My last post was 12 months ago to the week and I am pleased to report that everything is going along pretty smoothly. It is 5 years since my diagnosis and life could not be better!
I had toyed with the idea of finishing up the Blog but my son Lachy encouraged me to keep it alive as it may just help a few people understand the condition they are dealing with. And within a few weeks of that conversation a good friend Geoff Daniel was diagnosed with AML and he is now starting the long gruesome journey that I have been on over the past 5 years.
I am still attending Peter Mac every 3 months for ECP treatment plus IVIG infusions. I usually back this up with appointments with Amit and my dermatologist.
At this month’s appointment Amit confirmed that my condition had indeed stabilised – with a planned long slow weaning off the drugs. This could take more than 2 years but Amit is confident that I have seen the worst of the skin disease – yipee!
I must say I was very happy to hear this as any worsening of my skin condition would be alarming. There are times at present when I have some trouble breathing due to the physical constriction of my tightened skin. It is like wearing a corset.
I am still on a range of steroids and immunosuppressants plus ECP and UVA light treatment which, along with regular stretching and yoga, appears to be keeping my condition manageable.
I regularly see a respiratory physiotherapist who has given me a range of breathing exercises in order to strengthen my diaphragm and improve lung capacity. Regular Lung Function Tests confirm minimal reduction in lung capacity which is pleasing.
We are spending most of our time on the farm helping our daughter Claire and partner Toby who have taken over the lease this year. They have dived in the deep end without any great farm experience and now run a 2000ha sheep farm.
Recently we took our new hybrid camper up to Queensland with friends and spent a week sailing around the Whitsundays. It was certainly a trip of a life time.
I am lucky to be able to fly regularly, play tennis, ride my Adventure motorbike with friends and jump on a mountain bike from time to time. I must say 5 years ago I certainly didn’t expect that life would be so good!
I was a little down in my last blog due to the worrying progression of Sclerodermatous GVHD but I am very happy to report that recent treatments appear to be working.
The progression of the disease appears to be halted and I have noticed a significant improvement in my skin elasticity.
Amit Khot tells me he has “thrown everything at it but the kitchen sink” – meaning he has me on three different drugs Prednisolone – a corticosteroid, Ruxolitinib – an immunosuppressant, and Tacrolimus – another immunosuppressant plus ECP (extracorporeal photophoresis).
Apparently it is a little unusual to provide patients with so much ‘firepower’ but in my situation Amit was not confident just one drug was the solution.
However, ever the scientist he worries that with so many treatments he will never really know which one is working!
Adding to Amit’s dilemma I had trialed Red Light Therapy (an LED light array in the range of 615-630nm) hoping that I might be able to reverse the fibrosis or tightening of my skin. There is very little empirical evidence and research on this method of treatment and Amit believed I was wasting my time.
Although I certainly received some short term benefits after three months of treatment I discontinued it as there were no signs of sustained improvement. I can categorially say the RLT was very helpful with muscle soreness and cramping – results were almost immediate.
Not to leave a stone unturned I moved on to another light therapy – UVA1 (340-400nm). Unlike the RLT this light therapy is reasonably well researched with a number of scientific studies published for treatment of Scleroderma (a similar skin condition to mine).
I managed to track down a light machine from the US via a local supplier and have been using this treatment for over a month now.
First results are promising but it is too early to be definitive.
i am also using a massage gun to help soften my skin. I got this idea from Dougall’s mate Pope.
There was a time where my mobility was significantly reduced and I had been experiencing increased difficulty breathing. I am less concerned about this now and am much more comfortable day to day.
Amit has been skeptical of light treatment saying there is no real evidence that it will assist but a specialist Dermatologist, Ryan D’Cruz believes it will deliver some benefit and is fully supportive of a trial.
