I really appreciate the information you all bring to the site. My daughter, 17 yrs old was diagnosed JAK2+ ET two years ago. It has been challenging with the pediatric hematologists, because of their lack of knowledge for this particular condition in pediatrics. I am extremely apprehensive to start my daughter on hydroxy, which was recommended by the two local hematologists that we have seen. Her platelets range between 700’s - over a million (when she gets sick).
The pediatric MPN Dr that we telemed with initially backed up my vision of keeping her medication free, until our most recent appointment. She asked my daughter about the symptoms she has which are, frequent headaches (which run in our family) fatigue and neck/back pain. She is currently taking the turmeric with CURCURWIN. Then the Doctor suggests maybe starting her on Hydroxy or IFN, to POSSIBLY help minimize or eliminate some of the symptoms. I’m totally confused by this since she originally supported the no meds concept. Nothing has changed health wise with my daughter since the initial diagnosis. After reading your experiences, I am even more afraid to start those meds at her young age. Does anyone have any advice for us to consider? How long do you have to stay on these meds once started? Could either of these medications possibly cause everything to reset to normal ranges within a short period of time? All of her other labs are within normal limits, with exception of high platelets. We are transitioning to an adult MPN specialist at the Mayo Clinic soon, as she is turning 18.
Again, thank you all so much for posting your experiences , offering kind words of support and wisdom , and sharing insight on how to manage this dreadful situation.
Well wishes for you all,
Angela
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There are two questions embedded in what you have asked. Is cytoreductive medication indicated? What cytoreductive medication would be better?
Is cytoreductive medication indicated? In the absence of thrombotic or hemorrhagic episodes or evidence of Acquired von Willebrand Disease, then PLT numbers alone would not justify cytoreductive treatment. Sometimes cytoreductive treatment is indicated for symptoms like fatigue, headaches and neck/back pain when these symptoms are attributed to the ET. The question is whether the intrinsic risks of cytoreductive medication are justified by the potential benefits. The tricky part being that headaches and fatigue are also part of the potential adverse effects, but also one of the potential benefits.
What cytoreductive medication would be better? Many MPN Specialists would recommend IFN over HU for younger patients based on the long-term risks associated with HU as well as the intrinsic risks of HU use with people of childbearing years. Note that hematologists not regularly treating MPN patients are less likely to be familiar with IFN use for MPNs. This is an issue best discussed with a MPN Specialist familiar with IFN use for younger MPN patients.
IFN vs HU is not a black-and-white issue. Part of the equation is what your daughter's treatment goals, risk tolerance and treatment preferences are. She will soon be the sole decision maker for her treatment. What does she prefer? Does she believe that the potential benefits of cytoreductive treatment justify the intrinsic risks? Based on the risk/benefit profile of each choice, which would she prefer?
Note that you can stop any of these medications after you start if you find that the choice is not suitable. You simply return to the original baseline. That is exactly what I did the last time I tried HU. It was not suitable for me so I discontinued it. I later initiated treatment with the IFNs, which significantly improved my quality of life. That, however, is my treatment outcome as a 68-year-old with PV. We are each different in what we need and how we respond.
It sounds like the primary concern is symptom control rather than cytoreduction per se. Another option os to try other interventions to address the most bothersome symptoms. As an example, some type of headaches (migraines) respond very well to CGRP inhibitors. It may be that there are other symptom-relief options are worth exploring.
It is a good thing that you are planning the transition from pediatric to adult care. This is a very significant transition for all young adults, but much more so for a young adult with a rare disease, There are some excellent resources here for the young adults and their parents. gottransition.org/ .
Thank you so much for your reply! It is such a relief to have a wonderful and supportive community to turn to with all of my anxiety and questions about this situation.
Hi. As your daughter is turning 18 soon, perhaps it’s better to wait until she transitions to an adult specialist with MPN expertise. She’s in it for the long haul so maybe a long term view would be helpful.
There is ever increasing recognition of the particular needs of younger people with MPNs in terms of management, treatment and support. (Certainly here in the U.K. and at Guy’s Hospital in London - and presumably you’ve checked out the MPN Voice website.)
With changes in awareness, earlier diagnosis and a much broader offering of treatments, the MPN landscape has changed completely and it is no longer regarded as a disease of older age.
