Hi Scott. I will be painfully honest. I have PV and read in the Social Security Disability Benefits website that PV left untreated can be fatal within 4-18 months. I do believe my test results reflected I had PV at least 7 years before diagnosis. I am still here by the Grace of God with no treatment for that long. Hopefully, that’s the case for you as well. Thank the Lord you are still here because you too could have possibly lost your life due to delayed treatment. I am not that knowledgeable about ET, but I think it would be helpful for you to investigate this matter on your own by researching it via the web. Let us know if you find anything…I highly recommend the American Society of Hematologists website as well as the resources found here.

My diagnosis was Primary PV, with Jak2V617F mutation. What really concerns me is my Father of origin (I had a stepdad too) died with CML. CML is considered one of the MPNs. I wonder sometimes if I inherited this mutation from my Father. Do you know if anyone else in your family has anything similar? I have lots of people on my maternal side with cancer…so many it’s unreal. Even my Stepdad passed in 2021 with liver cancer. Scott, I am glad you are doing better now. It would be very interesting to me if you find any info on delayed treatment. Sometimes I think we will never know the answer.

Dear Scott,

Many people suffering from MPNs have a story similar to yours. Years on end of hight blood counts that were dismissed.
In my case, my thrombocytes were abnormal since 2012/2013 and I was diagnosed after an uphill battle to get this diagnosis in October 2020. I was 32 when the values went up and stayed up, 38 when the investigation started (genetic tests, BMB), 39 years old when finally officially diagnosed.
The issue with staying undiagnosed for so long is that we are put at great risk. It is true that in people younger than 60 years old w/o JAK2 mutation or a previous clotting or bleeding incident the treatment is very conservative, i.e., low dose aspirin, but we are all monitored and when the blood counts and other tests start showing changes, action can be taken.
Left undiagnosed, people end up with strokes, embolisms, DVT, heart attacks, etc., that could have been prevented via monitoring and timely treatment.
Given that this illness also causes chronic inflammation, this will affect the overall health of the body too. So having the diagnosis can also help keep an eye on the health of other organs and systems in the body too.
The worst part of being left undiagnosed is that in some people ET and PV can transform into myelofibrosis. This can be life-threatening illness if left untreated.
I just learned recently that I inherited my non-canonical MPL mutation from my father. He was never diagnosed with ET, as health care did not know of MPNs and thought he had high platelets due to his breast cancer. After chemotherapy for breast cancer, his platelets normalised for a while. When chemo stopped, they creeped up and health care passed him through endless scans for solid cancers. He had ET and after some years it progressed to myelofibrosis. When he died, he barely had any type of red, white blood cells or any platelets. He suffered tremendously, as lack of diagnosis meant no treatment.
MPNs might be rare chronic blood cancers, but they are not rocket science and not having a timely diagnosis can lead to unnecessary suffering and loss of life.

Best regards,
Tatiana

Scott,
I don't believe that treatment with HU and Jakafi have shown to prevent progression of disease. So delay of treatment may not be as damaging as feared. If your condition was severe, you'd have had a lot of symptoms without treatment. Platelets of 600 and 700 may not be that high. I think high wbc, rbc and platelets can all increase risk of blood clots. So if you got past the risk of clots without having clots, you may be controlled. What does your doc say about this topic?

Hi Scott. probably if you had been treated with hydrea during these years it would not have evolved to myelofibrosis