Newbie help please. Knowledge is power!

Yes Sandi I know what you mean. I took mycophenolate for 3 months as it seemed to work once I was on 2g a day by which time I felt in a permanent fog most of the time. Then my platelets crashed and stayed down so I came off it. Within a week the fog was gone.

Ferv there are several other options available.

Sandi & Mrs B
Thanks for getting back to me,

Here are the options on my UK hospital's regional pathway site:
2nd line treatment options
- Rituximab
OR ANY OF THE FOLLOWING
- Mycophenolate mofetil this was the only option mentioned... apart from splenectomy.
- Danazol
- Dapsone
- Vinca alkaloids
- Cyclosporin A OR
- Splenectomy (noting Current guidelines suggest to defer splenectomy to at least 1 year from the time of ITP diagnosis)
Have they missed something that might be more appealing or are these the other options you meant?

I found this article...anyone know more?
20 patients were prospectively enrolled onto a single-arm phase IIb study to describe the safety, efficacy and tolerability of oral dexamethasone 40mg Day 1-4, oral cyclosporine 2.5-3mg/kg/daily Day 1-28 and intravenous low-dose rituximab 100mg Day 7, 14, 21 and 28
www.bloodjournal.org/content/early/2015/05/13/blood-2015-03-631937?sso-checked=true

I've been waiting for an appointment with Drew Provan....but he's retired!

Thanks for your time
Ferv

That article was discussed here:

pdsa.org/forum-sp-534/6-general-itp-discussion/16141-new-articles-posted-here-update-11-21-15.html?start=120#48706

I would add that the idea of taking these three medications simultaneously, all of which lower the immune system, scares me. Taking the dexamethasone and Rituxan and leaving off the cyclosporine does not much change the results, and it would seem less risky to me.

You did not mention Promacta or NPlate, which are your next best treatment if you can get it prescribed. I understand that is a big "if" for you.

Another treatment option not listed is "watchful waiting". The last I read was that your platelets were at 30, but I know you have been lower and had symptoms of bleeding. Still, you might want to slow the taper and see how low your platelets can go without symptoms becoming a problem, then treating with the minimum prednisone to keep you in a safe range. You know you respond well to steroids, so you have an easy rescue if symptoms get out of hand. All of the treatments you listed have risks. Remember, at some point the treatments become riskier than the risk of bleeding. What was your lowest count, and how bad were your symptoms?

Then there are the natural methods, which I am not too keen on, but others are, if you are interested

I am 68. My hospital's route for me over 3 .5 years has been:
bone marrow scan to check problem is platelet destruction rather than production
70mg prednisone daily for 3 weeks , ineffective no change from count of 3
Ivig , no change,
Indium scan to check source of destruction ,for me in both spleen and liver ,so splenectomy out, not seriously considered anyway
Eltrombopag at varying doses , worked for 2.5 yrs but gradually became less effective, ending on10 at max dose
Change to Nplate over 4 mths ,after an initial rise to 32 then again gradual deterioration to 10 at max dose.
Discussion about Rituximab next
My decision to watchfully wait -lasted 5 weeks- fell to 3, onTranexemic acid 6 per day, 2 platelet transfusions
Then had 4 weekly infusions of higher dose Rituximab
At start of 4th infusion had fallen to 1 , nose has been oozing and gums bleeding for 2 weeks, tranexemic acid up to 8 per day,ferrous sulphate added as Haemoglobin also low despite ok ferritin. No evidence for AIHA. May have poor absorption.
Decision to include 4 days of Dexamethasone 40 mg per day. After one day nose bleeding stopped and gums much better.
Just on day 2 and still improving
Depending on outcome may ask for 2 more 4 day pulses as evidence in 'Blood' indicates 3 pulses has good outcome in younger patients than me but still hopeful
Given my history and age I do not expect to ever achieve a count anywhere near normal. Would love to be 20-30 but would be happy to stay above 10 as fortunately I do not have limiting symptoms at that level: enjoyed a holiday by train in France this year while at 10. If Rituximab/dex fails to do this I may ask to try Eltrombopag again even if only at 25 mg to keep above bleeding level for me(about 5) . NHS Scotland will give the TPOs as second line drugs, before Riuximab.

Ferv which hospital are you attending?

I'm a renal nurse . I would not recommend Cyclsosporine it is known to cause kidney failure.
Try stick out for romiplostin (N Plate) I seem to be getting on quite well with it on a very low dose

Hi Rob, thanks for the input. I couldn't find the thread on the forum page link, but will return and have another look.
Promacta and Nplate absolutely not available in UK unless 2nd line has been tried and possibly not after that!

