Myocarditis

[GoldieR]

Good morning all

I haven’t posted before but thought now was a good time. I was diagnosed with PV JAK2 positive in December 2022 when I was 61. Up until February this year, I was only taking aspirin and I’ve had a few venesections.

I am currently under shared care with Guys Hospital, together with my local haematology team. On the advice of Guys, I started taking Besremi this February on a low dose of 100mg every 28 days. So far, I’ve had 3 doses, my last one on Sunday 30 March.

On Friday afternoon, 4th April, I started having quite severe middle chest pain, like a tightening feel, and felt a bit breathless. This followed a slightly stressful episode at work so I thought it would subside when I got home and rested but it didn’t. I went to A&E at my local hospital. ECG was fine but my troponin level (cardiac marker) was very elevated (2000). They suspected a heart attack so was admitted and I am currently in the cardiac ward. I had an angiogram on Monday which was completely normal, no arterial blockages so unlikely to be a heart attack.

I had a cardiac mri on Tuesday which shows inflammation of the heart muscle (myocarditis). I am still waiting for the full report so still an in patient and the doctors are waiting to speak to Guys. My question and the question of the doctors is whether the cause is linked to Besremi and/or PV. I have been in touch with Guys about this and will be speaking to them today. Heart problems are listed as rare adverse effects on the very scary Besremi information leaflet! It’s a bit of a mystery as other causes could be post virus, cold or COVID but I haven’t had a virus recently as far as I know. Just wondering if any of you could shed any light on this

Sorry for the long, rambling post! Any of your thoughts would be very welcome!

Best wishes to you all

[artydutch]

I had a heart attack before I knew I had MF. My troponin levels were high but angiogram showed no obstruction at the time. The cardiologist suggested it was a blood clot causing the MI. Once diagnosed with MF the hematologist suggested the MI was caused by my MF as this causes sticky blood. It may be the MPN rather than the Besremi that caused the MI. Good luck.

[GoldieR in reply to artydutch]

Thank you for your reply. Sorry to hear about your mpn journey. In my case, it is really impossible to prove whether it is the mpn condition itself, the Besremi or something unrelated that caused the myocarditis but to be on the safe side I have been advised to come off Besremi as it could have been a very rare adverse effect. I just hope it resolves itself soon.

Best wishes

[hunter5582]

Sorry to hear about this complication. You were wise to seek care rather than just ignore it.

While there are some rare Besremi cardiac adverse effects listed (cardiomyopathy, MI, arrhythmia), Myocarditis is not one that is commonly listed. It may be possible that it is a very rare complication. Best to get expert consultation on that.

Given that myocarditis is an inflammatory condition it is reasonable to wonder whether there is a link to PV, which is also an inflammatory condition. Other aspects of PV may also bear on this issue. It seems possible the PV could predispose to myocarditis even if it is not a common complication. Best to get expert consultation on that as well.

It is always possible that the myocarditis is not directly related to the PV or the Besremi. However, the body is an integrated system. "Unrelated" conditions can interact and exacerbate symptoms.

Please let us know what you learn and how you get on.

[GoldieR in reply to hunter5582]

Thank you very much for your reply Hunter. I am out of hospital today. I have just spoken to an MPN consultant at Guys who advices me to come off Besremi as it could have caused the myocarditis although it is an extremely rare adverse reaction. I will be closely monitored instead and will be repeating the cardiac mri in 3 months and have regular blood tests to check for troponin levels. Hopefully, it will subside soon and that it is an acute rather than a chronic form of myocarditis. I will keep you posted.

Best wishes

[Jennytheb]

Sorry to hear about all of this, was a further disappointment for you having been diagnosed.

I am also Jak2 positive ET and am very well suited to Hydroxycarbamide but it will be good for you to have a thorough check up and hopefully some good news for you going forward.

This will be an excellent site for you, we are all in it together on here!

All the best, Jenny

[GoldieR in reply to Jennytheb]

Thanks very much Jenny. Yes this a fantastic supportive group for this very complicated condition. Everyone responds differently to the various treatments so it’s very much trial and error. I will probably take hydroxycarbamide as the next step after being monitored as I would have to try that before being considered for any other newer treatments.

All the best

[Jennytheb in reply to GoldieR]

Wishing you the best going forward!

