few Q's from someone new to this rollercoaster!

Hi All,
Just found this site this morning and am so grateful. Our 12 year old was just diagnosed with ITP about 2 weeks ago. I'm a mom seeking perspective and counsel from others. It's been a really wild, scary 2 weeks -- 5 CBCs, 2 ER visits, 1 overnight in hospital for treatment, and still we feel like what the hell has happened here!?!? A few questions I'd love help with:

- Does it always feel like a roller coaster at first? feels like we are drowning in not knowing what to do!

- When Mia dipped to 6K they treated her -- 60mg prednisone and IV anti-D. Is this standard? My dad had an autoimmune disease (pemphigus vulgaris) and I watched the prednizone nearly kill him -- I'm so afraid of that stuff but I know it can be necc'y sometimes. Should I be as scared about the prednisone as I am?

- her #'s popped up (to 19K, then to 130K) but it feels like they are dropping now -- some blood in nose, grumpy, bruises appearing.Will get CBC first thing in morning..... Will we learn over time to get a sense of this?

- are there dietary changes that can be helpful/supportive?

- plan to fly to CA on Thursday to see grandparents -- is this a dumb idea if we are at the begining of this and things appear to be in flux?

wish there was a class to take or something. Hard to gain a sense of the big picture we are finding from the doctors. We believe our hematologist is good - told so -- at Boston's MGH in pediatric hematalogy -- but how do we really know?

So grateful for any answers. thanks.

Hi Firkins,

i was waiting for someone more knowledgeable than me to answer you, but its a bit quiet at weekends. So, here are my answers

Does it always feel like a roller coaster at first?

Probably. The problem is that you naturally get very caught up in numbers and counts, and then you get excited and then disappointed when they go up and down. One thing thats worth doing is looking at counts in multiples of ten or twenty - 1 is pretty much the same as 8, 11 is pretty much the same as 19, etc etc. My son's haematologist only really uses 3 categories -under 20 (can be dangerous), over 30 (pretty safe), and over 50 (pretty much ok for anything).

60mg prednisone and IV anti-D. Is this standard?

I can't really answer this. In the UK it is unusual for children to be treated on the basis of a count alone, or for that matter, on the basis of bruising and petechiae. Children here tend to only get treated if they have bleeding symptoms or wet purpura. From what I've seen on this site, the best american doctors also follow this approach, because the risk from the treatments can be greater than the risk from ITP. You wouldn't want to be on steroids long term, but I don't think the occasional dose is that bad - it seems to be the standard first line treatment for ITP

Will we learn over time to get a sense of this?

Whether your daughter's count is dropping? Yes, after a while you will learn to recognise the signs and not require a count - for my son, petechiae on his legs means around 13/14 and bruising means under 5. In the days when his count used to go over 30, he used to get a peculiar dirty looking effect on his legs that meant between 30 and 40. He refuses to let me look at his legs more than once a week now!!!!

are there dietary changes that can be helpful/supportive?

The UK ITP Association is funding a scientific study into food intolerance and ITP, so there must be some real anticipation of this. I have read about people going down this road, but not of any dramatic successes. I tried dairy free for my son for a fortnight a few months ago, and it had no effect.

plan to fly to CA on Thursday to see grandparents

I would definitely ask your haematologist about that one, because your daughter's diagnosis is so new, and no-one really knows how stable she is. Some doctors seem to advise against flying, but we've never really found this a problem. I hope you get to go.

drs - how do we really know?

You don't. However, the questions I would be asking myself are - are they prepared to listen to you and your daughter?, do they panic you into treating?, are they prepared to consider not treating as an option?, do they tell you the potential side effects of treatment?, how many other ITP patients do they have? are they on the PDSA list? (since you are in the US). Having a doctor who doesn't panic you into treatment over a low count is probably the most important in my book.

I hope this is a little bit helpful. Good luck with it all. Its very worrying when its all new, but I just wanted to assure you that ITP does not mean the end of life as you know it. Children can have very normal lives with the condition, even if it turns out to be chronic and counts remain low. And children remit all the time - even those who have had it for a number of years.

Ali

Ali,
thank you so much! this is all wildly helpful and reassuring -- esp for today the parts about the doctor (not sure how well she listens!) and the flying (we're so much at the beginning we are a bit naive...)

Want to write more but I'm getting kids off to school. Really, thank you thank you
Firkins

ack. levels this morning at 5,000. still no overview from doctors about this. so frustrated with our doctors. going into mgh clinic now. need list of questions -- does anyone have them?? feeling like we are drowning here and so mad at doctors who give us zero infor and just keep treating her without telling us side effects, pros/cons.
Help! thanks!
Firkins

Does your daughter have bleeding - if so, the doctors should treat.

