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!

Wake up call 

Every once in a while I need a reminder that my ITP is a chronic auto-immune disease, and that just because I feel well and full of energy and a functioning brain, I am not over it. I am not cured, because it’s an auto-immune problem for which no-one knows the cause. Since we don’t know the cause, we can’t eliminate it, and it’s probably exacerbated or mediated by various genetic and nutritional deficiencies I’ve had since birth. And so the problem of low platelet numbers will recur time and time again. I received a wake up call last week when my latest blood test showed an alarming figure of 17, three below the safety level my doctor and I had set for going to hospital. To be honest, the levels had been dropping over the previous three months, by about 50 percent each six week test, but we’d quietly ignored the decrease as my numbers were still high, and I was doing well on all the other counts – haemoglobin, cortisol, adrenals, liver function, kidneys, everything except my IGGs, which were still a bit behind.

At the end of April I had a flu shot, as winter was approaching with the flu season. Two weeks later I flew to the US (21+ hour flights each way) and had three wonderful weeks with my daughter and her husband in Boston, and three and a half fabulous days in New York. I paced myself, resting on every second or third day, and every day for a couple of hours in NYC, but other than that had a fantastic time, travelling, eating well, drinking cocktails, meeting new people, visiting museums, theatres and art galleries, and exercising once a week in their home gym.

When I came home to Sydney, I was tired but still high on pleasure, and apart from normal tiredness – no jetlag – felt fine. In fact I felt better than fine. I felt fabulous! A week later I had my blood test, and a phone call the next day from my doctor, concerned about the dreadful figure for my platelets. When I saw her, I assured I was feeling fine, that I had no spontaneous bruising (plenty of bruises from travelling, but I knew the origin of each bump and knock), and that my gums were only bleeding a little when I cleaned my teeth. We had a serious discussion about risk management, and agreed that I would go straight to A&E if I had any fall, trauma or was knocked down in the street. As before, the risk is invisible but serious – internal bleeding, especially into the brain if I had a head injury.

Since winter had already started, and although we were enjoying milder temperatures than usual, early mornings were foggy and rainy, so I agreed to stop going to the gym for the next few weeks. Early morning is the best time of day for me to go to the gym, so I’ll just have to exercise at home for a while. No sense risking getting knocked down getting on or off a bus!

A week later and I’m rejoicing that I have a head cold. Violent, explosive sneezing, runny nose, slight temperature, mild sore throat. Nothing major, just a common or garden cold, an URTI, but it’s something to keep my immune system busy, so it leaves my platelets alone. No-one enjoys a cold, but I’m actually delighted this one has started. Next week’s blood test could show a nice increase in my platelets What the statistics from my three and half years of blood-tests show is that I need at least two episodes of illness – flu, stomach bug, heavy cold, migraines that leave me dehydrated from nausea – to give my platelets a chance to recover and bounce back to normal.

So here’s hoping that this week’s cold will do the trick. I don’t want anything worse. I had a bad stomach bug in late November that was absolutely horrible – vomiting, diarrhoea, shakiness for days – that messed up my plans for Christmas. But did bounce my platelets up to 146. If this cold works, I should have three to five months of good numbers before I need another infection. Bring it on! Meanwhile, pass me the tissues, I feel a sneeze coming…

Reasons to be cheerful

Apart from the happy memories, photos and new clothes from my holiday, the fact that I mostly feel well and have patchy but fairly regular freelance work I can do at home are very good reasons to be cheerful.  Living in a beautiful city with lots of cultural activities I enjoy with friends is a bonus!

More Thoughts on ITP

ITP has a new name, or at least a more efficient attempt at nomenclature. New research on the condition was reported in the March edition of the American journal, Hematologist. The very first news was the name change, according to Professor of Pediatrics at New York Presbyterian Hospital, Weill Cornell Medical College, Dr James Bussel.

“There is now a standard nomenclature. ITP is now immune thrombocytopenia; the “i” is no longer “idiopathic” and “purpura” is no longer part of the name,” he reported.

Well, so now the medicos no longer confess in the condition’s name that they don’t know the cause. It is now officially an auto-immune disease and perhaps they have some better idea as the original cause of the condition.

New Hypotheses on the Pathology of ITP

Dr Bussel reported that understanding the underlying pathology of ITP has changed. The old idea was that thrombocytopenia resulted from antibody-mediated platelet destruction.

Now there are two new concepts. The most developed hypothesis is that the same antibodies that mediate platelet destruction also impair platelet production by damaging the megakaryocytes (the bone marrow cells responsible for the production of thrombocytes) and/or blocking their ability to release proto-platelets.

That's one idea. Here's another: “An exciting but to-be-explored area involves the role of platelet reactive cytotoxic CD8+ cells. These cells clearly exist, but their clinical relevance is not known,” Dr Bussel reported.

Another area of intensive investigation involves T-regulatory cells, which have been reported to be deficient in ITP in several studies, but as yet there are no results.

Eradicating H pylori Infection Lifts Platelet Numbers

Some researchers have suggested than an infection with Helicobacter pylori, the bacteria responsible for causing stomach ulcers, may have a limiting effect on the numbers of platelets in people already suffering from ITP.

An article in the journal Blood in February 2009, reported on a systematic review of all the studies published dealing with H pylori infection in patients with ITP. The researchers wanted to test “whether the eradication of H. pylori infection can increase the platelet count in patients with immune thrombocytopenic purpura”, which they said was “still a controversial issue”.

They looked at 25 studies including 1555 patients, and saw a 42.7 percent increase in patients’ platelet counts from around 30 to 100, and a 53 per cent increase in patients’ doubling of platelets from around 30 after treatment to eradicate H. pylori infection.

The researchers said responses were better in countries with a high background infection of H. pylori, but concluded that “the detection and eradication of H. pylori infection should be considered in the work-up of patients with seemingly typical ITP”.

I’ll certainly be discussing a test for H. pylori with my GP!

On the Way Up, I Think

For the past two or three weeks I’ve been feeling stronger and healthier, which is a great improvement. For three weeks in a row my platelet count sat at 46, so my doctor and I reduced my steroid dose by a whisker – 0.5 mg – to 7mg a day.

The following week the count dropped to 32. I thought this was a disaster, but my doctor was much calmer. All my other blood chemistry was good – haemoglobin back to normal, non-anaemic levels, kidney and liver functions good, and the cytotoxic leucocytes – the white blood cells eating up my platelets like Pac-Man in my bloodstream – were back down to a normal level. She tweaked my Chinese herbs some more and cautioned calm.

This week, the platelets have crept back to 36, and my physical energy and brain power are both at about 70 percent of what they used to be. I feel scarily normal, and will have to work hard at not working hard – that is, pacing myself to take lots of rests, do as much exercise as I can without tiring, and enjoy time with friends. Although I feel capable of working, I must be aware that I’m not 100 percent.

Meditation and Staying in the Now

In the past couple of weeks I’ve started seeing a psychologist to help me with depression – a normal side-effect of a chronic health condition, which is apparently exacerbated by the steroids. After a couple of sessions discussing the meaning of life (the old ‘why me?’ cry, when struck by crisis like diagnosis with a potentially life-threatening illness), we decided that what I needed was help with focus. Staying in the now.

So I have a cd with meditation exercises, and as well as going for a walk every day, I must now sit down quietly for at least 10 minutes and try to still my anxious, chattering mind. I know from reading about meditation that it can help lower blood pressure. I wonder if there’s any study done into the beneficial effects of meditation on platelet counts?