My good friend Andy Irvine put me in touch with a contact of his who runs a drug development company in Melbourne – Certa Therapeutics. They are currently trialing a drug worldwide for Skin Fibrosis. Having made contact with the key person, Darren Kelly, I have initiated a series of meetings between Darren and Amit who is assessing the merits of accessing this new ‘ trial’ drug on compassionate grounds. Although this new drug FT011 appears to be a possible solution Amit is cautious and sees it as a ‘last resort’. We will see what happens.
Life on the farm
Things are going well in Tassie and I have enjoyed flying again. I was grounded by CASA rules during June and July due to my increased level of Prednisolone at the time.
COVID is not having an impact in Tassie at this stage, although after most trips to Melbourne for ECP treatment I have to self-isolate at home for two weeks which is a little annoying but not too bad in the scheme of things.
Hopefully there will be no news (being good news) for a number of months….
It’s been almost six months since my last blog and there have been a few developments……unfortunately not for the better.
Back in January I was concerned that my skin condition had regressed due to GVHD and it has become obvious over the last few months that the ugly condition has flared up again.
My new immune system is attacking my skin resulting in fibrosis between the dermis and epidermis layer. It looks a lot like cellulitis but is actually hard and stiff and it results in a slow loss of mobility as the skin thickens and becomes less elastic.
The standard way of dealing with it is to increase the steroid, Prednisolone, which is a very ‘blunt’ instrument widely used for a range of conditions. My hematologist, Amit Khot, pushed the dose up from 10Mg to 25Mg hoping to slow down the spread. I’m also on a new drug, Ruxolitinib (Jakavi), which can take up to 6 months to take effect, I’m only two months along this journey – so more time to wait.
Unfortunately there isn’t a blood test to determine if this type of GVHD has flared up or is under control, it is only assessed via clinical presentation. It changes very slowly and is extremely difficult to detect. It’s like being a frog in boiling water, it can progress undetected for months until the situation gets out of hand – and unfortunately it is not reversible using conventional methods.
In an effort to seek alternative solutions to reverse the damage I have investigated a range of treatments including light therapy. I have tried Red Light Therapy (wave length of (615-630nm) and although there is definitely a short term positive result it is unclear as to the long term benefits.
Amit continually says ‘ there is no evidence’ to support such treatments my view is I should ‘chase every rabbit down every burrow’ as you never know what you might find. In fact my research has uncovered a number of new drug trials that are focused on reducing fibrosis in organs including skin so hopefully in time something will eventuate that might provide a solution.
However my Dermatologist Ryan D’Cruz believes other forms of light therapy may be effective and he supports a trial of UVA1 (340-400nm).
The issue is to try and determine whether the change of drugs or other treatments are delivering results – especially difficult when these are being delivered concurrently.
Although it sounds rather grim my recent action and searching for solutions is more to avoid significant regression of the disease (and perhaps improve the situation) rather than simply accept a degeneration that will eventually lead to loss of mobility.
Apart from some annoying side effects of Prednisolone (lack of sleep, odema, cramping, peripheral neurophy) things are going reasonably well and I am still able to do most things that are on my daily list (with the exception of flying).
One thing that all doctors agree on is physical therapy such as stretching can only help. It is now very much part of my routine to stretch for 45mins most mornings.
We have been based back in Tassie for some time now, returning to Melbourne for treatment as required.
During the last few months we have had some great visits from old friends, including my school mates Peter Taylor & friend Katrina from Sydney and Ian & Christine Poole from Brisbane.
And most recently Stephen & Katrina Weir together with Lisa & Simon McSweeney from Sydney – just in time before the Sydney COVID situation got out of control!
Ken and Helen Lansdowne from Wagga visited for a few days on their recent Tasmanian travels. Ken and I played in the Wagga Highland Pipe band for many years. He has been a great supporter of mine during my extended illness.
And my Ward 7B buddy James Watson with his wife Victoria visited as well.
We visited Jan’s parents in Bairnsdale. They have recently moved off their farm into a new house in town.
As you might have gathered not much has happened on the health front for a few months (which is good news) but I thought I would update you now that I have some more news.
