Advice Please

Hello Everyone,

I was diagnosed with ITP about 25 years ago (I'm now 50 years old). I've never had any treatment and have generally had no symptoms - except that I bruise easily. Over the last 25 years my platelet levels have gradually been dropping from 80 downwards. Over the last couple of years they have been hovering at 30 with one drop to 19 and more recently another drop to 21. I have been referred to the haematologist at my local hospital who want to try me out on Dexamethasone for 4 days to see how my platelets will react. Unfortunately I have recently been diagnosed with Osteoporosis of the spine and Osteopenia in the hip and femur. I'm really worried about going on Dexamethasone as  I understand it can cause bone loss. 

So, firstly, I'm wondering if I can get away without having treatment at all - seeing as I don't have any symptoms except for bruising easily. If I do need to have treatment, then I'm wondering if there is any non-steroid medication that I could go on that wouldn't make my osteoporosis any worse.

Thank you for any advice that you can offer me.

Susie.

Hi SusieJ,
Interesting that your doctor didn't refer you to a hematologist sooner with those drops. I don't know what to tell you about Dex and osteoporosis (I too have that) - what was your count when you saw the hematologist?

Hi MelA,

Thank you for getting back to me.

I haven't actually managed to see the Haematologist yet as they are too busy to see me and are only doning phone call appointments (I'm in the UK and the NHS is a tad overwhelmed). I had my first phone call in August when they asked me to get a blood test. They only call you with test results if they're concerned, otherwise you have to wait until your next appointment. I had my next call with them this Wednesday when they told me my count from my August test was 21. I've just had another blood test and will get the result at my next appoinment in about 3 months time.

I'm wondering if Dex has had any negative impact on your osteoporosis. Do you have to take it long term or is it just something you take sporadically to help with platelets? I was wondering if I should ask about Rituzimab therapy as this doesn't seem to be steroid based - and seems to have bone-sparing effects.

Many thanks,

Susie.

Not sure waht happened but my reply isn't here - sorry Susie. So when your count went to 21k in August it was suggested for you to give Dex a try? I'm surprised it wasn't prednisone. That is terrrible that you have to wait months to find out what your blood tests results are, didn't know that about the UK.

I have never been on Dex - when I was diagnosed the treatments were prednisone or splenectomy, since a cure couldn't be promised with a splenectomy I went for prednisone (which turned me into a WITCH ). I also had one Win-Rho IV after a tetanus booster dropped my count to the gutter. I understand Dex is harder than prednisone so not sure I'd want it.

Wish I could help you with Dex & osteoporosis - I just don't know. Sometimes I wonder if prednisone may have been the reason for arthritis in my knees and shoulder, but who knows.

Is this a question you could ask your primary care physician about - if so I would contact him/her!

SusieJ, you might find the page on this site regarding corticosteroid treatments helpful. It's located at pdsa.org/corticosteroids.html . Or find it in the drop-down menu from the "Patients and Caregivers" tab above. My advice is to read everything in that section. The information this site provides for laypeople is the best you'll find anywhere, in my opinion.

It would be wise for you to get the advice of your doctor about what effect dex might have on your osteoporosis. Dex is much more potent than prednisone, but you'll take it for only 4 days. Then again, it isn't likely to give you a lasting boost in your platelet count. You're likely to need something for treatment again. If that will be a repeat of a steroid, the cumulative effect will eventually affect your bone density.

If you could try a platelet booster drug, it wouldn't decrease bone density, and the other side effects would be fewer. But, they're very expensive drugs, and not routinely prescribed as a first line treatment in the UK. It wouldn't hurt to ask your hematologist about it when you see him/her, though. The attitude about them may have changed by now.

I once took a low dose of prednisone for about two years. It definitely decreased my bone density, but I was able to regain some with extra dedication to a cal/mag/vit D regimen. I was more active in those days, which helped too. I suspect at my age now, my inability to do weight-bearing exercise would put me at a disadvantage there.

