Colin S – aged 67
In 2003 a routine military blood test indicated a haematocrit over 50 which led to an appointment with a haematology consultant for investigation. The initial thoughts were Haemachromatosis and I also had Rosacea. However, after a year of inconclusive analysis I visited my GP practice to ask for a second opinion but was told not to worry as my blood results were in line for age.
In 2008, towards end of my military service, I went for a full health check with military doctors to make sure I could leave military in the following year in the best possible health condition.
Over many years I worked 12 hour days and night shifts of seven days seven nights then three weeks off. This was brilliant, but often I had mild headaches, particularly during the night shifts. I used to drink a lot of water and kept well hydrated. I also had a few aural and visual disturbances where my hearing would be muffled with sparkly kaleidoscope effects in my eyes and the centre of my eye vision for reading would grey out. These lasted about 20 – 30 minutes before resolving itself.
Results of my military health test picked up that my blood results were out. I realised that this had happened before and went for another blood test at my GPs. This time the GP agreed that there was an issue and my haematocrit was 62.
I was quickly sent to the hospital where large quantities of blood were taken for genetic analysis. Before the results were available I had three venesections in one week to reduce the haematocrit towards 50, and then 45.
Before I retired in late 2009, I told the military doctors that I would get treatment through my home GP and local hospital. They hadn’t fixed me!!
Polycythaemia Vera was confirmed and treated by venesections with 75mg aspirin. Apart from the odd brain disturbance, I was completely asymptomatic.
My retirement plan was to spend the summers on a boat which we had moved to France in 2008 and spent the winters skiing in the Alps, so I really didn’t want anything to get in the way of that plan.
We moved full time onto the boat in mid 2010. The MPN Consultant agreed that the team could train my wife how to do venesections at the Day Treatment Unit and DTU provided the kit. She had been a research biochemist at the Radcliffe Hospital and was well used to taking normal blood samples, but a venesection needle is a completely different ball park!
When we were abroad I would pop into a local hospital and have a blood test every couple of months and if indicated Mrs S would do the duty ‘vampire task’. She is very good with no discomfort at all and much better than many nurses. One nurse was particularly bad in a Hospital in Paris and nearly had me ripping out the needle.
We have also had a couple of amusing? incidents. Once we only got half a bag, so stopped and I went out to lift the cycles back on board the boat. I felt a warm trickle down my arm, so stuck my arm out and continued to pump a Japanese style cartoon blood fountain. I found an empty bottle on the bank, and filled that till I reckoned we had got the full venesection done!
On another occasion, we filled the bag as full as possible until it was bursting. Unfortunately, my wife forgot to close the line valve and removed the needle resulting in a fountain of blood with the back pressure from the bag. I was amazed that the pressure hadn’t already blown the needle out of the vein.
In between boating and skiing we would pop back to UK and I’d pop in to see the DTU team, have a hospital venesection and pick up supplies etc.
I soon learnt though that blood tests abroad were pretty hit and miss and the numbers were often way out to what I expected so we just did a venesection every six weeks. One hospital I went to in Switzerland, for a ski injury, was fascinated by PRV and the haematology team were keen to learn more. This resulted an impromptu lecture to them all whilst being venesected, which was due.
In late 2018 I had a marrow biopsy which was pretty painful. Apparently I have very thick and stout hip bones. This showed grade 3 myelofibrosis. My spleen was also getting enlarged and protruding below left ribs. Blood tests also had high LDH, a sign of cell break down and inflammation and high platelets. JAK2 allele burden was 54% and RDW, a sign of erratic red cell production, was 25%.
I still did not have any significant symptoms apart from the odd visual/aural and I kept a couple of 300mg aspirins about my person to munch when one happened. (stroke? – perhaps a TIA in the brain micro capillaries).
The doctors wanted me to start Pegasys, but I was off skiing for three months and so I decided to delay that until I returned home.
In the three years since starting Pegasys it has had positive effects, but I still need the occasional venesection. My spleen has retreated behind the ribs, JAK2 allele burden is down to 6%, RDW is back to a normal 15%. My platelets are 120 and LDH at 120 and the visuals/aurals have disappeared. A BMB in April 2022 showed that the myelofibrosis was now grade 1-2. The consultant noted:
“We are fascinated by the improvement that some patients have to interferon treatment and so I will arrange for the pathologists to compare the 2018 biopsy with the most recent biopsy in our myeloid MDT meeting.”
I keep pretty active. Life on the boat is active and we only have bicycles, not e-bikes, for transport and visiting places. When at home during the lockdown I would do an hour cycle ride every couple of days.
The skiing is also pretty active and I happily ski from the first lift to the last lift. In 2015 I decided to make skiing more difficult and took up telemark skiing instead of alpine, which goes back to the origins of manoeuvrable skiing in 1868, and is a considerable challenge. Essentially the heel is free and the turning involves deep lunges.
As a style of skiing the sensations are fantastic and it is thoroughly enjoyable. I achieved a pinnacle in January 2019 at the GB Telemark Championships where I won a Gold Medal in the Open Team Classic Race.
Finally I eat healthily and meals are home cooked from fresh ingredients with lots of fruit and vegetables. I don’t smoke but do enjoy a drink or two.