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.

More Numbers, More Patience!

Another blood test, another visit to my GP, another set of numbers to crunch and digest. And more patience expected of me – never the world’s most patient person.

The bad news is that my platelets seem to be trending down as the steroid dose is reduced. The last four readings have been 153, 177, 149, and this week’s one, 127. While they’re still in the safe zone, above 100 (thousand), they are at the low end of safe, with the pathologist marking the last two readings as ‘mild thrombocytopenia.’

Alien Attack!

Of course, this then begs the question – why is my immune system intent on destroying my thrombocytes, and is there anything – other than destroying the immune system with corticosteroids – we can do to stop it?

It seems fairly likely that a virus infection, possibly the mild dose of ‘swine flu’ I had in October, triggered the immune system to view my thrombocytes as alien invaders to be destroyed quickly and efficiently. If they really were aliens – say, daleks or cybermen – I’d be very proud of my immune system, fighting them off with no sign of The Doctor anywhere close by.

But, wait – these aren’t aliens – these are necessary members of my corporeal community with important roles of their own! Apart from causing my blood to clot at any injury, my platelets also help wounds to heal. I just found out that their name, ‘thrombocytes’, literally means ‘clotting cells’ (Greek: thrombus – clot, cytos – cell).

What we need to do now is to find some way of getting my immune system to recognise that my platelets are on the same side of the war against the real, biological aliens – that they’re friends, not foes.

And in the meantime, keep the numbers up as the steroid dose goes down.

Unhappy Kidneys

I’m still peeing large amounts, but no longer vast cataracts of liquid, thank goodness. So my kidneys are gradually improving, but that funny, almost metallic, taste in my mouth that makes me want to suck peppermints all the time, is apparently a sign of the kidneys being damaged. Probably by the prednisone, but possibly by other lifestyle matters as well, such as my chronic irritable bowel.

The worst part of this, apart from a natural concern about the state of my kidneys, is that the taste in my mouth is affecting my enjoyment of coffee. Suddenly coffee doesn’t taste so good any more!

If my kidneys are still not happy next time I see the doctor, she will prescribe some herbs (Chinese or European, I’m not sure which) to cheer them up. These will probably taste foul, but I won’t know, until my kidneys are functioning properly and I no longer have that strange taste in my mouth.

Still on the Wagon

Apart from celebratory drinks with friends over Christmas, I haven’t touched any alcohol for three months. Not even on my recent birthday! I’m not a heavy drinker now I’m not in my 20s, but I do enjoy a glass or two or red wine with a meal, and/or the occasional whisky. In the summer, I like a Cinzano Rosso or Sec over ice.

Now, remaining teetotal, while not a huge struggle, is an ongoing disappointment. A gentle pleasure denied me by this stupid disease. So I’d hoped that the steroid dose was low enough at 1.5mg that I could be allowed a glass of red wine with my evening meal. I was even prepared to barter my daily mug of coffee for an evening tipple.

Nope! No way! In fact, the barter had to go the other way. My doctor was prepared to allow me my daily coffee (though she’d rather I didn’t) in return for a promise to stay off the demon drink. I was tempted to quote St Paul’s advice to her: “take a little wine for thy stomach’s sake” , but I don’t think she’d have been amused. (Besides, modern translations probably say 'non-alcoholic wine', and what’s the point of that?)

It seems my poor body is not strong enough yet for strong liquor – not even an environmentally friendly and very smooth blend of South Australian shiraz and merlot.

Weaning Ever So Slowly

What with the kidneys, and the headaches I've had off and on, and the lethargy/feeling like a lump of lead, or the days when I can't stay awake, it seems I'm still cutting down the steroids a touch too fast. So instead of going from 1.5mg to 1mg this week, I have to take a smaller jump - more of a hop, really - to 1.25mg. I'll need a pill cutter for this, as the 1mg tablets are hard enough to break in half, let alone quarters.

This extra step adds at least another week to the 'getting off the bloody steroids' timetable. Maybe by the middle of March?