A sustained count under 30 probably should be treated. If you were to have an accident with substantial blood loss, or if you'd need emergency surgery, the outcome might be dire. But in the end, I believe the choice to treat or not should always be the patient's.

Good luck figuring out how to proceed in your best interests.

Thank you MelA. I really appreciate your response. Best wishes, Susie

Thank you so much for your post. I really appreciate it. Best wishes, Susie

Hi there,

Well, I am not familiar with NHS. Steroids are often the first prescription, though dex could also be a hold over until you are able to talk to a hematology about your recent labs. Telehealth appt. could be good if they have your labs. Let them know your concerns. They may not be taking your bone health into account.

Yes, steroids are horrible, but I suppose sometimes a necessary evil. I understand your hesitation. What you have been prescribed is not long term, though could turn into that if it works for you... Short term I think you figure out what is important to you and find the balance. If you start spontaneously bleeding, you may have to take steroids for a bit. However, your drops are slow and you are still in double digits, so that's good. So seems you have time to figure this out. Any symptoms?

I would ask about other ITP medications. As, I'm sure you know, a medication that works for me, may do nothing for you. There is Promacta, Nplate... Sometimes infusions such as rituximab, IVIg (though usually for emergencies)... Depends on what helps your counts, what works for you schedule and time-wise, and what they are willing to prescribe. Steriods is certainly not the only option.

Best ~

Hi b2h,
Many thanks for your post. Good advice. Thanks so much.

SusieJ

Hello Susie,

I too am in the UK, and I was diagnosed with ITP in May of this year (2024), at the age of 73. So I am a newbie to the world of ITP!

If you read other threads and the personal stories on this web site, you will notice that the results of treatment are extremely variable. And this was the case for me too.

After I was diagnosed, I was treated with Prednisolone (a daily tablet), plus various other medications (Omeprazole, Aciclovir, Azithromycin, Colecalciferol). This increased my platelet count, but the improvement lasted for only 2-3 weeks, after which my platelet count started decreasing again.

The medical team then decided to discontinue the Prednisolone, and switched me to Romiplostim (a weekly subcutaneous injection) plus a daily Mycophenolate tablet. This initially boosted my platelet count, but the improvement lasted for only 3-4 weeks, after which my platelet count started decreasing again.

So the medical team discontinued the Romiplostim and switched me to Rituximab. This is administered as an infusion. You sit in a treatment chair in the Day Care Ward, and a pump is connected to a vein in your arm to pump the Rituximab in. Because Rituximab can cause an adverse reaction in some people, they give you tablets to damp down your immune system before starting the infusion. I think these tablets are Piriton and Paracetamol. They also start the infusion with a very low dosage for 30 minutes, with a nurse checking that you still feel well. If all is OK, they double the dosage rate for another 30 minutes. This is repeated several times, each time doubling the dosage rate. Those who did maths at university will realise that the last dose by itself is marginally bigger than all of the other doses combined.

I had four Rituximab treatments, spread over four weeks. I was pleased to find that after the last treatment my platelet count was back to normal. No additional treatments of any sort are required. However, Rituximab is not without its disadvantages. Specifically, it works by eliminating the naughty B cells in your blood. The B cells are the ones that destroy your platelets. With no B cells you are now "immune suppressed", and are vulnerable to contracting opportunistic infections. It also means that you cannot have any vaccinations that use vaccines with live components. As it happens, the influenza and Covid vaccinations that are offered annually in the UK do not involve live components, and so are safe. But with a suppressed immune system, those vaccines are likely to be ineffective.

Your B-cell count does not remain suppressed permanently. It will recover gradually over the next 6-9 months. This means that your platelet count might start decreasing again. There is a review paper available on the internet here:

[url] pmc.ncbi.nlm.nih.gov/articles/PMC6545833/ [/url]

The authors review all of the papers published by clinical teams reporting the results of using Rituximab in their treatments of ITP patients. The reviewers concluded that in those patients that showed remission after the Rituximab infusions (that includes me!):

- Only 40% still showed remission after 2 years.
- Only 20% still showed remission after 5 years.

