It will be good to see the back of 2017.
Leaving aside the political and military mayhem that has gripped much of the world this year, the last two-thirds of 2017 have been a personal rollercoaster of pain, despair, hope and near death.
It’s the weekend after Thanksgiving. I am a little surprised to find myself back on my feet, 40 pounds lighter and a little unsteady but easing back into the hunt. Ahead are the December days ending this year’s journey through hell, the last acts of the year, the long-awaited ASH meeting in Atlanta in two weeks, the Winter solstice, the dropping of the Times Square ball to light up 2018.
Meantime, cachexia. My photo tells it all. A couple of weeks ago I was back in hospital. My kid took this photo. It’s like Kafka’s story In the Penal Colony. There, a prisoner is loaded into a machine that writes his transgressions into his body with penetrating needles. Look at the stripped off fat, wasted muscle mass, the stick arms, prominent shoulder bones, sunken cheeks. Behold: cachexia written clearly in the flesh.
(And that pot belly isn’t an MPN enlarged spleen. It’s the filling ileostomy pouch, an accessory glued to my belly following colorectal cancer surgery.)
So that’s the first message of this Return Tour of the Zhenya Senyak Road Show. Our MPNs are no guarantee against secondary cancers. In fact, the genomic instability, compromised immune system, and inflammation all associated with our MPNs place us at greater risk for mutations that might grow deadly. We need to be vigilant. And get a colonoscopy and keep on schedule. It’s quick and painless and an easy alternative to months of chemo, radiation and surgery.
But I’m not “Lazarus come back from the dead, come to tell you all.” Just passing along the hard-won wisdom of having screwed up my own maintenance out of arrogance or just laziness…and hoping you’re a lot smarter and more careful.
The Second Act of this Return Tour is to chat a bit about legacy. What’s ahead for our MPN community? We’ve lost strong friends and advocates in recent months– Arch, Harvey, Kathy and Peppe come immediately to mind. And there are many others. We have seasoned patients and caregivers and many new people have joined us. The Foundation is rolling out new initiatives and expanded publishing.
The shape of our new leadership is starting to come together. It’s a mixed bag of non-profit and frankly commercial organizations of patients and patient-entrepreneurs, of deeply experienced, committed people and half-baked Googlers. Yes I am prejudiced. I have counted on MPNforum to keep us on track. We were obligated to no one except each other, patients, caregivers, and volunteer scientists and hematologists. The Forum is fading into the background, its hundreds of articles and profiles will be on-line for years to come at http://www.mpnforum.com. Ellen Jacquart will be keeping the List of Hematologists current for now and from time to time we will publish special reports like Mary Cotter’s notes on the CR&T meeting earlier published this month.
But we will no longer be the watchdog investigating drug company claims, holding clinical trial sponsors and the FDA responsible for mismanagement that injures or kills our fellow MPN brothers and sisters. We will no longer mobilize support for creative scientific studies like the Fatigue Project or the Zebra Coalition. No longer launch investigative reports behind the CRISPR lab doors. Who will?
The Players. I am placing my faith in the future on four pillars: (1) The MPN Research Foundation and its leadership team. It would be hard to overstate how lucky we are to have this Chicago based group on our side; (2) Dedicated patients working independently like Julie Libon and her HikeMF project, Chris Harper and his Stem Cell Buddy crusade, Pastor David Denny and Diane Blackstock of the Myelofibrosis Support Facebook group, Ken Young and Nathalie Cook in Australia, a scattering of individual admins and contributors on Facebook, many new, bright patients and the daily advice and support offered by the email groups; (3) The scientists and hematologists specializing in MPNs who participate in patient support events, forums, and discussions. Where would we be without Ruben Mesa and Ruben’s Raiders in Scottsdale, Oregon and San Antonio, Dick Silver, Claire Harrison, Serge Verstovsek, Hans Hasselbalch, Jerry Spivak, JJ Michiels? We’d still be fumbling in the dark, bumping into each other with our pet theories.
More problematic and yet highly promising are (4) the commercial enterprises circling our community. Incyte of course with its billion dollar drug and ton of educational materials, websites, events and marketing strategies funds everyone…And there’s Andrew Schorr’s Patient Power with superbly produced videos, interviews with major credible MPN experts. (The problem I have always had is these are often sponsored spots, supported by drug companies and medical institutions, advertising appearing as journalism.) You will not hear critical comments on a Patient Power spot, no challenging questions. It’s a platform…always professional and sometimes useful, openly commercial (https://patientpower.info/sponsors/become-a-sponsor).
Another organization that has a clear claim on our loyalites is Ann Brazeau’s MPN Advocacy and Education. Ann has a long history of working with MPN patients. originally through the Foundation, and has created a sponsored vehicle to bring top MPN specialists to local areas at very low or no cost to patients. She also advocates for MPN patients in internationally, in Washington and at the VA. These are not income producing activities and her work gives us opportunities for up-close and personal patient/physician interaction. (mpnadvocacy.com/)
As a community, we’ll be OK. In recent years the MPN international community has come together on the internet and at events. With our growing awareness and greatly increased numbers comes the power to influence research and events. We’re tantalizingly close to a cure or at least long-term reliable management of ET, PV and MF through immunotherapy and gene therapy. Human applications and clinical trials have already started.
We need a strong, scientifically based independent group to monitor our options — specially clinical trials and new drugs. To illustrate the need, here’s the third and final act of my Return Tour. A quick look at the checkpoint inhibitors.
