April 2020 – Welcome back! Skin GVHD flares up!

In my last ever Post of February I had mentally moved through the 2 year post transplant tests and had believed that I had fully recovered from Leukaemia and things were moving forward well. I was in a very happy space and there would be nothing more to share – how wrong I was to be! Here’s an update…….

A Leukaemia patient should not be surprised when the disease throws you a curved ball.

For me that is now Sclerodermatous Skin GVHD (bascially my immune system (well my brothers if you must know! – attacking the soft tissues in my skin believing it is ‘foreign’).  I had recalled talking to Hugh Williams (brother of Ross, ex Wagga, now St Helens, Tas) about his Leukaemia treatment not long after I had started to recover.  Hugh is two years further advanced than me in his treatment and his main complaint was GVHD of the skin – a condition that affected his skin severely with a tightening and restriction over many areas of his body.  His way of dealing with this (apart from the drug regime) was to spend up to 2 hours each day with stretches and exercise for relief.  At that stage it was not on my radar.

Over the past two years I had often made mention of sore muscles and bones.  And a year ago I had a nasty outbreak of Lichen Planus on my hands.  As it turns out this was a pre-cursor to the Skin Sclerodermatous GVHD which has manifested itself as a nasty tightening of my skin, with scar tissue forming just under my skin layer across a large portion of my body.

The skin hardens up around your body and looks like ‘cryovac meat’ – nice vacuum sealed dimply muscles – just want you want.

This is a chronic condition and will take a long time to resolve.  Fortunately I appear to have caught it reasonable early (in my mind 4 months later than first discussed with the specialists) but never the less in time to address the issue.

Unfortunately with Sclerodermatous Skin there is no specific blood marker that easily identifies the condition – unlike say kidney or liver issues with are easily identified.

The clinical presentation of the disease is the key.  The main issue for people is loss of mobility as the skin gradually tightens up around your body.  Eventually you lose movement in your joints – so much so that you can’t bend over, touch your toes or even sit comfortably;

Luckily at this stage I am finding I still have my full range of movement but am most affected in by tightness around my legs and significant tightness around my abdomen/gut/trunk area.  The end game here if things don’t go well is loss of movement and ending up in a wheel chair – not for me!

I had been complaining to Amit for some time about feeling like I had developed a ‘beer gut’ which the associated comfortableness. We had passed this off as a side effect of the steroid Prednisolone that I was weaning off last year.

However as it turns out this was just the beginning of the Sclerodermatous Skin issue.

Amit’s response was to turn up the steroid dose initally to 10mg, then 20mg, then 40mg and after little impact 80mg a day  – which is a significant dose and one they don’t like you staying on for too long due to the many complications from side effects, including loss of bone density and muscle mass (and endless fatigue).

They add other drugs to this as long term immuno-suppressants, such as Mycophenolate   (In fact I am now back on up to 26 individual tablets a day – almost at the peak of my illness where it hit 44!).

As Amit says this will take a ‘long time’ to resolve.  Patience is once again part of the plan.

As part of seeking answers I have also consulted a Dermatologist at Melbourne City Dermatology, Emma Mooney, who first diagnosed this situation and took a biopsy to to confirm the condition.  She also referred me to a specialist Dermatology clinic at RHM with Dr Ryan De Cruz.  Emma was the first mover on this condition for me and I am grateful.

So where to from here?

Although quite worrying there are a range of treatments but as Amit says ‘unfortunately there is no clear path forward’.  We have to try the drugs and measure the clinical response looking for slow improvement.  There are a range of alternative drugs, each with various sides effects that might be used.  And as a third and final line of defence there is a process called Extracorporeal Photophersesis that involves a ‘dialysis ‘ like treatment of your blood on a regular basis over many months that is know to have a positive impact.  That would involve many months in Melbourne again for treatment.

Two Year Tests

My two year post transplant tests were all very positive. Via a comprehensive TeleHealth Session with Professor David Ritchie at Peter Mac, we talked through the tests completed in January.  This process is relatively new for RMH and it aimed at the Long Term Effects for Leukaemia patients.  It was very imformative.

Things tested and discussed included:

1. Hormonal Health Issues – testosterone levels, bone denisty, vitamin deficiencies, thyroid issues. Affect of constant steroids.
2. Cardiac Health – heart condition – response to drugs.
3. Immunologic – blood type, recovery of immune system, blood graft etc
4. Secondary Cancers – skin and other types
5. Psychological Health

David quoted numbers, ranges and test results, (largely at my request) and most if not all were in the normal range.  It was a very positive and glowing report card!

My recent blood tests showed most results in the normal range with a few outliers that the consultants were not overly concerned about at this stage.

Vaccines – I still a require the ‘live’ vaccines like measles, mumps & rubella but they have to wait until I am completely off immunosuppressants.

Where to from here

So although this blog brings you my current thoughts and concerns I see it as only a minor (and annoying) set back in my journey and one that I am determined to work through.

After such a good report at the two year mark I felt things were well underway but the life of a leukaemia patient is often unpredictable and can take many unforeseen turns.

I am sure I will get on top of this current situation and will endeavour to keep you posted on improvements from time to time – certainly not on a monthly basis but perhaps a few times throughout the year as things change.

As before, many thanks for your support and feedback.  It has been an important part of my journey and one that has meant a lot to me.

If there is anything good to come or the current COVID-19 Pandemic the ‘lock-down’ on our farm has meant plenty of time to catch up on jobs and keep well away from possible infection that would certainly take a rapid toll on my health given my current severely compromised immune system.

I am certainly enjoying my time with Jan catching up on pages of jobs around the farm, lots of reading and of course plenty of virtual drinks and ‘get togethers’ via Zoom with friends and family.  We even managed a black tie dinner for Claire’s birthday with 15 groups and 28 people.  It did get a little noisy at times!

So life goes on and compared to a lot of people I am having it easy!  When you think about it there are plenty of people much worse off – so no complaints here!