Time for a re-evaluation

Over the past two weeks my platelets have risen quite nicely, and so far have stayed up.  My doctor and I have continued reducing the steroid dose, and this week agreed to stretching the weekly blood tests to fortnightly.  It's official - my condition has "turned around, stabilised" - at least for now.

On Wednesday she and I had a serious discussion about what we've learnt over the past three months, and what direction we - or more importantly, I - want to take on this journey with my auto-immune disease.

After I'd done all the research I cited in my last post, I sent her the link to Crystal's story, requesting her views on the haematologist's comments, which I'll quote here: "The goal for treatment of ITP is only to treat or prevent bleeding.  There is no other goal.  The platelet count tells us something about the risk for bleeding, but even patients with very low platelet counts for a very long time may have minimal bleeding symptoms, or none at all. "

He went on to say "patients with ITP who have very low platelet counts are often treated with many different medicines even though they have never had any significant bleeding. And the outcome is often that the treatment of ITP is much worse than the disease."

I'm a little bleeder

Readers of British background and those of us who watch British tv shows will get my pun. It seems I'm actually a low bleeder. Even when I was down to 12 recently, I had very little spontaneous bruising, just a couple of smallish marks on the backs of my thighs. When my gums bleed while I'm cleaning my teeth - maybe once a month - the bleeding stops as soon as I swirl my mouth with icy water. If I cut myself, it stops after a couple of minutes pressure or a tight bandaid. Even when the regular pathology nurse is away, and the replacement nurse is a little clumsy, I only lose a few drops of blood, although the blood flows fast enough into the vials while it's being taken.  So even with low platelet numbers, my blood does apparently clot fairly rapidly.

Doing the numbers

My doctor cautioned me to remember that platelet numbers quoted in the US studies such as Crystal's Story, are not directly comparable with Australian or UK numbers, because the measurement systems are different. Australian and I believe European systems (which would include UK), measure platelet numbers at10 to the power of 9 per litre of blood. So even when I was down to that scary 12, I actually had around 12.000,000,000 platelets in each of my approximately five litres of blood. And they seem to be doing their job, even though the average count should be 150-450 (times 10 to the 9th).

What we learned

Living with an auto-immune disease is a daily experiment, with an experimental sample of one, since no two people have exactly the same physical, emotional, psychological or spiritual experiences, even if they share the same disease. And like the good haematologist, Dr James N. George, who is Professor of Medicine, Hematology-Oncology Section, Department of Medicine, Department of Biostatistics & Epidemiology, College of Public Health at the University of Oklahoma Health Sciences Center, my doctor believes in listening to the patient and learning from their experience. This is what we've learned from the past three months:
1/ I can crash while feeling really well, without any warning signs and no clear reason why;
2/ During a crash, applying high doses of steroids doesn't have much effect, but when it turns around, small doses are quite effective;
3/Just when you think you know what to expect from the illness, something changes. "Welcome to the wonderful world of auto-immune disease."

Where to from here?

Over the next few months we will continue to reduce the steroid dose, with aim of weaning me off steroids altogether. Maybe not by Christmas - a goal we've aimed for each year for the past three years - but some time in the next six months. Then the next phase of the experiment will begin. Living on the edge - life with ITP without corticosteroids.  The safety net will be IV immunoglobulin and/or platelet transfusion if I get dangerously low. 

But that's a while down the track.  Right now, it's enough to know I've stabilised again, and we can get back to weaning my body off those horrible steroids.

Reasons to be cheerful

All of the above, my lovely family, good friends, and a new writing course I'll be starting next month.

Another roller-coaster ride, so it's back to the research

This week's number was not a beautiful one like last week's. From 96, I dropped in seven days down to 41, a loss of over 50%.  Meanwhile my face is still swollen and uncomfortable, and my energy is unreliable, trickling away quite fast on some days, taking a while to get started on others. No wonder I've been a bit down this week,  a little weepy, or at least quite grumpy! 

On Monday I tried to get back to the gym, reasoning that structured  exercise would be good for my mood, but a combination of my bus being late, leaving me to stand around in the cold wind for 20 minutes and my energy running out before I got to the gym, meant the best I could manage was a gentle 15 minutes on the exercise bike and five minutes doing bicep curls and rotator cuff exercises for my damaged shoulder. The gym staff were, as ever, friendly and helpful,  and my membership fee suspension has been extended for another fortnight, bless them, but it was disappointing.

However, I can still work, thank goodness, if a little more slowly and my brain is still able to make sense of scientific and medical research information. So today I went searching again online for any news or recent clinical studies on ways to treat ITP.  In doing this research I've been reminded of a fact I keep losing sight of.  Unpleasant as my experience of ITP is, I am way better off than a lot of people! I do not have  refractory ITP, as I was beginning to imagine. Refractory ITP occurs when even surgery - splenectomy (removal of the spleen)  - does not produce a lasting increase in platelet numbers. I just have simple ITP.  My platelets respond OK to largeish doses of  the cortocosteroid prednisone (known in the medical world as Pred), and until recently they behaved reasonably well as we weaned me off the horrible things. I was down to 0.5mg a day for three months before my count got so low as to scare both my doctor and myself.

Options for ITP treatment

So here's what I've been reading.  First up, a straightforward account of the protocols of treating ITP: Immune Thrombocytopenic Purpura Treatment & Management. This is where I learnt that my ITP is not refactory.