In mid February I had another consult with a specialist Dermatologist at Royal Melbourne who has established a clinic specifically for GVHD issues. Dr Ryan D’Cruz was able to check me out in person (the first two consults last year were via TeleHealth) and confirmed that good progress had been made with my ECP treatment, which is on-going.
I raised some concerns with Ryan that some areas of the Sclerodermatous GVHD had regressed slightly. Unfortunately with this presentation of GVHD there is no blood test or result that will definitively show what is happening. Results must be based on clinical observation. The changes in skin condition are very slow and might take months to be recognised.
For me this is quite a concern as any further regression of my situation would be far from ideal.
With this in mind I decided not to take any chances and discussed the options available to increase my treatment. Ryan suggested increasing the frequency of ECP back up to fortnightly but left it to my Hematologist, Amit Khot to make the new plan. He also suggested that Amit look at prescribing a relatively new drug, Ruxolitinib which although not on the PBS for Sclerodermatous skin was known to be highly effective as an immunosuppressant.
My consult with Amit resulted in an increase in Prednisolone to 9mg daily and a doubling of Tacrolimus. He also prescribed Ruxolitinib (which requires special approval) to gain funding.
I am back on monthly consults with Amit and will remain on monthly ECP sessions for the next 3-4 months. Hopefully this will lead to another slight improvement in my condition.
As I explain to people my skin condition is not restricting much of my activity. I can do most exercise activities (bike, walk, stretches, flying, gardening, farm work etc) although at times the pain level will vary.
So overall I consider myself to be pretty lucky but as with all GVHD issues (80% of bone marrow transplants suffer from GVHD) we don’t know what is around the corner. According to Amit the presentation of GVHD will ‘wax and wane’ over time with gradual improvement expected.
As there is not much more to report I will continue to keep this blog open and will update everyone as and when there is some new information to report.
It has been over four months that we have been ‘holed up’ in Melbourne with just one week back in Tassie during September. Of course Melbourne would be the worst place in Australia to live at the moment due to the harsh COVID lockdown.
Never the less we have managed to pass the time easily with plenty of walks and bike rides. Unfortunately we haven’t been able to visit friends. And of course our major social outing has been the weekly trip to Peter Mac, talking to the very friendly nursing staff.
The results from the ECP (Extra Corporeal Photopheresis) treatment have been very positive. It certainly has stopped the spread of my skin disease (Schleradermatous skin) and in many areas has softened my skin making it much more comfortable to move and get around.
For many people this condition results in severe mobility issues which worried me considerably. However I believe I am through this stage and the future looks promising.
I had experienced bowel problems with severe pain and diarrhoea over two months and at one stage found it was causing me more grief than my skin condition. My medical team were at a loss to determine the problem thinking it might be a side effect of one of the immuno-suppressant drugs, Mycophenolate, or perhaps a low grade infection that had not been detected via my weekly blood testing regime.
A colonoscopy was ordered and results confirmed CMV in my gut, which has not been detected in blood or stool samples. This was treated with Valganciclovir – an anti-viral drug which cleared up the situation within days. What a relief.
Jan is now a media star being interviewed for a new Podcast Series developed by the team at Peter Mac, called the “Straight and Marrow” – a series to help people understand Bone Marrow Transplants.
The plan from here
Having consulted with Amit we now have a new plan. I have been weaned off most of my drugs (such a relief having maxed out at over 30 tablets a day). I am now on just 7.5mg of Prednisolone, completely off Mycophenolate and just 0.5mg of Tacrolimus daily, plus a few others including Amoxocillin, Magnesium and Bactrim – what Bliss!
Amit has reduced the ECP treatment to fornightly which means we are free to return to Milford- yay! We will be commuting back to Melbourne regularly but hopefully the COVID situation will settle down and we will be able to come and go without having to quarantine each time we return to Tassie (self isolation on our farm luckily).
Thankyou to everyone who has send messages and contacted me – I really do appreciate your support.
