I’d be grateful for any advice or guidance on starting Interferon for ET CalR mutation.
I was diagnosed with ET approximately 12 years ago when I was 57. I took Hydroxycarbamide in increasing doses over an approximate 6 year period when the efficacy of the drug began to wane.
I was then prescribed Anagrelide which I took for two years until the pandemic hit. I was supposed to have had a BMB after two years but with no face to face appointments with my haematologist , I was left on it for a further two years. The consultant who had been treating me retired and a new consultant appointed and she called me in and said she’d like a BMB done as soon as possible. The result was that I had grade 2 bone marrow scarring and she took me off the Anagrelide and put me back on Hydroxycarbamide. However, she also suggested that I consider starting Peg- Interferon.
I started back in the Hydroxy while I thought about it and after an initial platelet rise it then stabilised at around 450. However, it was raised again at my last blood test and my Haemotology Nurse who monitors the blood tests said she would discuss this with the Haematologist. The upshot was that they both thought it was time to start interferon.
I received a package from the hospital via my surgery with the pre- filled syringes. I’m in a complete quandary now as I’m unsure about proceeding as I have an added complication. I was put on statins four/ five months ago because of a slightly raised cholesterol test. Since starting it I’ve noticed some strange joint issues, particularly in my fingers. They are sore and I have the symptoms of ‘trigger finger’ in my index finger of both hands. It’s quite painful so I thought I’d better discuss this with the doctor before adding a second variable to the mix. I have been given a telephone appointment in six weeks time- the first available appointment it seems at my surgery.
Should I wait until I’ve spoken with the doctor about the statin issues or should I plough on with the Interferon? I’ve messaged my Haematologist and not received a reply yet. I’m going to a wedding next week and will need to drive a round trip of around 600miles from Cornwall to the north and back on my own and I’m concerned about the possibility of feeling unwell as well as the uncertainty of delaying the Interferon unnecessarily!
Any advice would be gratefully received.
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Reggieroo
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I would suggest that it is worth considering Pegasys in your situation. I am not sure why Jakavi (ruxolitinib was not recommended given that you have Grade 2 bone marrow scarring (fibrosis) . You may want to ask your hematologist about this option. In light of the Grade 2 fibrosis, suggest you ask your hematologist about your current diagnosis as well.
Regarding the joint pain, this is a known stain side effect but muscle pain is more common. Joint pain is also a known hydroxycarbamide adverse effect. Joint pain can occur for other reasons as well. The timing of the symptom may be your only clue as to the cause.
It is a calculated risk as to whether to start the Pegasys before or after the trip. While many people do not have an immediate reaction to PEG, some do. You did not mention your dose. Most start at 45mcg, which is usually 25% of the standard 180mcg prefilled syringe. You have to waste the rest. The norm is for a nurse to train you to do the injection unless you already know how. It is best to do the first injection in a medically supervised setting just in case of a very rare severe reaction. One of our MPN friends who is a doctor went to her local hospital and did the injection there without supervision so that she would be in the right place should she encounter a problem.
Please do let us know what you learn and how you get on.
I appreciate your response. I have been very conflicted about my current treatment. Jakavi has not been mentioned at all as a consideration. So, I will definitely contact my Haematologist regarding that as I’d like to have a discussion about all treatment options. Getting an appointment to do that in the short term will not be easy but I’ll definitely press for that.
I know that side effects from Hydroxy can develop at any time so it’s difficult to know whether it’s the long term use of that or the recent taking of statins that are causing the joint pain and stiffness in my fingers. However, I think that reading your response I will hold off injecting the Interferon as I haven’t been given any instruction or direction for injecting it. Nor have I received any advice from my team about the possible problems. My information regarding it has come from independently reading the leaflet that came with it and some internet research- which, isn’t always helpful and can be confusing. I would like to have a medical professional to show me how to do it but when I enquired about this with my haematology nurse she said she’d never performed one with a patient and wasn’t able to show me.
So, I will continue with the Hydroxy and send a message on Monday to my Haematologist asking for an appointment to consider other treatment options. I feel that with a long drive to the wedding next week I will hold off on interferon until I have had a discussion with my Haematologist.
Could you tell me why you think Jakavi might be a better option for me? I know from your posts that you do a lot of research and analysis of MPN treatments so I would value your opinion on it.
You cannot safely take any medication without instructions, including dose. If you have never self-injected, you need to be shown how. It is very easy to do once you know how. What the hematology nurse said about not knowing how makes no sense. This is a simple subcutaneous injection. All nurses are trained to do them.
The next step I would suggest is getting an updated diagnosis. Your hematologist should explain the significance of the Grade 2 bone marrow scarring (fibrosis) and the implications for your diagnosis and prognosis. I would not make any treatment decisions without being clear about my diagnosis and all treatment options.
