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!

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!

Life is a Chronic Illness

A friend was recently diagnosed with breast cancer for the second time. Although her lump, which was pre-cancerous, was successfully removed, she has to have radiation therapy as a precaution, to reduce the likelihood of the cancer returning in a more aggressive form. Her oncologist told her “Breast cancer is no longer a death sentence. Breast cancer is a chronic illness.” I was there to support my friend, and it occurred to me that life is a chronic illness. While not immediately life-threatening (if that's not n oxymoron), it does kill us in the end. The trick is learning to live with this chronic condition, to take each day as it comes and make the most of it, not knowing when the end will come.

Now, I know this thought isn’t original. I’m not sure which philosopher put it into words first – probably not Marcus Aurelius, who I'm currently re-reading, although he had some helpful things to say. Do not act as if thou wert going to live ten thousand years. Death hangs over thee. While thou livest, while it is in thy power, be good. (From Meditations.) So why am I meditating on life and death? Because I’ve hit another little bad patch in my journey, and I need to remind myself that being ill is not the be-all and end-all of my life.

Low energy, shakiness, weepiness – so what’s new?

The most annoying thing about this chronic condition is the way a relapse takes me unawares. It’s a pain! I can go along feeling fine, full of energy, brain working almost as well as it always has (maybe a little slower, if I’m honest), and wham, suddenly I have a day when I’m feeling shaky, weepy, and an intense desire to lie on couch and feel sorry for myself.

The reason is I have been ignoring the little warning signs that this is creeping up on me. At some level, I have been believing that I well. Fixed. Cured. 100 per cent better. And behaving like I’m as fit or fitter than I was at 25. Exercising at the gym 3 or 4 times a week. Working hard editing, writing. Going out with friends. Filling every day with activity. When, to be honest again, I should know – and accept ­– that at best I’m only ever going to be 90 per cent or 80 per cent, and I can’t behave like I have the drive and energy of my younger and healthier self.

Low iodine and slow adrenals

In my last post, I mentioned the possibility of mild hypothyroidism. My most recent blood test reinforced that possibility. My blood iodine levels were mid-range at 48 – moderate iodine deficiency, so now I’m taking one drop of iodine tincture in a glass of water once a day.

Iodine is one of the micronutrients the body requires to produce the thyroid hormones. Although it is found in fish and seafood, the best natural sources of iodine are seaweed and kelp, which not many people eat, except as wrappers for sushi rolls. Japanese people, whose diet is high in seafoods and seaweeds, seldom suffer from iodine deficiencies, but many Australians unknowingly have a mild lack, and this is a concern with maternal and infant health. Other sources are eggs, yoghurt, milk, strawberries and some cheeses, but their iodine is dependent on the soil in which the grass or the crops was grown.

Worldwide, 139 countries have soil deficient in or entirely lacking iodine. Australia is one of them – particularly Tasmania, where I spent my childhood. Severe iodine deficiency, especially in pregnant and lactating women, causes horrific brain development problems in the foetus and young babies. Growing up in the 1950s, I saw many cases of cretinism and goitre, terrifying to a small child. By 1960, the cause had been discovered and we were given iodine supplements. The best sources of iodine in Australia are actually bread and salt, which have iodine added. (Milk used to be a good source, when iodates were used as part of the sterilising process in dairies, but this no longer happens.)

Adrenal exhaustion – not again!

Eighteen months ago, I was totally weak, limp and useless, as I was suffering from adrenal exhaustion. I was afraid this latest set-back would be the same, but I was over-reacting. Sure, I have a bit of adrenal fatigue, thanks to the low iodine, slight anaemia, having migraines on hot and humid days, and just doing too much, but I am 75/75 – ie – 75 percent well, 75 percent of the time. I just have to get it through my stubborn mind that I MUST rest more and do less, and keep taking my daily supplements of B12, folinic acid, vitamin C, iodine, and the Chinese herb, cordyceps. Find a balance between action and repose, to make the most of my chronic life.