If not,

a) when would they treat?
b) what would they treat with (the normal ones to start with would be steroids or IVIg) - there are drawbacks to both, so make sure they give you the lowdown.
c) What order would they use treatments?
d) When do they recommend that you contact them? - bleeding, wet purpura, just weekly.....
e) are there any activity restrictions that they would recommend
f) do they have a longer term plan in case things don't improve

There are no easy answers wiht ITP because what works for one person doesn't work for another. But there is a document called the international concensus for the management of ITP (or something like that) which gives a general guide - try googling it.

Don't panic too much - 5 is pretty much the same as 15. Kids manage fine on those counts most of the time. Let us know how things go

Ali

thanks so much, Ali! you are my London life-line! We are at MGH now and waiting for hematologist. Your questions will be helpful. Yes, she is bleeding a bit from nose (a sign we are learning that levels are below 10). And she's had 2 blood draws this morning and they have bled pretty heavily before stopping -- is that "bleeding"? it's not like her nose is gushing blood.... Hard for us to understand all this. Will ask hematologist. I am very, very grateful for your help.

We are now at MGH, levels at 5. Will be treated with 75 mg IV Anti-D and 60 mg of prednisone. Hope is that the treatment will last longer than the 10 days we got from last one. They are "surprised" her levels went from 130 to 5 in 4 days. I finally got a hematologist to sit down and answer all my questions which was a relief.

A couple of hematologists we've seen through the last 2 weeks in ER's etc have said that low platelets counts will NOT make you tired. But i'm pretty sure, and so is my daughter, that when hers drop she feels a deep, bone weary fatigue (plus nosebleeds - the key tip off for her).

Any one else's child also feel this FATIGUE we are told she isn't really feeling but we know she is? THANKS all. So glad I found this blog and thank you Ali esp

Fatigue is a recognised symmptom of autoimmune illnesses generally and specifically of ITP -try this link.

www.ncbi.nlm.nih.gov/pubmed/21323737

Haematologists often don't seem to recognise it, but my son's doctor is one of the foremost ITP specialists in the UK, and he says there is definitely a link.

Not sure why they would try the steroid and Anti-D again when it had such a short lived response first time round. And 130 to 1 in a day is possible for some people with ITP, so I don't know why they would be surprised about that either!

Hope it works better this time round though.

Ali

The international guidelines that Ali talks about are here..

bloodjournal.hematologylibrary.org/content/115/2/168.full

Ali: thanks for checking in on Mia/us today via this site. i am so grateful. Lifeline to someone outside hospital. Your questions really helped and got a new hematologist who listens better and was very patient.

We tried IV AntiD again because it worked well the last time just didn't last long. So upped the dose by 50% to 75mg with the prednisone and hope for it to last longer. Next step would be Iv ig or whatever iti is called.

Daughter is really tired and sound asleep. They said if levels up by Wed she can fly on Thurs to see grandparents as planned. Just taking it day by day.

thanks again

Ali and Ann - is IV anti -d the same as IViG? Just curious.

I agree w all Ali suggested - you want a hematologist that listens to you, and I wish that were easy to find. Took me over a year to get to the right one.

And usually treatment w kids is not as aggressive, because ITP can spontaneously go not remission w them, unlike adults where that is rare. So they should be looking at a host of things ( how she feels, age, etc) rather than just the numbers.

And I think it's really weird that hematologists say that fatigue has nothing to do w ITP. Almost everyone on these boards will tell you the opposite. And sometimes it's more than palin ol tired - sometimes its full on exhaustion - I can sleep for 11 or 12 hours.

Also I assume they've ruled out other things for Mia? Or did they go straight to ITP as a diagnosis? It would be very weird and foolish if they did that, as low platelet count is also a symptom of other more important stuff that they need to rule out first.

Good luck Firkins - this site is sooooooo helpful, and there are lots of parents on here who will be able to help you along your journey.

One more thing Firkins - no one has mentioned this yet, and it's nothing you should panic about. But I wanted to let you know that at 60 mg of prednisone, a 12 year old girl could get pretty darn moody. That's such a hard age anyway, and that's a really high dose of steroids, so just keep a heads up for mood swings or anger issues, and try to cut her a little slack. She may not make the best decisions on 60 mg of pred as well, so just keep an eye out.

At 60 mg I was bouncing off the walls, and had a rage that would hit me at a pin drop!

Hi Kittie and all,
thanks so much for your posts and questions. Again and again, I'm grateful I stumbled on this website and discussion group. I have great friends who are really there for me, but they just don't know about ITP -- so this thread has been a wonderful thing over the past few days. Ditto exploring aroudn the site.

Anway -- they treated her yesterday they said because: 1. she had bleeding & bruising, and 2. at 5. Not a lot of blood but it was in her mouth and nose. They did wait and see in the early going a couple of weeks ago, but her numbers kept dropping. She was treated the first time at 6 (with nose bleeding) and shot up to 130 at one point about a week later, only to drop to 5.


Kittie - grateful for your points -- but the doctor assured me it was not just hte numbers (though 5 was one she would treat) but the bleeding too, and her tiredness. (although she also said fatigue doesn't come iwth low platelets - ! but all know it does).

