I was diagnosed with ET with fibrotic changes and jak2 13 years ago. I have decided to change my hospital and have my first appointment end of month. Just wondering if anyone has done this and maybe what questions to ask. I have not seen my old specialist for nearly 18 months just phone consultations.And I don't usual ask anything just accept my medication but feel I need to know more about my treatment and anything else that maybe available. I am 63 year old and currently taking 2000mg of hydroxicardimide and Aspirin also thyroxine 175 mg. Can anyone give me any advice.
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JeniMac
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Sorry I don't think I can offer you any advice other than I'm asking my GP next week for a referral to a mpn specialist, I've only been diagnosed for 3 years but for peace of mind it would be reassuring to talk to an expert It might involve a 6 hour round trip
I would try to get connected to a MPN Specialist, not just a regular hematologist. Here is a list. mpnforum.com/list-hem./
If you are talking to a regular hematologist, do not be afraid to ask how much experience he/she has with MPNs. If the doc is not a MPN-expert you can also ask with whom the doc will consult abut your MPN care. This is in essence a job interview. You are the one doing the hiring.
Assuming you like this doc, then be sure to discuss your treatment goals, treatment priorities, all medical issues, your questions, and treatment options. I always go with a written agenda. I give a copy to the doc. It looks something like this.
Treatment goals: Define for your doctor what your priorities are. Can be very specific at times. (these are mine)
1. Extend quality of life.
a. Protection of cognitive function is the single highest priority.
b. Protecting vision, hearing, other senses and mobility/physical abilities second priority
2. Extend length of life (only when QOL intact)
a. Philosophically – better to get less tx benefit and preserve QOL with any tx
b. My medical POAs have very specific instructions regarding my care and when to d/c all tx.
Treatment Approach Define what your approach to treatment will be. Make this specific to your treatment preferences. ALWAYs tell your providers all the interventions you use
Updates/Issues/Questions
Write out the symptoms and issues you are having and a list of your questions. Always include a holistic review of what is going on, particularly with specialists. Be thorough for a provider being seen for the first time. Only give updates/changes to an existing provider.
Treatment
Review all treatment options – let the doc know what you are doing now and seek input on what else to do next. Ask about ALL options, not just the one the doc recommends. Ask the doc to explain why the recommended option is better than other choices. Ensure that it is your goals and preferences that drive the decision about your treatment. This includes your own risk tolerance and what risks you prefer to take.
Medication Guidelines Provide your doctor guidelines about how you will make decisions related to medications. Base this on your priorities about what happens to your own body.
• ALL medications have a risk/benefit profile. Benefits must always be weighed against risks.
• ALWAYS review each of your medication choices and compare the risks/benefits of each of your choices. There is nearly always more than one choice.
o It is up to your provider to recommend a medication.
o It is up to you to decide.
o This is your RIGHT and your RESPONSIBILITY.
Contraindicated: this means you will not take meds with these adverse effects
Avoid/Consider Alternative: you will take these meds but will consider other options first.
Caution/Monitor: you will take these meds but will monitor closely for adverse effects.
Medication Adverse Reactions: Be sure the doctor knows any history of adverse reactions
Thank YouThere is no Mpn specialists in my area and my doctor said my new consultant is more qualified than my last. I will definitely take your advice and have everything written down.
I have phone consultations with an MPN specialist at approximately 3 month intervals as well as seeing my regular general haematologist. This works well - my local one sends on my blood results and any observations.
For me, Jeni, I think its definitely worth going the distance, literally in my case, to find a good MPN specialist. This means a 3-4 day road trip in my miniature camper van so I treat it as a holiday and an adventure.
Hi Jeni, I know Hunter has recently given out a link to a list of MPN specialists.I had heard so many favourable reports about Prof Claire and her team, up at Guy's, on this page and on various MPN Facebook groups that the choice was a no brainer. My first haemo nurse clinician, back in Surrey, had no idea why there was a concurrent risk of both lots and bleeding with ET handmade no suggestions as to how I might find out. Here in the West of England, I tried to explain to the haemo why he didn't need to be freaking out about high potassium readings and referenced some research about pseudohyperkalaemia being common with high platelets. He informed me he was a haematologist, not a research scientist and when I got upset, the line went dead. I got straight back to my GPs and asked if they would refer me to somebody who knew what they were talking about, such as Professor Claire.