Reasons to be cheerful

• Music on my laptop, for when I have to lie on my couch.
• Poetry cds, poems read by their British or American authors for couch time
• Good books and a local library that gets all the latest ones
• Walking through the Botanic Gardens or my favourite parks when I have the energy
• Work that uses my brain and provides disposable income to buy concert tickets, books, cds, occasional new clothes,
• Time with friends and family – either in the flesh or on Skype

Poor genes, poor health – but not poor me!

I’m approaching the 2nd anniversary of my diagnosis with ITP. I feel stronger and happier than I did this time last year, but it’s been a strange journey, and along the way there have been far more questions than answers!

Every three weeks I have a blood test, and my GP and I analyse the data and come up with a new or extended hypothesis of the underlying causes of my low platelet count and my prognosis over the next few weeks. As well as the all-important platelet count, we have a cycle of other markers we monitor – cortisol, haemoglobin, red cells, white cells, lymphocytes, monocytes, basophils, eosinophils, calcium, vitamin D and vitamin B12, plus a string of blood chemicals and hormones identified only by initials – most of which I only have the very vaguest understanding of what they do.

No simple answer for thrombocytopenia

Two years on and the one thing that is clear is that there is no simple, straightforward answer as to why I have thrombocytopenia. Why my platelets bounce up and down, and struggle to reach 100. Why 50 or 60 is an OK level for me, though it would be a tremendous psychological boost if they would sit on 100 for while.

On 50, or better still, 60, I function pretty well most days, with maybe one dodgy day a week or 10 days. By ‘dodgy’ I mean slow moving in the morning, or shaky for the first couple of hours, so my bad days are nowhere near as horrible and debilitating as they have been, and are, currently, less frequent.

Over the months, we’ve looked at various possible causes or contributing factors, such as H pylori ,gut parasites, and low-grade Lyme disease. Plus we have identified some of my genetic deficiencies – being gluten-intolerant and also unable to metabolise folate from food. Now we have another one – low level hypothyroidism.

Hypothyroidism a possible villain?

In the latest blood test, we checked my Vitamin B12 level, something we do every three months, together with homocysteine. As usual, the B12 was lowish – still within the very broad range of 145-637, but this time right at the bottom. And the homocysteine was way up, almost double what it should be. Both the high homocysteine levels and the inability to metabolise folate are linked to the MFTHR gene and can occur together with low levels of thyroid hormones, and could indicate very borderline hypothyroidism.

That my presumed hypothyroidism is very marginal is shown by the fact that I exhibit none of its most noticeable symptoms: tendency to gain weight and inability to lose it; slow thinking; thin hair and hair loss; puffy eyes; oily skin; and other more delicate comparisons – such as constipation vs irritable bowel.

What it appears I have is ‘secondary hypothyroidism’, thanks to the last year’s adrenal fatigue and my ongoing adrenal insufficiency. So possibly the borderline hypothyroidism is not so much a cause as what the medicos call a co-morbidity – connected, but not necessarily cause and effect.

Chronic condition from long-term problems

To sum it up, here’s my equation:
a lifetime of less than optimal health (as a result of choosing my parents badly), compounded by environmental factors* + an unknown viral or environmental stress in 2009 = ITP.

Not a clear cause and effect, or diagnosis and cure, but good enough to be going on with, now that what was life-threatening two years ago is pretty much under control.

Reasons to be cheerful

• My weight is pretty steady at an appropriate weight for height and build: 60kg to my 160cms and thin bones;
• I have good energy (most days)
• I enjoy going to the gym 2 or 3 times a week
• I have enough freelance work that I can manage
• I love doing coffee or going to a play, concert, discussion group or art gallery with family and friends
• I have time to write my short stories
• The jacarandas are flowering
• I feel good!

* Tasmania, my home state, has very low iodine levels in the soil, causing many people to develop gross hypothyroidism and goitres. This connection was not recognised until I was about 10, so I would not have had an optimal iodine intake during my early growing years.