Reaching four years

Reaching four years

In a couple of weeks I'll reach the fourth anniversary of being diagnosed with ITP.  I am almost adjusted to living with a chronic autoimmune disease.  I say almost, because last week I had to see the haematology professor for the first time in three and a half years, since my GP took over managing my condition.  Most of those four years have been documented in this blog.

But as it was coming up to that anniversary, and my platelets had dropped quite low after my wonderful holiday in the US, my doctor and I  agreed it was time to get some advice from the haematologist.  Having worked out that I'm not a bleeder and that I'd like to risk life on no steroids at all, eventually, I was nervous about this visit, convinced I'd be forcefully advised to stay on the prednisone, or if not, some other unpleasant, and to my mind unnecessary, treatment.  (The professor and I had not really hit it off during the time I'd had to see him for weekly visits!)  I took my latest blood test results as requested (platelets 29, everything else good, apart from the kidneys, from not drinking sufficient water in our very hot spring weather).

To my relief I was not seen by the professor but by his registrar, a younger woman doctor who listened to me and my arguments for not having treatment after the prednisone weaning. She agreed that four years on steroids is too long, given the bad effects on my bones and skin and adrenals.  Then she quietly and patiently explained what treatments I would need, in hospital, if my platelets dropped below 10.

10 is my new danger line

My doctor and I had had agreed on 20 as the number at which she would advise me to go to the hospital ER. But when I came back from Boston and we discovered I had dropped to 18, I was clearly so healthy - none of the nasty flu, stomach bug or other winter ills going around - that she just advised taking extra care to avoid accidents and we boosted the steroid dose again.

The haematology registrar explained that below 10 was dangerous because, as well as the risk of internal bleeding from a fall or a blow to the head or abdomen, there was the scarier risk of spontaneous internal bleeding - blood leaking into the brain or internal organs.  Now that IS scary!  I was quite happy to agree with her that if I did drop below 10 during or after the weaning, I would go immediately to the ER, and receive intravenous immunoglobulin, and then go on weekly infusions of rituximab for a month.

50 is my new target

It's unlikely I will ever get back to normal platelet numbers, short of spontaneous remission, so although it would be wonderful to have platelets in the 150+ range, that's not a realistic goal.  "It would be lovely if we could get you sitting around the 50 mark," the registrar said. I have been over 50, but I was on a relatively high steroid dose. "39 or even 29 are quite good figures for you."  So 50 is a goal to aim for, but not to be too distressed if I don't make it.

I see the professor in a month, by which time I'll be down to 4mg of pred a day. A milligram a month means it will take three  months to get down to 1mg, and then we'll be reducing carefully by halves and quarters, as that last milligram is the hardest. Three years ago I reduced too fast, my adrenals could not cope with the steroid withdrawal and produce enough cortisol to compensate, and I ended up with adrenal exhaustion.  I'm determined not to let that happen again.

On the bright side

I'm disgustingly well.  I still haven't caught any of the nasty viruses going around, despite people coughing and sneezing on buses and in doctors' waiting rooms. My spring vegetables are growing really well on my balcony, so I'm eating fresh lettuce and rocket and spinach every day.

And there's soccer on the free-to-air tv channel!

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.

Another setback

Last week's blood test result - 46. This week's blood test result,
which I got yesterday was - not 60 or even 50, as I'd modestly
hoped, but - 12. 46 was certainly not as great as I'd hoped, but it
was pretty OK, and definitely out of the danger zone. 12 is back in
scary territory!

In other words, although the steroid pulse my doctor and I did 10
days ago had definitely worked - a jump of 34 - it hadn't held once
I was on 5mg for a week. I'd lost everything I'd gained, while
putting up with all the usual unpleasant side-effects of a relatively
high steroid dose. 

So we're trying the pulse again - this time over  a longer period.  I started on 25mg last night and will take that for three nights, then on to 15 mg until I see my doctor again after next week's blood test. If the results are encouraging, we'll drop down to 10 mg for some days. It will beabout three weeks before we get back to 5mg, and  only if my platelets are much higher than 46.

As well as being under 'house arrest' again for at least until next
week's blood test, I can look forward to three weeks of increasing
discomfort from the horrible steroid side-effects.

