In 1999 I was 52, enjoying my work as a TAFE librarian, and living in Kiama on the south coast of NSW. I began having hot flushes but as I was two years past menopause, I ignored them. Then my right ankle became very painful and swollen so eventually I had to see a GP. He was puzzled and ordered a blood test. When the results came I was summoned to the surgery and given the news that I probably had polycythaemia rubra vera.
I remember him looking it up in a big red book and informing me that I had about 10 years – he didn’t say what would happen after that. I had to wait several weeks before I could get an appointment with a haematologist, and of course I used the time to scare myself silly by reading everything I could find out about PRV – it was mainly a problem for Ashkenazi Jewish men, and yes, I had ten years to live.
Fortunately I also found MPD-NET (now MPN-NET), a wonderful support group started by Joyce Niblack. Now I was in touch with people who were going through the same journey as me, and who were very willing to answer any questions. So by the time I saw a haematologist (Dr Kim Cartwright at Wollongong Hospital), I already had some idea of what was happening to me. I had a bone marrow biopsy that confirmed the diagnosis, and started on regular venesections. Dr Cartwright said she was sorry that there was very little information about PRV, and no support group, but I already had a support group on the internet.
Incidentally, my ankle was still swollen and painful, but this seemed to have been forgotten in all the excitement. Eventually the swelling went away!
The venesections controlled my blood counts but began to raise my platelet level too high, and also I began to have nosebleeds. So the decision was made to start hydrea. From the MPD-NET I was aware that there was considerable discussion about whether hydrea was leukaemogenic, and if interferon was a better option. My doctor was willing to try interferon if I really wanted it, but at that time I don’t think it was on the PBS for PRV, and of course there could be very unpleasant side effects. So I started on hydrea and have been taking it ever since. So far my only problem with it is that every few weeks I get quite severe mouth ulcers. Everyone has a cure for these but none of them have worked for me! I just change my diet for a couple of weeks so I don’t starve. This problem is fairly recent.
I was partly influenced and encouraged by another member of MPD-NET, an Australian called Ian Sweet. Ian was a couple of years older than me and lived in Canberra. He was diagnosed with PRV at about the same age as me, and was taking hydrea. He had researched blood disorders and their medications very thoroughly, and was of the opinion that the danger of PRV changing to AML (Acute Myeloid Leukaemia) was minimal. There was (and is) a school of thought that hydrea hastens this process. Sadly Ian did develop AML and passed away in December 2013. Was it a result of taking hydrea? We will never know.
I was going along nicely until 2010, moved house, retired, and had 3 monthly visits to the haematologist. Then I developed a sigmoid sinus thrombosis (blood clot in a vein in my brain) and spent two weeks in hospital. I am now on warfarin permanently. I began to suffer with very severe headaches, requiring several visits to hospital, consultations with specialists and numerous tests.
Finally in September 2014 I was admitted to a private hospital, where the physician diagnosed migraine! He prescribed Sandomigran, my headaches disappeared and I got my life back. The clot in my brain was caused by the PRV, the headaches probably not.
In 2016 we moved to Albury, and on asking my friends in MPN-Oz and checking the net, I found that there is no haematologist in Albury! As I knew a good GP I decided to follow his advice, and was referred to a medical oncologist at Border Oncology. I was impressed with his knowledge and thoroughness, if slightly taken aback when he informed me that I was too old for a transplant! I assured him that I had already decided not to go down that path.
I don’t feel that having PRV has made a great difference to my life. I do tire easily and I have to visit doctors and pathologists far more than I would like to. There is so much more information out there now for people with MPNs. My daughter has a connective tissue disorder and is in constant pain, taking massive doses of painkillers including morphine. Seeing people like her puts my problems in perspective.
Incidentally I am JAK2 negative, which means I am one of less than 5% of PRV patients. I am also CALR negative. I was diagnosed with osteoporosis in 1999, and I believe that this is common with PRV. I have started prolia injections for this.
Luckily my husband is very supportive, and at 70 is very fit and healthy. I have given him a couple of scares when he thought I wouldn’t come home from hospital. For years he has driven me many kms to appointments, and we are both happy that now I attend the new cancer centre near our home. It is so important to have a supportive family, friends and of course the wonderful people on MPN-NET and MPN-OZ.
Jenny Shepherd
October 2016
Postscript:
MPN AA team members are very sad to learn that Jenny passed away in August 2021. We are so grateful for her generosity in sharing her story, and our best wishes go to her family.