Now to the good news

Yes, there is good news, despite all my whingeing. While my weight remains the same, there are more days when I can get into my baggy green pants, and even occasionally squeeze into the stretch jeans (hiding my muffin top under a loose shirt or smock). Various swollen bits are going down – my bras almost fit, my face is a bit less pumpkin-like, and yesterday’s great achievement: I could get my feet into a real pair of shoes again!

The hot, steamy, tropical weather has ended too, with the start of early autumn. The humidity has gone, there is less bite in the sun, but it’s still gorgeous weather. Though this has no connection with weaning off the steroids, or being good about staying off the booze, it has certainly improved my psychological and physical ability to deal with both of those!

Ups and Downs on the Steroid Rollercoaster

Today I had my second session with my new ‘wonder worker’ GP. I reported how I’m dropping the steroid dose (1 mg every 2 days) and how much better I've been feeling. In particular, that my brain is a lot clearer, and my energy levels higher, though neither of them as good as they were before November 11 (ITP day).

While she was delighted with my progress, the good dr brought me back down to earth, warning me that the last few milligrams - in particular the last milligram - of prednisone is the hardest to wean the body off, and it might take some days of taking halves or even quarters of that last 1 mg tablet.

As she pointed out, when I did the steep drop from 20 mg to 15mg, I was reducing the dose by 25%. Last week I went from 13 mg to 12 mg (7%), and this week from 8mg to 7mg (12.5%). Next week I’ll be going down to 4mg, with the drop from 5 to 4 being a whole 20%.

But when it comes down from 2 mg to 1mg, that’s a BIG drop of 50%. She warned me I might have some really nasty experiences dealing with such a proportionately steep drop - even the possibility of depression again – as well as palpitations etc.

But hey, that’s two weeks, and two blood tests away!

Oh, What a Tangled Web…

No, I haven’t been deceitful – I’m referring to the immensely complex and convoluted physiological web that is the human body, especially when you add in various pharmaceuticals.

One of the unexpected side-effects of all this intervention has been the sudden oedema (swelling) of my legs. In particular the left leg, which swells up gradually during the day & never fully subsides during sleep. It becomes very tight and shiny and quite hot, & I cannot put the fat foot in any shoes other than my loose plastic clogs, or my very old gym shoes, just barely laced together.

This is partly a response to my too enthusiastic adoption of salt tablets to correct my sodium imbalance (even though I’m taking less than the minimum three the label suggest), combined with my own (unadvised) halving of my daily dose of Karvea, to reduce the enormous volume of pee each night. Wrong, wrong, wrong!! Apparently I need to keep peeing vast cataracts every night, to drain out all the fluid I’m storing in my face and now my legs.

Joint Pains, but Not from the Steroids

As part of this week’s general discomfort, along with the return of nightly leg cramps (though not as severe as before), I’ve also been experiencing pain in all my arthritic joints. And in my neck, shoulders and ribs. For these, I can blame the steroids, but only indirectly.

The problem is I’m cattywampus, and my joints are all complaining. ‘Cattywumpus’ is a lovely word I’ve learned from my daughter’s American friends. It means askew, off beam, ain't quite right, what I’d call ‘skew whiff’.

Normally I’d be having a massage about every 6 weeks, and seeing my osteopath every 3 or 4 months for an adjustment, as well as doing all my appropriate exercises. But a lack of income, combined with one of the few positives of steroid treatment – masking of joint pain – meant I’d ignored my skeletal needs. Now the steroid dose has dropped low enough for my complaining joints to get my attention.

Did Someone Mention Vitamin D?

When my other GP told me some weeks ago that I was low in Vitamin D, I did the right thing. I gave away my sunscreen to a friend whose pale skin burns at the mere thought of sunshine, and bought a calcium supplement fortified with Vitamin D.