Steroid side-effects

If you've never been on high steroid doses this is what I and many
others experience: sudden drops in energy, shakiness, palpitations,
indigestion and belching, pain in the oesophagus that feels like a
knife stuck through me, disrupted sleep - waking up and being
unable to get back to sleep because your mind is spinning with
thoughts. Not exactly fun!

Brain fog

Both my doctor and I hope I won't be on the higher doses for so
long that they affect my adrenals and produce the dreaded brain
fog.

Brain fog is similar to what many people receiving chemo for cancer develop.  Your brain feels full of cottonwool or thick fog; your neural synapses seem sluggish or unwilling to fire. It is debilitating and depressing to be unable to think clearly; often it affects your ability to make sense of what you're reading.

Damage to the adrenals also leads to fatigue. So it's no wonder I'm
feeling a wee bit down this week!

At the moment, my adrenals are coping and thank goodness my
brain is only a tiny bit slower, so I can still work effectively.

Always look on the bright side of life

I've been through all this before, but thankfully not for quite a
while. And each time, I've bounced back and my brain has returned
to action. So far this year I've been remarkably well, and for much
of last year, apart from debilitating migraines in summer. In fact,
the problem seems to be that I'm just too healthy! Even spending
three weeks in Boston in the spring sunshine and coming back to
winter in May didn't cause any health problems, if I ignore my
immune system munching up my platelets faster than my bone
marrow can make them.

And although my doctor telling me the bad news threw me a few
minutes of teary anger, I walked out of her surgery laughing. I said
I was heading to the nearest café for a restorative coffee and how
glad I was that coffee protects the liver against  steroid by-products. Shetold me about a patient going for a coffee enema to  detox,
who was surprised she was buzzing for 48 hours after. Yikes!! I'll
take my coffee orally, thanks!

Wake up call - part 2

Well, the cold was a fizzer - it wasn't a cold but a seasonal allergy,
as I suspected when despite frequent explosive sneezes, my 'cold'
didn't get any worse.  So, the next blood test showed a truly horrific
12.   Luckily for me, despite horribly wet and at times windy
weather, I hadn't fallen over or been knocked down, although I had
a couple of small bruises which I couldn't remember getting, and
my gums weren't bleeding. Time for the big guns, to arrest the slide
before I did get into danger.

We decided on a steroid pulse, a quick in and out to hit the immune
system without upsetting my adrenals. First night it was 50mg -
100 times the dose I'd been on for the past four months. The
second night it was 25 mg. By then I was starting to feel side-
effects: palpitations, indigestion, discomfort in the hiatus hernia in
my oesophagus, inability to get back to sleep after going to the
toilet.  In the morning, before my next blood test, I felt some
shakiness and was a little slower than usual.

That blood test was encouraging. My platelets went up two and a
half times to 30. I saw my doctor the next morning - a day of
flooding rain and very slow traffic - and we dropped the steroids to
15mg, with a maintenance of 10 mg/night until my blood test next
week. By then there's a good chance the platelets will be up north
of 50, and we can breathe easy again.

Back on the Chinese herbs

Although the platelet number was encouraging, my haemoglobin
levels are still low, and so are my sodium levels. I had low sodium
back in January 2010, & I was encouraged to put salt on my food.  I
invested in a salt grinder and some lovely sea salt crystals, while
keeping the iodised powdered salt for cooking, as I need to
maintain good iodine levels. This time, we noticed my sodium levels were down at the same time as the haemoglobin. There might not be a connection, but it's worth looking up. Back then, my
antihypertensive medication was implicated, and since then I've
been put on a daily paracetamol dose to relieve arthritis pain. I
thought that might have contributed to the sodium loss, but the
reverse is the case. There are warnings against too much sodium in paracetamol preparations

To boost the haemoglobin, protect me from the worst of the higher
steroid doses and generally assist my nutritional profile, I'm back
on the Chinese herbs, twice a day for two weeks. I have no idea
what they are - a list of 10 Chinese herb names that meant nothing
to me, as I only know European and Australian herbs.  But they
taste like the ones I had back in March 2010  and are actually quite drinkable, so I think they're the same mix.

On the bright side

The drenching rains of the past two weeks have stopped, and we
have sunshine,blue skies and mild temperatures (18C in the middle
of winter). The snow peas and rocket I sowed a month ago are well
advanced, the everlasting spinach is still going and my geraniums
are still flowering. Being mostly housebound means I have been
reading more and I've finished knitting the cardigan I started three
months ago.  In spite of the ups and downs, life is good!

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!