Bronchiectasis and MPN

[Kriddle01]

I'm 35yrs old with Bronchiectasis and just diagnosed with MPN. I have an enlarged spleen and suffer from extreme fatigue. Currently awaiting more tests as my results were overlapping with the possibility of MF. I am still trying to get my head around the news. Can anyone give me a little insight on how this is going to affect me? Is there a long term? I have a 2yr old and want to be around as long as I can.

[Mazcd Partner MPNVoice]

Hello Kriddle01, it is very difficult when you are first diagnosed, and still waiting for test results, there is such a lot to take in. I would suggest that you read as much as you can on our website mpnvoice.org.uk, I am sure it will help you.

You will get lots of replies from the lovely people on this forum who will share their experiences with you, and hopefully this will make you feel better about the future.

It might also benefit you to have a look at the videos of our 'virtual' patients' forums, where you can hear the latest news from MPN consultants and also patients giving talks, they are on our YouTube channel.

youtube.com/channel/UC-S_Ic...

Best wishes, Maz

[Kriddle01 in reply to Mazcd]

It is alot to digest, thank you for your response. I will have a read though the info on the websites and hopefully take some control over what is happening.

[hunter5582]

Welcome to the forum. Glad you found your way here. this is a great place to get support and information from others on the same journey you are on.

I was diagnosed with ET about 30 years ago, when I was in my 30s. It progressed to PV about 7 years ago. At age 65 I am still alive and kicking I have had a rich life and continue to do so. Please plan to do the same. There can certainly be challenges to managing a MPN, particularly when there are co-occurring health conditions. Sometimes these conditions are linked in ways that may not be readily apparent. At the core, MPNs are inflammatory disorders. The deregulation of the JAK-STAT pathway does more than promote hematopoiesis. It also increases the production of inflammatory cytokines. This is thought to result in many of the secondary symptoms we experience. You note having Bronchiectasis, which is a condition in which inflammation plays a role. Suggest talking to a MPN Specialist about this topic.

Do be aware that most doctors, even hematologists, do not know much about MPNs because of how rare they are. To get optimal care you need a doc with MPN expertise. Here is a list of MPN-expert docs. mpnforum.com/list-hem./

You will doubtless want to learn more bout MPNs, but may find random Googling less than effective. Mazcd provided some excellent resources. Here are a couple more.

The presentation on Molecular Biology is very good. The rest are too.

mpninfo.org/conferences/201...

Here is a nice primer on the role of Inflammation in MPNs

youtube.com/watch?v=FzyoPAG...(Not working as expected?)

Hope that helps.

[Kriddle01 in reply to hunter5582]

Thank you for your reply, my hematologist wasn't very helpful when discussing MPN, just used alot of big words that up until now I knew nothing about. Research is the key I feel and getting to know my own body. Funny how many of the symtoms I had just put down to being over worked and underpaid 😊 i have a follow up appointment next month that will hopefully give me a diagnosis of my subgroup, the hematologist says that my results are overlapping into MF as I have scarring in my marrow. But fingers crossed it is manageable.

[hunter5582 in reply to Kriddle01]

Hoping that your hematologist is a MPN Expert/Specialist. If you don't know, it is Ok to ask how many MPN patients the doc has treated. It is always advisable to get a second opinion with a condition like a MPN.

I have two hematologists on my team. I have a local hematologist (handles my on-going care) who is a wonderful doctor, but not by his own definition a MPN expert. I am only his 6th MPN/2nd PV patients in 20+years of practice. I also see a MPN expert hematologist at the John Hopkins MPN Center (several hours away). The MPN Specialist consults with my and the local hematologist about my care plan. This arrangement works great.

It is important to learn what all those big words mean and how they apply to your presentation of the MPN. It is the only way you will be able to make informed decisions about your care. The good news is that there has been significant improvement in MPN treatment options in recent years. Some of the newer treatment options are very promising (e.g. Besremi - aka ropegylated interferon).

DO let us know what you learn after your next appointment.

[Wyebird in reply to hunter5582]

The video you posted was extremely informative. It explained so so much. THANK YOU. I wonder if Maz is aware of it. I honestly think it would be advantageous if on the MPN voice website.

[hunter5582 in reply to Wyebird]

Dr. Fleischman is very informative and doing some really great research. She is also doing a Familial MPN study. My daughter and I are participating.

Here is more about Dr. Fleischman mpnlab.org/

[Wyebird in reply to hunter5582]

Gosh thanks I e saved and going to read later. Currently suffering fatigue due to op so can’t concentrate. Still can’t understand how someone so significant hasn’t come to my notice before. I’d love to hear what you and your daughter have to do in order to help her research etc. Ones again a huge thanks .

[hunter5582 in reply to Wyebird]

Participation in the study is really easy. Involved family members just sign up and receive a kit in the maul for various samples to do the NGS study. Super easy. they are interested in several family members. Me = JAK2+ PV (was ET) and positive for NF1:c5425C>T

Daughter = JAK2+ ET (possibly PV) and positive for NF1:c5425C>T

Son = JAK2 negative with erythrocytosis and positive for NF1:c5425C>T

Brother = Non-Hodgkin's B-Cell lymphoma and positive for NF1:c5425C>T

I do believe the evidence supports a genetic predisposition to MPNs and related disorders. Maybe there is some kind of link to Neurofibromatosis Type 1, which all of us have. Or not. No one really knows. That is why the research is so important.

[Wyebird in reply to hunter5582]

Gosh you all are definitely a family to study.

[Wyebird]

Devastating news but try and remember on reflection you will be looked after and hopefully when your meds kick in you will feel better. Also try and be positive and think about all the other horrid illnesses there are out there. That’s what keeps me going.

[Linnie5]

I too have Bronchiectasis ( currently not taking any meds for it). Only emergency pack. And have thrombocythaemia jak 2. Yes I am tired all the time but pace yourself and don’t stress about the little things. You will be fine. I’m on hydroxycarbamide and aspirin. And lead a full life. I’ve been told to stay off sugar and keep wheat low to protect my spleen. Good luck and let us know how you get on. 🤗