Of course, once I started on the magnesium supplement to counteract the leg cramps, I had to stop the calcium, as the two chemicals compete with one another for the same sites on the cell membranes, so it’s best not to take them at the same time. Being concerned about my thin bones, I questioned Dr ND today about calcium and Vitamin D and how I was to protect myself from osteoporosis.

There followed a convoluted biochemical lecture on the types of Vitamin D in the body – stored and activated – which I confess I didn’t follow completely, and will need to read the literature on, before I get my head around it, let alone explain it to anyone else.

As far as I can gather, I have too much of the activated type, and I don’t need to be trying to store any more of the other one (from supplements and/or sunshine). While the thin bones are a concern, it seem we have to put then to one side until we have completed the arduous task of weaning me off the prednisone, and getting the steroid residue out of my system.

Looking On The Bright Side

Yes, there are bright spots from today’s visit. I’m doing better than expected in dropping the dose down without too many problems. I do have more energy and brain power, even if my strength and endurance are still way below what I’d like.

And best of all, despite the swollen legs and feet, and still swollen face, my belly fat is going down!! Today I was able to squeeze into my wide-legged khaki-green linen pants – the same pants that last summer were too loose and needed a belt to stay up, and only a month ago would not go near me.

I may not be able to get my shoes on, but I can wear my favourite summer pants again. Yay!

A Life Lesson Finds Me Grumpy

This week I’ve been faced with learning another of life’s lessons, and for all I ‘m trying to be positive, it’s left me grumpy and feeling sorry for myself.

A few days ago, I went to my local hairdresser to get my hair cut and to chat with the pretty girl who does it about options for colour, as the reddish purple I‘ve been using lately fades too fast in Australia’s strong sunshine. It also doesn’t suit my red, swollen pumpkin face.

This girl, Anna, has long blonde hair, and looks like an archetypal Disney cartoon heroine.

When I first met her, four months ago, she was lively and animated. This day, she looked tired. Knowing that she had, some time in the recent past, also been on steroids, I was complaining about how I hated them - how fat I am, how I barely have anything that fits me, how I hate my puffed up face and the lack of sleep, and the loss of energy, etc, etc.

Then she told me she was living with something like chronic fatigue, and that she frequently has days when an hour after getting up, she needs to lie down again, because she's exhausted. She had been working full-time at the hairdresser's, but has had to cut her hours back, as she's not strong enough, and has been advised to try to rest.

"I’m only 25, I don't want to be resting!", she said. Poor kid! I felt so selfish & egotistical! I vowed to stop complaining right there and then.

One Good Day, Two Bad Ones

But it’s not that simple, and I figure I can complain here, even if I keep my mouth shut in public. I know I’m not as sick as Anna, but there are far too many days when these blasted steroids make me feel as if I am! If I have one good day, in which I feel almost normal (as long as I don’t catch sight of my face in the mirror), it’s almost inevitable that the next, and often the one after that, will be spent lying on the couch, barely able to move or think.

For example, yesterday morning I went for a gentle walk before breakfast. I went to the big local park, usually an easy 10 minute walk for me. I didn’t push myself, but enjoyed stretching my legs and breathing the fresh air, and got to the duck pond in 20 minutes.

Then I sat for about 20 minutes, watching the birds, looking at reflections in the water, loving the trees around me, enjoying the sight of people walking their dogs. I walked slowly home, sitting at a bus stop halfway along for another rest break. I was home about an hour after I’d left and I was feeling good!

After breakfast, I took my steroid dose, and promptly lost one and a half hours to the shakes, palpitations and energy drop. I lay on the couch, wrapped in a sweater, and dozed.

About mid-morning, I felt it lift a bit, and went into my office to attempt some freelance writing. I managed about two hours, slowly, of writing and reading research, but by lunchtime knew I had to give up. The afternoon was spent on the couch - and there was no cricket to watch, the only daytime tv I like! My eyes were so blurry I couldn’t read, but I managed a little knitting, in between shivering and dozing.

