My husband and I both diagnosed at the same time with ET JAK2

[Na56]

Hi everyone. ET is not a rare illness in our household. I’m 69 and Dave is 74. Dave has no symptoms and I’ve got itchy skin patches. This is a great chat line and I’m learning a lot from you all. Any recommendations for a hand wash to help the very itchy patch on the palm of my hand? Thanks Irene

[Mazcd Partner MPNVoice]

Hi Na56 and welcome to our forum, and WOW, this is the first time I have ever heard of a husband and wife being diagnosed with a MPN at the same time.

Sorry that you are having this problem with an itchy patch on your hand, I am sure you will get lots of advice from the lovely people on this forum, but do ask your haematology nurse specialist for advice as well and ask if there is any lotion/cream etc that you can either buy over the counter or get on prescription, which she/he can prescribe for you.

Best wishes, Maz

[Na56 in reply to Mazcd]

Thanks Maz

[SammieLou in reply to Mazcd]

Gosh that’s incredible so rare too.There was another lady I was talking too who’s husband was diagnosed too they had lived near a radioactive place together

Have you been on Facebook ?

Type in Facebook our disease

Lots of positive groups on there to answer your questions with thousands of people like us x

[Na56 in reply to SammieLou]

Thanks for your suggestion about Facebook SammieLou. We’re not aware of any environmental causes of our ET but it does seem strange that we both have been diagnosed. Our haematologist was very surprised and our blood tests were checked twice to make sure there was no mistake. Best Wishes Irene

[SammieLou in reply to Na56]

I cannot recommend Facebook enoughAll the very best to you both

So many helpful educated people on facebook with our disease x

[DJK12]

I guess many of us have hand issues especially with all the gels we've been having to use recently. My hand issues started when I was on Anagrelide and many years later I still have to be very careful.

I've learnt never to use ordinary soap and always to wear gloves (with cotton liners) for washing up.The soap substitute I use for hand washing and showering is a pump of Dermol 500. (It can be prescribed) Always dry your hand thoroughly especially under any rings. I am then liberal with Aveeno lotion also in a pump - it has oat extract in it which is very soothing.

If it persists your pharmacist might suggest a low potency steroid cream which doesn't need prescription. Otherwise the GP can prescribe a stronger one which might get it under control and possibly you would find it manageable after that.

Best wishes Diana

[Na56 in reply to DJK12]

Many thanks Diana

[hunter5582]

Definitely follow up with your MPn care team and a dermatologist. I use a ceramide based cleanser and a ceramide cream. I use the CeraVe brand, but there are others, I do find them very helpful. I would also suggest looking for potential triggers, Best to avoid certain chemicals, perfumes, dyes, etc.

Do note that that sort of symptom can also be a medication side effect.

Hope you find relief soon.

[Na56 in reply to hunter5582]

Thank you Hunter for your advice. I have been using the Cerave moisturising cream and I’ll now try their cleanser.

[Hopetohelp]

Can’t help with skin rash except to say I always use Aveeno daily moisturising yoghurt body cream every time I wash my hands and don’t have a problem. I am amazed both of you have ET. Guessing you can’t tie it into something external that could have affected you both? I did read somewhere that fireman sometimes are prone to it because of smoke inhalation but not sure how true that is

[Na56 in reply to Hopetohelp]

Thanks for your suggestion. Dave and I both worked in offices and can’t think of any environmental cause but it is very strange. Best Wishes Irene

[Meatloaf9]

Hi, sorry to hear of your diagnoses. It does sound very strange. I would wonder if you both could have been exposed to some environmental or chemical trigger. Benzene has been implicated in causing blood cancers. My blood counts started changing a couple of years after I had my house treated for termites (a 4 year old house). They used dursban TC (I think it has been banned now) and I later found out it used benzene as a solvent. Coincidence?? Who knows? My original diagnosis was ET, now 4 years later it is PV.Best to you both going forward.