Pegasys and Jakavi are both viable options for treating a MPN like you describe. These two medications have different mechanisms of action. Jakavi (ruxolitinib) is a JAK-inhibitor, downregulating the JAK-STAT pathway that is deregulated by the CALR mutation. It is known for good symptom control, including reducing splenomegaly and possibly fibrosis. Pegasys (peginterferon alpha 2a) is an immune modulating medication that is anti-proliferative and may reduce fibrosis. I would not suggest whether Jakavi or Pegasys is a better choice for you. That depends on your treatment goals and MPN profile.
I would suggest reviewing your diagnosis and treatment options with a MPN Specialist. MPNs are rare disorders and most doctors, including hematologists, have little experience with them. Here is a list. mpnforum.com/tsr-the-list/
I did my first injection at my hematologist's office after being shown how by a nurse. I stayed under observation for about 20 minutes to ensure there were no issues. There were not. I have never had an immediate reaction to the interferons.
Here is just a little bit of information about these two options. Note that there are more choices than these two, including other JAk-inhibitors such as momelotinib.
Thank you for all the links that you have sent me to enable me to read more about my condition, treatment and prognosis.
I will spend some time reading through it this weekend.
With reference to the nurse in the haematology dept - she said she’d given subcutaneous injections but never with pre- loaded Interferon. She said very few patients had been prescribed Interferon so she hadn’t had any experience of using the pre- loaded syringes. She did suggest I go to my doctor’s surgery and ask them for injection instruction and she did discuss the dosage with me, which was, as you said, 45mcg’s.
However, one revelation from your reply that shocked me was that not all haematologists are experts in MPNs. I’d naively assumed they were. My haematologist is not on the list you sent. Although there is one on the list at the hospital where I receive my treatment. I’m not sure if I’m able to ask to see them as I have always seen the same Haematologist since my previous one retired three years ago.
I do think that it is very difficult to navigate this medical maze of MPNs without specialist knowledge. I feel like I fumble through the internet during my late night browsing rituals, making accidental discoveries and trying to decide what is relevant from the information / disinformation- trying to understand the implications to my own diagnoses and treatment . It’s not easy and can be very confusing.
Thank you for taking the time to signpost me to some research that might benefit my decision making. I appreciate it very much.
You are certainly welcome. I hope the information helps. It is important to know that you are not alone on this journey with a very rare disease.
You may find it helpful to attend some of the MPN Voice Forums that provide excellent information and the opportunity to meet others with MPNs. mpnvoice.org.uk/get-involve... MPN Voice also has a MPB Buddy program that you may find of interest. mpnvoice.org.uk/get-involve...
It is very important to consult with a MPN Specialist about your care. MPNs are rare disorders and most doctors, including hematologists, have little experience with them. Optimal care requires MPN expert input by a provider who is up-to-date on MPNs. So much has been learned in recent years. Our treatment options have improved greatly but accessing this can depend on having a knowledgeable provider. It is your prerogative to change providers to a MPN Specialist. It is not about the doctor's ego, it is about your health.
You have really open my eyes to this whole world of MPN diagnoses. I’ve already had a look at the links you’ve sent me and, I have the say the first one contains flashes of acknowledgment but it’s pretty hard to decipher the message unless you have an in depth understanding of the jargon and medical references! You, I’m sure you can extrapolate the inferences… but I’m left in limbo - thinking- How does that impact on me and my diagnosis?
How on earth do you begin to understand the readings and how they impact on your diagnosis? Seems I’m on a long journey in snippers alley!
The way you understand the readings is to educate yourself about what they mean. It takes time to build a base of knowledge, but is it worth the time and effort. Here are a few more resources that may help.
Article on updated diagnostic criteria for the three classic MPNs, ET, PV, MF. Table 1 does a nice job summarizing the criteria. ncbi.nlm.nih.gov/pmc/articl...
The next step will be to consult with a MPN Specialist to ensure you understand your diagnosis and opt for the most optimal treatment plan. This is a long journey with plenty to learn. Have confidence that you will learn what you need to know.
Yes I will try to get to grips with it. I’m afraid I’ve been a bit of an ostrich over my diagnosis and treatment. I’ve never really had any symptoms and only found out by accident that I had ET.
It’s never manifested itself in my everyday life…. Apart from when I was on Anagrelide- I became very short of breath. I particularly noticed it when I was walking my dogs or playing tennis, which I do 3/4 times a week. Once I was taken off it and resumed hydroxy my haemaglobin levels went up and my breathing was better!
I was also very sick when I got Covid. I got it before I was vaccinated and it really knocked me for six!
I am a bit frightened of changing to interferon though. I’m worried about the long list of side effects and concerned about the impact in me.
