Frustrated

[Loose_ends]

Hello,

Over two years ago i was referred to hospital after a routine blood test suggested i have PV.

I had all the tests including a BMB. The biopsy was not definitive and i was JAK2 negative. However, the consultant then and to this day, says the test are suggestive of PV.

As it stands, the consultants i have seen all say that i have secondary Polycythaemia. I've had 20+ venesections since November 2019. I have blood tests every 8 weeks, then a review a week after the blood test.

My liver, kidney's and other organs are in good shape. The chest X-rays and CT scan have not revealed any cause for concern.

For 18 months, the consultants have settled on the habitual explanation of saying my lifestyle is the cause of secondary PV. I used to be a big drinker and smoker. 5 pints 10 cigarettes a day in the week and over at least 25pints and 60 cigarettes over a weekend.

I still drink and smoke, maybe 10 pints over a weekend and probably 20 cigarettes. I only smoke when i drink. I rarely drink during the week.

The difference between then and now is huge.

I know smoking and drinking is not good for ones health and i'm not looking to justify doing so, but is it really likely that my current consumption is the continuing cause of my PV?

I'm also not looking to seek an upgrade from secondary PV to primary PV, i wish for neither to be honest, but i feel something is being overlooked.

The consultants have dismissed fatigue, numbness in my legs, blurred vision and severe headaches being related to whatever PV i have.

I'm just stuck in this 8 week loop and i'm a bit lost in it all...I guess quitting smoking would provide some answers...

Do i ask for another BMB and other tests or or do i just settle on the consultants opinion that i've only myself to blame and only i can fix the issue?

[EPguy]

Your Dr's opinion on the smoking matches what is known. Many reports come up on the connection. But did you get checked for Jak2 Exon 12? This is a less common mutation seen in PV. Very rarely PV has neither Jak2v617f (the one you likely were checked) nor Exon 12.

On your Dr's 2ndry Dx:

<<The most common causes of secondary polycythemia are

... Smoking or lung diseases>>

webmd.com/a-to-z-guides/wha...

This report lists other conditions that also relate to 2ndary polycythemia.

--

Another report discusses 2ndary polycy with a revealing title "Smoker’s Polycythemia:..."

These effects are familiar to MPN patients but in MPN it's triggered by genetic marrow defects:

<<Smoking deprives the bone marrow of oxygen that

pushes its production of red blood cells (RBCs), the carriers of

oxygen. Elevated hematocrit (HCT) resulting from excessive

erythrocytosis can increase RBC mass, increase blood viscosity,

reduce blood return through the venous system (sluggish of blood

flow), and increase platelet adhesion.>>

scivisionpub.com/pdfs/smoke...

--

A smoking history is also a risk for PV, but your Dr has apparently concluded secondary from your total information. Negative Jak2 and possibly BMB do point that way.

[Loose_ends in reply to EPguy]

Thank you EPguy, a lot of information and took a while to get through.

Appreciate it very much.

[Mostew]

Blaming yourself isn't going to help but 'cleaning up your act' will.If you feel it would genuinely want to quit smoking it might be the perfect opportunity.

6 pints at weekend instead ? Good quality one?

What's you diet like . ANTIinflammitory is recommended.

Our mind and body work together .

Go easy on yourself and enjoy life as it is

None of us want to have an MPN but it can be an opportunity to grow.

[Loose_ends in reply to Mostew]

My diet isn't too bad, probably the same as most single guys with not enough time on their hands. When i do cook for myself, its all fresh ingredients, plenty of vegetables.

Whether i'm getting a balanced diet with enough of all the vitamins and minerals needed is an unknown.

It could be argued that i'm enjoying life too much, but thank you for your support.

I mainly drink cider occasionally swapping to lager.

What beer would you recommend?

[Mostew in reply to Loose_ends]

I’d go for unpasteurised unfiltered. .. not a beer drinker but know you some places do them….Old habits are hard to break …..

[Mostew]

Ps forgot you smoke with a drink .so probably not practical to cut 1 out without other .. ...... !

[Tropicaldays]

I would get a 2nd specialist opinion.I was in a similar boat to you so hired a specialist and view was then not PV for sure!

Takes time but worth doing for a black/white answer.

[Loose_ends in reply to Tropicaldays]

Although the consultants as surmised by EPguy are probably right, based on the info they have that i have secondary polycythaemia; which is more than likely due to my lifestyle, i'm struggling to reconcile that. This is probably poor logic, but i was hammering it a few years ago. I have dramatically cut my consumption of fags and booze, and if anything, HCT and Hb are more erratic than since cutting down.

[Hopetohelp]

Cutting down will help as it did for me. There is a balance between addictions and living a life of what you enjoy in order to be the least stressed as possible as stress does not help our condition. As Mostow said, mind and body work together. Might be worth trying a second opinion

[Loose_ends in reply to Hopetohelp]

I will have to try and get into that mindset. Trouble is, i've been living life on my terms since i was 16. Its going to be a hard nut to crack.