Over the past month I have been interviewed 7 times by young resident doctors practicing for their specialist exams. They have each taken me back through my complete treatment from diagnosis to current treatment and after an hour it feels like a good therapy session.
One of their last questions is “what do you see yourself doing in 5 years?” My answer is simple – the 6 Fs. Fitness (health), Family, Friends, Flying, Farming and having Fun. Quite simple really!
I will continue my blog later this year or early next year subject to any new news but for now, thanks for reading!
It has now been well over two months since I started undergoing intensive ECP treatment, twice weekly and I am pleased to say things are improving!
This treatment is considered to be on the ‘fringe’ by many doctors but the Clinical team tell me it is effective in 80% of patients . Results become apparent over months and years rather than the days and weeks with conventional medication.
We had a catchup with Hematologist Amit Khot last week and the news was positive. After a few tight pinches of my skin he announced that there was definitely a softening of my skin and the treatment was worth pursuing. He was so happy with my progress that he has reduced the treatment to once weekly.
He did say that for some patients without a sign of improvement at the two month mark he recommends stopping the treatment.
During our visits to Peter Mac where I might be in the chair from 3 to 7 hours we have met many patients undergoing similar treatment. It makes you realise that there is always someone in a worse position than yourself. Two of my new contacts have horrific skin presentations – almost if they have suffered 90% burns to their skin with massive discolouration, blisters and tightness that makes my skin look like a babies’ bottom. Sometimes you realise how lucky you are!
With the improving conditions my breathing has improved as my diaphragm has ‘softened’, it was quite tight and restrictive prior to starting the treatment.
I now have a full range of movement and despite a tight abdomen I can complete most exercises.
Unfortunately there is no real objective test of skin elasticity other than the ‘pinch test’ so I have been developing my own using a mini penetrometer (an engineering device for measuring soil density). The doctors are skeptical but I am confident I am on to a medical breakthrough!
I’m back up to 20 odd tablets a day, including a high dose of Prednisolone which has led to a significant reduction in muscle mass. I have lost almost 10kgs in the last three months which puts me back to mid 2018 (post transplant).
I long to do just one push up again! Thankfully I know I can return to full health as I have achieved this before, but it is so disappointing to slip back so far. As I’ve said previously it is like a huge game of snakes and ladders and I’ve just landed on the big snake near the home run – now for a ladder!
One recent ladder to mention is my recently diagnosed Type 2 Diabetes. This was entirely drug related and now that my dose has been reduced on both Prednisolone and Tacrolimus my blood sugar levels have returned to normal and I have kicked the need for insulin – yah!
Melbourne Lock Down
It is not the ideal time to be in Melbourne for treatment as Stage 4 lockdown certainly restricts our movements. Our major social activity for the week is heading off to the hospital and catching up with the nursing team – a bit sad but reality.
We do manage the allowable one hour’s exercise a day (sometimes more but don’t tell the teams of police and ADF personnel patrolling the streets of Melbourne at the moment). This generally involves a walk in the morning and a bike ride in the afternoon.
We lashed out and purchased some E Bikes last month (after a huge recommendation by good friend Peter Clucas) and we love them. It has made bike riding very enjoyable, when you hit the ‘turbo’ button the hills seem to disappear.
Zoom has kept us in touch with family and friends. We are looking forward to getting back to Tassie, hopefully at the end of the month.
Thank you for your phone calls and messages – it means a lot to me!
We moved back to Melbourne in late June for the Extra Corporeal Photopheresis (ECP) treatment I mentioned in my last blog. In many ways this was a relief but the recent COVID flare up in Melbourne has added another dimension to how we get about and live life in these strange times. Lockdown in Melbourne is certainly different to lockdown on the farm in Tassie!
A port had to be inserted in my chest under my skin for regular access and this took a week to heal. Treatment started in the first week of July and will continue twice a week for two months before being slowed down to weekly and then fortnightly, monthly etc. It could go on for years.