I really appreciate your support and explanations as well as the advice to learn more about how to interpret results and build a knowledge base that will help me understand my condition better.
I have asked for a buddy in my area now using the link you sent me . I have also left a call with my Haem Team to call me at their earliest convenience to discuss my concerns. Although I’m still unsure about changing my consultant at this juncture as they have been instrumental in arranging a telephone consultation for me with Professor Claire Harrison. I had an initial conversation with her in April last year and I have another later in the Autumn where I can discuss my current plan.
I have been told by another MPN voice contributor that Jakavi isn’t available for ET in the UK. I will check that out when I speak to the team.
Finally I have looked to see if I can join forum in the UK but the nearest one this year is in Leeds which is 600 mile round trip for me. However, I’ll keep an eye on that to see if I can find one nearer to home. I will also join the next online forum which deals with my condition.
You can have two hematologists involved in your case if you wish. This is what i do. I consult with a MPN specialist regarding my care plan , but this doc who is too far away to be practical for ongoing care. I also have a local hematologist who handles my ongoing care. This arrangement works great. There are others on the forum in the UK who make this same arrangement.
Dr. Harrison can do the best job of explaining the results of your BMB, including the finding of Grade 2 scarring (fibrosis). Please do let us know what you learn.
I will. I’ve left a message for a call back with my Haem team. I’ve sent an email to join the buddy system and I’m checking out any online forums that might help me gain more background knowledge.
So I’ve made a start. I’ll keep you posted on my progress.
I’m so grateful to everyone who responded. Makes me feel less alone in my journey!
Unfortunately, Jak inhibitors are not licenced for ET in the UK. Jakafi (Ruxolitinib) has recently been approved for PV patients for whom HU has failed, but ET was excluded.
So I don’t suppose there is much point in me discussing that with my consultant? I would like to discuss a few other concerns though, so I’m going to call them today to see if I can get an appointment.
There is never any harm in asking questions (or there shouldn't be!). The more you understand about the treatment options, and the more your haematologist understands about your experience of the condition, the better. These conditions are not like a broken leg or an infection, where the remedy is obvious and the same for everyone. The haematologist can't just look up in a book and find "the treatment for ET". You and the doctor between you need to work through the options to find the best treatment for you. It will always be a compromise between the effects of the disease and the effects of the medication. I wish you all the best, and it is vital to find a consultant who will discuss everything you are concerned about, including interactions with other medications and conditions.
Yes I agree. My Haematologist has been proactive in helping me but the problem is with the fact it’s not always easy to get an appointment. I only usually see the consultant once a year. The rest of the time my condition is managed remotely. I have blood tests every 6-12 weeks depending on my current stats and then a follow up call with a haematology nurse.
I do need to be much more proactive I can see that now! I have been a bit complacent and because I’ve had so few overt symptoms up till now I’ve just accepted the status quo!
Yes, it can be hard to stick up for yourself, especially when you know how pressured the health service is. But it is best for them to find an effective treatment earlier rather than later, too.
Yes you’re right although I’ve always found my new Haemotologist to becoming my corner and approachable. They facilitated a telephone consultation with Professor Claire Harrison for me and they always listened to me. The problems have all been related to being unable to get an appointment because of the pressures in the NHS.
I would not be reluctant to give the PEG a try, After all, i did and it suited me. Doing the first subcutaneous injection at the hematology office is a good idea. You can learn how to do it (very easy) and have your first injection observed just in case of the extremely rare serious adverse reaction.
I hope you will have the chance to meet directly with Dr. Harrison. She would do a great job walking you through your BMB results and explaining what they mean. That will facilitate your decision making and help you to feel comfortable with your options.
I am feeling better about it all now. I sent a message to Dr Harrison and received a positive reply.
I also have my appointment date to get my first supervised injection at the hospital.
I also have a follow up appointment with Dr Harrison in the Autumn but that will be a telephone consultation.
I leave for the wedding tomorrow so I’m pleased I’m not dealing with new meds with such a long drive. I’m also visiting my son in Leeds for a few days after the wedding. I’ll be mentally prepared when I get back to getting with it.
I really appreciate your input and all the support and advice.
I really think you should start the Peg treatment. I’ve had Peg for several years now and I’m on statins and have had no issues. I’m a bit surprised that they haven’t given you an appointment to show you how to do the injections though.
I have ET and have been on interferon for about a year. I take statins and several other drugs with no real issues. You definitely need to take the first injection at the hospital and be instructed how to do it. A nurse at the medical day unit showed me how to do it, although she had never shown anyone else how to do it but she figured it out. I am very squeamish and can now do it easily. Enjoy the wedding x
That’s good to know. I have been dreading starting it because I’m concerned that I will feel ill every time I inject. My terrible reaction to the Covid vaccine has made me a bit reticent!