QUESTION: The doctor said yesterday that because Mia responded so well to the IV antiD the first time (went from 6 to 130, even if it lasted just 10 days) that that was an excellent sign it was indeed ITP. Other blood levels arenormal. they have not done bone marrow test. Also rather extensive history of autoimmune disease in my family -- Does this make sense to people??? Should they do more to rule out other diseases?

We saw the moodiness the first time. It was so nuts that my husband and I (not to make light but..) it was almost like a SNL skit, "Grumpy Girl." It passed after a couple of days. My friends whose kids have kickass asthma tell me their kids get even higher doses. ack! I hate, hate hate prednisone as it wasted my dad who took it for his autoimmune disease.

The hematologist yesterday really listened and answered ALL my questions -- some sent by Ali from UK- which was great. I like her much more and so does Mia and my husband. Not sure we still are with right hospital/doctor but we feel confident for now. When the dust settles, I'll do more homework on that.

thank you alls o much.
best, Firkins

Thats good timing for treatment so that you can visit the grandparents without worrying. Hope you have a good time.

KittieG wrote: Ali and Ann - is IV anti -d the same as IViG?

No, not really. Anti-D is also called Anti-Rho because it has anti-rho immunoglobulins whereas IVIG has more general immunoglobulins. Anti-D particularly targets the red blood cells, marking them, and they go to the spleen for deletion (computer term) which is why it is not done for people who are anaemic and why the urine turns red as the blood cells are excreted and the haemoglobin falls. None of that happens with IVIG.

Also I assume they've ruled out other things for Mia? Or did they go straight to ITP as a diagnosis? It would be very weird and foolish if they did that, as low platelet count is also a symptom of other more important stuff that they need to rule out first.

My ITP was diagnosed by the lab technician looking at the blood film. All new, large platelets and nothing else of note and nothing wrong with any other blood tests, liver function etc, and he knew what it was. Generally that's the way it goes.

Hi Ann and Kittie,

Interested in if the doctors have done enough to ensure Mia does indeed have ITP. Here's what we have been told was part of diagnosis. interested in your thoughts (or others out there!)

- Has had several CBC's over past couple of weeks. Her red and white cell counts are normal - just platelets that are low. Even after the first treatment of AntiD (2 treatments total) her white and red stayed relatively level. Red dropped a tiny bit which we were told was normal.
- Heavy bruising and petichiae (sp?) came on relatively suddenly after a cold & short fever
- She responded "well" to the first round of IVAnti D- her levels went from 6 to over 130 over about a week. The hematologist said this was a strong indicator she does in fact have ITP. (yes? no? thoughts?)
- autoimmune diseases in my family: diabetes I, rheumatoid arthritis, pemphigus, i test postive on ana test but am asymptomatic (for whatever that's worth).

SHOULD THEY BE DOING MORE TO RULE OUT SOMETHING ELSE?

thanks all. this site is the best -- meaning, really, the people on it are.
Firkins

they have also done 2 blood smears to look at the actual cells.

firkins, it all sounds very normal diagnostics to me. If Mia had leukaemia they would know so don't worry about that. And it's true that responding to ITP treatments confirms diagnosis. Reds dropping with anti-d is to be expected as that's what it does.. attacks the reds in the hope to distract the body from attacking the platelets.

thanks, Ann!

I agree - sounds like they're doing the right stuff. Obviously if ey decide they want to do some other tests then go with it, but sounds good and like she is a ITP person.

And glad you've gotten a hematologist you like. That makes Inge a lot easier for sure!

Sounds right to me. My son, Brady, never had a BMA or anything like that. Similarly to your hematologist, ours said that the fact that Brady responded well to IVIG and WinRho and the fact that everything else on his CBC was always completely normal was indicative of it being ITP. Brady didn't have a virus or anything that we were aware of immediately prior to diagnosis but that's also a typical indicator and sounds like Mia had that cold/fever. So - your hematologist is definitely in line with where ours was when Brady was diagnosed.

At the beginning it's hard not to worry about anything and everything else, so we've been there and understand. Hang in there!

Hi Beth, Thanks for this email which is really confirming and relieving. I keep saying this, but glory do I love this site. I'm so glad to have found a group of people with ITP and moms of kids with ITP who are reassuring, grounded, helpful.

Mia just had 2nd treatment in 2 weeks - upped the IV AntiD by 50% to see if she'd have a longer lasting response. However, her bruising is continuing, her petichiae (sp) is not going away, and this morning she had a slightly bloody nose (has been a sign before of low levels for her, eg. <15.) She'll have a CBC this afternoon, and we have our fingers crossed, but I can tell she is discouraged. Learning she has an innate sense of this stuff (which makes sense really). The bummer is she and her dad and sister were supposed to fly tomorrow to see his parents in CA so that might not happen. At least not tomorrow. One day at a time.....

Thanks for your response and to all for your support. I hope to be offering it to others as we live into this new reality and learn more.
Firkins