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!

I'm Still Here!

Earlier this week I received an email from John in Los Angeles. He had been recently diagnosed with ITP, and searching the web for information on his condition, found this blog. He told me he was grateful to have a patient’s view of the illness and how it has affected me. John, if you’re reading this, thank you for spurring me to write another update. Just remember, though, we are all individuals, metabolically as well in our personalities, so what works or doesn’t work for me, may or may not affect you the same way. But at least you’ll get some idea of the dimensions of this ‘orphan condition’ that so few people know about.

Quick Look Back

Four months ago when I last posted in, I was feeling a little sorry for myself thanks to a slight recurrence of adrenal fatigue, and the diagnosis of mild hypothyroidism. As if I didn’t have enough, what with arthritis, osteopenia (thanks to small bones and the weakening effect of prednisone on them), and eczema, aggravated by, but not merely caused by gluten-containing foods. So, I was having to remember to be strict about what I eat and drink, remember to take my iodine, Vitamin B12, folinic acid cordyceps, slow release Vitamin C, magnesium (to counter muscle cramps), plus my normal ‘seniors’ medication to manage my high blood pressure and cholesterol levels and strengthen my bones No wonder I was feeling a bit sorry for myself!

Since then, I’ve had an operation on one eye to remove a growth on the back of the retina, and tomorrow I see the optometrist to see how much my vision has improved, and hopefully, to choose new glasses. I thought the eye op, which involved a stay in hospital overnight, would bump up my platelet count. Anything that occupies my immune system usually gives my platelets a rest from being munched, but no! For three months I cruised between 60 and 50, as we cautiously dropped the steroid dose by 0.25mg. Then last month – bump – down to 44. Not really alarm bells (my ‘rush to hospital’ figure is 20), but enough to scare me.

Thankfully, I caught one of the nasty winter colds going round (even more thankfully, it wasn’t the flu), so last week my platelets were a magnificent 92, my PB for this year! How long they’ll stay up there is anybody’s guess, but it felt like I won gold!

Thin bones getting thinner

Dem bones, dem bones, dem thin bones: I had a two year bone density last week, and only today did I have the courage to read the report before I take it to my doctor. As I feared, two years on from my last test, and two and a half years of taking steroids, my bones are getting more fragile. I have a marked risk of spinal fracture and a moderate risk of fracturing the left femur. Not fun!

On the other hand, I’ve only been taking the Fosamax (alendronate sodium monohydrate) for six months, and I understand it takes a few months before there’s enough in my system to have an effect. And, on the plus side, I’m still going to the gym regularly doing resistance training, which builds up bone strength as well as muscles, and last week my trainer graduated me from the small gentle hydraulic leg press to the big one with real weights.

Feeling pretty good

Despite all this and the messy cold, I’ve been feeling pretty good for the past month or so. I have enough energy, most days anyhow. I walk, I spend time with friends, go to art galleries, concerts with them. I joined a creative writing class which has been real stimulus for my brain, socialising with other writers and learning from a great poet. Some of my writing has been recognised, with a short story being published in an anthology, Between the Sheets and a poem accepted for a university literary magazine, to be launched at a writer’s festival next month.

I think too, that I’m finally learning something about acceptance and gratitude. Certainly gratitude. I’m far more conscious of feeling that for simple things, even for having a good day, in a way that I didn’t before this illness struck. And most days I accept that on a good day I’m 80/80 (80 percent well, 80 percent of the time), but that I will have days when I’m only 50/50.

Reasons to be grateful

All of the above, plus

· glorious sunny winter days in beautiful Sydney (top pic)

· the port wine magnolia flowering outside my balcony (left)

Let's hear your story!

I’d really like it if other people reading this blog would comment on how ITP has affected them, what works, what doesn’t work, how they manage, and above all, how they keep cheerful. Let’s hear from others in our special little group!

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!

Hail Coffee! Saviour of my Day

I’ve been feeling rather low and grumpy today, thanks to next to no sleep last night – new side-effect, muscle cramps in feet and ankles keeping me awake – and very low levels of energy and brain power. Plus my credit card got eaten up by an ATM because I typed in my PIN wrongly!

Craving a coffee to wake my brain, I thought I’d better check out the bad news on coffee drinking and liver function. Drinking alcohol stresses the liver (not that I’ve had any for over three weeks); guaranteed coffee will do the same, right? Wrong! I found a Medscape article describing various recent clinical studies that show drinking up to four cups of coffee a day can actually improve liver function!

This is great news, as yet another of prednisone’s annoying, possibly dangerous, side-effects is the risk of liver inflammation, which can be recognised by an increase in the liver enzymes alanine aminotransferase (ALT) and aspartate aminotransferase (AST).

According to this information on liver function tests, ALT and AST are enzymes located in liver cells that leak out and make their way into the general circulation when liver cells are injured. The ALT is thought to be a more specific indicator of liver inflammation, since the AST may be elevated in diseases of other organs such as the heart or muscle. ALT and AST are often used to monitor the course of chronic hepatitis and the response to treatments, such as prednisone and interferon.

As well as other liver enzymes, the study by Tanaka et al quoted in the Medscape article also investigated the potential relationship between coffee consumption and ALT and AST. Once again, coffee intake was significantly related to decreased serum concentrations of both enzymes. While it’s not yet known which compound is doing the good work, something in coffee is protecting the liver cells.

Skinny bones and Not Enough Vitamin D

As well as being short – 5 foot 3 inches “in the old language”, I have very fine bones. I used to think they were elegant, and a sign of noble breeding, blue blood(!). Now, I look at them as fragile, and potentially hazardous. I already have a history of broken toes, and a family history of osteoarthritis and osteoporosis among my older female relatives.

Knowing that the steroids can lead to bone thinning and reduced calcium absorption, I discussed calcium supplementation with my GP. She sent me off for another blood test – this time to check my Vitamin D levels. Another curse of being a woman over 60 is that we can’t make as much Vitamin D through the action of sun on our skin as we used to, no matter how much of a lizard I am, and how rarely I wear sunscreen or long sleeves.

Vitamin D is converted from cholesterol in the blood by sunlight and helps increase calcium absorption in the intestine, which builds stronger bones. Australians should be able to receive about 90 per cent of their intake from sunlight production. Experts are now warning older people not to do so much of the ‘slip, slop, slap’, sun protection routine of sunscreen, shirts and hats.

I’m not sure if it’s just us older women who lose the Vitamin D making ability or older men as well, but anecdotally, more women suffer hip fractures from osteoporosis than men.

So, the blood Vitamin D results are back, and yes, I have only 50 per cent of the serum levels that I should have. I’m now taking a calcium supplement fortified with Vitamin D.

Chinese Herbal Medicines

I’ve made contact with a local GP who practises both western medicine and TCM and has treated at least one other patient with ITP. Unfortunately, she can’t see me for another couple of weeks.
I’m hoping to be put on a course of TCM herbal remedies to help strengthen the liver, increase absorption of calcium from my diet, improve my Vitamin D making abilities, stop the muscle cramps, shrink my face, unblur my eyes and give me a good night’s sleep.

In the meantime, I’m off to make myself a pot of coffee!