Can anyone advise me what the next step may be?

Hi, my daughter was diagnosed in October last year with a platelet count of 3 and is currently on 16, 4 months later. She hasn't had any medication as yet, though received a platelet transfusion at Christmas for heavy menstural bleeding, which is only just under control nowthat she is taking an oral contraceptive pill. I'm really worried for her, my positive full on little girl who has always loved and embraced life has been knocked for six by this, and I don't know where we should go from here. Can anyone give me an idea if I should be looking for a second opinion, and if so where to begin. Should we be looking at giving her a dose of steroids or the IVg treatment, which her friend was given recently, who now has a count of 186.

I'm really quite scared for her, I've always remained positive and hoped this would be short term, but now Im not so sure. At what stage will the doctors do further investigative tests, and what may they test for? I understand a bone marrow biopsy is usually done at six months, is this painful?

I'd be really pleased to hear from anyone who can offer me any information at all, we feel so in the dark, I think we really need some direction, some plan, some idea but she sees the consultant every 6 weeks for a few minutes and we just dont seem to be getting anywhere.

9 Replies

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  • Hello Gem, I can fully understand your feelings of worry and concern. ITP is such a random condition and how we all react to it and to the many treatments is very different. In terms of how ITP is treated, unfortunately there is no one single best treatment but instead a range of treatments which the specialist may try depending on the age, life style and previous medical condition of the patient. In general terms with children and teenagers the platelet levels can quite often return to normal without any treatment at all and this may well be the way your specialist is thinking at the moment. The immune system can simply correct itself. In most cases if the platelet count does not correct itself the specialist will normally prescribe steroids (Prednisolone) accompanied by a drug called Omeprazole and also Allendronic Acid. Because steroids can cause quite a lot of heart burn they issue Omeprazole to counteract it and also steroids if used for the long term can cause Osteoporosis so Allendronic Acid is prescribed to help avoid this.

    If the steroid is only prescribed for a short time you may well find that Allendronic Acid is not prescribed at all. The steroid in most cases will lead to platelets rising , usually within about a week or so. When prescribed steroids they are given based on the patients weight and you are prescribed 1mg per 1kilo . When prescribed steroids you will find that you are asked to take them for a period of usually a month or possibly two then assuming the platelets respond well the steroid dosage is gradually reduced bit by bit, for example perhaps by 5mg per week. But remember all cases are different and it just depends on how each individual responds.

    If you would like a second opinion you are absolutely right to do just that. Is the consultant you see an ITP specialist ? If not do ask to be referred to one. The ITP Support Association can suggest a specialist if you email them on Info@ITPsupport.org.uk

    As for your question about the bone marrow biopsy this would not be something that they would do at this stage in my opinion.That is conducted after they have tried to treat the ITP and if they do not see a positive response in the platelet count.

    In terms of the various treatments for ITP do please have a look at the ITP Support Association website a itpsupport.org.uk. The problem with ITP is that because we all respond to the different treatments in different ways it is all a bit trial and error in that it is a matter of finding which of the treatments works best for each individual.

    Hope all this helps but if you feel that you would like to email me directly please feel free to do so on Anthony@itpsupport.org.uk

  • Hi Gem, Sounds like your daughter's hema is taking the watch and wait route. If her platelets have gone up from 3 to 16 on their own thats good, bc is a good thing too for itp. I started with a platelet count of 9k 4&half yrs ago and after lots of blood test recieved 60mg Predisonolone a day, after 5 days my count was 147k but as soon as i started to taper my count crashed then stopped working all together but took 7mths to taper off them, my mood swings were shocking & when i tapered i had atleast 1day of the shakes each taper. I got Lanzaprozol to stop them from giving me an ulcer and put 2 and half stone in weight on. My Hema said oh its ok we can just remove your spleen you dont need it, i refused & later found out if your spleenless your something like 50x more likely to have heart trouble and your liver can take over destroying platelets anyway. Then she wanted a bone marrow biopsy although 99% certain it was just itp from the other blood results being fine, if the hospital was worried they would have done this very quickly. She wanted to do it there in the room with a local anasthetic but i refused as they screw a needle through the bone then draw the marrow, i went for the sedation and local as i would have been just about out of it with sedation. That was much better and could not have done it if not. I then tried Azathioprine tablets started at 150mg day and within 1 wk my count went from 9k to 14k then 21k the following week, when my count went up and up until about 360k i reduced to 100mg then 50mg and just over 1yr ago i came off them and my count has been stable at around 250k & been discharged from hospital and now to get yearly counts checked through my GP. Azathioprine sent me into remission but when your on it you should not get pregnant due to it sometimes causing feotal defects (born with dual sex) but fine when your off the meds. Hope this helps, your daughter will be tired with lower counts as they cause lethargy.

    Julia

  • Hi Anthony and Juliah

    Thank you both for your replies which have been really helpful. I'm sorry Juliah that you have had such a rough time and am very glad to hear that you are now in remission.