[Na56 in reply to Meatloaf9]

Thank you for your best wishes. We aren’t aware of any chemicals we’ve been exposed to that could cause our blood problem. We’re organic gardeners too. We’re obviously a strange couple! Kind regards Irene

[LT55]

Crazy that you and your husband both have ET!

About 2 years before I was diagnosed with ET, my husband had ITP (which is extremely low platelets), which landed him in the ICU in a very precarious situation. All good now for him, but we do find amusement that he and I have conditions at the opposite end of the platelet spectrum so to speak.

Sorry...no suggestions for the itchy patch, but you have gotten good advice from many!

[Na56 in reply to LT55]

Thank you for your interest and great to hear your husband is doing well. I hope you are too. Best Wishes Irene

[Janis12]

Gosh, how unusual, two people in one household with the same rare condition. My husband died 25 years ago as a result of Motor Neurone Disease which is quite rare. We were part of a small cycling group who become our close friends. When my husband died one of the other members of the group then went on to develop MND...incredible! Relating to your skin problem, I have just started with an itchy forehead so I am looking at the responses to your question to get some ideas. Good luck.

[Na56 in reply to Janis12]

Thank you for your message and I’m sorry about your husband. I can recommend Cerave moisturising cream as also recommended by Hunter. It helps on the itchy patches on my legs and arms . My hands are constantly being washed so it hasn’t worked it’s magic on my itchy palm. Hope it works for you. Take care Irene

[Janis12 in reply to Na56]

Many thanks for that info....what a carry on we have !!

[Vtr1000]

Hi Na56, have you tried 1% hydrocortisone cream it’s available over the pharmacy counter. You can use it for 5 days. For myself I had an itchy patch on the back of my hand and it got rid of the itching over the 5 day period. I now use Cetraben cream. You’ll find the forum very helpful. There’s always help with any MPN question. Good luck with your quest for a cream.

[Na56 in reply to Vtr1000]

Thank you for your helpful suggestions. Irene

[Planti]

Very strange that you both have an MPN. I can see why they would have checked the results carefully but it may be that there are many people that have not been diagnosed due to a low alert level for high platelets.In my case, my father and uncle had MPNs and died long before Jak2 mutation was found. I now have ET too which was diagnosed within a few months of escalating platelets due to my insistence in being tested, and yes, I have the Jak2 mutation. Diagnosed late 2020.

You sound very well aware, and of course the added burden on washing and gel use disrupts the natural skin barrier. You are probably aware of the risks of Hydrocortisone containing creams. Anything you can do to close the skin on your hand which may have some disruption is good, heavy barrier ointments and gloves for a short time, then constant use of some sort of tolerable barrier and of course the best and most difficult thing of all is to avoid scratching. I like the CeraVe products too.

Most thankfully I have no symptoms of ET, I take Pegasys which at first caused some troublesome nausea but is possibly responsible for fatigue, but also for complete haematologic remission. So I am pretty pleased right now.

Welcome to this forum and all the help and support that you could ever wish for.

Lauren

[EPguy in reply to Planti]

I saw your earlier post about skin troubles with PEG. Has that persisted or changed? Great that you have CHR.

Hunter has posted about MPN in his family. There is an ongoing study on the subject:

mpnresearchfoundation.org/n...

[Planti in reply to EPguy]

Hiya, yes when I was first diagnosed Hunter told me about this study. As I am in Canada my participation was a bit limited by the samples I could send in the mail. My brother, who like me, has the germline variants that give an estimated 4x likelihood of developing the mutated Jak2, but has not been tested for the mutation, also sent samples as did his daughter. Both my brother and his daughter have several different cancers or tumours that are not technically cancerous but locally invasive. So far I have just this one.No results from the study yet. My uncle was interesting as he was a WWII fighter pilot then high ranking officer and participated in a long term health study of servicemen with yearly blood tests so his PV was discovered promptly. As he had PV from a young age and he travelled all over the world he consulted different haematologists and was a bit of an expert. Sadly there was really no treatment and he developed Leukemia and died age 62. Would be very interesting to see if his medical data, or samples were still stored somewhere in some bunker.

