What to expect at Hemaetologist appt?

[Jennifermcd]

Hello All,This forum has been a great source of information, thank you! I’m wondering if anyone can tell me what to expect at my first Hem appointment next week. I was referred by GP after high HCT/RBC’s (that have been creeping up for years) for the JAK2 test which was 7 weeks ago so now I’ll find out the result. What are next steps if it is positive? Does the JAK2 totally confirm PV or are more tests needed? And, if it is negative? I wonder what they do if negative - which tests they order from there and what else it could be. I’ve been keeping a journal of symptoms, the latest of which is dizziness when lying down or putting head back (does anyone else get this?) I definitely want to go for a second opinion no matter what is found by this doctor since PV and these high red blood levels are such a rarity... how many of you have gotten second opinions? Many, many thanks, Jennifer

[MCW22]

At my haematology department the first thing they do at each appointment is weigh you. Then take bloods. After about a 20 minute wait while the blood is being tested you then go in to see the consultant who gives you a copy of the results and discusses the next step based on the results. Write a list of questions you may want to ask and if possible take someone with you. They may remember things that the consultant said that you don't. If they don't offer you a copy of your blood results, ask for it, then you can compare each time yourself at your leisure when you are more able to take it in.

Carol

[Jennifermcd in reply to MCW22]

Thank you Carol. I hope I will get some definitive results.

[socrates_8]

Hey Jennifer...

Firstly, try to stay calm for the moment until you've seen your specialist and they have provided their opinion/diagnosis.

You might find that there is no MPN and your bloods thus far were activated virally thru some other event/infection? Anything's still possible at this early juncture...

If on the other hand, your specialist has other news(?)... Then he/she will best advise you at that time as to what next steps might be required.

There are many sypmtoms that can be attributed to MPNs which are also easily attributed to many other more simple diagnosis too...

Extreme fatigue is possibly the most common complaint that most people hear of w/ an MPN. However, there might be a plethora of them too...

It's a hard place to be, (in limbo...) while awaiting further answers...

Just try to be kind and patient with yourself for the moment Jennifer, and once you have more information you will be much better informed if indeed a second opinion is even required...

Best wishes Jennifer and keep us informed as to the results of your tests...

Be gentle on yourself...

Steven

(Sydney)

[Jennifermcd in reply to socrates_8]

Thanks so much Steven, I really appreciate your reply. It's easy to get worked up about this and a voice of calm reason is always good! Hope you are well.

[Ebot]

Expect blood tests (!) and possibly a scan to check your spleen etc. If the JAK2 test is negative they will likely test for other markers. If it’s positive then that is your MPN confirmation. And if your HCT is high they may well suggest a venesection. You may also be referred for a bone marrow biopsy as that provides a good bench mark and valuable information as to the state of the disease at this time. I wouldn’t be seeking a second opinion unless you feel the care you are getting is below standard or you are not being seen by an MPN specialist. The key is to be happy with, and confident in, your haem team. (Actually I was seen initially by a respected Professor of haematology who carried out the first raft of tests but once the results were through insisted he didn’t know enough about MPNs and referred me to his colleagues at Guy’s for which I was / am hugely thankful.) Definitely go armed with a list of questions and if you can, take someone with you as a second pair of ears. It can be a lot to take in. Wishing you all the best.

[Jennifermcd in reply to Ebot]

Thank you so much for your thoughtful reply, I know it's one step at a time and need to remind myself of that. I have a page of questions and list of symptoms. I'll be on my own, so hopefully I will be able to take it all in!

[hunter5582]

Check out the WHO Diagnostic criteria for polycythemia for the most succinct answer to your question. They changed in 2016, so be sure to look at that version. The short answer is that if you are JAK2+ then you will likely have some form of MPN - either Essential Thrombocythemia or Polycythemia Vera. The three types of blood cells the doc will look at are platelets, erythrocytes (red blood cells), and leukocytes (while blood cells). While ET and PV are fairly common diagnosis within the very rare class of disorders that are MPNs, there are also MPNs that are unclassified. You can be somewhere on a spectrum of MPNs that does not fit neatly into one category or another. The diagnosis can be made solely with blood tests. but some docs like to include bone marrow biopsy as well. While the BMB may not be needed to distinguish your MPN, it does let the doctor look at whether there is evidence of developing fibrosis as a result of the MPN.

