Some Life Lessons Take a Lifetime

Patience and Pacing

If you let go a little, you will have a little peace.

If you let go a lot, you will have a lot of peace.

If you let go completely, you will know complete peace and freedom.

Your struggles with the world will come to an end.

~ Ajahn Chah, A Still Forest Pool

I believe that challenges (including people) show up in life because there is something I need to work on, learn, or accept. Sometimes I'm an exceptionally slow learner.

Patience and self-pacing have never been my strong suit. I like to live fully and make the most of every heartbeat: no regrets.. don't take life for granted.. live out loud.. expand my comfort zone.. learn something everyday.. help others.. push through fears.. you get the picture. Living with urgency has served me well. I've always had more interests than time. I lived a full, active life; I was highly productive and able to achieve many things. Sure, I'd crash periodically. But I could always recover and carry on.  

Pacing for Dummies

Then along came blood clots and Polycythemia Vera... my body said "No more!" to my career in community development at a particularly difficult time in the real estate and lending markets (circa 2007).  I went from fast-forward to the ICU. No one saw it coming. 

It took my body more than 3 months to recover from the surgery and my normal energy never returned. When I went back to work, I was hard-pressed to work 8 hours; I would collapse on the sofa as soon as I got home. Less than a year after the surgery I had to resign from my job. I was crushed!

I then kept my brain active with some part-time consulting and a lot of volunteer work that had flexible timelines. I also got more involved with my kids' activities. 

I believe the illness occurred to make me slow down and to re-direct me to be more present for my children during the teen years. I became the mom that could cart smelly kids to/from sports practice and help at school functions that took place during business hours. It became a gift for my soul. 

Pacing for Big Dummies

When I pushed through the extreme fatigue, headaches, and other symptoms of the Polycythemia Vera, the Universe devised a new challenge: Behcet's Disease (a rare auto-immune disorder). 

The physical manifestations were excruciatingly painful. Both the disease and the treatments caused severe physical issues that limited my mobility. For a time I needed a cane to walk, couldn't climb stairs, and certainly didn't leave the house unless absolutely necessary. I gained 60 pounds (and had the steroid "moon face"). I was so miserable, self-conscious and depressed. I became resigned that old age wasn't in my future. 
That slowed me down big time. I tried to make the most of "good days" even though a productive day would cost me a couple of days in bed afterwards. 
Apparently, that wasn't enough.

Patience and Pacing for Obtuse Big Dummies

This February came the curveball we didn't anticipate: aggressive secondary myelofibrosis. My bone marrow is no longer able to produce enough blood. Without a blood stem cell transplant, I could look forward to maybe two years of a transfusion-dependent life. 

Since none of my siblings "matched" me, we put our hopes and faith in the US and International Marrow Donor registries. 

The waiting was a real test of patience. I felt like the crocodile in Peter Pan who swallowed the clock: tick tock, tick tock, tick tock pounded in my head.

Then my chance for a miracle: a 22 year old young man from Germany is my perfect match! Talk about a 2nd chance!

45 Days Post-Transplant

The "typical" allogeneic transplant (donor cells) shows engraftment between days 10 - 25 after the transplant. 

What is time anyway?

My new stem cells are still not fully-engrafted. The whites are all donor cells. But they aren't producing platelets nor red cells yet. I'm still transfusion dependent; I need platelets every week and red blood cells every couple of weeks. 

The doctors and clinic staff remind me that it will take a lot longer because my bone marrow is hard (full of fibers), making it difficult for the stem cells to find a place to settle in. Over time, the bone marrow should return to its original spongy form (at least enough for a successful transplant). 

Here is where my patience is thin. Objectively I know that my body is starting over ~ in producing blood to support the organs. 

The feeling of helplessness and lack of control can be overwhelming some days. I try visualizing spongy marrow, talk to my precious donor cells, and count my numerous blessings. I'd eat broccoli and brussel sprouts if it would help (they would need to show me studies first). 

I do know it will all be worth it in the end. And I try my best each day (some day's "best" is better than others). One thing that helps A LOT is reinforcement from family, friends, and the medical staff that this is just part of the deal; I haven't done anything wrong; and resting is the best thing to give those fighting stem cells the chance to dig in and do their jobs.

I'm hoping to become a calm, cool, collected person through this experience. One who feels and exudes patience. One who enjoys life reasonably without creating physical burnout the next day. Patience and Pacing. These are my lessons.

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.

Let's Kick It Up a Notch (or more) -- to Mayo Clinic

I had a Bone Marrow Biopsy done at Northside Hospital on January 24th. The bone marrow biopsy and aspiration report came back with some news:  lots more reticulan fibers and fibrosis "consistent with post-polycythemic myelofibrosis."  The report didn't look good from this patient's perspective but I was pleased that I wouldn't have to wait too long for Dr. Mesa's review.