I think it comes down to doing your own research and being truly thoroughly prepared, as has already been advised.
Hi Hunter, You always seem very knowledgeable so please can I ask your opinion. I am on 2 500 tablets daily of hydroxycarbamide every day and the latest prescription is saying to take 2 every 3 days so instead of 56 over 30 days it will be 20. This surely cant be right? I contacted the haemo nurse who said to carry on with 2 a day and she has emailed the haemotologist but if the past is anything to go by I wont hear anything. What do you think..if it is wrong they have made a big mistake!
That does sound like n error. I have never heard of a dosing pattern like that. If your blood cell levels are too low or if you are having adverse effects dose titration would make sense; however starting at 500mg bid one would think the first step would be 500mg qd or alternating days of 1 cap - 2 caps.
This may be a transcription error, which is bad enough. I would be more concerned if it was a prescribing error. I expect the former is the case since i do not know f any responsible doc that would make such a significant change without discussing it with the patient.
I think you did the right thing continuing on your current dose until this is straightened out. Hope you get is resolved soon.
I am in the same situation. I periodically consult with a MPN Specialist 2 hours away. He advises me and my local hematologist about my care. This works great.
Sorry to bother you but I had a bmb 13 years ago and had fibrotic changes would it be worth asking for another one to check on further changes,or would this show in my blood tests
It would depend on a couple of things as I understand it. What type of fibrotic changes were they? Reticulin fibrosis vs collagen fibrosis. "Recent evidence has shown that the amount of bone marrow reticulin staining often exhibits no correlation to disease severity, while the presence of type 1 collagen, as detected by trichrome staining, is often associated with more severe disease and a poorer prognosis."pubmed.ncbi.nlm.nih.gov/179....
There are several things in blood work that might show up as evidence of progression. Bear in mind that progression could also be to PV, not just MF. Looking at the trends over time of all of your CBC could shed some light on disease status.
There are two other things in blood tests that could answer questions about your status. 1. Has there been any progression in mutant allele burden? If you have the number from 13 years ago this can be rechecked. 2. Are there non-driver mutations present? This is predictive of your level of risk of progression. There are Myeloid panels that can assess this. This is one of them. files.labcorp.com/labcorp-d...
Note that not all docs are in agreement about the value of checking these 2 things. My MPN Specialist did agree that when I had signs of possible progression that these two things were worth checking. Based on the results, the decision was that there was no need to do a BMB at that point in time. Had there been any sign of progression, we would have done the BMB. Note that in 30 years I have never had a BMB. Not any of my 5 hemo-docs ever thought it was needed in my case. Again, not all docs would agree with this approach.
While I would not look forward to getting a BMB I would most certainly do one if my care team recommended it. We will do one if there is any sign of progression. I would push for it if I was concerned.
Thank you for your help and all the help you give to others. I have been very ignorant in my diagnosis but as I feel like I am deteriating in general I need to take a pro active approach.
The same thing happened to me. I ignored my MPN, my Neurofibromatosis and other things. Reality gave me a wakeup call. You are right about taking a proactive approach and managing your MPN and other health issues effectively. We cannot afford to be ignorant and must advocate for ourselves for the care we need.
Good luck, it took Burnley General Hospital ten years to destroy my husbands entire redblood cells, and never giving him any information, other than to tell him in June 2020 he
had 12 to 18 months to live. He passed away in September
I am so sorry to here this it really is a bloody lottery there is no Mpn specialists in my area and I feel I have been on a hamster wheel for years hoping this is a better specialist if you ever want to chat I'm here take care xx
I was diagnosed with Et in 2008. I was pretty stable until about 2016, and then I started feeling progressively worse, and my numbers were out of whack, so finally, in 2019, I found an Mpn specialist. I brought a copy of my original diagnosis and the last 6 months of bloodwork. So that’s my advice, to bring your previous records to your new doctor. My previous hematologist told me I had progressed to PV, but through a BMB and tons of bloodwork, it turned out I had MF intermediate 1.
Thanks I feel same just can't put finger on it but body pains fatigue and dizziness and horrendous hot flushes to name a few I don't getA copy of my blood tests so I
Am hoping they have a copy. Feel like I have aged in the last 2 years hope hypur feeling as good as you can.
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