[SarahPV]

what a shock for you. No advice but of course others have weighed in on this fantastic forum of ours. All good wishes to you xx

[GoldieR in reply to SarahPV]

Thank you very much xx

[Alex9621]

I was on Besremi from March to November of 23. In October I started off with a UTI that went into the kidney. That cleared up then I had slight fever spike of and on for 2 months, lost 35 lbs, and finally 1-1-24 landed in the hospital with fluid around the heart. I was put on some medication the cleared my heart issue. The doctor recently told me I would never be a candite for any interferon again.

[GoldieR in reply to Alex9621]

That sounds like a very rare reaction, I ve also been told I won’t be a candidate for any interferon either. What do you take now?

[Alex9621 in reply to GoldieR]

I have not been on any medications as of yet. I have just been doing the baby aspirin and phlebotomy. My symptoms have increased, and I am now 61 so my doctor is stating he wants me to possibly start Jakafi or Hydroxy. He is stating is about quality of life now. I am very hesitant as I am a little scared after being on Besremi and I do have other medical conditions. My iron levels have also gone down recently but I have not had a phlebotomy since November. I want to try to see if I can manage the symptoms on my own by drinking more water, eating a little better, and moving more. I have slowed down a lot from fatigue, but I think if I push myself, I can do this without medications. Sorry for writing more than asked I have just been mulling all this over for a while now!

[GoldieR in reply to Alex9621]

Thanks for your reply. I was very reluctant to go any medications other than baby aspirin and the occasional phlebotomy since diagnosis until this February when I started Besremi on the advice of Guys. I will also now have to consider what to try next, other than the wait and see approach. Drinking lots of water, a healthy diet and exercise does help. Good luck moving forward!

[conno61]

I wouldn't be surprised if it was the "medication", I was taking peg interferon for ET and it gave me severe heart failure.

[Janis12 in reply to conno61]

I remember your response to my post relating to cardiac symptoms i was experiencing after about 9 months on peg interferon. I was so grateful for the tip off, I stopped taking it immediately, contacted haematology who agreed, was referred to cardiology who carried out loads of tests and could not find anything untoward. Interestingly, about a month after stopping the drug the symptoms vanished. I think I had a lucky escape which was a result of the incredible folk on this forum passing on their own experiences, I am so grateful for that. Best wishes to you.

[conno61 in reply to Janis12]

Glad my bad experience and story helped you. The response from the NHS medical professionals has in my experience been dire. They initially all agreed it was the peg interferon as there was no other explanation, but have since closed ranks and won't say for definite. I think they do this incase you sue them. Anyway, good luck on your journey, I've opted for no "treatment" going forward as all they could offer was hydroxy with no idea when I asked, how that would affect the heart medication I now have to take. I could just forsee another disaster !

[EPguy in reply to Janis12]

Your quick cessation was wise and as you say is likely a reason your condition was "self limiting".

[GoldieR]

Sorry to hear that, that is a very extreme severe reaction. How scary! I hope you have fully recovered from that. How long were you taking Peg for before that occurred? My mpn consultant believes it was a very rare side effect for me so I won’t be a candidate for interferon.

[EPguy]

Cardiomyopathy is listed as "Rare" in the Euro Bes label, page 44 here:

ec.europa.eu/health/documen...

They have a scale, Very Common, Common, Uncommon, Rare, Very Rare, and Unknown.

We see in this thread two members with the Rare Cardiomyopathy on Bes. I suffered a severe and permanent "common" side. More indication we should mind these adverse listings in the label.

The medical term for never again is "no rechallenge". Dr Gotlib had that for me.

[GoldieR]

Thank you for that information. Yes, 1 in 1,000 is considered “rare”. Even if there is a doubt about the cause, as myocarditis isn’t listed at all, I guess it’ll be a “no rechallenge” for me.

[EPguy in reply to GoldieR]

Good point on the distinction of myocarditis vs cardiomyopathy. I was not clued in to that. I was looking at the broader application the label added "diseases that affect the heart muscle" of which both fit. Looking to why the broader inclusion this report might be one source:

"Cardiac toxicity (with Peg-IFN) is rare and presents as arrhythmia, myocarditis, HTN, ischemic heart disease, pericarditis, pericardial effusion, and rarely, cardiomyopathy...aborting pegylated interferon therapy prior to irreversible damage"

journals.lww.com/ajg/fullte....

That part about aborting is where I messed up, but my case was not ambiguous.