How could the growing popularity and proven success of checkpoint inhibitors not be a good thing? Cancer cells, tumors, evade immune system T-cell invasion by cloaking themselves in a naturally occurring healthy protein on the surface of the cell. This fools the T Cells into accepting the cancer cell as a normal, healthy cell.
PD-1 (Programmed Death) is a checkpoint protein on the T cells. Think of it as a switch that keeps the T cells from attacking normal body cells. While patrolling the body, T cells use its PD-1 protein to determine if a cell is healthy or not. It attaches to PD-L1, a protein on some normal cells. When PD-1 binds to PD-L1, the T cell leaves the target cell alone. Some cancer cells use PD-L1 to evade attack.
The checkpoint inhibitor essentially unmasks the cancer cell by inhibiting the normal surface proteins on the cell — or the associated proteins on the T cell — thus opening the cancerous cell to attack.
Despite dramatic results in improving outcomes in several types of cancer, checkpoint inhibitor use comes at a cost. Inhibiting those normal surface proteins affects lots of other cells in other tissues in other organs. Science Magazine (17 November 2017) reports autoimmune diseases resulting from cancer treatment with checkpoint inibitors. Thyroid disease, colitis, Type 1 diabetes and other autoimmune diseases resulting from an immune attack on the body’s own tissues have been reported after use of checkpoint inhibitiors
We need independent scientific review of findings and clinical trials by an active, committed patient leadership group working in partnership with the Foundation, the core MPN specialists and scientists, patient advocates and commercial organization. That’s a lot to ask of a patient population struggling with a rare and debilitating disease.
But like one of the patients on his way to the Cancer Center told me about his treatment plan, “It ain’t easy but it can be done. Maybe.” Working together we can beat this damned disease. There’s no maybe about it. We’re almost there.
Comments on: "“It ain’t easy… but it can be done. Maybe..”" (21)
Zhen Hope you recover as soon as possible. Look forward to reading more articles from you.
Thankgoodness you are on the road to recovery.
I am so happy to see that you are on the mend…sending hugs and prayers <3
zhenya , good to see you back , you have been missed but many peoples prayers and good wishes have been with you x
Thank you all for your kind, supportive comments. I know our MPNs can sometimes be mind numbing. But I hope that buried in your welcome back greetings is some thought of scheduling your own colonscopy. The rate of genetic mutation increases as we age…Add to that the essential genomic instability of MPNs and we have a clear need to be vigilant about our “routine” checkups and health maintenance. Love you all. If I come out the other side of this – and I plan to – I want to find you there.
Thank you for the very informative article.
I’m glad you’re on your road back to life.
I look forward to more of you reporting. No holds barred
Dear Zhen, I am so sorry to hear that you are not well. I pray that you recover quickly.
Zhen, You have been a very special friend and leader. You make me think in ways I might not have questioned without your suggestions. I pray for renewed strength and better quality of life for you. I hope you are able to make it to Atlanta and we can share a meal together. Sending HUGE HUGS your way.
Hello Zhen! So good to hear from you and glad to hear you have turned a corner. Thank you so much for all you do for us. Best wishes to your continued good health. Stay strong.
Zhen, I am so glad to hear from you and to lay my eyes on you. Sounds like you’ve been to hell and back…so glad you are on the back side and improving. You have been my inspiration for over 10 years. Pardon my selfishness, if I want you to stick around. Take care and try to keep kicking up those heels, old friend.
Zhen, thanks for letting us hear from you. You have inspired me for over 10 years now. Sounds like you’ve been to hell and back but so glad you are back and improving. Take care and keep on kicking up your heels, my friend.
Zhen, you inspire us every day through your courage and selflessness. Your voracious and inquiring mind have moved us forward as an MPN community, and like amazing individuals like Joyce Niblack you change the world around you. Feel better and I look forward to seeing you again soon.
Zhen, you are spot on with your assessment of the MPN community and players. As a well-researched and focused watchdog entity, MPNForum does fill a role that none of the others mentioned can. It is an essential component of any community to have someone pointing out inconsistencies or issues, especially when done out of love and concern and the drive to do right by patients, as has always been your motivation. Thank you and your partners and contributors.
Zhen, Thank you for your courageous reporting of your own condition, and for raising awareness of cachexia. Like many others, I’m so glad you are improving after this ordeal. Also thank you for reminding us to take our colonoscopies. I have to take them twice as often as most because of family history. And we should routinely check for other cancers as well. May you live long and prosper…with plenty of rest and comfort after this horrendous year!
Great to hear and see you dear Zhen. A bright star in my life. I send my deepest appreciation for your always interesting and informative newsletters. Warmest regards and get well soon.!!!
Zhen, keep fighting the good fight. So good to see your comments today. So many of us wish you well and care deeply.
Zhen, my friend, you are often on my mind . 2017 has knocked you about it seems but I’m very pleased to see you’re still fighting. Pls stay strong.
Zhenya, I was thinking about you earlier this evening, and was so glad to find this new article in my email.You have come through so much. Thank you for everything! Tomorrow I make that appointment with my gastroenterologist that is a little overdue. Onward to 2018!
Hi, Jane… Glad to hear it. It may be the best investment we can make — quick, painless, and effective! And thank you for the good thoughts.
Zhenya – I’m sorry you have been through such a difficult time – but glad you are on a comeback trajectory. Praying that you gain strength everyday.
Zhenya: Good to hear that you are doing better. I so appreciate your writings. Sending you many good thoughts.