My journey with Polycythaemia Vera

[WreckerS26]

Hi, I'm a 71-year-old male who previously enjoyed reasonably good health. my problems began in 2017 when I started getting a tingling sensation in my toes following a knee replacement I had had back in late 2016. Initially it was thought the symptoms were related to my knee replacement, it wasn’t until a few years later after I had I stated getting quite an intense pain in my toes and a purple discoloration in my feet that my GP requested a blood test. Things progressed quite quickly following this & after numerous tests I was diagnosed with Polycythaemia Vera. My main symptoms are quite an intense tiredness, pain in my joints especially my feet, bone pain, itching & worst of all breathlessness having been a very amateur but keen half marathon runner now to be out of breath walking upstairs is hard to swallow.

[hunter5582]

Sorry to hear about the struggles with PV. The symptoms you list are common to this condition. Fortunately, there are interventions that can help. It is very important to consult with a MPN Specialist. MPNs are rare diseases. Most doctors, including many hematologists, have little experience with them. Consulting with a MPN Specialist either as your primary provider or in a shared-care arrangement is essential in order to receive optimal MPN care. Here is a list. mpnforum.com/tsr-the-list/

An appropriate treatment plan can make a huge difference in the secondary/constitutional symptoms we have to manage with PV. While cytoreduction to control the erythrocytosis is an important element of PV care, there is more to successfully managing it. Symptoms like erythromelalgia, joint pain, pruritis, and fatigue also need to be addressed. One thing to address is the systemic inflammation caused by the JAK2 mutation. Many of the symptoms we experience are related to the overproduction of inflammatory cytokines. Issues like the erythromelalgia (pain/swelling in toes) are a microvascular issue. Joint pain can be exacerbated by the level of inflammation we experience but can also be a result of increased uric acid/gout sometimes associated with PV or the medication used to treat it.

Fortunately, many of these symptoms can be successfully managed. I have been managing a MPN for over 30 years. It was ET but progressed to PV about 11 years ago. My care needs and treatment plan has changed over time. When I shifted to my current treatment plan, my quality of life improved. I feel better now than I did 10 years ago.

I have a MPN Specialist, an excellent local hematologist, and Integrative Medicine doctor, and a great PCP on my care team. My current treatment plan includes the following.

Cytoreduction - Besremi. I have responded much better to Besremi than to hydroxyurea. It is more effective and easier to tolerate. It has also reduced my JAK2 allele burden from 38% to 10%. I do need to use a low dose so supplement with a venesection about 1x/year.

Inflammation - Under guidance of my Integrative Medicine doc - Curcumin, L-Glutathione, Pro-Resolving Mediator. This is very effective for joint pain. Works better than any NSAID I ever took.

Itching - which is a Besremi side effect for me. A daily dose of cetirizine controls this nicely.

Erythromelalgia - well controlled with a daily low-dose aspirin. Note that some people need aspirin 2x/day or an alternative antiplatelet medication. That is something to consult with a MPN Specialist about,.

We are all different in how our MPN presents and in how we respond to the treatment options. We each need an individualized treatment plan guided by a MPN expert doctor.

Wishing you all the best and success moving forward.

[Tomsgranny]

Hi there.

My story is very similar! I’m an 80 year old female, I had a shoulder replacement in March 2018. I was then diagnosed with Atrial Fibrillation in November 2018 and then with PV JAK2 positive in May 2019.

I too was fit, walking at least 5 k every day, but with erythromelalgia, joint pains, fatigue and breathlessness I can no longer walk more than 20 yards without stopping.

I have been on a drug trial, taking Ruxolitinib, for the last three years and my bloods are better controlled although the white cells stay stubbornly high.

I am currently have my heart function tested and my haematology team are very thorough, so I just take each day as it comes!

[JP1952]

I understand just how you feel and I send my sympathy. I am 73 year old female diagnosed in 2019 , I also was a runner , 3 times a week and, loving the Saturday morning buzz of park run and the 10 Kms and half marathons - only managed 2 in the end. Also a keen gardener and helping my children however I could.Like you symptoms are fatigue, lack of energy, bone pain, and breathlessness. The itching disappeared with Rux so was a Godsend but I still have burning, swollen feeling in my legs.

My life is still good and compared to many I am lucky. I hope you have good times in your life too.

[Bluetop]

Sorry to hear the PV has had such a bad impact. I am 73, daignosed with PV 9 years ago and have since been treated with hydroxycarbonate.

This treatment has been succesful in managing my blood counts -although the medication does need adjusting from time to time. I am very fortunate not to suffer from PV apart from erythomyalgia in my fingers and toes and deteriorating nails. I have no idea for how long the hydroxy will work for me and always keep up to date with the interferon option (Pegylated). I take daily 75mg asprin and I use curcumin as back up to manage inflammation.

I hope your haematologist can help you to better manage your symptoms.

[gjh8733]

Welcome WreckerS26 to our Club! I can relate to your history regarding being always very active and keeping fit. I was diagnosed at age 62 ( 5 yrs ago) after being checked out for persistent headaches and swollen red feet

Treatment wise I have tried Hydrea and Besremi - both had significant side effects - and am now on Jakafi for the last 9 months. It has been a Godsend for me as am back at the gym and am outside biking 3-4 times a week. Fatigue and joint pain are gone and have regained most of the lost muscle mass albeit with some additional fat which is a side effect of Jakafi.

[saltmarsh]

Lots of solid input here for you to consider. I'm 77 with pv for 5 years; taking HU for the last 3. I'm pretty active but find I need to juggle my vitamin supplements (multiple, D3, B12 and iron) to stay ahead of the fatigue. Trust me - for most of us there is a path - we just need to find it. And you definitely need to see an MPN specialist. Good luck and stay positive.

[Pogm]

You are getting great advice on this forum and will continue to do so. We are all in the same" ballgame". I was diagnosed with PV following an episode of Atrial Fibrillation in 2018. I started with just phlebotomies and blood thinners, but the pruritis was unending. After many consultations with a MPN specialist, I started Besremi and it is helping. I also use a small daily doseof Jakafi to control the itching. MPN specialists are invaluable, you and they will be able to work out the best treatment plan. Good luck and each day is a blessing!!

[Peter18]

I too am 71 years old and I was diagnosed with PV in early 2018, being prescribed daily hydroxycarbamide + low dose aspirin which I have been taking ever since. Tiredness and sporadic aches in limbs have been the most noticeable symptoms, along with occasional blister-like blotches on toes which can take several weeks to fade. Apart from such trials, morale has remained good, D.G., despite change in life-style. Very best wishes to you.