Next I looked up Rituxan, as it had been suggested to me by two or three commentators on this blog as an alternative to the Pred, as a way of avoiding the horrible side-effects.  But, as the above article points out, Rituxan (also known as Rituximab and Mabthera) is a third level treatment - ie - after Pred and intravenous immunoglobulin or Rho(D) Immune Globulin haven't worked. But I read about a Danish multi-centre study into the use of Rituximab specifically in ITP patients, as the drug was actually developed for use in non-Hodgkin's lymphomas and other haematological cancers.  It's also used 'off-label'  in difficult cases of multiple sclerosis and lupus, and is a possible therapy for ITP.

However, it turns out that Rituxan by any name is not a replacement for Pred, but an adjunct, as the Danish study shows.  Nearly all the patients in the study were given Pred prior to their weekly  infusion of Rituximab.  Some received as much 100 mg each time. To be fair, that was only once a week, but that's a heck of big dose! And the results, from an overall count of  35 patients, less than half got a response of 50-100 more than their previous platelet score. So, while I'll discuss Rituxan with my doctor, and the haematologist when I eventually get to see him - my appointment is two month's away, I'm not pinning a lot of hope on it.

A cause for optimism

Lastly I found a site for patients called Platelets on the Web, which among other useful information, gives four patient stories of their experiences with ITP.  The one that gave me cause for optimism was that of a 75-year old woman who had incredibly low platelet numbers - down to 3 at one point - who was eventually able to go off medication after even a splenectomy did little to improve her count, and then a year or so after her initial diagnosis, had a complete spontaneous remission, and remained healthy for many more years. It's called Crystal's Story.  The take-home message I got from reading that - apart from the hope for spontaneous remission -  was her doctor's comments about "the value of cautious, conservative treatment for patients with ITP. The goal for treatment of ITP is only to treat or prevent bleeding.  There is no other goal.  The platelet count tells us something about the risk for bleeding, but even patients with very low platelet counts for a very long time may have minimal bleeding symptoms, or none at all. "

He goes on to say "patients with ITP who have very low platelet counts are often treated with many different medicines even though they have never had any significant bleeding. And the outcome is often that the treatment of ITP is much worse than the disease."

Woo hoo - a doctor who understand his patients!

 It's good news week

 Well, this week is good news week, and the lucky number is 96!  That's a long way up from last week's dismal and scary 20, or the 36 the week before, and eight times better than that terrifying 12 I had a month ago!  So last week's steroid pulse worked.

Never mind that the right side of my face is tight and shiny, puffed up into the beginnings of the dreaded 'pumpkin face' I had for several months back in 2010. Or that my sleep  at night has been badly disrupted, waking to pee, and not getting back to  sleep for an hour or two, or on one really bad night for two and half hours. Plus all the other high steroid side-effects I've already described.  No wonder I've been grumpy lately!

Finding some good in necessity

There has been one unexpected benefit from the pulse and that's been the steroid 'hyper'. While it's a pain at night to have your brain going so fast you think it will take the top off your skull, in the daytime that much energy and ability to think fast is exhilarating. I've completed a huge lot of work over the past two weeks thanks to this amazing source of energy and surprisingly clear thinking. Not creative work, but nit-picking proofreading for commercial clients, which helps to pay for all my expensive supplements and high quality meat and fish protein. That's very satisfying, since I've been stuck at home for so much of the past few weeks and the hyper mood does not allow for quiet reading and listening to music! 

Sudden energy slumps

The downside is the  way that energy suddenly drops, like it fell out of your body down a chasm.  Whump! It's gone, and I'm left drained and brain dead for several hours.  Until the brain starts up again about 10 pm...

A holiday from the pulse

Since my number was so unexpectedly beautiful, my doctor and I agreed on a week's holiday from the pulse.  We'll see if my face goes down, the other side-effects subside to bearable, and more importantly we'll see if next week's blood test shows the platelets up, at the same level, or down.  My doctor thinks we've turned this low patch around.  I'll believe that when we get a run of good results.

On the bright side

 It's so good to be able to sleep again at night!  To wake and go for a pee, come back to bed and zonk straight into dreamland is wonderful!

Walking through the university early in the morning for my weekly bloodtest is always a pleasure, no matter how shaky I feel when I start out. Here's a secluded corner of the uni grounds:

Feedback from fellow ITP sufferers

Last week I had an email from John, who was diagnosed a year ago. It was lovely to have an email conversation with him, especially as he told me he found this blog helpful.  He described the major changes to his life in the 12 months since his diagnosis - " new routines, new worries, learning to adapt to changes in my life" and the fluctuating low platelet numbers. I'm sure I'm not the only person with ITP who can relate to John's experiences! I would love to hear from others about their experience with ITP.  You can email me or simply comment on this blog.

John was hoping that having a weeekly injection of a medicine called N-Plate would bump up his platelet production. He and his doctors are hoping he can get to 75. 

Romiplostim

N-Plate's pharmaceutical name is Romiplostim. It stimulates the bone marrow to create large numbers of platelets at a time to overcome the immune system's ability to destroy them.  It is not intended to create a full platelet count but to get the platelets over 50.

In the US, the FDA approved Romplostim as a long-term treatment for adults with ITP  who have not responded to other treatments such as steroids,  intravenous immunoglobulin, Rho(D) immune globulin or a splenectomy. In Australia, it is considered very much a last resort treatment, after everything else has failed.

Good luck with your N-Plate, John. Do let us know if you get to 75 and stay there.