It’s definitely worth exploring your daughter’s options further and having that conversation with the MPN team responsible for her care going forward. All the best.
Current research indicates a the earlier, the better, for Interferon. Using a low dose Interferon will minimize side effects, while giving the body time to adapt including a possible (partial) remission.
HU only reduces the blood cells, without providing the possibility of a remission.
From my point of view, starting with HU at that young age, without the need having to, would not be wise.
I'm part of a group of about 60 younger patients (ages 20-50) in Europe and most of them are on Interferon.
I would definitely see what the adult MPN specialist says. HU would scare me at this age if it was me. I would consider it with the understanding that it is temporary. Their are some people who take it for many years but like Hunter said it is definitely not good if she wanted to have a baby and even though the risk hasn’t been proven that it causes leukemia it still lists it. So, if she is on it from 17 through her life that would worry me. Other issue is skin cancers. That is a real thing with HU. Yes, interferon has its issues, but not everyone gets them. I am on Besremi and I like it. I am not 17, but I am 41. It has been amazing for me. And I would think with your daughter being so young hopefully she would respond quickly and without side effects. Wishing her the best!
Totally agree, the problem with Interferon is, that you will not see a fast effect. In case you need a fast effect, then maybe HU is a good starting point to reduce the high platelet values and then switch to Interferon. Though the HU dosing usually is very low, the amount over time, given the age, with HU only, is very high.
I was diagnosed ET Jak2+ in December 2020 and my daughter was diagnosed in February 2021.
I have PV now and take 90 mcg of pegasys weekly also take a low dose aspirin daily. Daughter now 22yrs old takes the low dose aspirin daily and has blood checked every 6 months. The aspirin has helped her with the headaches. She has always been active and eats very well for a university student. She still has stiffness in the back and neck occasionally, especially when studying.
We are both under the care of the same family doctor and MPN specialist. We live in different parts of Canada but she flies home for in person appointments. She still travels and is involved in all her activities.
She had a 2 hour telephone meeting after diagnosis with an MPN specialist and a gynaecologist who recommended birth control for her ( she was having terrible periods, long heavy cycle) and talked about what a future family, pregnancy and babies would look like for her.
Her platelets range between 650-800 and they have told her she can go much higher before they will intervene. I was hitting 950 so I was lucky to be offered Pegasys and am thriving on it!
Upon my diagnosis the specialist said “ your body has a secret, it can make blood clots” well it’s not a secret any longer and we have all adjusted to make sure it doesn’t happen.
Good luck! Find a specialist you both like and trust!! 🌸🌺🌼
Why don’t you email Professer Harrison at Guys Hospital London UK who is an expert & excellent MPN . She works with young people & no doubt would kindly help with advice. Always good to have second opinions 👍 Julia . UK
Hello, I am sorry your young daughter has to deal with this. Was she ever put on daily low dose aspirin? This would be my first option, then get labs done a month later. The problem with high platelets is causing clotting and embolism.
Discuss all options with her team that will allow her to be more comfortable.
I started hydroxy daily when fort diagnosed in March 2023. My new set of labs showed I was responding well and now I take one every other day plus aspirin.
Best wishes to you and your daughter in finding the best treatment!
I think it's totally understandable that you want to keep your daughter off of these medications - they're not "light" medications, and from my research and experience with an MPN, they shouldn't be the first thing that's jumped to by doctors/specialists, especially if it's for managing symptoms.
Inflammation plays a significant role in MPN symptoms, progression, and blood clot risk. If you have the JAK2 mutation (I don't know about the other mutations), it can cause your body to naturally be more inflamed. This can lead to a number of MPN symptoms.
Inflammation is impacted by lifestyle, so if you want to avoid medication, I'd suggest really putting effort into making lifestyle changes/optimizing your daughter's health in general.
Lifestyle/health things you can look into include:
-Following an anti-inflammatory/mediterranean diet
-Improving gut health/microbiome
-Exercise/physical movement
-Getting good sleep
-Learning how to manage stress well
-Looking at vitamin/nutritional needs that aren't being met
If you want to work with a medical provider to make these changes, you could consider an integrative/functional medicine practitioner or naturopath.
This video by MPN specialist Dr. Angela Fleischman does a good job explaining inflammation and MPN's, and the impact of nutrition: youtube.com/watch?v=Dh0H418...
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