Hi everyone, I was diagnosed with ET last Wednesday (still getting my head around it all). My platelet count is slightly over 1,600 and I really wanted advice on those experienced in this field, what I should be asking at my next consultant appointment.
I have been put on the Interferon treatment at 90 micrograms. I am 40 years old. I started the treatment this Monday and didn't feel too bad with the symptoms. I just seem to be experiencing a weird heavy / achy feeling to my right leg - which subsides after some nurofen/paracetamol.
I am with at Sheffield Hallamshire under Dr Francis - does anyone know if he's a MPN specialist? Or what's his credentials are. He's a man of few words... That I do know! 😃
Any advice on my current treatment plan - Dr Francis - and what questions I should be asking at my next appointment, would be greatly appreciated?
Thank you!
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Biscuitlove
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Well please be reassured that ET is very manageable. The idea of the medication is to reduce your platelets and many consider Interferon to be the best option for younger patients. It seems a relatively high dose so perhaps you could ask why at your appointment. You can simply ask your haematologist whether he/she is an MPN specialist.
Are you having a bone marrow biopsy? Do you know what (if any) mutation you have?
Thank you Otterfield! I will definitely ask about the high dosage - assume it's because my platelets are so high..but an assumption on my behalf!
I did have a bone marrow biopsy.
I believe I have no evidence of JAK2 and I'm still waiting on the results to what cell mutation I have. I should have an update at my next appointment.
Hello and welcome. This is a great place to find support and information from a MPNer perspective.
I was diagnosed with ET over 30 years ago. It progressed to PV about 9 years ago. I have lived a great life and continue to do so at age 67. There have been some interesting health-learning opportunities along the way, particularly in the last several years. There is plenty to learn about MPNs and, fortunately, plenty of time to learn it.
With platelets > 1600, cytoreduction is indicated for most people with ET. While there is a primary concern about thrombosis, there is also a serious concern about hemorrhage with platelets that high. There is a risk of Acquired von Willebrand Disease with PLT that high. There are some specific tests your doc would order to assess for that (e.g., von Willebrand Panel, Prothrombin Times).
Your level of risk with ET depends on a number of factors. Do you know whether you have one of the driver mutations, JAK2, CALR, MPL? DO you know your Variant Allele Frequency (AKA Mutant Allele Burden)? Do you have any additional non-driver mutations? What cooccurring medical conditions do you have? What MPN symptoms are you experiencing? There is a lot more going on with ET than just numbers of blood cells. The dysregulation of the JAK-STAT pathway also causes an overproduction of inflammatory cytokines. This is thought to be responsible for many MPN symptoms. MPN symptoms can include, fatigue, headaches, dizziness, insomnia, erythromelalgia, and more.
At your age, Pegasys would be a first-line choice for cytoreduction. While many of us start at 45mcg, some do start at 90mcg when a more rapid response is desired. PEG tends to be a slower acting medication than some of the other options, but better tolerated for long-term use due to risks associated with long-term use. Like most medications, PEG can have adverse effects that are dose-dependent. I have tolerated the interferons better than any other treatment options and they have been more effective for me.
Dr. Francis does not appear on this list of MPN Specialists. mpnforum.com/list-hem./ That does not mean he is not one, just that he is not listed. You will have to decide how you want to define a MPN Specialist. I would include having treated at least 50 MPN cases, active involvement in MPN training and research, and does a good job listening to patients and answering questions.
Here are a few resources to get you started on your MPN learning experience.
Wow!!! Hunter5582. I am so glad I wrote this post now and a lot of questions asked, I simply don't know the answer to -but helps me to ask the right types of questions. Thank you so much!! I really, wholeheartedly appreciate it. x
Welcome to the forum. You have had some excellent response, I can’t add anything else really with regards to your diagnosis and current treatment plan.
There are members on the forum who are seen at Sheffield Hallamshire hospital. From memory, ‘Conniesdad’ (forum member) recently had a successful stem cell transplant under the care of Dr Sebastian Francis. I think he rated him highly.
Some general haematologists have an ‘interest’ in MPNs, which doesn’t necessarily make them an MPN Specialist. It’s always good to check the MPN research that haematologists have participated in. I’m unsure of Dr Francis’ title but he appears to have participated in some MPN research, which is obviously a good thing.
I think others have offered you some excellent information so I’ll just say when I was diagnosed 4 years ago I had similar numbers. I was 42. I have the Cal-R mutation which often has a higher platelet count. I also was put the same dose of Pegasys as you. Fast forward 4 years and my platelets are now stable at around 200 and I do one 45 micrograms every 10 days. ET certainly doesn’t rule my life which is great as it can be shocking and scary when first diagnosed.
Please reach out if you have any questions or just want to speak to someone with similar experience x
Thanks JoJoWonder. Would make a lot of sense if I did have CALR because I have a night level of platelets but no symptoms what's so ever. Did you have a slight scarring to your bone marrow? And is that related to this type of mutation?