It takes me back to mid 2018 when I was going into Peter Mac twice weekly for blood transfusions. Luckily Jan is still happy to help out and comes along each day to support me. We both agreed when we got married that we would support each other ‘…….in sickness and in health’….. – not thinking it would actually come to this! I certainly have great respect and admiration for my wife who has been happy to support me through this long and trying time.
Amit Khot and his GVHD team run by Clinical nurse Paige Marino and Alex Rivalland have been working with this treatment for a number of years and say it is highly successful although it takes ‘time’ to see results. Luckily I have plenty of time and am looking forward to seeing a response.
The treatment is straight forward, I am ‘hooked’ up to the Apheresis machine (blood extraction and processing) for about 2.5-3 hrs. 1.5 litres of blood is extracted, the red cells are spun off and returned and the white cells are injected with a drug (methoxsalen) which makes the T Lymphocyte cells to be susceptible to UV radiation. The white blood cells are then exposed to UV radiation before being transferred back to my body dead T Lymphocytes and all. The idea is that my immune system sees all the dead T Cells and ‘encourages’ my bone marrow to reduce its production of T Cells in an effort not to continually attack my skin.
The ECP machine
The control panel
There are four Aphoresis machines in Peter Mac and three at PAs in Sydney to service the population of Australia and New Zealand. They are used for a range of treatments involving immune response/rejections including ‘hard organ’ transplants such as heart and lung transplant rejections.
Jess, the leader of the 3B Outpatients clinic was the nurse who oversaw the collection of my brother, Tony’s Stem Cells back in January 2018 and it was a delight to meet up with her again.
Josh’s book is very inspiring
Jess who remembered us from Tony’s stem Cell transplant more than two year’s ago
She was kind enough to lend me a copy of a book written by one of their Patients, Josh Komen, who still travels from New Zealand four years after first receiving ECP treatment. It made a huge difference to his life and his book is certainly inspiring. In “The wind at my back” Josh describes his long and successful but very painful and challenging battle with AML over many years.
During one treatment we met a couple from Werribee who had been coming to Peter Mac for over a year for his ECP treatment. Ray’s condition was much worse than mine with a very stiff body and legs and unfortunately he was confined to a wheel chair a lot of the time because of his mobility issues. The clinical team had been telling me that my condition was certainly one of the ‘milder’ cases which I had found hard to believe until I met Ray. There is always someone worse off than you!
Last week I attended a Telehealth Dermatology Clinic seeking answers to my many questions. In particular will the ECP treatment reverse or soften my skin which has become quite hard. Harini (the dermatology registrar) spent quite a bit of time explaining the GVHD process on the skin and its various layers and indicated that the
hardening of the skin was due to the Thickening Collagen Bundles between the Dermis and Epidermis layers. For some patients the ECP is very effective in softening this condition and for others there is not much change. Let’s hope I’m in the first group!
For the majority of patients the ECP combined with immuno-suppressant drugs is effective at halting the spread of the condition.
I am currently using ultra-sound treatment on the effected areas although Amit tells me there is absolutely no medical evidence that will help but if I perceive a benefit it is unlikely to be doing any damage so go ahead. Of course the physiotherapists out there (including my mother) would swear this treatment is effective in a range of situations.
I am working with a physiotherapist, Kennedy Lay, for over two years now and he has encouraged me to implement a set of stretching exercises that take over an hour each morning. This is certainly helping. (Kennedy now in his 30s has recently taken up a cadetship with Qantas at their new training facility at Toowoomba but is happy to support me through Telehealth).
I am having blood tests on a weekly basis and all counts are generally in the normal range (although the CMV – Cytomeglavirus) started to climb to worrying levels. This was treated with a new drug Valganciclovir and is back under control now.
At the moment I am on a total of around 36 tablets a day with 16 different drugs. I have returned to my old mantra of “one day at a time” as this episode has taken me back approximately two years in my recovery.
After my two year work up I was excited to think that I was through the worst of the complications and all indications looked good, but as Amit previously said “we don’t know what might show up with the GVHD and after all we are dealing with biology”.
