In September 2009, when the nurse in emergency at Epworth Hospital told me that I would be admitted, I’d had a heart attack, and no I would not be hosting that long planned dinner party that night, I couldn’t quite understand what this all meant. Here I was, a fit active 46 year old, training to run a marathon, and I’d had a heart attack?!. The previous day (Friday), whilst at work, I’d dismissed the chest pain, sore arms as some form of angina – it’d been a stressful week at work – though the sore chin was odd. The Aspro (aspirin) my wife gave me the previous night helped immensely – I felt 100% better. (I hadn’t been able to easily get in to see my wife’s GP the previous evening – I didn’t even have my own GP.)
No doubt I downplayed my symptoms. Frankly, the idea of an actual heart attack was not on the radar. When I eventually saw my wife’s GP on the Saturday morning, after hearing what had happened, she looked at me and said I think you’ve had a heart attack and either your wife drives you straight to emergency or I call an ambulance right now. (The GP reminds me of how lucky I’d been).
Anyway, a stent was inserted. 95% blockage in the L.A.D., my cardiologist told me. I had indeed been very lucky.
But what caused it? I had few of the classic situations that are associated with a heart attack, through some family history. The cardiologist simply said – my hand had been shown. There seemed no real enthusiasm to dig deeper. Outplacement and education etc was offered.
Fast forward 9 months. On holiday in Western Australia, more symptoms emerged, crippling pain in the shoulders, wrists, hands, knees. There had been other signs: incredible itching after going swimming and a ruddy face, almost with a purple tinge at times. But it was the joint pain that got me back to my cardiologist, and then a referral to a haematologist (although his other title as “oncologist” was concerning). My haem was great. He quickly diagnosed me with Polycythaemia Vera (PV). My blood counts showed highly elevated red and white blood cells, and platelets. Everything was right up. My haematocrit was about 62, well over the normal range. A rheumatologist I saw was also a little perplexed, and rheumatoid arthritis was ruled out. A preliminary diagnosis of a form of “gouty arthritis”, which can be associated with PV, was made. (There was no final diagnosis on that side of things, but allopurinol keeps things under control).
I did actually know something about PV already – because I had (of course) Googled all manner of sites to try to work out what I had. I thought PV was a real possibility. What I read on the net about it – significantly decreased life expectancy – had me (and my wife) very concerned and distressed. My haem was quick to point out that there was a lot of outdated material on the net, and that certainly if it was untreated, there was a real risk of another thrombosis, but that there were more options now and there had been huge developments in understanding the myeloproliferative neoplasms (MPNs), of which PV was one. (The others being Essential Thrombocythaemia (ET) and Myelofibrosis).
I now believe that the PV accelerated my pre-existing tendency to a heart attack. This sounded logical to me, as my blood was simply “too thick” (because of the elevated counts) and susceptible to blockages.
There was a rapid flurry of venesections – phlebotomies – or “blood letting”. They brought my blood counts down quite quickly. My haem was keen to put me on Interferon, and this happened within a few months. He took into account my (relatively) young age, and he did have some concerns about Hydroxyurea, which I understood was another possible medication. He was quite clear that the venesections alone were inadequate, particularly given I had already had a thrombosis.
The injections of interferon (IntronA) (3 times a week) were initially undertaken by my wife and took a while to get used to. Eventually I did them myself and they became second nature. I still do this 3 times a week, though this is about to change.
Interferon has some side effects – “flu like symptoms”, particularly the day after injection, anxiety and possible depressive symptoms. I had these but not enough to deter me from using it. Crankiness is part of it, but then isn’t that often part of getting older? There has been an impact on mood and outlook – foggy brain makes it more difficult to focus, concentrate and to plan for anything beyond the immediate and familiar routine. (This latter aspect I feel can be overlooked but is quite real).
I know some others with PV. Everyone copes in different ways. Support groups both in person and online, are useful. I subscribe to various Facebook groups and a US based web digest. I have been to a couple of meetings in Melbourne organised by the Leukaemia Foundation, a blood cancer conference and a specialist MPN conference.
The odd thing is that like many people with these diseases, I look fine. It is hard to explain the significance of the disease when you appear OK.
I know that there have been recent major advances in the state of knowledge of MPNs, particularly with the study of various genetic mutations (including JAK2 and others) that are associated with the diseases. I am confident that the research is leading to a better quality of life and increased life expectancy for those suffering the group of diseases. The possibility of progression of disease is always there – progression to secondary myelofibrosis, or acute myeloid leukaemia. But it seems to me that with treatment (and perhaps a bit of luck), such progression is very uncommon.
I am about to embark on a change of medication from IntronA to Pegasys. Pegasys, pegylated interferon, has just been put on to the PBS in Australia for MPN sufferers. This has been great news and the campaign was spearheaded by my friend Nathalie Cook. I gather that Pegasys will mean once weekly injections, instead of three times a week, and likely fewer side effects.
I try to keep up with all my usual activities as much as possible. Exercise (gym and weights) helps a great deal in quality of life. I still see my very supportive haematologist every few months, and have one set of blood tests in between appointments. We are able to have a quick email just to check that the results are OK. Everything is “stable”, to use my haematologist’s terminology, which is a good thing.