I was going for watchful waiting just as you say. When I reached 5mg my dr said the ITP was not steroid dependent and I should come off Pred ASAP. Why I complied I'll never know and the result was the drop. My count is at nearly 50 on 10mg and am reducing slowly 1 mg a time. I think the dr just wants me off steroids and is anxious I will have side effects blaming them, so I am feeling very pressured.
What is a reasonable minimum level to hope for?
My symptoms were mainly large shiny purple spontaneous bruises, just a few petachea and purpura and also bleeding gums. I caved and took pred at count of 16. By the way I'm 66 so more at risk from bleeds.

I feel vulnerable because I am on my own and this is why your thinking is so valuable to me. My thinking has gone a bit 'predbrain'
Thank you Ferv

Mrs B they won't give it to me unless I take MMF.
I didn't ask for it...the haematologist just came out and told me "You have to take this (MMF) before you can get the drug that is most likely to work". Seems like a conversation she may have had before.
I'm at St Peter's in Chertsey. Was waiting for my GP referral to go through to Barts when I chased found out Dr Proven has retired and they are in ..CHAOS! No chance any time soon, so trying for St Georges Tooting.
I'm so glad to hear that a very low dose is working for you, very glad indeed. Which hospital are you at?

Where is Ann - she is the expert for treatments in the UK [in my opinion].

I did the Rituximab (700mls x 4 weeks) + Dexamethasone (40mg x 4 days) + Cyclosporine (200mg morn/night) combination without any side effects but unfortunately it had no effect on my count and prior to that I was given Dapsone which was also ineffective. After these treatments it was then on to NPlate where I had great success.

It frustrates me to no end that they put patients through so many treatments that don't work until they finally get to the TPO's. Waste of time and money and causes unnecessary side effects.

Unfortunately we seem to follow the UK model where the various treatments have to be done in a specific sequence. I initially did the usual Prednisone and IVIG before moving on to the other treatments previously mentioned and I was lucky to get access to NPlate as it is only offered to patients who have had their spleen removed due to the high cost. My specialist had gone to a conference where they were finding that patients treated with NPlate within 6 months of diagnosis were going into an unexplained full remission and so I was given access to NPlate even though I still had my spleen. These findings were discussed recently in a PDSA media release on the 25 Nov 2015 and this is exactly what happened to me after just 9 months. Of all the treatments I had though it was only Prednisone that really caused me any grief just as it has to others on this forum. Hopefully with this new data, the treatment of ITP may change and access to the TPO's be given more readily so as to avoid the high cost and risks of IVIG, Rituximab and the other treatments.

Robert

That is inspiring for the future, I'm so glad that research on TPOs is beginning to be responded to by more enlightened doctors leading the way!

Thank you for bringing it to my attention,
I'll look it up
Ferv

Hopefully some things will change though - other than rescue IVIG and steroids the treatment course suggested for my daughter was TPO's (we went with Promacta). When she was initially diagnosed 2 years ago and the treatment options were outlined TPO's were not the initial choice, but in the 18 months after diagnosis until she really needed treatment her doctors started promoting TPO's first. I am not sure how much of an obstacle insurance protocols are - we were fortunate that we did not have a problem with them covering it.

Fervenz asked:

What is a reasonable minimum level to hope for?
My symptoms were mainly large shiny purple spontaneous bruises, just a few petachea and purpura and also bleeding gums. I caved and took pred at count of 16.

"Watchful waiting" is a very individual and subjective process. Individual in that everyone's symptoms are different at a given count. Subjective in that each person has their own comfort zone. It also depends on how quickly one responds to steroids, versus how bad the side effects are.

Personally, as a husband of an ITP patient (Ellen) who responds well to steroids but hates them with a passion, I become very nervous when I see large shiny bruises. I would become very watchful at that point, and if counts and symptoms didn't start turning around very soon, or if any new symptoms started getting worse, I would want to see treatment started. If she were 66, I would probably begin to treat at the point of large bruises. But that is totally subjective.

Rob

I have much dispassionate advice... so subjective is fine by me!
No those bruises don't look good, and if there was a chance of finding the lowest steroid dose I may have missed it.
I will try to hold out for Romiplostin as Mrs b04 suggests, but not sure how to go about that. That are pushing MMF so hard. Oh, I don't want to take another immune suppressant at all.

So far eltrombopag was best for me for 30 mths. The dosage is easier to manipulate than N plate and it is cheaper , especially at a low dose, so may be more acceptable to your NHS trust. I would go for that first. My hospital,Aberdeen Royal infirmary ,went for that first after ineffective Prednisolone. You could get your consultant to contact the haema dept there. Boss is Prof. Henry Watson.

