7 years though and we didn't know about it. She is 12 now and we are heading down the splenectomy route now. I sometimes worry that she could end up being a failed splenectomy case because many other treatments don't work for her. (See below on the treatments she has tried.) She is currently on Nplate and it does work for her, but we're having a hard time with keeping it stable, but then again...she just recently started back on it so it will take some time for the drs to adjust her dose to where it needs to be to get her at a stable dose. The nice thing is that she is able to do her physical activities that she wants to (for the most part) and it really has almost NO side effects. It is also quick and easy, just one shot per week. The downside is that you have to go to the dr weekly to get the injection, but it is not an all day event like IVIG is. Rituxan/Rituximab also works for many kids and that is similiar to IVIG where it is an infusion, but they get it once a week for 4 weeks. It didn't work for Caitlin, but it works for many other kids. Stay in touch and let us know how things progress with Josh. I truly hope he doesn't have to endure a transplant! (Kim is the only adult with ITP that I know of that has gotten the transplant...hopefully she will see your post, but if not...try the adult side and post with "bone marrow transplant" in the subject line.)

Poochie (sorry, don't know your name:)

My son, Timothy, has had ITP for six years now (since age 11). He was very treatable at first, and had good solid remissions, but in the last three years has become more and more refractive. Now he is refractive to IVIg, WinRho (and allergic), Steroids, Rituxan, and Promacta. He has a history of a count of about 1k sometimes lower (yes...zero) and held that from about Sept. 09 to February 10. Finally they started him on NPlate, and he has gotten into double digits, safe counts (you can read his recent counts in my signature), and even a few time normal counts!

Has your hemonc not considered Nplate? Though I guess if there is disfunction of the bone marrow itself, that would be a reason to transplant, right? Is it that his bone marrow is not producing platelets or that there is a problem with it?

Ask them about NPlate...if that is not the case. If its simply a platelet issue, it might be a better option before transplant.

Hi,

My son Zach was diagnosed when he was 2 years old. He is now 17 and he still has ITP. He only received IVIG treatments during the first 10 months of diagnosis, receiving 8 in all. He slowly stabilized to a safe number and went into remission for a couple years when he was about 7. However, when he was about 10 he once again had low platelets. We opted for prednisone for a few months, and then his counts went back into the 70 - 90k range, which is where they continue to stay. He does have the odd dip into the 30's but we only treat now if he needs, such as having his wisdom teeth out. It does get easier, although we are very lucky with Zach's "high" counts. Hang in there, and hopefully his counts will increase.

Take care,
Shelly

I posted last nite about my son Josh living with ITP for the last seven years,
and have been so grateful for all your replies!! I guess I should have mentioned
that before his spleenectomy , we treated the first year with WinRho ,and when
that started to lose its effect we switched to steroids and then Rituximab which
he had a poor response to. The only effective treatment is IVIG , but that
does not last very long. He has virtually no immune system and was told recently
that the platelets he does produce are defective. Anyone ever heard that?
His nickname at the hospital is "The King of Crash", because he will be at
40k one day and within 12 hrs his platelets will crash to 5k. I always dream
of the day when a doctor invents a little pocket tester like the diabetics
have and we can just prick his finger to check his count instead of going to the
docters so often! Wouldnt that be wonderful!!!
His spleenectomy almost a year and it was great, but when we went back for
a three month checkup his doctor had moved!!! And taken all his records with
him!! We have since had a new doctor trying to piece everything together. He
is the one who has a issue with his immune system. The kid is always sick it
seems. He goes to a small private school to help with the germ issues, but
that only helps a little. He is a big time nose bleeder, especially when
he's sleeping. Weird. Its a fine line as you all know , between letting them
be kids and wrapping them in a bubble! Thanks for all your replies.......wish
I had joined pdsa years ago!

The only thing we have not tried for Josh is Nplate
I keep reading about. Any words of wisdom on this
drug?

Hi again,
Sounds very much like my Tim, minus the splenectomy. I've always been against it because his grandmother has chronic refractive ITP and is post splenectomy. Her splenectomy failed, and with the risks involved with it (immune system issues not to mention bleed risk during and after surgery because of the low platelets) I would not consent to it. As I had said before, he was very low. He could be at a normal count and then the next day crash to near zero...no kidding. His hemonc was very conservative with him at first, but his pediatrician did not mess around because he saw that Timothy was as your son, the king of crash.

As far as Nplate, I would ask about it. It doesn't have all the side effects like the other drugs, and so far has worked for Tim whereas nothing else does. Last week he was at 172k! This is not his normal-he's averaging mid double digits...say 50-60k, but even that is a safe count compared to his pre Nplate count of 0-1k!

I would personally ask about it before a bone marrow if the bone marrow is only due to platelets. Transplant is pretty risky if you don't have to do it, I would opt for the Nplate first...at least ask.

My advice is...if he has something wrong with his platelets, don't do it. It could only cause more problems for him. Nplate is an injection classified as a "TPO drug" and this is a protein made in a lab that forces the bone marrow to produce more platelets. If his platelets are "defective", then it is only going to make more defective platelets and I'm not sure what good that would do for him. Plus, Nplate can cause bone marrow reticulin (my daughter has had this, but took a break and restarted...could very well happen again so they watch her closely). If the bone marrow reticulin "grows in abundance", then the bone marrow forms fibrosis, or collagen and the bone marrow will stop producing all cells (wbc, rbc, and platelets). Noone has had this because they watch it closely. You can do a search online...I think the website is something like: www.nplatenexus.com or something like that.

Most people with low platelets do not have problems with their immune system unless there is something else going on. Has Josh been on a whole lot of steroids and other immunosuppressants to cause this? Or is he immunosuppressed just because of not having a spleen? I'm sorry to hear that he is sick all the time...I don't think that is normal so maybe the drs really know more of what is going on than what they are telling you. Our hematologist refuses to do long-term steroids because it isn't good for children so she does "short pulses" of high dose steroids. It works a little bit for Caitlin where it brings her in the safe range, but the last time she was on 120 mg/day for 5 days, her count still stayed below 20. Good luck and I hope Josh at least starts feeling better soon, poor guy!

Alissa will reach the seven year mark in October of this year. For the last year she has been getting NPlate. Her counts have been everywhere, up to 350 down to 11 last week. For the majority of the time she has remained over 50,000. This is the first time she has dropped to 11 in a year. We watch her labs closly. The Nplate has really given her some peace of mind.
Amgen has a list of md's that are experienced with giving NPlate. I would contact them for names if Nplate is something you are considering.

Debbie
Alissa's Mom, (dx 10/03)

Has he been tested for WAS or CVID?

www.immunedisease.com/patients-and-families/about-pi/types-of-pi/wiskott-aldrich-syndrome.html

www.immunedisease.com/patients-and-families/about-pi/types-of-pi/common-variable-immunodeficiency-cvid.html

Has he had any tests that show he has an immune deficiency or is that just a speculation?

Hi there,
You need to have a Immunology work up done by a Immunologist.. Ivig has a drop off in 2 to 3 wks.My son infuses Subcutaniously twice a wk.. it keeps his counts steady.(IgG levels) They will not do a bonemarrow transplant for a immune defieciency unless it is SCIDs which is the "boy in the bubble disease" If they did ,every Immune deficent person would be lining up.
Look up Immune Deficiency foundation.IDF.it's actually one of the write ups sandi found but the actaul site will tell you alot.
Just want you to know my son suffered for 4 yrs since his Hemo had no clue as to what she was looking at and kept telling me that the ITP had nothing to do with everything else that was going on with my sons body.Boy was she wrong.
good luck , and hope you get some answers
Patty