MY MPN JOURNEY BY TEREENA COCKS.
I have had, what many may call an “interesting” and “very challenging” haematological cancer.
It all started at age 15 in 1982 ( I am now 57) when I was diagnosed with ITP (idiopathic thrombocytopenic purpura) after falling from my horse and being black and blue down one side of my body. A GP visit and blood tests done, resulted in a phone call later that day advising that I had 11 platelets – and a rushed trip to the Children’s hospital – being driven in the car by my very nervous Mum – (we live in Strathalbyn 1 hour from Adelaide).
A bone marrow biopsy was performed, and ITP diagnosed. I was treated with high doses of Prednisolone – which worked well to bring my platelets back to a normal range. I suffered terribly with leg cramps and required assistance from my parents at night to help massage my legs to stop cramps. As a side note, I had just had the Mantoux test at school only a week or so before ITP occurred.
In 1989, I left Strathalbyn and moved to Adelaide to study Enrolled Nursing. I graduated nursing, but then changed careers to become an Ambulance Paramedic in 1991.
Fast forward to 1992 I started having symptoms of ITP again – bruising easily, bleeding gums, enlarged spleen. Interestingly, I had only a week or so earlier had another Mantoux test after exposure to TB from a patient transported by Ambulance ( I was the attendant).
I went for another bone marrow biopsy and abdominal CT scan. The biopsy showed a recurrence of ITP and CT showed an extremely enlarged spleen and abdominal lymphadenopathy (which they thought could be lymphoma).
Surgery was recommended – as it was thought the spleen may rupture spontaneously due to its very large size and the risks of my job- should I be involved in a high-speed vehicle accident or a heavy patient lift. I had a laparotomy and splenectomy on 3rd April 1992. I was able to return to my work as an Ambulance Paramedic within 8 weeks and felt well with normal blood tests.
In March 1994, I seriously injured my lower spine lifting a very obese patient and had to be admitted to hospital for treatment. Whilst resting in bed, my left hand became very blue and cold and it could not be re-warmed. A Doctor came to assess it and could find no radial pulse. I was transferred quickly by ambulance to the Flinders Medical Centre for further treatment.
I was sent for an immediate venous angiogram, and it showed that I had an extensive blood clot in my left radial artery and that my platelet count was over 1 million.
This was my first introduction to having an MPN.
Essential Thrombocytosis was confirmed. I was started immediately on Hydroxyurea and aspirin and discharged to home about a week later. Nothing was explained to me about the disease, nor the hydroxyurea. I was told I must never get pregnant while on hydroxyurea as it causes major birth defects. I was then told that I must never stop taking it as it was the only treatment at the time and without it, I would be at risk of clots and bleeding. At no time did Doctors ever discuss if I could freeze eggs before starting hydroxyurea. I continued taking hydroxyurea at varying doses right up to having my stem cell transplant as will be discussed further down.
March 25th 1994 – Got engaged
On March 25th 1995 I married the most wonderful husband I could wish for.
Over the ensuing years, my platelet count would cycle high and low and I suffered with multiple blood clots – venous and arterial and spontaneous bleeds. I suffered haemarthroses in knees and shoulder joints, bleeding into my thigh muscle and uncontrolled nose bleeds. I had problems with stroke-like symptoms that were attributed to my very high platelet count causing my blood to be hyper viscous.
My haematologist tried many different medications in an attempt to control my disease. Anagrelide had to be discontinued due to causing severe heart arrhythmia – holter monitor showed my heart rate to be in excess of 200 beats per minute at multiple times during the day and night. I was taking aspirin as a blood thinner and suffered a large spontaneous bleed into my right quadricep muscle (corked thigh). At the same time, I had a clot form in my left external jugular vein. I was prescribed warfarin and required regular monitoring of my INR (clotting factor). Unfortunately, my INR was very unstable – either too low or too high levels with an increase in spontaneous bleeds. Vitamin K was given many times to rapidly lower my warfarin levels. I was put onto many anti-platelet medications and very high doses of hydroxyurea. Doctors tried Interferon a2a and Busulfan but could not control my recalcitrant MPN.
Around the year 2000 I noticed that every time I showered, I became terribly itchy. I thought I had a soap sensitivity and changed to a low allergen soap. It did not help. Even sunshine would make me itchy. Even my dogs knew to stay away from me for a while after I had a shower – I was SO itchy! My haematologist did blood tests and found that I had mutated to Polycythaemia Rubra Vera. I started having monthly venesections.
I found from the internet that Zyrtec (antihistamine) tablet taken in combination with Zantac (anti reflux drug) worked wonders on controlling the itch. That is due to having both a H1 (Zyrtec) and H2 (Zantac) histamine inhibitors together.
Sadly, Zantac in the form of Ranitidine was removed from the TGA and it took a while before the safe replacement was available in the form of Famotidine (Zantac 360)
In 2003, I was forced to retire from my Ambulance job after 15 years of service due to worsening health.
In 2004, my husband and I then decided that it was now a good time for us to travel the world on a 4 month holiday – from July to October. We travelled to the UK, Europe, USA and Canada. It was tricky as I had to regularly have INR tests to check my warfarin levels and had to be admitted to hospital in England and Canada and barely escaped being admitted again in California – all with symptoms related to my blood condition.
It turns out that our decision then to take a holiday together was very timely. We have not had another opportunity for any overseas travel since then, and now never again for me.