Today is a little better. My brain is working at half-speed, so I can just manage this blog, but my energy is barely above couch level. And my positive attitude is having difficulty maintaining altitude.

I know I will get better. I know I will be off the steroids in another six weeks or so. If I’m lucky, the ITP will truly be idiopathic and never return, though it’s far more likely it will recur on occasion, necessitating some use of the steroids in the future.

Sadly, I also know from what Anna told me, that it can take at least two years after stopping before the steroid effects wear off completely and I resume my normal face and body. If ever!

Always Look on the Bright Side of Life

Ever since 'The Life of Brian', I’ve tried to live by that motto, as I’ve faced many of the life crises other women my generation experience – marriage breakdown, retrenchment, severely reduced income, some truly nasty things in my past needing to be acknowledged and dealt with.

Why should this health problem be any different? So, I’m going to sing along with Brian!

Pumpkin face

I’ve reached the end of my first week of learning to live with ITP, doing my research and observing and experiencing the side-effects of the steroids, as I continue to try to run my small business.

Firstly the good news. My platelet count is currently 169,000, and my haematologist has cut my oral prednisone back to 40mg for this week, with permission to drop it to 30mg next week, until I see him again in a fortnight.

The bad news – well, it isn’t really bad, just annoying – is that, while I’m experiencing many of the common side-effects many people suffer when taking oral steroids, I’m not enjoying the psychological benefits.

When I mention I’m on steroids, people say to me ”Oh, you’ll have lots of energy and a sense of euphoria!” I wish!

I actually have less energy – not a major drop, but enough that I notice it – and while my mood is positive, there’s no way I’d describe it as euphoric.

I know I’m benefiting physiologically – my platelet count is rising, and I’m no longer at risk of major internal bleeding. Believe me, I’m thankful there is medication I can take that is effective!

Annoying Side-Effects of Prednisone

These are the side-effects I’m experiencing. They may differ for other people.

• The swollen “pumpkin face” or “moon face”, which can get quite hot and tight

• Shakiness, especially 1 hour after taking my daily dose, but also at intervals during the day

• Reduced energy levels

• Occasional palpitations or tachycardia, especially at night

• Disturbed sleep – finding it hard to get to sleep, or to go back to sleep after waking in the night

• Irritation of my hiatus hernia below my oesophagus, generally in the evening, but occasionally during the day

• The brain stops working, briefly, mid-sentence, or mid-thought.

Consequences of Long-Term Oral Steroid Use

There are also some concerns about the effects on the body of long-term use of oral steroids. If I do have chronic ITP, then it’s likely that once every year or so, I might have to have a couple of months of steroid medication.

These consequences can include:

• Some shrinking of the adrenal glands as their burden of producing cortisone has been relieved. This is managed by reducing the steroid dose slowly. To allow the glands to take over again.

• Increased susceptibility to infections, as the cortisone is damping down the immune system

• Muscle weakness

• Osteoporosis from reduced calcium absorption (here’s some bone density info from MedSafe)

• Aggravation of existing conditions such as diabetes, glaucoma, and high blood pressure

• Increased cholesterol and triglyceride levels in the blood

• Weight gain and uneven distribution of weight, so a swollen face and belly with thin arms and legs

• In some cases, the skin becomes fragile, leading to bruising and even tearing

• Psychological side effects include irritability, agitation, euphoria or depression and insomnia.

So the challenge will be to get the very real benefits of the prednisone, while minimising or blocking the equally real dangers. Like much of life, it’s going to be a balancing act!

TCM – Traditional Chinese Medicine

I have been sent a clinical article on the use of Traditional Chinese Medicine in treating ITP.

It’s very interesting reading, but with my western science background, the concepts expressed are a little strange. Not entirely unfamiliar to me, as I have interviewed veterinary practitioners of TCM and other alternative therapies, but just not in my immediate conceptual comfort zone!

However, TCM herbal therapy seems like a real possibility in the strengthening of my body to deal with the long-term steroid effects, so I plan to investigate it further over next few weeks.