Thanks for all your very interesting posts and links, you and Hunter are stars!

Cheers, Lauren

[EPguy in reply to Planti]

Thanks for the kind encouragement...

There are some reports on the idea of starting INF in hi risk patients before the disease starts. See quote here. But I don't know if any Dr would actually permit this treatment.

ashpublications.org/bloodad...

<<identification and upfront treatment of individuals with JAK2-mutant CH (CH is a preMPN state of Jak2) raise the possibility that IFNα could have the potential to prevent the development of MPN>>

[Planti in reply to EPguy]

Oh, I get the occasional flare of itchy bumps around my waist every few weeks that don't last long but they are not very troublesome, started after Peg. I just leave them and they go away in a few days. L

[EPguy in reply to Planti]

It seems then the facial effects you wrote about have improved some. Between Covid infection and MPN I've aged plenty.

[Planti in reply to EPguy]

As soon as my dose went back down to 45, the swellings around my eyes vanished and have not returned. Nothing compared with what others have to put up with but as I had no sx of ET before treatment it was er, not welcome. You have a long memory.

[EPguy in reply to Planti]

More evidence that using the smallest effective INF does is really important. We are frequently seeing that here.

Credit to the web site for the memory, all it needs is the inclination to look there. There is a lot of useful info in the old posts, but it can be overwhelming.

[Planti in reply to EPguy]

Interesting that my haemo, who is the provincial MPN go to expert, tells me she thinks that you have to take enough INF to make a difference to the bone marrow and suggested that unless my counts go way down I stay on same dose. There is no way here in BC to get an allele burden done so I have no idea what that might be. She says it has not been proven to be of benefit and it is all about costs. I would still like to know. So many questions. I am hoping that after a few years I won't think of it. Of course being out of the pandemic would help with more distractions.I can't imagine the province paying to give INF to prevent anything.

[EPguy in reply to Planti]

Seems conflicting advice from your Haem, take high INF dose to improve marrow, but Dr is unclear whether it's beneficial to do so. (I think she's right there is no solid studies so far that clearly prove this benefit)

This is a trade off some of us are also thinking about, the Besremi trials were done with doses in the 300mcg or so range, or even more, do we need that much to get the marrow benefit?

You're reasonable to want your allele burden (AB), and also a full gene analysis. This info may be of specific use as new understandings and therapies evolve quickly. If your specialist requests it, might the medical service cover it?

[Planti in reply to EPguy]

Er, in a word, no, it is not covered, nor can I even find a place to send it and pay for it myself as we are not allowed to get things by paying if they are not available to everyone. This is what universal medical coverage means for the average Canadian. Ditto for the gene analysis. She knows that I am very interested and says if/when it is deemed knowledge that is proven to have value and is then sanctioned, I will get it. Coolish comfort.Must not think too much about it.

[EPguy in reply to Planti]

I now understand your frustration. Hope you find a solution in some way.

It seems every medical system we learn about on the forum has various unique problems.

[Na56 in reply to Planti]

Thank you so much for your welcome and information. Irene

[Wyebird]

Welcome ! Gosh crazy fir you both to be diagnosed. I swear by argan oil instead of handcream. When I suffered an itchy scalp it helped enormously.

[Na56 in reply to Wyebird]

I’ll certainly look into argon oil. Thank you Irene

[hunter5582]

While it could just be coincidence, there is research underway to potential envirnmental triggers for MPNs. One set of research relates to the Pennsylvania Cluster here in the USA. Here is a bit on that. There is more if you care to look.

atsdr.cdc.gov/sites/polycyt...

wwwn.cdc.gov/TSP/NEWS/NewsL...

It is of great interest to see if we can learn what triggers the development of the disease state. We now know that the JAK2 mutation is acquired early in life, long before it emerges as a disease. We also know that there are people with the JAK2 mutation present who do not show any signs of a MPN. Finding what things trigger the emergence of the MPNs will be a big step forward.