Wednesday, June 26, 2013

Jakafi to the Rescue

Myelofibrosis Treatment Banishes Behcet's... Coincidence? I think Not

About one month after my Autumn 2009 PV (polycythemia vera) diagnosis, I started getting unexplainable lesions in most inconvenient locations (I've shared this over a year ago). After several months of excluding many possibilities, it was diagnosed as "Behcet's Disease/Syndrome" or "Probably Behcet's" in March, 2010.  

I found it odd that I would acquire an MPN (myeloproliferative neoplasm) and an auto-immune disease around the same time (mid-40's). My internist said anything is possible; my hem/onc and rheumatologist said there was no connection. 

While the medical literature didn't show any links with Myeloproliferative Neoplasms (MPNs) and auto-immune diseases, the question persisted in my mind.

Chemical Reactions or Physiological Changes?

I took Hydroxyurea (HU) to manage the polycythemia vera, along with occasional phlebotomies. When the Behcet's became unbearable, I was treated with prednisone for over a year to manage the flares. I then switched to Remicade infusions when I couldn't stand all the side effects of prednisone. The Remicade worked like a dream for six months. The last six months of using it, however, I endured debilitating inflammation and joint pain. My rheumatologist pointed to the PV and my hem/onc pointed to the Behcet's. Even getting second opinions in both fields kept me in a painful mobius loop.   

Last November, a gastroenterologist friend said that the inflammation symptoms were consistent with what some of his Crohn's patients experience after a time on Remicade.  I was in a conundrum: which pain do I prefer -- the hands, feet, and joint pain or the lesions pain in other equally important body parts. It was a false choice. 

At the same time, all my blood counts were on a slow but stead descent and we knew something new was happening. I quit taking the Remicade and decided to use prednisone when a flare was just beginning. It took about eight weeks for the Remicade to fully leave my body.

Enter Jakafi... After being diagnosed with post-PV Myelofibrosis in February 2013, I began a low-dose regimen of Jakafi to shrink my spleen, ease bone pain and night sweats.

My last Behcet's encounter was the first week I started Jakafi. I hit it with prednisone to keep it from going full flare. It subsided within 4 days. I haven't had another flare since.

Apparently, there has been research on the JAK pathways and their relationship to auto-immune diseases. Pfizer now has TV ads for Xeljanz (tofacitinib), a "JAK inhibitor for Rheumatoid Arthritis."  RA is another auto-immune disease.

I realize that I am but a sample of one, but this experience gives me solace that my hunch that these two rare/oddball diseases share something in common. The JAK pathway!

When Focus Needs Perspective  

We are living in a most remarkable time of scientific discovery. 
Researchers are able to focus on changes at the molecular level and tie to proteins, genes, kinases and other terms I can barely pronounce.  

Many times I've been frustrated by the very narrow scope of this research because it doesn't help me NOW when I'm hurting. 

I must remember the adage: Follow the money. 
Research funding is tied to very specific research questions and tight timeframes, influenced by the funder(s). Funding is extremely competitive. And those research dollars rarely fully-fund a project, much less encourage researchers to follow new paths that emerge as they do their work. 

So blood cancer researchers continue their diligent work and auto-immune disease researchers continue their work. All the work is vital to understanding the disease processes which will ultimately inform us on effective prevention strategies. 

At the same time, a funding mechanism that supports tying all this knowledge together must occur to make the whole larger than the sum of its parts.
Stand Up to Cancer is a funder of innovative and collaborative research and information sharing among experts of different types of cancers.  

Perhaps it's up to patients/trial subjects (whole beings) to share our experiences, observations and questions. We can help the subject matter experts "connect the dots" in ways that may be outside a study protocol, but useful nonetheless.

After all, we are not only the patients, we are the consumers of the eventual treatment or cure.


wayne RODRIGUEZ said...

It is always a smart idea to get an second opinion in cancer diagnosis Ontario . Because, other general medical cases are usually straight forward. After diagnosis, treatments are made. So there is no need for a second opinion in most cases. But in case of cancer, there is much fear, many questions and misunderstanding.

Mary T. said...

I'm in the same situation you are, I have Rheumatoid arthritis and Uveitis and also PV with some fibrosis. I'm currently taking humara with some results for my eyes and also, by some strange coincidence my blood counts. Most likely because all three illnesses ahve the same Jak pathway.
I have been bugging my rheumatologist to switch me to xeljanz, but she said it hasn't been proven to stop eye inflammation, yet.
It would be nice to get a three for one medication.
Very best of luck on your bone marrow transplant.