16 year old daughter diagnosed in August 2012

My daughter who is 16, was diagnosed with ITP last August.
She's been having nosebleeds since she was about 2 years old. I also had a lot of nosebleeds as a child so I just thought it was a hereditary thing. At last year's annual check-up, her pediatrician sent her to an ENT and to a hematologist, just to rule anything out. The ENT cauterized one nostril, which he said had 4 big juicy vessels on the surface, which were causing her nosebleeds. That was last July. Only 1 small 2 minute nosebleed since. August was her appointment with the hematology clinic at our Children's Hospital. Blood tests confirmed very low platelets, and once anything more sinister was ruled out, a diagnosis of ITP was delivered.
The odd thing is, despite her low platelet counts, she is pretty much asymptomatic, other than occasional bruising. No petichiae, no gum bleeding ever, hardly any nosebleeds in the last year, normal periods, no prolonged bleeding, no fatigue, great appetite, great mood, no depression. You get the picture. With the hematologist's consent, we've pretty much taken and wait-and-see approach, that is, until this past Monday when her count was at 8. We decided to treat with ivig after having previously treated with prednisone. The prednisone acted rapidly but the effects were short-lived: about a month and she hated the side-effects: bloating, moon-face, ravenous appetite, acne on her face, chest and back and the growth of hair on her upper lip. Ivig went well yesterday until last night when a bad headache began. She had the worst headache of her life, a sore neck and nausea and all she could take was Extra-Strength Tylenol, which barely took the edge off. There was no pain management plan prior to the ivig despite the fact that bad headaches are a common side-effect. This frustrated me to no end. I asked the doctor and she told me to give Tylenol in the event of a headache. Luckily, after sleeping for most of the day today, she is finally turning the corner. Her head still aches but a lot less intensely. The sore neck is almost gone and her light sensitivity is much better. She even had pizza for dinner and watched some tv.
Of course now, she is vowing never to have ivig again. Any tips on preventing or treating the headaches with something more effective than Tylenol? She drank a lot during the procedure, which lasted about 5 hours total and she drank water and tea today. I don't know at what rate the infusion was given, but the nurse told me that they deliver it slowly, especially the first time.
Her counts with the ivig were improved but they only lasted 2 weeks. Now the hematologist is talking about rituximab (rituxan) if another round of ivig doesn't work. Why would it work when it's just a rescue treatment? After everything I've read about it, I'm really hesitant as is my husband. My daughter is at her wit's end with weekly trips to the hospital to have bloods done and to see the hematologist.
We recently saw our naturopath who suggested 6000 IU of Vitamin D daily. Of course, the hematologist said that it was much too high a dose and to lower it to 800-1000 IU. Have any of you seen any improvement with Vitamin D, which is supposed to be great for auto-immune diseases?
Thank for your bearing with me through my ramblings.
Looking forward to hearing from you.

Hello. Sorry to hear about your daughter.

Vitamin D - has she had her levels tested? Vitamin D should be dosed based on the level. If her Vitamin D isn't low, then yes, 6,000 IU's daily is too much. D can be toxic at high levels if the person does not need it. As far as whether or not it helps autoimmune disorders, that is under debate.

scienceblog.com/20157/vitamin-d-may-exacerbate-autoimmune-disease/

Treatment - Yes, IVIG is usually just a band-aid. Some people can prevent aseptic meningitis by taking certain measures such as staying hydrated, using pre-meds before and during the infusion, and having a slow drip. I'm not sure what her doctor means by "if another round of IVIG doesn't work". It seems like it did work. A sustained response isn't expected with IVIG.

You are getting to the more potent treatments now. The Rituxan decision is a hard one. It does have a good chance at remission, so that would be a good thing to consider. However, she seemed happiest when she wasn't treating and since she is not symptomatic, that is also something to consider.

Good luck!

First of all, I don't know what has happened with this site, but I see my profile photo and name beside comments on various boards that aren't mine. Anyway.

My daughter Emma has been "stable" with a platelet count of about 10 for about a month and a half. She is still pretty much asymptomatic. She had 3 small nosebleeds, 3 days in a row, but they stopped after 10 minutes, which the doctor says was great. The hematologists at the hospital are taking a wait-and-see approach with her, having her come in for a cbc every week. So far so good. One of the doctors has mentioned ivig + iv steroids with pre-meds, 2 days in a row. Another one has casually mentioned rituximab, but says to wait a bit. This morning, she had the h. pylori breath test, which was a piece of cake. We'll have results in 2-3 weeks. I've never wished for a positive result before (other than my 2 pregnancies), but I'm hoping she's positive and that antibiotics will help her platelets. I'm not naive enough to hold out much hope, however. It's a "hope for the best, expect the worst" situation, although the hematologist told us that it does cure the itp in some patients, so who knows.