Also, do you see a MPN specialist or. Hematologist Consultant? I'm not at this point worried about switching but I'm interested in the pros and cons. What I've heard so far has been around listening and being more closely aligned to clinical trials / research x
I haven’t had a BMB. After discussing with my Haematologist I decided I didn’t want to have one. I had my diagnosis through genetic testing which confirmed the Cal-R mutation. The reason I decided against a BMB is that my treatment programme will not change if scarring or irregularities are shown in my bone marrow but I will have the knowledge that there’s an issue. If there was a treatment plan to slow or halt progression (from my understanding Interferon’s are best for this anyway) I would have one in a heart beat but as it stands nothing would change. I feel I don’t need to live with that worry. It’s a personal choice. The other thing is that results are somewhat subjective. From my understanding they are analysed by an expert whose opinion may differ to another expert. Not a can of worms I wish not to open. In the future if my health declines I will definitely under go investigation including a BMB. I live responsibly with my condition, make time for check ups, take my meds religiously, attend forums etc but I want to live my best life and not let it be a defining factor. You can become obsessed with it when first diagnosed because being diagnosed with cancer is hugely emotive.
My Haematologist isn’t an MPN specialist but is very experienced and I feel we are aligned in our outlook. I have spoken to a number of MPN Specialists over time by visiting in person forums. This is a fantastic and very supportive community. If you are able to visit an in person forum I urge you to. You’ll get all the up to date information and meet incredibly inspiring people who also live really well with MPN’s. I wish you the very best in the future. I wish I could go back 4 years and tell myself it was going to be okay ☺️
hello Biscuitlove, and welcome to our forum. It can be very daunting when you are newly diagnosed, there is such a lot to learn and understand, I would advise that you look at our website mpnvoice.org.uk there is lots on there that will really help you make sense of it all.
You will see from the replies from the lovely people in this community that although MPNs are rare, there are lots of us who have them, I myself have ET, I was diagnosed in 2003, so I am hitting my 20 year anniversary this year. As Otterfield has said, it is very manageable and in general people with ET without severe clotting or bleeding complications have an excellent chance of living out a near-normal life span. It is always best to write down any questions you want to ask your haematologist, and Dr Francis is an excellent haematologist, he has attended our patients' forums and given talks. We suggest writing down your questions because it is easy to get side-tracked in a consultation and you may forget to ask something, so if you have a list you can refer to it, and if you can, take someone with you to your consultation so that you have someone else listening to what is being said and may remember something that you don't.
It is also a good idea to find out who the clinical nurse specialists are in the department, they are very knowledgeable and can answer many of your questions in-between your appointments, so if you have any queries or concerns you can contact them for advice, so do find out who they are and how to contact them.
And as per the advice from Jo Jo Wonder about attending one of our in-person patients' forums, I would urge you to come along to one of our forums as they are a great place to meet other people with MPNs and swap hints and tips, and to also hear talks from haematologists, specialist nurses and patients. We are holding quite a few in-person forums around the country this year, one will be in Leeds which is a combined forum with the team in Leeds, Sheffield and Rotherham, hopefully Dr Francis will be there as well giving a talk, you can find the details for this and all other forums here mpnvoice.org.uk/category/ge...
I agree , definitely write down your questions and start the list well before (eg a week) before the appointment and add to it as things come in to your head. Once I have the full list of questions I sort them in priority order on computer as time can run out at the consult. If the doc is okay with it record the consult on your phone, I am always amazed how different it sounds when you play it back in the calm of home, I then take notes and file and date it.
If you can’t record it, as Maz said take a friend and try to take notes when you are there or if your friend is suitable ask them, good luck.
Sheffield covers Doncaster .and Barnsley . I’ll always be grateful to Barnsley the consultant who upon request sent me to Guy’s.
I know Sheffield was at one time a centre for excellence for cancer but not sure if it covers MPns . Is scarey to be diagnosed . I also know that interferon is good for you because if your age. You will find this site invaluable. You will never be alone.
As usual my fellow MPNers have already given loads of great advice.
I’d just add that I started on 90mg Interferon weekly & then gradually reduced amount & frequency over time. NB-once my platelets were down I asked for a reduction in dose & frequency so it might be worth asking once you get yours down.
I’m now taking 45 mg every 4 weeks & my platelet graph line is safe & steady.
I had a BMB about a year ago (which was ok) to log some baseline readings to refer back to in future if necessary.
Other than that always remember to drink loads of water/squash as this helps massively.
Once again it’s brilliant to have you on board with the group. It’s been a great reference & support for me since I reached out &, as your journey progresses I’m sure you’ll be able to assist others with your experiences.👍
welcome - I was diagnosed with ET a couple of years ago and am on Hydroxy Carbamide . I’m 73 years old an actor and on tour (at the moment Ireland) and still loving my work. ET hasn’t stopped me working, just maybe made me more focused on what I really want to do rather than just filling time. Alarm bells for me when you say ‘he’s a man of few words’ I think it’s important to know you can talk in depth about how you feel and what your worries are. Although I’m sure there will be the amazing Cancer care Nurses who are brilliant. Look after yourself and be kind to yourself. There will be bumps on the way but here’s the place to find the solutions - or many solutions that fellow ‘sufferers’ have found. Paul
Hello Biscuitlove! I’m 80 next month and I was diagnosed with CALR+ ET about 15 months ago. Blood tests and bone marrow biopsy undertaken. Prescribed 1 aspirin (75mg) daily and 90mcg Pegasys interferon jab forthrightly. Platelets were 1200-1600 at diagnosis and 450 two months ago. I am treated by a London NHS hospital, having first consulted the consultant MPN haematologist privately. I see the same person on the NHS. My consultant is knowledgeable and approachable. So far, so good. Itching, suspect being interferon, has largely vanished. The aim is to slow down, as far as possible, transformation to MF. I don’t dwell on my condition and I eat, drink, take overseas holidays and make merry much as before.
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