Steven S

Steven S

Aged 76

Managing my MPN against a plethora of medical issues

I was diagnosed with ET about 30 years ago. This progressed to PV about eight years ago. I also have another kinase-based disorder – Neurofibromatosis type 1 that complicates things a bit.  NF1 is a non-driver mutation for MPNs, increasing my risk of progression to AML.  It is also a separate disease state that causes it own issues.  The NF1 lead to a brain tumor that I had to have resected back in June 2019. A year before that, I had to have a heart surgery for tachycardia (catheter ablation). Six months prior to that I had a Green laser PVP; that was a terrible surgery!  That experience taught me a very important lesson in how important it is for patients to advocate for themselves.    I have also recently had another type of tumor removed from my mandible that was a lipoma with osseous metaplasia.  It has been a rather interesting couple of years!.

I am hydroxycarbamide intolerant and phlebotomy-only treatment didn’t work for me due to the adverse effects of chronic iron deficiency.  In May 2021 I started  treatment with pegylated interferon, Pegasys .  I am quite fortunate that a weekly dose has resulted in good control of the erythrocytosis and thrombocytosis and I have not encountered any adverse effects.  It seems that PEGylated Interferon is the right treatment option for me and I am hopeful that I may achieve hematologic remission and even molecular remission as well.  Given the additional NF1-nondriver mutation, the chance to reduce my risk of MPN progression portends a more favourable outcome.  I really am fortunate to be having such a positive response.

Managing the MPN in the context of all the other stuff has been a real learning experience.

So how to cope? I think we all find our own ways and here are some things that work for me:

1. Support from my family, friends, and faith community.
2. The MPN Forum: (my friends and MPN Family).
3. Maintaining your sense of humour and finding ways to have fun, no matter what.
4. Surrounding yourself with things that are positive and lift you up.
5. Mindfulness practices – I practice Qigong.
6. Say the Serenity Prayer every day and take it to heart!
7. Educate yourself about your condition(s). Knowledge is power.
8. Look to find a high-quality treatment team who you trust.
9. Advocate for yourself. Assertive patients receive higher quality care. Passive patients do not.   Remember that you are in charge of your care. It is your goals, priorities and preferences that must drive your treatment. Empower yourself to deal with the MPN.  I have become a very strong advocate for patient empowerment and education and I try to support anyone navigating care systems to get their needs addressed optimally.

I have been blessed to have a relatively indolent form of a MPN and have led a long rich life which I plan to continue, despite what happens with the MPN. Sure, there have been some challenges with the ET-PV and the other related and unrelated medical issues I face. Sometimes you have to do, as the military folks say; “Embrace the Suck” or just “Suck It Up Buttercup.”  However – it is not all “Suck.” There is plenty of good in life to enjoy and embrace. As the prayer says, ‘I seek the serenity to accept what I cannot change, courage to change what I can, and wisdom to know the difference. ‘

One thing many people worry about is longevity.  I do not.  This is my philosophy about that.

Longevity

1. I understand what the MPN and NF longevity studies and MPN risk calculator show – these are statistical projections, not an individual prognosis.
2. I expect to live a relatively normal lifespan for an American Male (76.1 years), but not likely well into my 80s. However, anything is possible.  Treatment options are improving.  Perhaps there will be something curative in my lifetime.
3. I have no plans to die on a schedule.  No MPN/NF time clock ticking away.  My focus is on living a high-quality life for however long that is possible.  All treatment is geared toward this goal.
4. I do not fear death.  As a person of faith, I know something better awaits me; however, I am not in any hurry to exit this stage of my existence.  There’s plenty left to do and enjoy in this life and I plan to stick around for as long as I have a decent quality of life.