Guys update

[Spanelmad]

Hi all, update on marsked PVYesterday had the first appointment at Guys, wow the difference in approach is palpable!!!

After developing neuropathy in both hands and feet Peg had been paused at the end of April (45mg).I have a degenerative spine and rheumatoid arthritis so it's not clear why this has happened ,it could be the editor an arthritis flare and the nerves in the back having a party!! Neuro referral in.

Guys are redoing all genetics( including some not originally done).Amazed that there doing this so quickly after the cyber attack.

After the examination( never had before) spleen found to.be enlarged.

Full history taken ,including family my sister was with and asked about .hers.

Agreed to ultra sound and BMB in the next couple of weeks at Guys.

Results due in about 8 weeks . If its PV switching to Rux. My comment to anyone out there that doesn't see an MPN specialist is get a request in for that second opinion, the care is completely different.

[Lyndjs]

Thank you for sharing. I'm sorry to hear you're suffering with arthritis and neuropathy in your hands, but so pleased you've been seen by the team at Guys. So happy to hear they are looking into everything for you. I hope BMB goes comfortably for you.

Please keep us updated on your journey.

Take care,

Lynd x

[Spanelmad in reply to Lyndjs]

Waited a long time for the appointment so was pleased it went well.

[hunter5582]

Glad to hear you had such a positive experience with a MPN Specialist at Guys. This is all the more important when you have co-occurring conditions that complicate things.

Wishing you success moving forward.

[Spanelmad in reply to hunter5582]

Was pleased it went ahead as lots of patients were cancelled due to last week's cyber attack

[PhysAssist]

Hi Spanelmad,

I'm not really surprised at all that you've seen a large [positive] contrast.

Best of luck with all of that.

Don't be too concerned re: splenomegaly finding- this is one place where the imaging is way more definitive than the physical exam.

Also- have you had any symptoms of the purported spleen enlargement? Early satiety, abdominal fullness, abdominal pain- especially in left upper quadrant?

God bless - keep us informed, and keep breathing [deeply]!

Best,

PA

[Spanelmad in reply to PhysAssist]

Hi PA, no saiety as such, but I've been changing my diet and have lost a stone recently.But it's as you've described a fullness around the middle with a little pain.As you.say still.breathing just worn out.Hope your doing ok

[PhysAssist in reply to Spanelmad]

Hi Spanelmad,

May we assume that you meant satiety [as in feeling full with minimal oral intake] , because I do hope you're safe.

You comment about losing a stone had me feeling sympathy for the pain you must have gone through in passing it [as in kidney stone or gallstones]- and then i realized that meant it as a unit of mass/weight. 😄

I have been OK, thank you for asking.

My Hct was, but is not currently being compliant to the goal of <45 % [instead i has been solidly 49 % despite phlebotomy], but my WBC and platelets have been really well-controlled- almost too low in fact. I'm planning to request a f/up of my allele burden test and abdominal U/S to see if there has been any budge in those parameters in my 2 years+ since diagnosis, and nearly 2 years of INF treatment.

Weight loss [especially if it's unintentional] can also be a symptom of SMG [splenomegaly] I think because patients start to eat less without really realizing that specific symptom of fullness as representing a degree of early satiety.

Quote: "The most common physical symptom associated with splenomegaly is vague abdominal discomfort. Patients may complain of pain in the left upper abdomen or referred pain in the left shoulder. Abdominal bloating, distended abdomen, anorexia, and/or early satiety may also occur."

Source: ncbi.nlm.nih.gov/books/NBK4...

BTW, apropos of my breathing comment- dyspnea/shortness of breath out of the ordinary can also be present as a consequence of restriction of diaphragmatic excursion- i.e., it's harder to take a full deep breath.

Regardless, hang in there and trust the process [especially since you're in better hands now. 😇

Best,

PA

[Spanelmad in reply to PhysAssist]

Ok I see take the ! with the "brit", sorry no kg here!!! Yes it's vague pain and the weight loss all though trying to, is more than expected.Had a full exam of lungs during the appointment but they were concerned that respiratory have not scheduled follow up since the bilateral PE.So more appointments to look forward to!

The breathing hasn't really got back to normal.Although found this morning that I could sing along to the radio without turning bright red.

[Spanelmad in reply to PhysAssist]

Glad to here you going on well even with the wbc a little low.Hopefully not to many infections

[PhysAssist in reply to Spanelmad]

Hi Spanelmad,

I'm in the US, so for us it's pounds, not Kg's- and thus there would be a chance that you might think we meant money, and not weight- it's a weird world we live on.

I'm being careful [about infection], but [hopefully] my WBC's won't get quite that low.

I'm glad to hear your breathing has made some progress at least.

I had forgotten all about the PE.

Sorry,

[Spanelmad in reply to PhysAssist]

No need, I managed to sing my heart out driving back from my sisters house complete joy.

Plus you've kept me awake ,so I'll sleep tonight.

[PhysAssist in reply to Spanelmad]

Well, put your heart back in where it belongs- you're gonna need it.😁

I'm glad my blathering was good for something/someone- especially for you.

Sleep well, and dream pleasantly if at all.

Best,

PA

[Exeter21]

Yes they were amazing with me too sorted my treatment & drugs & are so caring to help not like our normal conveyor belt hospitals.

Really recommend them Claire Harrison has helped turn treatments around with her knowledge & teams up there.

Julia . Exeter 👌

[Spanelmad in reply to Exeter21]

Felt like a person not a number! They couldn't of be more helpful ,dispite the fallout from the cyber attack.Glad all is going well with you

[Bobadog]

Hi Spanelmad,

I also developed increasing neuropathy whilst taking Peg ;45mg fortnightly.after 1 year.

I switched to Rux in March this year:10mg twice daily.

Now taking 5mg am and 10mg pm as haematocrit dropped .37 from .48 in 6 weeks .

Rux is Effective on the lower dose for me.

Rux has only recent been approved by NICE in the UK for PV,so watch this space…

I also suffered with severe acute lumbar/sacral pain last year.

Referred to rheumatologist locally.

MRI scan showed ‘insufficiency fractures’ in the sacrum.

Referred for Dexascan which showed osteoporosis.

Now taking Alendronic acid for osteoporosis (not sure about the effectiveness of this drug ,but I will give it a chance)

Perhaps the PV has long term effect on bone bone marrow health exacerbating any OA symptoms?

All the very best ,

Bobbie

[Spanelmad in reply to Bobadog]

Has the neuropathy improved at all? As for the Alendronic acid that's standard for osteoporosis and they should do follow up dexa scans at some point.Interesting thought about the OA, mine is definitely worse since starting PEG.

Hope your not feeling too bad .Sacrum pain is the worst.

[Bobadog]

Hi ,

Yes ,the neuropathy disappeared completely in a couple of weeks after stopping Peg

I think a more graduated exercise programme,should help the other joint ‘rubbish’ going on ,which at my age ,63, is a common occurrence

I don’t think the Peg particularly caused an increase in my joint pain.

Perhaps the nature of the Jak 2 PV ‘ driving ‘effect on the bone marrow over the last 10years,plus repeated venesections,(which may increase the RBC production over time),affects bone health etc?

Anyway ,forever onward…let’s all try and keep moving as much as possible,within our capabilities ,in a sensible way,ie no over exercising!!

Take lots of care,

Best wishes,

Bobbie

[Spanelmad in reply to Bobadog]

Yes I agree pace yourself ,not easy with a working cocker spaniel!!

[Bobadog]

…or with 2 flat coat retrievers!😂

[Spanelmad in reply to Bobadog]

Nearly flat on my face just now on the afternoon walk