My condition was getting worse during 2011 despite playing tennis and soccer. Thought it was age (was 52 at that time), but after a blood draw and bone marrow biopsy I got the diagnosis: high risk myelofibrosis with a life expectation of 1 year.
Almost immediately we decided to start the stem cell transplant procedure. Life quality with high risk MF was too minimal and we thought it was worth the risk.
Now, 5 years later, I think we didn’t realize the high risks of this procedure at that moment.
Streptococcus A bacteria.
After 2 months we got the green light. They found 3 donors with 100% match. Unfortunately one day before admission to hospital (Feb, 2012) to start the transplant I got streptococcus, a bacterial infection due to low white blood counts. It almost killed me in 24 hours. Admitted in ER, for an operation because the only way to get rid of this bacteria is massive antibiotics and exposure to oxygen. Most common, this bacteria is on arms or legs and very often the only way to stop it is to amputate the arm of leg. So very, very scary. The most scary thing I had in my life. Fortunately the bacteria was on my upper chest where they removed skin from shoulder to shoulder and 20cm wide. It healed well and looks like a skintransplant from a burnwound. Six weeks after the operation I was discharged and recovery started
Of course the SCT was postponed. I recovered, bloodcounts remained bad but stable and we started some vacations with the family knowing there would come another moment when we should start the procedure all over again.
There was an opportunity to start with a clinical Trial, pacritinib. I volunteered for it because I was hopeful this would be a solution to change MF to a chronic disease and I would avoid the risks of a transplant. The pacritinib trial did not work out well (got toxic) and we stopped it. Also I became transfusion dependent due to low hemoglobin..
Beginning of 2014 we started ruxolitinib for several months but it did not improve quality of life.
In 2014 my blasts started rising. MF was morphing into AML. It was not a surprise as we saw an increase of blasts during the regular blood draws. So always keep an eye on increasing blasts!
We decided on two objectives:
As both objectives are life threatening, I arranged my last will and some other stuff before admission.
First get rid of the AML, and second, one week later, start the stem cell process. After the first procedure a BMB revealed that AML was gone and we started the SCT procedure.
There was no doubt to start this. It was do or die. Blasts were 68% (normal is <5%).Two days after transplant, and with white blood counts as low as zero, I got huge heart and lung problems. Was transferred to ICU where they worked to save my life. CPR, tracheostoma, artifical respiration.
May 15, 2014 is the day of my rebirth. Nurses and doctors were very helpful and informative during all these weeks. During transplant family and friends were not allowed in my hospital room because of my vulnerability.
A SCT with an MF patient is slightly different and more difficult than usual. First the spleen needs to shrink, otherwise it will surge all the new stem cells, even before they can graft in the marrow.
Second, there is scar tissue due to MF which makes it more difficult for the stem cells to engraft. So it is important to choose a hospital and a doctor who is not only an expert in SCT but also in MF.
First they put a PICC line in, which makes blooddraws, medication and chemo much easier to be done. The chemo consists out of different kinds. ATG, a “rabbitvirus,” three times in 3 days, together with a high dose of prednisone. I felt sick, feverish and couldn’t sleep due to the high dose prednisone.
Next there was a chemo called melphalan and a chemo called fludarabine. During all these weeks I was still on ruxolitinib because it also affects the spleen size.
It all did its work. My bloodcounts dropped and my spleen shrunk to normal size (and it was huge, bottom was below my bellybutton). We got the green light to let the stem cells go.
And after…GVHD kidney.
The second half of 2016 my health declined. It appeared to be GVHD affecting the kidneys. Very rare complication (1% of the patients). And again we got it under control with prednisone and immunosuppressants.
After 6.5 weeks in Intensive Care, I was transferred to a normal hospital room and 4 months after admission I was discharged. Weak, no muscles at all. Could not turn in bed from one side to the other. We started rehabilitation with physical therapy.
Took half a year to learn walking again. First with walking aid and wheelchair. October 2014 I got graft versus host disease(GVHD) on my skin, mouth and liver. Fortunately we got it under control with immunosuppressants and prednisone which could be stopped after a year.
Costs and travel.
The costs for all medication, procedures for AML and sct, PT and PCP are covered by the mandatory health insurance in Holland. We pay about 110 dollars a month and have a copay of 415 dollars a year. We live 20 miles away from the hospital so it was easy to travel.
No more work for me. Chemo and complications have done too much harm, but definitely I do not regret the decision for a transplant. It was not easy, but sure worth it.
After getting dx high risk MF it was impossible to work, and also after the sct and all complications it is not possible to work anymore. In 2016 I had 91 appointments in the hospital. And this year already ove 30. Main complaints are fatigue and lack of concentration dus to chemo and complications.
I had a great job as an CIO in a hospital I was responsible for all ICT ( Spell out) and telephony in 1 hospital. That job was gone very quick. But fortunately I am never bored and started some new challenges which I can do whenever I feel better (and I am happy there are moments I feel better).
A lot of my time, and satisfaction, I put into a Dutch MPN FB group. Just with one goal. Learn from each other to get the best available quality of life. Creating awareness among Dutch MPN patients that MPN is very rare and that they should stay in charge of their treatment and be an active partner with their doctor.
June 28, I am organizing a sponsored boat trip on a sailship with 70 Dutch MPN patients where they can meet, talk, eat, drink and exchange experiences.
The boat trip was sponsored by the Dutch MPN association and “Vaarkracht,”, Dutch initiative to give cancer patients (and sometimes also their caregivers) a day with joy and no worry.
I am very happy. I have time to rest and relax (watch TV) and visit hospital when necessary and I can do some nice things when I feel better. It has been, and still, is a rollercoaster. After my kidney GVHD I am not so convinced there will be no problems anymore so if possible we try to enjoy every minute, every day of our livesHope this can bring more clarity in who I am and what I am doing while still recovering from sct and complications. Bye and greetings from Holland.
Most important advice Don’t wait with enjoying life till later, because if later comes earlier it is too late. That is what these last 5.5 years have brought me. And stay positive. It will contribute to a better outcome. There will be bumps but you can beat them!