I might be lucky and be one of the 20% where remission lasts 5 years, but it is more likely that I will be one of the 60% for whom remission does not even last 2 years. What happens then? I don't know!

All of the above treatments were carried out by the NHS (the National Health Service in the UK), and I don't have any criticisms concerning the speed or quality of the treatments (but your experience of the NHS may be very different from mine). None of the treatments caused me any problems with side effects, but that is not the case for everyone (as reported elsewhere on this web site).

As an earlier respondent mentioned, you should certainly tell your haematology consultant about your osteoporosis problem, as it could well cause him/her to change their suggested treatment. Which treatment to accept is not an easy choice, and I really hope that this works out well for you.

-- from Clive in the UK

Hi Susie,

I hope you are getting on alright. I recently found this patient leaflet which I thought might be useful to you. It contains information about treatment options and supporting ITP patients to be involved in treatment decisions. [ www.itpsupport.org.uk/download/ITP%20Shared%20Decision%20Making%20Toolkit%20FINAL%20Version.pdf ] If you do find it helpful, maybe you could fill it in and send it to your haematologist?

If you are still looking for treatment suggestions, I would also mention eltrombopag or similar medications as non-steroid options. These stimulate your bone marrow to make more platelets (if I remember correctly) and personally I had no side effects from eltrombopag at all, and it worked for me for years. I don't know if there are any contraindications for osteoporosis or other medications you may be on, though.

In terms of blood test results: if you get your bloods done at your GP practice, you could ask of they have online services, such as the NHS app. If so, you might be able to access your blood results on there. You could also ask the reception team at your hospital department (haematology?) if there is any way for you to request blood test results. Ideally they would assign you a specialist nurse who you could contact.

Best Wishes,
Raph

Clive
(Sorry I cannot find a way to turn your quote into coloured italics like we used to on the old system to indicate we were quoting)......"I might be lucky and be one of the 20% where remission lasts 5 years, but it is more likely that I will be one of the 60% for whom remission does not even last 2 years. What happens then? I don't know!"

If you have responded well Rituximab courses can be repeated if your count drops again. .

Clive, I have been in remission since 2011 from Rituxan (here in Canada) (Rituximab, I'm assuming in the UK).
I'm probably in the minority for success.

Hi Susie,
You could ask your hematologist if delaying treatment and monitoring your platelet counts is safe instead of starting Dexamethasone.

It’s possible you may not need immediate treatment if your only symptom is easy bruising, but your low platelet counts (19–21) suggest a discussion with your hematologist about risks versus benefits is crucial. Non-steroid options like TPO-RAs or Rituximab could be safer for your bones, and optimizing osteoporosis management (e.g., bisphosphonates, calcium) is key if steroids are used. Share your concerns about bone health with your hematologist to tailor a plan that balances both conditions.

lauralordelaure wrote: It’s possible you may not need immediate treatment if your only symptom is easy bruising, but your low platelet counts (19–21) suggest a discussion with your hematologist about risks versus benefits is crucial. Non-steroid options like TPO-RAs or Rituximab could be safer for your bones, and optimizing osteoporosis management (e.g., bisphosphonates, calcium) is key if steroids are used. Share your concerns about bone health with your hematologist to tailor a plan that balances both conditions.

In my opinion, this moment for a new patient should be addressed with a hematologist, like you said.  
 
Then, the thrombopoietin test could be telling, before any medication, to see if there is a shortage of thrombopoietin which is responsible for platelet production. This will help you, I believe,  on second line drug choices down the road if options start running out.
 
Then try papaya leaf extract and adjust dose with the bi-weekly blood tests. If you find this works, then you have your maintenance and your safety net if the number drops further, to keep you away from the steroids, and/or hospital. 

Of course, don't expect complete acceptance of the papaya leaf extract from the hematologist... Its something that can't be denied, when it works, but their curiosity is often limited. You need the blood tests, so hopefully you find a relationship that works. He will watch the numbers, and as long as they are not going into the low serious zone, things should be good.