We decided over the holidays to try to get a consultation with Dr. Ruben Mesa, hematologist/oncologist at Mayo Clinic Scottsdale. I really must thank my mother for making this happen.  She prepared a concise yet thorough letter to Dr. Mesa explaining the changes in my symptoms and asked questions regarding my suitability for a clinical trial that we've been pursuing.  All this before we had the BMB.
  
Dr. Mesa is one of the top experts in the world on Myeloproliferative Neoplasms (MPN), which includes Polycythemia Vera and Myelofibrosis.
Since Mayo Clinic Phoenix is hosting the Joyce Niblack Memorial Conference on Myeloproliferative Neoplasms this coming weekend, we wanted to schedule the consultation for the same trip.  This conference is organized by the MPN Education Foundation and is rich in sharing the latest research in language patients and their loved ones can understand.  This will be the 3rd such conference mom and I will have attended.  I'll be reporting on the conference as it occurs.

So yesterday (Tuesday) I had the initial consult with Dr Mesa. 
Unfortunately, the chronic leukemia has progressed from Polycythemia Vera (PV) to post-PV Myelofibrosis (MF). This means that my bone marrow has gone from producing too many red blood cells to producing not enough of any blood cells (eventually makes one transfusion-dependent).

He explained that all the MPNs are on a spectrum -- my PV appears to be on the aggressive end of the PV spectrum.  [Blood clots in 2007;  uncontrolled hemoglobin and hematocrit in 2009 (when it was diagnosed) to now:  low blood production, unexplained weight loss, increased anemia, bone pain, excessive fatigue, etc.]

Myelofibrosis (both primary and secondary), like the Polycythemia Vera, is a chronic leukemia -- meaning one can live for quite some time with appropriate medications and monitoring.  (Acute leukemias are much more aggressive). 

He needs more info to determine where my MF is in the DIPSS 4 stage range.  It's likely somewhere in the middle -- not early and not severe.  This is good news.

The 20+ pounds I've lost in the last 2 months is likely from increased calories that the cancer is burning.  I'm still not to my Weight Watcher's goal weight, so no concern about it yet.  I finally found an advantage to being chubby ~ it gives cancer more to chew on while the doctors prescribe more toxins to help you get better.

We were hoping that my treatment would change from hydroxyurea to pegylated interferon.  Despite the potential side effects, the peg-interferon has reversed fibrosis in many PV cases.  Unfortunately, the interferon is likely not an option for me now (too late in the disease process AND it may exacerbate the inflammation problems).  We were considering this in 2012 and it never came to fruition. The hydroxyurea (pill chemo) has run its course, too.  No need to continue suppressing the bone marrow.

One of the new JAK-2 inhibitors will likely be a good fit, along with other meds to address the anemia.  He mentioned Jakafi (it's a pill, not an exotic tropical island where cancer patients sun and heal).

There are no medicines to cure secondary MF at this time;  what is available can slow the progression of the disease and reduce the likelihood that it transforms to acute myeloid leukemia.

Before Dr. Mesa develops his recommendations, he needs more information.  So off for more tests:
*  I had lots of blood drawn that is on its way to Mayo Rochester for evaluation.
*  I had a chest X-ray in part because he noticed my fingernails are "clubbing" which is a sign of pulmonary problems.  It could also be a result of all the inflammation.
*  I had an ultrasound on my big spleen and gut.

Dr. Mesa also wants to consult with a rheumatologist for the Behcet's and the other mystery inflammation (see my earlier posts). The inflammation is not related to the blood problems.  While it may not be due to Behcet's, he wants the rheumatologist's opinions. The rheumatologist can't see me until next Tuesday morning, so I have to extend my trip.

Also, he believes that at some point a bone marrow/stem cell transplant may be a good option for me.  This is the only known cure for MF.  He says you don't want to do it too early nor too late in the disease process.  He wants me to consult with their SCT doctor.  That appointment is this Thursday morning.  I'll know a lot more about SCTs as a result.

Dr. Mesa also says that my "youth" is a big advantage (most are diagnosed around age 65). 

The kids know I haven't been feeling well and are glad that I'm seeing a world-renowned expert. Their love and teenage chaos pulls me out of self-centered funks and remind me of all things good. Laughter is truly the best medicine!

I will know a lot more on Friday and will also learn a whole bunch about the state of MPNs and other patients' experiences at the symposium this weekend.

Stay tuned!  Never a dull moment!