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!

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.

It’s good news week!

This month saw me reach my anniversary – 12 months since that strange and frightening day when I was diagnosed with ITP and bundled off to the emergency department of Sydney’s wonderful Royal Prince Alfred Hospital. By coincidence, I had my regular fortnightly check-up booked for the very day. And I was NOT looking forward to my anniversary!

Turns out this was good news day, not the doomsday I’d been dreading.

No, I haven’t suddenly been cured. No, neither my doctor nor the researchers studying ITP are any closer to discovering what causes it. And yes, my pituitary gland is still “screwed up” (to quote my doctor), and likely to stay that way, to a lessening degree, for the foreseeable future. Yes, the steroids are still threatening to tip me over into Type 2 diabetes if I don’t watch my diet very carefully, and they’re thinning my bones and my skin.

BUT, the good things definitely outweigh the bad.

On the bright side

I'm feeling remarkably well and most days have reasonable levels of energy. My platelets are sitting in their usual position around 50, but they're not causing any obvious problems. My blood pressure and haemoglobin level are fine, and the horrible gluggy cholestyramine is doing an excellent job of keeping my cholesterol down and fixing my kidney function, so my kidneys are no longer struggling.

Best of all, because I have been sticking to the no carbs diet, I am no longer insulin resistant. So, as long as I keep on keeping off carbohydrates and sugar, and get enough regular exercise, I should be able to keep the dreaded T2 diabetes at bay.

Regular exercise important for people with chronic illness.

I know regular exercise is important for good health, but the problem for people with chronic illnesses is if you have a bad spell (like my recent gastric flu episode), you very quickly lose condition. It becomes hard to do the things you could do when you were healthy, like walk up hills, climb stairs, carry grocery bags, or wash the floors.

Once again I have my local health service to thank for the opportunities to build up my fitness during this period of comparatively good health. I’ve been accepted into the RPA’s rehab gym, which is mainly for patients with lung and cardiac conditions. I started there this week, doing a little bit of gentle cardio on the treadmill and exercise bike, and some even gentler resistance training for my arms and upper body.

When, in a few months’ time, I get too fit to qualify for this gym, there is another section of the health service that offers continuing gym membership, supervised by physiotherapists, for people with chronic conditions. This service is not free, but is well worth the small monthly fee to continue to maintain and improve physical fitness in managing chronic illnesses.

Reasons to be cheerful

Apart from all the good news from my doctor and the pleasure of doing a little targeted exercise, I have learned to love my diet. Why? Because, not only am I losing lots of weight, but I now have to eat foods that will maintain my weight, and prevent me getting too thin.

Because I must avoid carbohydrates as much as possible, within reason, to defeat the steroid tendency towards insulin resistance, I need to replace the energy I would have got from eating bread, rice, pasta, porridge, cake, etc. And that means eating more fat.

My doctor has virtually ordered me to eat more cream, cheese, butter, bacon, even chocolate, alongside the serves of protein, vegetables and fruit. As she said: “You can have a lot of fun on a no-carb diet!”


It won’t be forever. When I finally get off the steroids, I will be allowed a more ‘normal’ level of carbs in my diet, and once more I’ll have to think very carefully about eating cream, butter, bacon or chocolate. But until then, boy, am I gonna enjoy my no carb regime!

More Thoughts on ITP

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

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

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

New Hypotheses on the Pathology of ITP

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

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

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

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

Eradicating H pylori Infection Lifts Platelet Numbers

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

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

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

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

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

On the Way Up, I Think

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

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

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

Meditation and Staying in the Now

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

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

Down and (hopefully) Up Again!

Last week’s blood test was a shocker – my platelet count was down to 33, with the pathologist flagging it as ‘marked thrombocytopenia’. Yikes!!

I feared they were falling from the previous reading of 50, as my gums had started bleeding when I clean my teeth, and I had one possibly unexplained bruise.

So my doctor had a little chat with me – and pushed the box of tissues over as my tears started to flow. This is serious stuff. Whether I like it or not (definitely not), I am seriously ill, and if those platelets don’t start climbing again, I could end up back in hospital.

So, despite my wishing it otherwise, we have increased the steroid dose to 5mg a day. I tried to bargain for 3mg, but was over-ridden. “Better to overshoot and be able to cut back later than to undershoot and have you back in hospital,” she said. I know when to give in gracefully.

Fortunately the Chinese herbs are having positive effects on other aspects of my blood chemistry. While my white blood cells (the neutrophils in particular) are still low, they are back over the danger line. My haemoglobin count has also improved, and is almost out of the anaemia trough.

Because this herbal mixture was giving me unwelcome bloating and some diarrhoea, it’s been tweaked to remove the offending herb. I’ve also found the mix is easier to take if it's made like a cup of tea – ie- diluted twice as much (not halving the dose but adding more hot water).

Food for Thought

My diet has been modified even further, mainly because of my muddle-headedness (or wishful thinking). When my doctor told me to cut out all raw fruit and vegetables and sugar, and eat only warm, cooked food, I thought she also said “and maintain a completely wheat-free diet.” Hence my discovery that eating porridge with salt is much more pleasant than having it laden with golden syrup or brown sugar.