For me the toughest thing is dealing with the setback, especially the lack of strength and fatigue, knowing that it is again a long way back to good health. However knowing I have been there before I am up for the challenge. It is just a matter of time.
Type II Diabetes
One of the major impacts of the many drugs I am currently taking is high blood glucose levels. Amit has been monitoring this over the last few months and referred me to the Diabetes team who confirmed that I was now considered to be a Type II diabetic. They have assured me it is most likely related to the drug load and will hopefully return to normal once the drugs are reduced, but it may be that the condition is with me for the long run.
The normal range for blood sugars is around 4-6 mmol/l on a fasting test and between 6-10 mmol/l during the day. My levels are well above that at 12-16 during the day. The Diabetes team didn’t seem to be overly worried saying above 16 is of concern and when the levels rise over 20 it is considered to be very serious.
So I am now testing 4 times a day (with a home test kit) and self injecting insulin once a day.
Victoria Watson, wife of good friend James who was treated for ALL at the same time as me delivered a food parcel during the COVID lockdown in Melbourne. James has made a full recovery and no longer takes medication. He is doing so well!
In my last post (the one after the all time final) I gave an update on my new skin condition, Sclerodermatous GVHD. Time for another update. (The Johnny Farnham Blog lives on!)
Things have moved on and my skin condition has worsened. The response from my medical team was to increase the drugs by taking Prednisolone to 80mg daily (which is quite high and it is a nasty drug with lots of side effects, muscle wasting being the main one, and potential loss of bone density). Not long after they added a second immuno-suppressant – Mycophenolate. And then when this didn’t appear to be doing the job they added a third line of defence another immuno- suppressant, Tacrolimus.
So as you can imagine, being on 3 immuno-suppressants, its a bit of a tough ride – these drugs are quite toxic! Not to mention the 12 other drugs I am also taking. So all in all I’m now gobbling up around 32 tablets a day. This takes me back to the post transplant days two years ago. I am having weekly blood tests but the skin condition is largely assessed on a clinical basis rather than through the blood results. Most of my critical blood counts are in the normal range to close to.
As my good friend James Watson (an ALL survivor from Peter Mac who incidentally is off all drugs and doing marvelously) says, “it’s the set backs when things seem to be going so well that are tough to take”.
And from Cathy Koning (a new friend through the Leukaemia network, who is finalising a book on her experience)
“For me recovery is not actually a single line from illness to some medical intervention to feeling better and thanks, see you later. The cancer train stops at many stations. It is a series of many setbacks. And recoveries – from the leukaemia/chemo, from sepsis, from being in ICU, from the actual transplant, from GVHD, from shingles, managing diabetes (more than once), from pneumonia and from the serious side effects of drugs such as Prednisolone.”
Just a few months ago I was crowing in my ‘final’ blog how well the two year tests went believing I was fully cured and life would get back to almost normal. How wrong I was!
Amit Khot had always said that GVHD affects 80% of Leukaemia patients and it is unpredictable. After all “we are dealing with biology”.
So for me it is back to my old mantra “One day at a time”.
The good news is that Peter Mac have set up a new Clinical team to tackle the GVHD issue for patients and there is now a strong focus on this issue. The team have recommended a fourth line of treatment Extra Corporeal Photopheresis (ECP). Basically a process like dialysis that takes blood, spins off plasma and red blood cells and returns these unharmed then treats the remainder with a drug to sensitise the T-cells to UV light. The T-Cells are then killed by exposure to UV radiation, and returned to your body to train your bone marrow not to produce too many T-Cells which are causing all the damage to my skin. This process has been around for a number of years and has minimal reported side effects. I have added a leaflet explaining the process if you’d like to know more!
Peter Mac has three ECP machines and a nurse dedicated for each one. The process takes around 3 hours and I’ll have two treatments a week for the first two months, then its effectiveness will be reviewed and and based on my response the number of treatments should back off after that.