Anyone else with really low platelets taking a wait-and-see approach? What about rituximab? I have a lot of faith in our team of hematologists at the Montreal Children's Hospital, which is a McGill University teaching hospital. Thank goodness we live in a big city with great, free medical care.

Hi -- you posted a while ago, but I haven't been on the site for a while.

Sounds like you are in good hands with a great medical team and you are calm abut the whole thing. All of this counts for a lot, I think!

Your daughter's symptoms sound very similar to my daughter's path with ITP -- same levels (1-12'ish mostly), lack of symptom, and -- luckily -- a hematologist (in our case at MGH in Boston) who was willing to do wait and see for a long time with us. This a great nonAmerican (!) approach I learned from so many good people on this site. Since my duaghter was non-symptomatic we could afford this luxury for the most part.

At any rate -- we did Anti D (nothing), Ivig 3 times (twice bad reactions, icky) and finally didn't work at all, prednisone (icky but good as a band aid as, for example, over her bat mitzvah weekend when her platelets bottomed out!

We did do 4 rounds of Rituxan after MUCH thought and reflection. We were very nervous about it due to warnings, etc and because of the bad experience we had with Ivig. (You can see our posts on this if you search my name on this site.) I think, as Sandi says, every family has to come to their own decision. It was a process of real discernment for us: everythign was very unclear until the moment it wasn't! We ended up doing the rituxan, heart in throats, and it was a breeze in terms of administration. Mia also seems to be getting results from it, 6 weeks out. But as with all things, ITP there are never guarantees.

I wish you clarity and calm as you work to figure out the best course for you and your daughter.

ps we also do the h-pylori test -- to no avail -- but it was worth the try!

Hi,

My 14 year old son has mostly taken a wait and see approach for the last four years - counts have been mostly under 20, but he is very asymptomatic fortunately. Kids in the UK rarely get treated for ITP, and it has little or no difference to outcomes. I think wait and see is a great (non) treatment and its really been the best thing for my son.

Having said all that, he recently tried rituximab and as Firkins said about Mia, it was a doddle compared to IVIg. The steroids that came with each dose wiped him out a bit, but it was otherwise problem free. I'm not sure it has worked for him (yet) or maybe only marginally, but I think it was worth a try at this point because he doesn't appear to understand the concept of "restricted" activity!

Its worth remembering that most kids seem to accept and live with ITP a lot easier than their parents - maybe we should all take a leaf out of their books?

Good luck with it all

Ali

Thank you both, Mia's mum and Alison for your replies. It makes me feel less alone knowing that you and others are going through similar situations.

Our daughter Emma had 2 days of ivig + steroids last Wednesday and Thursday at the hospital because her platelets have been between 8k and 11k for the last month and a half. She was given ondansetron for nausea and extra-strength tylenol for pain. Wednesday, Thursday and Friday were pretty good days and we thought the worst was over until she developed an excruciating headache accompanied by vomiting on Saturday afternoon. We called the hematologist-on-call and he suggested that we go to the emergency room. They ended up keeping Emma for 6 hours and giving her 2 doses of maxeran for her headache. Luckily, it got rid of it and broke the cycle. I guess we won't be trying ivig again as it is the second time she has it and the second time she gets the really really bad headache. The doctor called it a "super reaction".

The team of hematologists will meet and then we'll meet with them to discuss other options. I'm for the wait-and-see approach, especially since she's mostly asymptomatic and doesn't play sports but I also worry about her getting hit in the abdomen or falling and hitting her head when her platelets are low. Such a confusing illness.

Hi -- I'm so sorry to hear about the ivig response and the headaches. UGH. This is exactly what happened to MIa --right down to the timing, a few days out -- twice after Ivig. Like you, we moved on! We were very happy doing "nothing" -- just watch and wait for a long, long time until she started getting single digits. I was probably still okay with it as her Mom, but her dad wasn't so much, Mia was concerned and the hematologist got a bit more "serious." We did have the same concerns you do about getting bonked or in an accident even though Mia, like Emma, is not athletic.

You are not alone! It's a very confusing disease, as you say, and irritating because there's no "magic bullet."

Wishing you the best -
Firkins
(mia's mom)

You're really inspiring my husband and I to now consider rituximab. I've always been terrified of any drug being given to my children but the more I read, the more I see that people seem to be having some measure of success with it. We'll have to discuss our options with Emma's doctors.