    The Hamatologist my daughter is seeing is not an ITP specialist, and I have now emailed the ITP Support Association asking them to recommend one for me, thank you for that info. I still have very mixed feelings on steroids, or perhaps I should say on the dose that she would be perscribed - 50mg, and am very worried about the potential side effects on a teenage girl. Anthony, you suggested that steroids may be perscribed for 1-2 months which is the time the haemotologist had initially perscribed them for her (in an attempt that her platelets would have risen in time for her to ski - though she chose not to take them). Since then I have read on the ITP Association website and also in the Great Ormond Street leaflet given to us by the GP that steroids in children should only be perscribed for 4-7 day for ITP, and this is something i will mention to him at our next appointment in a couple of weeks time, if should be his next course of action. My preference would still be the IVg - have either of you had this? I understand its very expensive, so I guess probably used only when necessary.Thank you again for your replies, I know everyone is different in their response to treatment but I feel you have far more information and experience than the doctors.

    kind regards

    Gemma

  • I don't think you can get an itp specialist here in the uk, the Hematolagists are the specialists due to it being a blood disorder although they still don't know enough, you will probably learn more than most medical proffesionals through learning as you go and reading the pdsa web from real people who are also going through it. I was never offered ivig but think i would have if i hadn't have tried Azathioprine, If i remember rightly It lasts around 1mth per treatment, good idea for ski trip but try beforehand. There is also Rituximbab which is becoming more widley availiable in the uk, before when i asked for it i was told it was a good cancer treatment & it wasn't being used for itp although it is used loads in the us. If your daughter tries pred for 1-2mth at 50mg a day, it will take her 5mg week to come off them, you can't just stop them unless you take them for less than 1wk as it can kill you if you just stop. Most people find steroids awful, i will never ever use them again but it did give me loads of energy, i decorated my staircase the first week of taking them, i was so hyper & couldn;t sleep. PDSA ITP support group is fab but many of the treatments available in the us is not used here due to cost. Good luck

    Julia

  • Gemma., I think that the answer you get may depend on which consultant you get. My son's does bone marrow tests at 6 months, but does not usually treat children. IVIG is horrible stuff. We tried it out nearly three years into ITP, and it gave my son the headache from hell for about 5 days - we would not do it again in a rush.

    If it helps at all, my son had sub 20 counts for the first six months of ITP, and then out of the blue, had a year of counts which varied from about 10-120. He had a very normal life in that period, its just unfortunate that it didn't last! Just because your daughter has had this problem for a number of months does not mean that it won't get better - even people who have the chronic form sometimes get better!

    Good luck

  • Hi Gemma. However hard it is, do keep positive. It will work out in the end either by itself or with medication.

    It has been said by Anthonly, we all respond differently to the drugs, so it is, very much a case of trial and error. I have been on most and ended up with Ivig, at one time spending a day a week on infusion. Count went up for a few days then back to the normal 10. After a year or so of this, I was put on watch and resue unless I needed surgery. That was ten years ago and until recently the count has remained around 10. It has suddenly started to go up and at last checkup was an incredible 38!. Dizzy heights indeed. I now go for blood checks on a yearly basis.

    There ARE Haemotologists that specialise in ITP in this country and the support group can advise on this. The trouble is, medicine is so vast a subject that haemo specialist like all medics cannot know everything and ITP only appeals to a small percentage of consultants. My consultant is the first to admit this is not his pet condition and has referred to the Royal London for advice in the past.

    Ivig can give you a headache. It can be especially bad if you react to it. This is why it is usually administered extremely slowly for the first batch to see if you are tolerant. The good thing is it is taken from blood and is a lot better than some of the manufactured medication but that is a personal opinion. You are correct, it is very expensive. I do not know the cost now, but when I was visiting on a weekly basis I was told the medication costs alone were about £1,000 per visit. God bless the NHS.

    Finally, if you can make it, attend the ITP convention in York later this year where you will have the opportunity to meet and talk to specialist consultants from the UK and the USA and be able to mix with other Mums, Dads and sibblings not to mention the oldies like me. It's a great day out.

    Keep us informed of developments

  • Hi Gem

    When I had IVIg I it gave meningitis which was a whopper of a headache. I came off it straight away and never touched it again. There are other treatments to try.

    It can be a roller coaster and the fear is real. Come to the Convention in York and meet other ITPers. I have not been before but I have been persuaded by sailor to go.

    See you there.

    NickyD

  • Thanks Everyone for the really interesting and helpful info. My thoughts now are for her definately to see an ITP specialist and possibly continue with the watch and wait theory! The side effects from all the medications sound horrible. I've already booked for the ITP convention and we're really hoping it will throw some more light on the condition.

    See you there

    Gemma

  • i was diagonsed with itp in 2002 have over the years been on different treatments due to dramatic drops in platlets lowest being 1 , had a splenectomy 2 weeks ago today , feeling good so far still taking it easy though , just nice not to wake up with a different bruise only time will tell if its worked but so far so good ,keep staying positive .

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