They treat around 20 patients a year, including people from all over Australia and NZ and say that some people don’t respond so well and choose not to continue the treatment largely due to the time commitment. But in my case Health is number 1 and I have plenty of time to dedicate to this nasty disease. I’d like to get on top of it ASAP and the process should start next week sometime – I will keep you posted!
At the consult two of the Nurse Consultants (both of whom had spent time with me during my treatment so remembered by ‘thirst’ for knowledge and numbers – read ‘the guy that asks lots of questions’) – reassured me that the ECP treatment is very effective, with an 80% success rate and usually some softening of the skin. Things certainly won’t return to normal (whatever that was), but at least I will be able to continue on with life.
They reassured me that my condition has been caught early enough to treat and they often see people with much worse Sclerodermatous skin.
Whilst annoying and sometimes painful if the condition was stablised and did not progress I could certainly live with it going forward.
Being severely immuno-suppressed the main concern for me is infections, either viral or bacterial. God help me if I catch COVID -19 – it will be quick! Luckily Jan is a stickler for hygiene and keeps reminding me of the things I need to do to stay safe.
On the Farm
We have been enjoying Tassie’s strict lockdown procedures as this has given us lots of time to complete the myriad of small jobs that seem to hang around. The Garden and farm now look ship shape. We are installing two new centre Pivot irrigators and this requires a big clean of of rocks to allow pasture to be sowed without damaging the machine.
Passing of my Dad
A few weeks ago my father, (aged 86) passed away after a short illness. In fact he spent less than one day in hospital which was his preferred way to depart the world. Although a sad moment it was a great chance for a family get together and to share memories of someone we all loved so much.
In my lastever 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:
Hormonal Health Issues – testosterone levels, bone denisty, vitamin deficiencies, thyroid issues. Affect of constant steroids.
Cardiac Health – heart condition – response to drugs.
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!
Welcome to my last blog post covering my Leukaemia journey from August 2017 to February 2020 – almost two and a half years.
In mid January I underwent a series of two year post bone marrow transplant tests including a bone marrow biopsy (to detect signs of leukaemia), a bone density test, heart test, lung function and others. I had 13 separate blood samples taken for a range of blood tests.
My wife, Jan and I met with Amit Khot, my haematologist in late January to receive the good news that I was technically in ‘molecular remission’ – basically cured of cancer, with less than 5% chance of relapse. This was wonderful news – many people don’t survive this nasty disease. I am in the lucky category!
Almost all my blood counts are now in the normal range and I feel fit and healthy – almost back to my pre-diagnosis health. The main issues I currently deal with are quite minor and ‘at the fringe’ being GVHD, largely of the soft tissues in my mouth and some minor aches and pains of muscles and bone joints. These things can be easily managed and don’t affect my day to day activities much at all.
I must say I am eternally grateful for many things and would like to thank the many people have helped me return to health:
The wonderful medical and support staff at RMH and Peter MacCallum – in particular Professor John Seymour and Dr Amit Khot
My gorgeous wife (and proof reader!) Jan who stayed by my side for many months advocating for my return to health
My wonderful family who have supported me through some pretty tough times and in particular our wonderful children Claire, Lachlan and Dougall.
My brother Tony who donated his stem cells (bone marrow) to keep me alive
Our friends who have wished me well and made special efforts to visit and keep in touch
I am now moving onto 3 and then 6 monthly blood tests and checkups and anticipate that my focus on leukaemia will wane as I move forward with my life.
I have recently contributed an article for the AML news – a special newsletter produced by the Leukaemia Foundation and you can read it on my Blog under the home page.
My treatment for Leukaemia has been an incredible experience, with some challenging situations and one of learning and growth. Whilst I would not wish it on anyone I do implore those with a similar diagnosis to stay focused and positive in an attempt to manage your own recovery.
Thankyou to each and everyone who has connected with my blog and have taken the time to send messages of support. This support was a key factor in my recovery.
I wish you all health and happiness in your own lives.