I'm 42, married with six children, and I live in Australia.
In 2013, I was diagnosed with CAL-R positive ET. I started taking aspirin and managed pretty well.
After a spontaneous bleed in my right quadricep, it was discovered that I also had a genetic bleeding disorder, dysfibrinogenemia.
After that, I stopped the aspirin and was just watchful and waiting.
In 2017, after recurring migraines and burning in my feet, I started on pegylated interferon. It worked well and lowered my platelets.
In early 2022, after an increase in my platelets and symptoms (extreme fatigue, 15kg lean body weight loss, night sweats, bone pain, and abdominal pain), a repeat marrow test on August 18, 2022, identified Post ET myelofibrosis. Genetics were CAL-R type 2 with ASXL1.
I started Jakavi in September 2023, and due to poor response, I was also put on Hydrea and Pegasys to slow disease progression.
After my platelets dropped below 1000, I stopped the Pegasys.
My doctors have recommended that I start thinking about a stem cell transplant (SCT); however, the best donor they can find is a 9/10 unrelated donor.
I'm currently participating in the INCA033989 CAL-R antibody trial but also on Hydrea to try to control my platelets. My platelets are still around 1.8 million.
Symptom-wise, I still struggle with fatigue, constant nausea and abdominal discomfort, brain fog, and am battling to maintain my weight and trying not to lose any more muscle.
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Hatchie
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hello Hatchie, welcome to our forum. I am so sorry to hear that you are suffering so badly with the fatigue and other symptoms, hopefully you get some improvement now that you are on the trial.
I don't know if you already know about a great organization in Australia, MPN Alliance Australia, they have a lot of information on their website which will help you, the team are lovely people, mpnallianceaustralia.org.au/
Also, a member on this forum, socrates_8 is based in Sydney and has a great website - MPN Mate mpn-mate.com/ which also has a MATEs forum where you can be in touch with other people with MPNs. Steve is also lovely and is very knowledgeable about MPNs.
Sorry to hear about the struggles. It sounds like you have a complex case. With platelets that high, you would be at risk for hemorrhage. The dysfibrinogenemia would make this risk even higher.
It sounds like a SCT would be a reasonable course of action. A 10/10 match is certainly preferable, so your caution is understandable. Perhaps you can recruit some people to test and find a match.
Meanwhile, glad to hear you can participate in the CALR clinical trial. this is the only way to move the science of MPN treatment forward. Hopefully, the treatment will work for you or at least buy additional time to find a better donor match.
Thank you both for your warm welcome and thoughtful responses. I truly appreciate the support and understanding during this challenging time.
Maz, thank you for the information about MPN Alliance Australia and MPN Mate. I will definitely check out those resources and connect with others who are going through similar experiences.
Hunter5582, your insights into my condition and the considerations around a potential SCT are very helpful. It's reassuring to know that participating in the CALR clinical trial could contribute to advancing MPN treatment options. I'm hopeful for positive outcomes.
To provide some additional context, all my family members have been tested for a match, and my transplant teams have extensively searched the Australian and international bone marrow registries. I've been advocating for people to get a swab to join the registry, hoping to expand the pool of potential matches for others in need.
Thank you both again for reaching out. I'm grateful to be part of this forum and look forward to connecting more with everyone here.
Hi Hatchie sorry to hear for what you've been going through.I also live in Au, been diagnosed with PV last year at age 35, however accidentally I found blood tests while I was in my early twenties with well elevated platelets back then. Have 2 young children and need to work hard to provide for my fam.
I was previously put on hydrea but could not tolerate due to high toxicity and extreme fatigue.
Now on Pegasys I manage to live near to normal life backed with healthy diet and supplementation. Drinking heaps of filtered/spring water always helps to cleanse my head. Wishing you best on your way moving forward.
To briefly summarize my experience, I'll split my response into categories:
Blood Counts: After three treatments, my blood counts improved overall, with a reduction in platelets and mild improvement in RBC. LDH levels also dropped to a normal range. Two weeks ago, I experienced an inflammatory event of unknown cause, which led to bilateral ankle tendonitis and chest pain. During this event, CRP levels rose along with platelets. I was treated with Prednisone, which raised all cell counts. Platelets peaked at 1.8 million, so I resumed Hydrea to control them.
Spleen: Following the first treatment, my spleen size reduced significantly and is now not palpable.
Constitutional Symptoms: The treatment has effectively controlled night sweats, weight loss, and abdominal discomfort. Severe bone pain events have been reduced, although fatigue remains an ongoing issue.
Bone Marrow: A repeat Bone Marrow Biopsy (BMB) is scheduled in two weeks.
During Infusions: During the first infusion, I experienced mild chest heaviness and instant fatigue. However, subsequent infusions have been without any reaction.
As this is a phase one trial, response times are not yet established.
Wow, it sounds like it works but creates a lot of inflammation. That is interesting. Hopefully the bone marrow biopsy will give more insight and maybe they can benchmark your allele burden to see how much it is helping.
I know how it feels to be a young mom and be faced with health issues. I can’t compare my journey to yours, but I can only imagine how you feel. I am 42 with a 5 year old. It is tough even without an illness! I wish I had advice for you. Other than hang in there and keep us all posted. I am here if you want to vent! I am a good listener. Will be praying for you and your family.
I wish you the best on the antibody trial! Hopefully you have great results. I’m curious about how long you were on interferon? Were you on interferon when you found that you had progressed to MF? How many years were you treated with interferon?
Hi Hatchie, I’m from the uk and one of several SCTers here. I had primary Myelofibrosis for which I had my transplant (10/10 from an unrelated donor) thirteen years ago when I was 58. My story from diagnosis to three months out is online or I can send a link. Happy to chat or answer questions on the SCT process from a patients perspective, if it would help.
Hi Chris, Thanks for reaching out. Please send the link to your story.
I spoke to my doctor again today and the reality is the 9/10 unrelated donor is my only option (unless millions of people with German/English heritage join the donor register).
With all your support efforts what are the lived experiences of SCT with mismatched unrelated donors?
Mismatched unrelated transplants are not unusual. I have spoken to as many, indeed possibly even more, going this route as a familial one. 9/10s aren’t as common but I can think of some. I know people in Oz, NZ and USA (several) who have had their stem cells from Germany where they have one of the best databases in the world; so a shame they can’t find one for you. It is difficult as we are all mongrels with all sorts in our make up. Donors are joining all the time, and you only need one, so things can change. My donor signed up 18 months before I needed her having seen a newspaper story.
I am having trouble pasting a link into this site so will try to message you.
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