Unfortunately, many docs - even hematologists, do not have much experience with MPNs due to their rarity. You should not assume the doc you are seeing has the KSAs you will need. You have the fundamental right and responsibility as a patient to ensure that your provider(s) has the knowledge and skill you need for your treatment. You also need to ensure that the doc is willing to work with you, listen to you, hear out your concerns, and be willing expand his/her own knowledge base as needed. Remember that this doctor works for you and is responsible both for and to you.

In terms of what to expect from this first hematology appointment, that is for you to determine. I would recommend treating it somewhat like a job interview. You are interviewing the doc to see whether you are going to hire him/her as your provider. Write your questions down and go prepared to the appointment. A few questions I would suggest are: 1. How many MPN patients have you treated? 2. What training have you had specifically regarding MPNs? 3. What testing do your recommend for diagnosis and why? 4. What is your treatment approach/philosophy regarding MPNs? 5. Are you willing to work with a consulting MPN-Specialist for my on-going care?

There is a lot of very promising research and emerging treatment strategies for MPNs. The role JAK2 signaling plays in MPNs/Inflammatory cytokines and symptom management is just one example. There are common treatment protocols out in the literature which may be right for many patients, but not for any one patient. You need and deserve a doc who will look at your individual profile and design a treatment strategy based on the unique presentation of your MPN, your cooccurring medical conditions, and your personal preferences and priorities. Please feel empowered to take charge of your own care and ensure your needs are met. Passive patients do not receive optimal care - assertive patients do. You deserve high quality care, but it is your responsibility to ensure that you receive it.

Here is one resource I found very helpful. It is a list of patient-recommended docs with MPN expertise. mpnforum.com/list-hem./ . Since MPN-expert docs are so hard to find, my own approach has been to employ a local hematologist for my ongoing care who I really like (I am only his 5th MPN patient in 20+ years). He actually listens to me, hears out my concerns, and is willing to consider treatment options outside his own knowledge base. I made contact with an MPN expert doc (from the list above) who now serves as a consultant regarding my MPN care. The docs do not always agree, but that is OK. Their disagreement is informative and ultimately I am the one making all treatment decisions for myself - so more information just better informs my decisions. I do have to travel to see the MPN-expert doc - but it is totally worth the time and expense. Getting the right treatment makes a profound difference in my health and quality of life.

All the best to you on this journey. I hope you find what you need and deserve.

[Jennifermcd in reply to hunter5582]

Hi there and thank you for such a detailed and considered reply. I needed it!

I looked up the WHO diagnostic criteria, thank you! and it looks like this:

"Diagnostic criteria for PV as per the 2016 revised World Health Organization (WHO) guidelines include three major criteria and a minor criterion. Diagnosis requires the presence of either all three major criteria or the first two major criteria and the minor criterion.

Major WHO criteria are as follows:

1. Hemoglobin >16.5 g/dL in men and >16 g/dL in women, or hematocrit >49% in men and >48% in women, or red cell mass >25% above mean normal predicted value

2. Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size)

3. Presence of JAK2V617F or JAK2 exon 12 mutation

The minor WHO criterion is as follows:

1. Serum erythropoietin level below the reference range for normal"

So far the only major one that I know I have are #1 --high haemoglobin and hematocrit. Will see about the JAK2 tomorrow. Not sure about erythropoietin. This is all so mysterious! But I will definitely be assertive during my appointment, thanks for the encouragement. I have my list to hand with symptoms and a lot of questions too.

Thank you again, your post has been so helpful.

Best Regards,

Jennifer