I'm so happy for your daughter's raised platelets. Let her remission be long!

We were terrified of giving her this drug, too, and it is not for everyone. We were at just about the year mark; Dougie's family (also on this chain) waited 3 or 4 years. Some never do it. Whatever you decide, I hope you find clarity and peace! (and of course high platelets)

Personally, I wouldn't even begin to consider rituximab until after a year - just because the chances of spontaneous remission at that point are still pretty good. Rituximab knocks out part of your immune system for up to a year and in my opinion anyway, thats a pretty heavy duty solution to what might be a lightweight condition. .

After Emma's hellish week last week, we anxiously saw the hematologist this morning and her count is at 473k. I know it will come down again eventually, but I'm so happy that we only have to go back to the hospital in 3 weeks, and not every week. What a (temporary) relief.

great news. Hope it lasts a while. You never know, as Ali points out, many kids go into remission spontaneously. Happy for the great platelets. Enjoy!

Well, here we are again......

After a couple of months of low counts (11,000) Emma decided to try ivig again before our annual vacation next week. I reminded her of her saying "never again" after last March's treatment, but she decided to go for it after being promised all the bells and whistles to prevent side-effects.

We spent the day at the hospital on Wednesday where she had hydration, steroids, ivig, maxeran (metoclopramide) and zofran (ondansetron) through iv. Everything went well Wednesday, Thursday and Friday. She didn't feel great but was able to watch tv, eat and bake cookies. This morning, she woke us up telling us that her head was starting to really hurt. We gave her a maxeran and an ondansetron for the nausea and she is now asleep. We have postponed our departure by a day and will leave for our 6 hour drive to Connecticut on Monday morning instead of tomorrow.

This is absolute madness. The treatment is worse than the illness, it seems. We were approached by the hospital to participate in a clinical trial of Promacta in children (she's 16 1/2). After careful research, discussion and consideration, we opted against it so now we are starting to consider rituximab. Decisions, decisions.

Sorry about the venting - I'm just so frustrated.

Hope all of you enjoy the weekend - it's hot and humid here in Montreal but we're keeping cool in the house. Thank goodness for air conditioning.

I see anti-nausea drugs in that list, but no tylenol. Was she premedicated with tylenol, and regularly given tylenol after? We were told that was crucial. We did that, with the benadryl, and plenty of fluids. My daughter had a horrible headache after ivig the first time, which caused her to go back into the hospital for observation in case of head bleed. Fluids, tylenol, and benadryl staved off these effects on the second and third times. She also had a steroid shot just before leaving the hospital on ivig #2. Since this was in advance of travel, could she have tried a short round of steroids? I know they have their unpleasant side, too, but the steroid side effects don't generally put the patient in bed. My teen was very resistant to the idea of using steroids, due to the weight gain issue, but it may be worth trying on occasion, for vacations and medical events, if she can't tolerate ivig. (Sorry, I now see she tried prednisone. How about the decadron pulses? The patient is on low dose decadron for five days then off for the rest of the month. My daughter didn't tolerate the post-treatment drop in count, but some on here do very well on it.) I'm sorry she's going through this. ITP, and all the stuff it drags along with it, is really tough on teens. Hope she feels better soon.
Norma

I don't understand why they didn't give her hydrococone with acetominiphen for her headache. I was given free access to that any time I needed it when I was in the hospital, and when I told my hemotologist I was having some pretty severe backbone a couple weeks ago, and he knew I am headed for lots of walking at Disney, etc, he prescribed 50 hydrocodone for me with a refill. I don't know why they didn't give her that.

My son did 4 rounds of Rituxin after about 2 months ago. I waited about 6months to do it as it made me very nervous. Then I realized it was one of our only chances for him to actually kick this disease. He's had it for 2 1/2 yrs. It was stable on DAPSONE (haven't seen that mentioned anywhere on this forum) which many dr's don't use and the one's that do don't know why it works. Unfortunately my son had no response to the Rituxin but I'd still do it over again as it was the 1st attempt to fix the problem rather than just band-aide the symptoms. We are looking to find a fix, not sure how though. Wondering if I should attempt enzyme therapy the homeopathic dr suggested.

Everyone is looking to find a fix. Let me know if one exists because in my 16 years on this Forum, no one has really found one outside of the known ITP treatments.

Dapsone is not used very often because it doesn't work for most people. I actually did try it at the beginning of my diagnosis, but it made me really sick so I had to stop using it.