In 2005 – the year that the JAK2 mutation was discovered, I was tested and returned a positive JAK2 mutation.
In 2006, I suffered with blood clots in my portal veins in my liver. Initially, Doctors told me that I would require a liver transplant, but later scans showed that only half of the liver (the lower lobe) had died (infarcted). I did not require surgery to remove the dead part – the body reabsorbed it. I have been left with a dysfunctional liver with very raised liver enzymes. I do not drink any alcohol and have never smoked. The lower lobe has never re grown.
I spent time recovering at home and in 2007, decided to upgrade my certificate of Enrolled Nursing to a Diploma – and undertook studies at college.
I gained a full time nursing position (in 2009) at a country hospital. I then started my studies at university to become a Registered nurse and won a scholarship towards this. Unfortunately, I had to withdraw from my course and also from my full time Enrolled Nurse position at the hospital (in 2011) due to a rapid decline in my health.
In 2011 I suffered with another arterial clot – this time in my left ulna artery. I was treated with surgery (thrombo-embolectomy) and IV Heparin and was discharged home on clexane.
Only a day or so after arriving home, I suddenly became very unwell – my abdomen had become very distended and I was in excruciating pain. An ambulance was called and I was taken to Flinders Medical Centre -almost 1 hour from where I live. I have never had such intense pain in my life! A CT scan showed I had a lot of free blood in my abdomen and was still actively bleeding. Laparotomy found a ruptured ovarian cyst and 2.5 litres of blood in my peritoneal cavity. I received multiple transfusions during this hospitalisation.
In 2012, after consultation with my haematologist and gynaecologist, they thought it was in my best interests (risk of spontaneous bleeding) that I have a radical hysterectomy. I went ahead with the surgery and was forced into menopause at 45 years of age.
I now had no spleen, no uterus, no ovaries, half a liver and previous appendix and gall bladder removal too.
In 2016, my husband and I took on a family business that we ran from our home. It was a specialty service for police, emergency services and military personnel to have their medals, honours and awards put together in a group of medals, ribbon bar or memorabilia board.
We were doing well – until symptoms of severe fatigue and night sweats and changes in my blood picture led to another bone marrow biopsy in 2017 and a diagnosis of Myelofibrosis. The Myelofibrosis made me transfusion dependent within a few months. I continued with weekly blood transfusions for a while – until I started getting extremely fatigued, breathless and having more night sweats – so I started on Ruxulitinib (Jakafi) at this time which helped control the symptoms.
In November 2018 my blood picture indicated that I was beginning to advance to AML (Acute Myeloid Leukaemia) which was confirmed with yet another bone marrow biopsy. It progressed rapidly with blast cells appearing in my peripheral blood. I was put onto the Stem Cell Donor search list. A stem cell transplant was my only option for possible cure. I had no matches with siblings, nor any other donor Australia wide. An international search ensued and a donor that matched well was found in Germany just in time.
At this time, we realised that we could no longer manage a business from home and decided to put it up for sale. Fortunately for us, we were successful in selling – just before I was admitted to the new Royal Adelaide Hospital (on 14th Feb – Valentines Day -2019) to start chemotherapy prior to stem cell transplant. I was sent home on 10th March for a few weeks before the transplant – which was really nice as we celebrated our 24th wedding anniversary at home together. I was readmitted on March 27th for the 7 day countdown to Day Zero (stem cell transplant day) which occurred on 3rd April 2019.
Adding on to the difficulties was the emergence of COVID 19 which started just as I returned home after my transplant. The fear of catching COVID with my severe immunosuppression forced me and my husband to have forced strict isolation at home.
Interestingly, my stem cell transplant was exactly 27 years to the day that I had undergone my splenectomy!
Having had Essential Thrombocytopenic Purpura (ITP) at age 15 actually was a precursor to my development of AML. Prior to my stem cell transplant, I had a series of genetic tests done. This showed I had 3 different gene mutations – a CHEK2 mutation and a TP53 mutation along with the JAK2 mutation.
There is no evidence that I am aware of an association of ITP following having a Mantoux test other than having an abnormal immune reaction.
The CHEK2 mutation is commonly found in MPN’s and other cancers and having the JAK2 mutation is also a clear driver for an MPN. I was lucky to have the opportunity to look back on my life, and with all the mutations and medical emergencies, I feel lucky to have survived. It certainly does shed some light on why my MPN was so difficult to manage.
What have I learned from all of this?
We are much stronger than we realise.
Who the relatives/friends/strangers are that will stand by your side throughout the worst and best days and nights.
There is still a lot to be learned about MPN’s and that each person has different signs and symptoms. There is no “one size fits all”.
There are many far reaching effects of MPN and symptoms should never be discounted. We know our bodies the best and sometimes must advocate strongly for ourselves.
There are people working hard on studying MPN’s finding new and innovative treatments and therapies for MPN patients. This helps to make the lives of MPN patients and their family/friends better and able to negotiate the ups and downs (like a rollercoaster) on their MPN journey.
As much as possible, surround yourself with people who uplift you. Having strong support and encouragement goes a long way in making each day a better one. A positive attitude along with education can empower you to have an active role in your care.
When you come down to making some of the big decisions in your life – don’t let anyone convince you that you are too high risk and probably won’t survive. Don’t rely on statistics to define your journey.
Always go to reliable sources for information. There are great resources available in Australia that have many resources that can help you and your family. The Australian MPN Alliance, The Leukaemia Foundation, groups on Facebook – are all very helpful.
