Does anyone with an MPN have a diagnosis of this? Soon after my ET diagnosis and starting HU 8 yrs ago I noticed shortness of breath with mild chest discomfort when walking fast and I dismissed it as possible HU side effect. The clinic treating me at the time said no, more likely lung scaring from previous pneumonia even though I’d made full recovery from that.
Gradually symptoms worsened and couple years ago I had an extensive range of lung and heart tests but still no firm diagnosis. The nearest I’ve got to one is: most likely coronary artery spasm or coronary micro vascular dysfunction. I was discharged back to GP with heart meds which sometimes help but also have side effects which then required more meds on top.
With the various lockdowns and difficulty seeing GPs face to face I’ve stopped some of the meds, pretty much just got with it but it does impact on quality of life, never mind the ET. A recent chat with a GP didn’t resolve anything and I’m waiting for a face to face appointment where I’m expecting to be told not to mess with the meds and to up the dose before I can to referred back to cardiology.
It’s likely a coincidence that these symptoms started just after ET diagnosis but who knows? I’ve learned my probable vascular condition is difficult to diagnose at local level and can only be done in a specialist centre so my just wonderful MPN clinic are going to look into a next step for referral to a specialist.
For now I’d be interested to know if anyone else has any experience of cardiac micro vascular dysfunction or vasospasm alongside their MPN.
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Rem31
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My angina came first Rem31 and though my platelets were noted to be a bit high it was not investigated.
One of the first pair of heart scans in 2001 did show patchy perfusion, but nothing major was blocked. ECGs were always normal because the pain had eased. Lung scans were clear. One doctor tried to say it was the rib muscles or indigestion, but I know those pains. The cardiologist said it was most likely microvascular spasm, or Cardiac Syndrome X.
Every time I was admitted more drugs were added until my pulse was around 40 bpm which really upset the pulse monitor.
A few years later, after ET was diagnosed, and another resting heart scan did pick up an altered ECG as drugs were injected and the pain got worse. The new Consultant then said the major blood vessels were not blocked so there was no surgical treatment, and he stopped most of the drugs and discharged me.
I am on 40 mg of statins which is to increase the elasticity of the blood vessels? and on Bisoprolol. I had to retire early on health grounds. The frequent episodes of chest pain/ hospitalisation/ absenteeism, meant that I was, “more trouble than I was worth,” in a busy NHS hospital. My colleagues said I went very white and quiet before an episode.
I believe the crushing chest pain and breathlessness is related to ET, though who in the NHS does joined up thinking?
One thing I have found is that staying very well hydrated reduces the frequency of the episodes. The sudden sweats with ET can be followed quickly by crushing chest pain. Sometimes drinking glass after glass of water can stop an episode before it builds up. (Dehydration= drinking lots of water and still not needing to wee for hours.) The worst time I had in A&E was 11 hours on a trolley with no water or IV. I keep GTN to hand, but it does not work every time, and it drops my blood pressure so I feel sick and shaky.
Retiring, however reluctantly, was the right decision. Less stress, regular meals, more sleep, regular and not too strenuous exercise. Overdo things and the next days are full of pain and exhaustion. Avoid getting cold.
You have my sympathy having two possibly related conditions which nobody knows about. It is comforting not to be alone in this frustration.
Oh my, you have been through the mill. Only recently have I woken up to the need for a proper diagnosis and some help dealing with symptoms but have now found it’s not straight forward.
I wasn’t looking forward to seeing my GP about a referral and so it a big relief the MPN team say they’ll be arranging for one to a cardiology team in the same Trust and which has expertise around micro vascular and vasospasm symptoms, aka cardiac syndrome x. I believe they are also involved in research.
If you haven’t seen it already have a look at INOCA International website.
I found it via British Heart Foundation and their 2019 patient forum videos are enlightening. There are consultants describing and decrying the treatment you have experienced and who are working to improve the patient’s lot.
Currently there’s no evidenced based treatment and, if I understood correctly, no NICE protocol for diagnosis. There’s a lot there and I haven’t viewed all the videos yet.
It’s very reassuring to know I’m not alone here. Although there have been some worrying moments I’ve not yet ended up in A&E with it but I do now keep the GTN spray close.
I certainly agree about water intake. Tiredness, exertion and breathing cold air are my main triggers. I’m interested that you feel your symptoms are ET related. If I get to the root of mine, I’ll be posting an update.
I have not experienced that myself, but it is reasonable to assume that MPN related mi9crovascular symptoms could affect the heart. Here is one article to that effect. onlinelibrary.wiley.com/doi...
That does not mean that this is what is going on.
Given how little most docs know about MPNs, I would be sure to follow up with a MPN Specialist. I would not assume that this is an issue of microvascular dysfunction. The dyspnea could still be from another ET-related issue, and/or something that the HU is making worse with time. Some adverse effects do get worse rather than better. The key to figuring it out will be working with a MPN Specialist to figure out what is going on.
Thank you and for the link. You’ve got me thinking about questions to be asking. I’m confident I’m in good hands with the MPN team treating me as its also involved in research but, as my ET has been fairly stable, all my appointments since the start of the pandemic have been by phone which isn’t ideal. My next one in November will be in person and it will be easier to raise more detailed questions then.