But of course that’s not what she meant, and she said it more clearly this time. “Maintain a completely gluten-free diet.” Umm, that makes life more difficult, especially first thing in the morning. I have trained my body to need breakfast, the most important meal of the day. However, I am always dopey for the first hour or so of the morning, even without the steroid/exhaustion-induced random hedgehog munching my brain, but I can more or less make porridge in my sleep. Plus oatmeal is good for controlling high levels of LDL cholesterol.

Now breakfast will have to be (a) left-overs from the night before – (can I face them?); (b) home-made savoury muffins reheated in the microwave; or (c) toast made from disgustingly bland and crumbly gluten-free bread.

Still Tired and Weepy

It’s been a long hard week, and I have serious decisions to make about my future in terms of how I survive financially when I’m in no state to work more than a few hours a week. Not only does my body feel exhausted, but my brain too – what my doctor calls “brain fag”. This is a problem common to many people faced with a severe illness that becomes chronic, and financial stress can make it harder to relax and recover.

I am sick and tired of being sick and tired! How many chronically ill people must echo my cry?

I know that with my doctor’s help I will a reach a stage where my ITP is managed; when the factors destroying my platelets are balanced by factors that support them without damaging my immune system too much.

I know that when that point is reached I will have much better levels of energy and a more regularly functioning brain – instead of the short bursts of both I have at the moment. And then life will return to something more like normal. But that’s a way down the track, and I have to face the short-term challenges created by a much reduced income.

Always Look on the Bright Side…

This week’s bright side: the higher dose of steroids has blocked out the pain from my arthritic joints and tight neck after only two days. Marvellous!

Also marvellous are my friends, who have rallied round me with empathy and useful good advice (not all good advice is useful). Thank you. You know who you are.

Going down!

Two more blood tests – now weekly - and it’s clear that as the steroid dose goes down, so alas, do my platelets. The pathologist is now marking my thrombocytopenia as ‘moderate’, with the latest reading at 53 (thousand). My haemoglobin count has gone down to 112 and ‘mild anaemia’, and my white cells are also disappearing too, though only just below the safety zone of 4.

So – it’s not a one-off idiopathic event, but as I feared all along, it’s ongoing and will need careful management.

At this stage, my doctor and I are experimenting – within clear clinical guidelines – to see how my body will respond to some Chinese herbs to boost my blood cells and support my spleen.
I now have a mix of 10 powdered herbs – zhi huang qi, bai shao-sheng, dang gui, dang shen, bai zhu-chao, shu di, chuan xiong, rou gui, fu ling and zhi gan cao – to take in hot water twice a day. They initially tasted pretty awful, but I’ve found by diluting them more than the instructions said, they not only taste bearable, but are less likely to give me diarrhoea!

I don’t know what the western or common names for these herbs are, but there is certainly liquorice in the mix! This makes the herbal ‘tea’ almost enjoyable once I worked out a dilution that meant I could mostly only taste the liquorice.

We have also stopped weaning me off the steroids, so I’m sitting on 1mg a day until I see the doctor in a week’s time, when we might have to bump it back up to 2 mg.

Let your food be your medicine

As well as the Chinese herbs, I’m on a strict diet to avoid stressing my body too much. I’ve been eating what I believed to be a healthy diet of green salads every lunchtime, occasional mid-morning or mid-afternoon snacks of raw celery and carrot sticks, as well as apples and cantaloupe pieces. I’ve never been all that fond of raw veggies, and apples are the main fruit I like, but I knew that salads and fresh fruit were healthy, so I persisted.

Well, seems I was wrong, again. At least for now. I’ve been told to only have well cooked vegetables and stewed fruit, as it’s draining energy from my body trying to digest the raw produce. That’s actually what I prefer, and how I used to consume my fruit and veg, until I decided to get really healthy!

The harder part of the diet to follow is the restriction on sugar. Sugar reduces the effectiveness of the immune system, by deactivating the white blood cells, specifically the neutrophils which attack bacteria such as Staphylococcus aureus and Escherichia coli. Since I have a lowered white cell count anyway, it would be foolish to diminish their fighting powers.

So no sugar in coffee, no cakes or biscuits, no honey or marmalade on my toast, and no sugar on my morning porridge. It would actually be a total prohibition, but stewed fruit would taste pretty awful without a tiny amount of sweetness (and I refuse to eat chemical artificial sweeteners)! Porridge tastes surprisingly pleasant without sugar and with just a tiny amount of salt.

Still tired, & now weepy

It’s depressing at times to find how easily I tire, and how little energy reserves I have. A walk to the park with a stroll around the duck pond, and then home again, can still knock me out for a couple of hours afterwards.

My brain feels like it has a hole in it, where the neurons have frayed apart, so I stop suddenly, mid-sentence, waiting for a neuron to bridge the gap and bring the word or thought back.

I have days when I’m shaky and a little weepy – some of which can be blamed on the steroid reduction, and some of it on the generally distressing situation.

On the plus side

On the bright side, my kidneys are almost back to normal – coffee tastes good again, yay!! My cholesterol count is rapidly dropping back to a safe level.

And, although I don’t know what I weigh this week, I can squeeze into more clothes and shoes, so I know the weight and swelling are going down somewhat. Still have the pumpkin face, but I have a sharp new haircut and a vivid purple hair.