Ectopic beats

[lynnieb]

My heart often has runs of bigeminy, with no clear cause. I have ET Jak+ve, with platelets usually around 1200. Another condition (neurofibromatosis) makes medicating me tricky so I just take Aspirin. Does anyone else have experience of this? I

[hunter5582]

Welcome to a very small club, people with both a MPN and NF. I have Neurofibromatosis Type 1, variant NF1:c5425C>T. I also have PV (used to be ET) with the JAK2v6217f variant at a 26% mutant allele burden.

Like you, I also have an arrhythmia. I developed paroxysmal atrial tachycardia. I did not tolerate the meds well so opted for surgery in 2018. It was successful, but I still get occasional mild sinus tachycardia. I am at risk for progression to A-fib, so I do monitor this situation.

The NF1 lead to a very rare adult pilocytic astrocytoma in my occipital lobe. We could not determine what is was on imaging and it appeared to be a possible higher grade tumor. We could not biopsy it as it had been hemorrhagic. Possibly due to the MPN or aspirin. I opted for surgery to have it removed in 2019.

It has been an interesting couple of years. I have learned a lot about the underlying mechanisms for both diseases. Both are kinase-based disorders. The RAS-MAPK pathway involved in NF1 is downstream from the JAK-STAT pathway. The RAS-MAPK pathway is also involved in MPNs. The implications of having both disorders is not well understood. The database is just too small. Every time I ask about it, the specialists say "That is is really good question."

When managing both NF and a MPN it is very important to have an integrated care team. We have to be out own care managers and advocates to ensure the integrated care occurs. I base my specialty care out of the same place - Johns Hopkins MPN and NF centers. I also had the brain surgery done there. This does help with keeping care coordinated. However, I have "ologists" in other places and work very assertively to keep all care coordinated.

Regarding meds for MPNs, the NF may or may not be a factor depending on your NF treatment needs. What is clear is that NF1 is a non-driver mutation that impacts the risk of progression of the MPN. This was one of the factors that lead to my decision to use PEGylated Interferon.

All the best to you walking this path. Not many of us are on it. It is good to have others who understand what it is like. That is the great thing about this forum.

[Virginia91 in reply to hunter5582]

Hunter you are an amazing and generous person.🙂

[Stephen399b]

Morning Lynnieb and Hunter

I have ET Jak2+ and was also diagnosed with Prostate cancer in January.

Like you I am having to be very careful with meds and treatment for each condition, so my haemo and oncologist work together on this.

In a routine BP/pulse check we found that my heartbeat was down at about 35bpm in one reading and it turms out that I have an ectopic heartbeat. I had the 24 hr monitor which showed that this was happening about 45% of the time, which apparently is very high?

I am due another consult with the cardiologist this week as it seems to have settled down but will probably have another 24hr trace. No symptoms that I can put down to this, as already tired from other meds. The cardio suggested beta blockers, but because of the other 2 conditions I am loathe to take anything else if I don't need too.

Interested to know your experiences

Stephen

[EPguy]

I have ET Jak2,. I'm on HU (~500/day) and aspirin. Blood numbers all good. I've had Ectopic beats (thanks for the new word) since 30 years ago. It could go for many minutes continuously. But it was ok for a long time till I started the HU. I've had cardio workups before and no worries and recent EKG was ok. Another full workup is scheduled for this month. But I don't have NF so maybe not comparable.

The sensation of Ectopic beats goes to my neck left side and it's most annoying. Based on the last year I think the HU has something to do with it.

[Fluffylittle]

I know this post is a few months old, and while I'm not currently on treatment for my ET (possibly masked PV.. this is currently up for debate), I am on a beta blocker to control my ventricular tachycardia. I also have a history of AFib. Hunter5582 (posted above) is actually my dad, and I'm in that club of NF and having an MPN. I would make sure your cardio team is looped in with your NF doctors as well as your MPN doc. It is really crucial that everyone is on the same page when treating comorbid disorders. I am fortunate that all my doctors are able to communicate easily when it comes to managing my myriad of issues.