Margaret

Thank you for the info. North of the border obviously has a more flexible system, not sure there's any leeway here. I'll give it a go..with the contact details you gave me too.
On Monday I see my consultant and I'll post his response for sure.

Wishing you +luck with Dexamethasone
Ferv

Ferv there is always azathioprine if they insist you try another drug before considering TPOs.

Eltrombopag may be cheaper than Romiplostim but for me, as a shift worker with osteoporosis, it wouldn't work with the dietary restrictions pre and post dosing.

Hi fervenz,
Aberdeen Royal Infirmary is a Clinical centre for ITP Like Barts and St George's.
Prof . Henry Watson is a consultant with expertise in coagulation issues like haemophilia and ITP.
My 4 days dexamethasone is up now and though I've stopped bleeding from gums and nose my count is still 2 so I am still taking tranexemic acid too. The dept is good at keeping in touch by phone and have assured me that they are working on a plan to get me through Xmas. Due to go in on 18/12 but may get the call before then.
I think that the differences in prescribing decisions are due to the Scottish medicines Consortium being responsible for NHS
Scotland 's drug policies.
I managed Eltrombopag by taking it either very last thing at night or even at times in the middle of the night if I ate late. Later when it's effect waned I was switched to Nplate. I managed to inject myself ok but it didn't work well enough even at high dosage. Although more flexible and generous the protocol here says that after 4 weeks at max dose without a count above 20 treatment should be discontinued. Max dose for me was 3 packs per week at cost of £400 per pack per week. Good luck with your fight.

Margaret

Thanks for the update and more info. I'm sorry to hear that the TPOs didn't give you a longer break, that's tough.
Aberdeen R I sounds like a good place to be treated, with your Prof Watson. I'll see what happens here in the south on Monday.
Good luck on the 18th and hope the team there do you proud with a plan that means you can relax and enjoy the holiday season.

Ferv

mrs b
Do you think azathioprine is less destructive than MMF?

Ferv:

I've taken both. I'd say they are about the same. They are both strong immunosuppressants.

Thanks Sandi... and thanks to everyone who has taken time to give me the benefit of their experience!

I'm clearer about my options now and what I will do when I know what I am offered on Monday.
I wouldn't have been in this position without the help on the site, whatever the outcome at my appointment I am very grateful for this.

Thank you all!

Ferv

I really don't think there is much to choose between them to be honest. Neither of them worked for me I'm afraid.
My consultant stopped mentioning splenectomy when I told her I didn't want one. There is no guarantee it will work and I cannot see the point in having a perfectly healthy organ removed.

My aim is to keep my platelets over 50 until I retire then have a rethink about what I will do after that. Splenectomy will not enter those thoughts

Yes, the argument for splenectomy is very weak indeed. I am antibiotic sensitive due to UC so it wouldn't be a realistic option for me anyway. I want to try to get to a low dose steroid maintenance, but I'm out of time they say.
Aren't you on a TPO? That's working OK so far?50 is good, hardly any symptoms, hope that happens. It's tough to work with the fatigue aspect, does that bother you?

Out of time? What do they mean by that?

I echo Sandi's comment about out of time.

Romiplostim does seem to be suiting me at present and I hope it stays that way as only on a tiny dose.

With regard to the fatigue aspect, quite frankly it's the worst because waves of it come over me without any warning.

Work can be hard sometimes. People don't understand and think I'm better as no longer covered in bruises.

Occupational health are sending me on a well being at work course next month. Could be interesting

What do you mean by "low dose steroid maintenance"?
Usually the doctor/s want one off prednisone as quickly as possible.

Can't address the fatigue issue, never had it with low counts.

'out of time'
Should have read ...out of steroid time! I have to move on to 2nd line.
Sorry if it sounded dramatic.

I still believe ITP emerged as a result of taking mesavent. So I was secretly hoping all this time, for a miraculous recovery once the drug was stopped, didn't happen....although my symptoms did begin to subside immediately, well before diagnosis and treatment.

Immunosuppressants probably increase reliance on antibiotics, don't they? I am highly reactive to these and suffer flare ups of UC after treatment with them. I am very scared of this happening. So have been refusing MMF. No other option has been offered.

Rob's post above echoed my thinking re steroids with more clarity. I am very responsive to them and maybe I can take a risk lowering the dose to something acceptable. Of course I'd like to be on TPOs, especially as I am only 8 months since diagnosis...and latest research is so positive for remission. TPOs are only second line in the UK in certain cases inside very strict guidelines. The wording is very difficult for me to understand, but it will depend on the hospitals interpretation anyway, not mine. I see the consultant on Monday and expect to be on MMF and follow the prescribed pathway...in the hope I will be able to progress to 3rd line treatment eventually.

Ferv