Anyone living with Essential Thrombocythemia with JAK2?

I just discovered this forum, and find it gives me so much information. I have ET. I have been on HU for about 19
years. Recently I have been dealing with an ulcer on my ankle that isn't healing, which I understand is a side-effect. My dr now wants me to stop the HU and start on interferon injections. Has anyone else had this experience? I do not want to start this new treatment, but unsure of what to do. I would appreciate any suggestions or options. Thank you for sharing.

Hi, @dewz13 You pose an interesting question! “Does anyone know if we can still donate organs with ET/Jak2?”
After reading several articles I’ve gathered that the risk of transmitting the JAK2 V617F mutation through organ donation is considered low, particularly if the donor has a well-controlled condition. However, the mutation can be present in the blood and may affect the recipient's health, especially if the recipient is already at risk for blood disorders.

There are several clinical papers discussing liver transplants with patient who receive livers from donors with ET -JAK2 V617F mutation.
One such paper is of a woman who had Essential thrombocythemia who passed away and could potentially be an organ donor. The recipient of her liver was a 68-year-old male with a history of end-stage liver cirrhosis secondary to alcohol consumption. He was given all the facts regarding the possibility of acquiring ET if they went ahead with the transplant. The doctors felt they could treat this condtion post transplant, if necessary. The patient was willing to accept the risks.

The transplant was a success, the JAK2 Mutated genes did show up in the patient’s blood test the day after transplant. But dissipated with subsequent blood work.
This led the doctors on the case to report, “We believe that it is reasonable for organ procurement organizations to offer organs from donors with JAK2 V617F mutations for transplantation into informed willing recipients within programs that provide appropriate follow-up, and in particular in situations in which the benefit from the transplant may supersede the potential and theoretical risk of MPN transmission.”
Here’s a link to that particular article from Hepatobiliary Surgery and Nutrition: https://hbsn.amegroups.org/article/view/23618/html

There are also research papers wondering:
Is it time to revisit contraindications to organ donation from donors with a JAK-2 mutation?
https://pubmed.ncbi.nlm.nih.gov/25778631/
Whether this is accepted across the board that’s hard to say. Not being a medical profession but just talking off the top of my head, I think with the demand on organs being high and availability low, that the transplant team would assess the potential side effects of going ahead with the donation if someone has a mutation with the JAK2 gene.
I think a reasonable team would weigh the long term success of the transplant against the risks before offering to the patient receiving the organ.

In my Allogenic bone marrow transplant, I received the stem cell donation from a donor. I was informed that my donor carried a latent virus in his system which I did not have. But that after the transplant I would most likely carry this virus as well. And I do! But my immune system is strong enough to prevent the virus from resurfacing. This donor was my 1 in 22,000,000 chance for life! I wasn’t going to toss that away for the potential of carrying a latent virus.
It think sometimes when it’s a choice between life and death, our ‘2nd hand’ organs or cells may come with a little baggage. But if the baggage can be safely ‘stowed’ away then I’m expecting the organs may be used. I know that I cannot donate organs because I carry 2 sets of DNA and that would be unpredictable for a recipient.

Bottom line, it would be best to consult with a healthcare professional or transplant center for personalized guidance regarding organ donation eligibility based on specific health conditions! How’s that? ☺️
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@loribmt
My husband (in remission with CML Leukemia) was accidently infused with infected platelets. He went into septic shock and died within 24 hours. They said the chances of that were- some astronomical number against- but it happened. So when I see incredible odds against something, I just blow it off. Cause if ---1 in a million chance---- and your the one............doesn't much matter.

@loribmt

Yes we are waiting to
Hear back from them.
Any tips.

@janemc you have brought me to tears. I really appreciate all the support that is here on this site. It’s been incredible to be a part of this so far. I’m hoping I could be just as supportive. Once I learn more about the Mayo Clinic and what happens there I will detail it here for anyone else who might need it

@sckf I was on HU for 9 months. Suffered with a variety of symptoms. Glossitis, bleeding gums, tingling feet, brain fog, repeated UTIs, bone pain and extreme fatigue. Oncologist/hematologist would never acknowledge that this was all from HU.
Self referred to NPM specialist at Cleveland Clinic. He listened.
Put me on HU holiday. It’s been 6 weeks off HU My symptoms have disappeared except for bone pain. I’m 90% better.
Will start interferon once my platelets rise steadily above 600.
Seek out an MPN specialist if you have not. They are much more updated on better treatment!

Hi, @bobbiejo80 Sorry to hear you’re also a headache sufferer with hydroxyrea. You mentioned you’ve been able to back down from your original higher dosage to now just 4 days a week. Did the reduction help with the daily headaches?

@jodyjazz I’m so sorry for the tragic loss of your husband. Occasionally people may develop hives or anaphylactic reaction. But having septic shock is really very rare. I can’t imagine how devastating this was for you, along with your husband’s medical team. Again, my condolences to you and your family.
Do you know what the platelet contamination was?

Hi @dmcj. If it’s been several days since you requested the appointment you can contact Mayo to see if everything has been received.
Here is a link to the ‘request an appointment’ page. It will have the phone number for you to call.

Was your daughter’s referral from a physician or was this direct contact from you or your daughter?
A physician’s referral isn’t necessary but I’ve heard that it can be helpful.

It’s ok to be the squeaky wheel. ☺️ How long has it been since the request for appt was sent?

@loribmt
My husband (in remission with CML Leukemia) was accidently infused with infected platelets. He went into septic shock and died within 24 hours. They said the chances of that were- some astronomical number against- but it happened. So when I see incredible odds against something, I just blow it off. Cause if ---1 in a million chance---- and your the one............doesn't much matter.