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newly diagnosed child

Hi my daughter was diagnosed in may this year after collasping at home at the time they were unsure of what was wrong with her so she was sedated and took into intensive care. It was there where we were told her platelet levels were 10000 and she needed a complete blood exchange. This was done but her platelet levels still continued to rise and after bone marrow we had the diagnosis of cml. she was started on imatinib and after stopping and starting them on different doses is still battling to control levels.They are either to high or to low, so does this mean the imatinib is not working as it should? she is curently on 400 one day and 300 the next and her levels are just starting to go low again. I also wanted to ask about Bone marrow aspirates, she has had her first one today and has to have them every three months, is this normal? and what should i expect from the results. how do you know what stage of cml you have. as she is newly diagnosed her consutant only ever asks her how she is feeling, and never explains anything. i feel completely lost at the moment as all the support seems to be for adults with cml and not children. does anyone has any advice for me on cml i should also tell you that my daughter has cystic fibrosis which complicates matters such as bma as she has to have ivs for 5 days with each one. i would appriciate any information anyone has to offer as at the moment i don't understand what cml is and what the future holds for us.

Thanks

Clair

 

Hi Clair

There is some good information on CML in the FAQ section of this site, explaining tests, blood counts etc, which is worth looking at as it may help explain what cml is and what normally to expect.  How old is your daughter and where is she being treated? 

I find it very strange that your consultant doesn't explain everything to you at least - mine was at pains to do so right from the start and while I didn't take it all in to begin with I did get it fairly quickly. You should feel free to ask them to explain what's going on, how it's treated, what stage your daughter was diagnosed in, why she needs 3 month bone marrows, what to expect and what impact the CF may have on the cml.

Trying to answer your specific questions as best I can:

If the levels are dropping, then I think the imatinib is doing its job. It's quite common at the start for levels to drop below normal. This is probably because the imatinib is killing off the bad cells but your daughter's bone marrow is not yet making enough normal cells. Usually over time this balance settles to a normal level (and if it doesn't and isn't fixed by varying the dose they may change medication - that happens to some too). It may be complicated in your daughter's case by the fact that being a child, the normal dose of 400mg has a greater effect on her than it does on an adult.  And maybe the CF has an effect. I would ask the consultant about that.

Three month BMAs is a bit more frequent than normal (although it used to be the norm).  More typical are BMAs at diagnosis, 6 months and 12 months, and annually thereafter unless either there is some cause to do one more frequently or a good reason not to do one at all (like complete molecular remission). So, I would certainly ask. It's why I asked where she was being seen - practice varies and my feeling is the less experience centres may do them more often than normal as they are less up to speed with current practice.  But there may be a specific reason so you should ask.

Stage is based on percentage of blast (immature) cells in the bone marrow. The vast majority of people are in chronic stage when diagnosed. Your daughter's platelets were very high. 450 is top end of normal. Mine were quite high at 1500 (most people don't present with such high platelets but again there can be reasons - in my case, I have a rare translocation which means my white cell count wasn't that high but platelets were). I was definitely in chronic stage though - so you should ask.

Hope that helps. It's a bit bumpy at first and you do need some guidance from your consultant, and the CF may be a complication for the cml (I don't know) - but for most patients over time things do settle down after the first few months.

Richard

Clair, my heart goes out to you - you have a terrible load to bear at the moment, but I'm sure the folks on this site can help.

I think the FAQ section on this site will answer a lot of your questions  on what is CML, how imatinib works and how counts might move. CML is normally identified by white cell count rather than platelet count - you need to make sure that you have the right information to understand what is happening. Suggest you go to your next appointment with a list of prepared questions based on the FAQ page information and don't leave until you have them answered - most consultants will be happy to support this.

There are a couple of questions I think the forum needs to know to help us help you. How old is your daughter, and what region do you live in? The age will help people know whose experience is most relevent to you - there are links on the blogs page to Rio Williams and Aaron Bullingham, both of whom were yougsters (but different ages) when diagnosed. The location is important because I think we would all feel you and your daughter need to be seen at one of the real centres of excellence for CML. CML is a pretty rare condition; within CML childhood diagnosis is unusual, and then we have your daughter's combination of conditions. The centres of excellence have the most experience of dealing with this condition and such sombinations, and that is where I think the treatment of your daughter's CML needs to be managed.

Bone marrow testing quarterly after diagnosis is not unusual, but does not continue at that frequency, and stops once a stable position is achieved.

I hope that's a useful start. I was diagnosed as an adult - I'm sure those who have experience with childhood diagnosis will be posting with practical support from their own experience in the coming days.

Alastair

Alastair is absolutely right that CML usually leads to high or very high white blood cell counts. Occasionally though it can present with high platelets. Mine were raised to nearly 1500 but my white cell count was only 14 (top end of normal is 10).  Most people present with a much higher white cell count than that but usually only modestly raised platelets or not raised at all. For stage however, the crucial thing is blast cell count, which is expressed as a percentage. Your consultant will be able to tell you what that was for your daughter.

He's also right about centres of excellence. I was referred to one because of my odd presentation and having been there I am so glad I was. With your daughter's complications, I would strongly want that too.

Very best for you and your daughter. 

Hi everyone thanks for replying to me, Jess my daughter got out of hospital this morning! Jess turned 14 last week and attends the oncology department at Birmingham Childrens Hospital UK. At the time of her diagnosis her white blood count was 14 so not high like you would expect to see. At the moment it is 4.8 hb 10.3 platelets 144 neutrophils 1 7.  i have never been told a percentage or anything else whilst attending appointments so when she attends clinic i will be going with a list of questions to ask her consultant. I think they are unsure of how the cml will affect the cf so they are being cautious. I am a little worried in case the diagnosis is wrong though but guess the BMA results will help us more.

Thanks again

Clair