They are referring me to the cardiology department (also involved in research including Microvascular symptoms) within the same hospital group so at least the communication link between the two teams should be good, I hope.
I wonder how much around patient care gets missed because medics don’t communicate across different specialisms and work largely within their own silos while patients are silently overwhelmed and struggle to know what to ask.
My referral won’t happen quickly as Covid has caused an enormous backlog with huge waiting lists in the UK.
That sounds like a really good plan. Having a MPN Specialty care team in the same institution as the Microvascular-cardiac team is a great way to go. I cluster some of my care at Johns Hopkins in the same way. It make a big difference. You still have to act very assertively to to ensure collaboration, but it is easier when the providers work in the same system.
Crotchety. I have PV jak 2 +.and now waiting for an angiogram to check for angina. A year ago I had a stroke like episode which left me with involuntary movements of the arm, now less uncontrollable. My MPN specialist thought it might be a microvascular stroke. ( It wasn't arterial) and wanted tests to be done but the neurologist, very kind person,- different hospital - felt it was PV related so those tests not done. Angina like symptoms have now become worse and from walking for miles have had to stop badminton and cannot walk up hill without chest pains. I fight against it - I have a happy, busy, social and family life but social efforts for one or two days, also trying to disguise how I feel and live up to my previous reputation as a mother of five, grandmother, long term career, always able to cope, leave me exhausted. I also have night sweats and sometimes daytime from walking. My view is that this angina is microvascular too. I am slightly underweight though this has stabilized since suffering weight loss over the last years. I have always been on the thin side anyway. I am 75, and on hydroxicarbamide, aspirin, various asthma drugs and now angina drugs. I would like to now whether I will have to stay on the angina drugs- haven't gone on all four yet as NHS having difficulty with supplies, or can microvascular problems be treated in any other way. I sympathise with other MPN patients with similar problems. It's nice to know I'm not alone!
Hi crotchety, you also seem to be having a tricky time. Like you I’ve no difficulty with weight, was always physically busy and with full on work life. Low/average BP and cholesterol. Gardening and long rambling walks were my thing until fatigue and ET took hold. Now my walks are more limited and the gardening gets done with frequent breaks. I think that’s largely down to the vascular symptoms. I’ve always associated my fatigue with ET but am now wondering maybe both conditions are at play.
In my relief at the angiogram showing no obstruction I thought a few pills a day would be sorting out my new suspected diagnosis and so I attended the follow up appointment with only one question: what’s the prognosis? Excellent. I accepted it but I have no idea what that answer was based on and there was no further opportunity to ask anything more as I was discharged. My mistake was not thinking ahead and going prepared. I might not be back at the start again had I been more questioning and maybe assertive.
I wish you well with the angiogram. Let us know how you get on.
Hi, Rem31, I'm sorry you have had no useful feedback. I don't know if there is any treatment for microvascular problems other than these pills, which cause side effects themselves. The thought of being on these pills for ever is not good unless they do make me less breathless and less exhausted. Part of the problem is that each health problem is dealt with by different hospitals. I had thought the angina was asthma but mentioned to the nurse that the inhaler wasn't working and anyway my peak flow was good and was then referred for angina. I really think it must be the PV causing little obstructions but was told to treat angina as a separate problem. No one seems to know. Could do with one person/ hospital looking at me as a whole and deciding whether I should be rattling with all these meds. Sorry- want my old self back. It all makes me rather crotchety! I do hope you can find a better solution. It is difficult as you don't want to take up NHS time or sound like a hyperchondriac. But fight if you can for some answers or find someone to fight on your behalf. Very Best Wishes, Crotchety.
I’ve had that tightness in the chest and it feels horrible like my chest is being crushed. I’ve not had it often, but I did worry it was angina. I also get a bit out of breath if I’m walking too fast and on a hill. I have ET and only on aspirin , but like you, I do think it could be related to ET although I’ve not had these pains often enough, so never actually gone to the GP yet about it !!!?
Hi Kizzy, as much as I don’t like going to the GP, please do get your symptoms checked out. It took me 5 years to do anything about mine so I can’t shout but testing did move pretty quickly once cardiac symptoms were indicated. What with current delays and waiting lists for non urgent care it would be best to get on the list now rather than later. Good luck.
Iwas diagnosed with P V ,as soon as the Hydrea got the blood flowing ,I had chronic pain in my chest,we called the Pompiers who rushed me to hospital,it was discovered that an artery had collapsed,I had a stent fitted,this is 12 years ago.Previous to the artery collapse I had several heart scans ,all looked o k,now I am checked monthly for P V & heart,blood tests too for both .I am treated in S W France where we live.....all very complicated isn't it.
I had a heart attack 5 years ago (age 36 at the time). After extensive tests including an angiogram, they presumed it was caused by a coronary vasospasm. I was put on a statin and a calcium channel blocker. Years later I had a stroke which ultimately lead to the JAK2 mutation discovery. My team is convinced the heart attack and stroke were a result of the PV and not a vasospasm so they have pulled my calcium channel blocker. Not sure how helpful that is.
Hi JT, thanks for replying. I think what you’ve highlighted is that none of our conditions are straight forward and diagnosis is not certain thing. Somehow I feel having that label and certainty is helpful in being able to cope as a patient but it just doesn’t work out like that. What you’ve been through can’t have been easy. With best wishes.
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