I'd also vote for Pegasys. There's a chance Pegasys could slow or prevent your Grade 2 fibrosis from progressing to grade 3 and possibly even reverse it to grade 1.
I’ll be speaking to my haematologist as soon as possible and checking out some of my concerns. If I do go ahead I’ll also follow Hunters advice and ask them to show me how to inject it while I’m at the hospital.
If it helps with my fibrosis- that would be a big plus!
I have ET and was taking up to 4 x Hydrea per day for around 2 years. My Haematologist has recently swapped me to Interferon injections x 1 per week and platelets are still stabilising. I need to take it for another recently diagnosed rare blood disease also, but we had intended to move to interferon. It is very easy to use, but takes getting used to re side effects, for me it’s digestive issues, for most other it is flu like symptoms. I’m in Australia and my Specialist Haematologist is the only Doctor who advises me on medication, II keep her informed on other medications I am on and I follow her advice. Interferon has to be kept cool in the fridge and in a pack with ice/cooler blocks while travelling - I’m flying 3 hours within Australia on Friday and have had to get a letter stating I need to take hypodermic needles through airport security. So it’s a whole new world of fun. But hey, it’s medication that will keep us upright, so just go with it if your Haematologist is ok with the other medication your are on - which they should be aware of before prescribing Interferon.
Also, If you are not use to self injecting you should get some training from a nurse at least. also be sure about the amount to inject. Here in Australia you start with only a third of the pre-filled injection.
Thanks for your reply. I am going to try and speak to my haematologist tomorrow to check out a few things I’m still having doubts about. One of those issues is whether you get flu like symptoms every time you inject? If so how long do they last for.
I am a bit concerned about it because every time I had a Covid Injection I was ill with flu like symptoms for a few days. If I’m regularly injecting interferon every two weeks will I feel ill every time?
Hi, I am 61 and been on interferon for 7 years. I have been on statins, warfarin and aspirin for 20 years. I’ve had no issues with side effects aside from muscle aches at night. For that I take 2 paracetamol every night before bed. My numbers are steady (170 from originally 1250) I have gone from 45 mgs per week to 90 mgs every 3 weeks i.e. 1 injection every 3 weeks. Based on my experience I would go for it. Good luck.
I have had quite a few replies now to my original post. All have been pro- interferon. I would just like to have a final discussion with my haematologist about the symptoms I’ve recently developed in my hands - the joint pain and trigger finger- to ascertain whether they think it’s the statins or hydroxy that could be causing it.
Actually I’ve taken an executive decision to halve my statin dose until I get to speak with my GP. The joint pain is much reduced and the popping in my fingers has stopped so I’m already thinking the statins were the cause.
Thanks for your positive endorsement of interferon. I’m just delaying my decision till I speak to my haematologist and after the wedding.
peg interferon is excellent. I changed from Hydroxy to Peg . I inject 45 every 4 weeks . You should speak with an MPN on phone as you always start on a very low dose . I do it mornings. Drink lots of water. If anxious then do it after your trip. I flew away & injected at beginning & was fine but only if you start on low dose. Julia . Exeter .
my instinct would be if possible not to start the Peg until your ready, is it an option to continue in the short term on Hydroxy, do your long drive , discuss Peg with your docs and when your comfortable start Peg then.. It’s seems starting Peg for your MPN is a good idea but explore your concerns first if you can.
I think the only people who can properly advise you on this are your doctors, so it’s worth pushing for an earlier appointment on the basis that you have worrying symptoms and don’t know whether it’s safe to start the new medication that you have been prescribed. Lay it on a bit thick if necessary!
Reggieroo it's such a big decision anytime you have to go on a new medication. There always seems to be one side effect or another, so you are left weighing the pros & cons of those effects vs your current symptoms and problems. I cannot speak to your interferon question because I don't take them, but I will mention that I was put on a statin about 2 years ago, not because I had high cholesterol, but instead because the doctor said since I have PV it would help to reduce inflammation. I'm on the lowest dose but still managed to have leg, ankle and foot cramps, in addition to the cramping in my fingers (the trigger finger). As you've said, it's very painful when your finger locks. Thankfully I do not get it anymore. My doctor suggested I take CoQ10 to help with the cramps. Apparently statins deplete something in us. Maybe ask your doctor about giving it a try. Hope you get all the answers you need to help you make informed decisions.
Sorry somehow I missed your post. That’s very interesting and I will speak with my doctor about it but my appointment isn’t for six weeks! So I’ve self medicated by halving my dose until I can get speak with them. I’ve still got cramps in my fingers which are locked in the morning but the ‘trigger finger’ has eased a little. I’m meeting a very good friend at the wedding at the weekend who is a newly retired GP so I will speak to her about it too. She’s always been very helpful!
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