But definitely leaning towards another opinion, i am entitled to that after all.

[Hopetohelp in reply to Loose_ends]

Yes you certainly are! Good luck

[Loose_ends in reply to Hopetohelp]

Thank you.I don't suppose you know who i should ask / the best way of getting a second opinion?

Whilst i may not be fully satisfied with the conclusions the consultants have made, i must say that overall, my treatment has been first class. Blood tests, reviews and venesection appointments have been pretty much bang on time. I've only had a few appointments delayed and only by a few minutes. The nurses and all but one of the consultants are brilliant.

The NHS gets a bad rap, but my experience is quite the contrary.

I hope asking for a second opinion does not upset/offend any of those that have provided my care as that is not my intention at all.

This goes back to my childhood. I've always questioned or rebelled against those in advisory or authoritative positions. Until it clicks in my head i do not accept, instructions / orders or anything that is tabled as fact....As you can imagine, this has caused me much trouble through the years.

I asked one of the consultants; if i only smoked one cigarette a week would that still be reasonable cause for secondary polycythaemia. As you'd expect, she swerved the question as she cannot be seen condone smoking and i could tell by her face this was a question i would not get an answer to.

Instead, she generalised and my jaw hit the floor. She said majority of people with PV she has seen were smokers, (She did not single out secondary polycythaemia in that statement).

Instead of being candid with me, i get the default smoking advice. Its things like that that spark my mistrust.

Oops, went on more than i intended there.

Thanks again though.

[Hopetohelp in reply to Loose_ends]

I too have had good experiences with nhs for our condition. I like my haematologist. They have stopped asking me about the smoking as I would probably crack up without it but my side of the deal is to cut down as much as I can and I feel better for it. I am not sure how to go about asking for a second opinion but many here have been referred to Claire Harrison who is an mpn specialist yet still remain under the care of their regular haematologist from what I can see. Might be worth a try.

[friendofpiglet]

I was diagnosed with PV JAK2+ 8 years ago. I was a smoker, and immediately stopped and to ease the burden went onto vaping (still vaping, and keep telling myself to run that down!). Believe me it works and the money you save will pay your fuel bills ,,,😃

Do you know what your EPO levels are? High would suggest secondary polycythaemia, abnormally low would suggest PV.

[Loose_ends in reply to friendofpiglet]

Hello and thank you for your reply.

Does EPO go by any other name?

I have copies of blood test results from the hospital and cannot see anything that relates to EPO.

[friendofpiglet]

Erythropoietin. Allegedly, it is used as a performance enhancer for competetive endurance atheletes. Low EPO levels are a minor criterion in the diagnosis of PV.

If you can't find it in your results, ask your haematologist.

[PhysAssist]

Hey L_E

Also, if you have not done so, consider asking about a sleep apnea evaluation, Mine required 2 overnights in a sleep lab, but when I referred SWMBO to our PCP because of her snoring with gaps of quiet followed by a gasp for breath, she was able to be tested at home.

The screening is even easier- and usually covered by even the cruddiest insurances here stateside, because it's just wearing one of those finger-pinchy pulse oximetery probes on a finger while you sleep. The little monitor comes with a memory, which they download and analyze to see if your blood oxygen dropped overnight at all, and if so, how often, how low, and for how long.

Sleep apnea is both common, and is a very common cause of 2nd PV., and us- beer-guys, [and you- chimney guys] tend to be at increased risk for it.

Best,

PA

[Loose_ends in reply to PhysAssist]

Hello, PhysAssist, thanks for the info. I will definitely put that to the consultant on my next review.us-beer and chimney guys made me chuckle, thanks

[PhysAssist]

Hi L_E,

You're totes welcome, of course.

If you can't have a beer and a laugh, then what's the point in going forward?

BTW, as I always seem to, I had a weird component to my sleep apnea, which we only know was related to it because it unexpectedly resolved when I started using C-PAP.

You may have heard of restless leg syndrome? Well, prior to my beginning treatment for sleep apnea, I had restless everything syndrome. Twitching, jumping, etc., etc. of my legs, arms, etc.

Nothing subtle about it either- SWMBO said it looked like I was running a marathon in my sleep. Also, I was getting up 3-4 times a night to pee- which made me worry about my prostate [which was/is just fine, thank you].

The nocturia was what triggered my GP MD to order the sleep studies, but after I started using C-PAP, SWMBO reported that I started sleeping more like the dead [or a log], and I never wake up because of having to get up to pee [and no, it's not because I just wet the bed and then lie in it.😜😇😎]

TBH, [aside from my PV [or now Fe-deficient anemia?] symptoms, I feel so much better than I had in the years before.

Best,

PA