The Outs and Ins of MPNs

My last phlebotomy was on June 29, 2012. It took about 15 minutes to withdraw 500 ml of that RBC-rich blood from my body. Now, almost eight months later, I'm receiving my first transfusion of packed red blood cells.  What a ride this is!

When my hemoglobin (hgb) and hematocrit (hct) was not stabilized with Hydrea last summer, I needed a couple of phlebotomies to get me back in the zone. Since last fall, these counts have slowly but steadily fallen. 

Now, with Hgb at 8.2 and Hct at 27.4, I can't walk up the stairs without my heart pounding. I get light-headed and dizzy when I walk or stand for much time around the house. (You know something is not right when you find yourself looking for things to lean against as you move around. Similar to when I was pregnant and had to scope out the nearest restrooms)

One of the great take-aways from the Joyce Niblack MPN Patient Conference held in Arizona this month was encouragement from experts like Susan LeClair, PhD and Ruben Mesa, MD that blood counts and ranges are not absolutes for every patient. We are encouraged to pay attention to our individual symptoms and let our doctors know.

This gave me the confidence to let my local hematologist know that I need a blood transfusion now (with at Hgb of 8.2)  -- I don't want to wait for it to reach 8.0 (the standard accepted level to begin transfusions). I've been very fatigued with it in the 9's for the last few weeks; when it fell lower this week, I had to cry "uncle."


Additionally, I plan to start taking Jakafi when it's approved by my insurance. Since blood counts often drop during the first few weeks, I want to start from a position of strength.


So yesterday I went to the hospital's outpatient infusion center to get my blood typed and cross-matched. Here is a good explanation of the blood transfusion process.

My "energy pack"

Today I'm getting 2 units of B negative packed red blood cells from a donor in Riverdale, Georgia. A nurse came in with a cooler that contained what I'm now calling my "energy pack." After verifying the info on the blood bag with the info on my wristband, the transfusion commenced.

As I sit here and write this while someone's generous gift is flowing into my veins, I feel quite humble. Someone took time (and energy) from his/her daily routine to help a stranger. It is an unconditional gift. No questions or judgments about my race, religion, age, gender, political views, sexual orientation, family situation, employment status, or the reason I need the blood. A pure gift of care and concern for others. 

One of the nurses explained that this donor helped me with the red cells, and another with the white cells, and a third person with the platelets. How is that for leveraging a donation? 

My mom was an ICU nurse while I was growing up and we learned how important blood donation is to saving lives. I started donating blood when I was 18 and in college. Because "B negative" is not a common blood type, the blood bank would sometimes call me in. I continued donating until my early 30's and quit due to chronic anemia. Later, in my 40's, I became symptomatic and diagnosed with Polycythemia Vera. I've been assured that my blood was good and safe for donation in my early years; the JAK2 mutation that triggered the PV occurred later. 

I have no idea how much blood I donated in my healthy years. But I'm sure it not enough to cover the blood I will be receiving over the coming months. Some have told me that they would like to donate to someone they know. I remind them that should they ever need blood (due to illness or accident), the donor who saves them will likely be someone unknown to them. 

Blood donation is the ultimate random act of kindness. I receive this gift of kindness and life with love and gratitude. It is yet another reminder of the interdependent web of life. 

These Tired Bones Need a Makeover

After several days of tests and consultations with experts at Mayo Clinic Scottsdale, I returned home with a worse-than-expected diagnosis. Here's the quickie Bad News/Good News version:

My bone marrow has gone from over-achievement to under-achievement. Instead of producing too many red blood cells, it is now in the "spent phase" and is not producing enough blood. This transition called my spleen and liver into action to produce blood. They are working hard, but will not be able to sustain their efforts forever. I'll soon need blood transfusions to keep my hemoglobin at a livable level. What irony! From phlebotomy to transfusion in less than one year.

A blood stem cell transplant is in my near future;  a successful transplant will cure me of the myelofibrosis AND the auto-immune conditions that rage inside.
Without a transplant, I'm at great risk for acute leukemia (no cures) and could expect less than three years on the sunny side of the ground.

Now that the test results and reports from Mayo are in my local doctors' hands, I'm preparing for the insurance approval / denial / appeal process. Quite frankly, I'm more anxious about getting the transplant approved (with the best transplant center for my particular leukemia) than the transplant process itself.

The kids and Robert are taking the news like champs. So are my mom, dad, and siblings. We're going to get through this together as we do all challenges ~ with love, laughter, the occasional cuss word, and toasts to the present and a bright future. 

Some have asked what they can do to help.  If you are so inclined, please consider:

1. Become a blood donor. You can be sure that me, or someone like me, is grateful for your gift of life.
2. Join the National Bone Marrow Donor Program. With a simple swab of your cheek, you will be "typed" (not blood type, by the way) and entered into the database. When a patient needs a donor, our info is compared. Donors are contacted and must agree to be considered for donation before the patient is contacted. Donors can say no at any time. Also, I won't need your bone marrow ~ just your blood stem cells (it's like donating platelets).
3. Keep us in your happy thoughts, prayers, meditations. My mom alerted her international, multi-faith 'God Squad' and I am here to tell you that I do feel the love. It manifests in hope, energy, and strength.  

NOTE:  I especially encourage my friends of Asian, African, Jewish, and Native American descent to sign up. Matches for are very limited for children and adults of color compared to white European descendants. 

Here are some specialty donor programs (they tie into the large database):  

Asians for Miracle Marrow Matches

BlackBoneMarrow

Gift of Life Bone Marrow Foundation

#  #  #

If you like more technical terms, read on:


I. My Polycythemia Vera (PV) has transformed to post-polycythemic Myelofibrosis (MF) in recent months.

• I have completed the “spent phase” of Polycythemia Vera and my bone marrow now has lots of reticulin, which crowds out the blood-making stem cells. 
• Extra-medullary hematopoiesis is now at work – the spleen and liver are beginning to produce blood due to the bone marrow fibrosis.  Over time, this puts extreme stress on both organs.
• The overall goal is to prevent the MF from progressing to an acute leukemia from MPN;  it is very difficult to treat and very high mortality.
• The auto-immune conditions and portal vein blockage add complexities to my circumstances. My “youth” is an advantage.

II. I will start a newly-approved medicine called Jakafi to help improve the symptoms of the disease.  
While it won’t slow the progression of the disease, it should reduce the spleen size, night sweats, itching.  May cause more anemia.  I’ll likely need blood transfusions (when hemoglobin falls below 8.0

III. Need to plan for a blood stem cell transplant in the next year or so.
Because of the rapid rate of disease progression, I'm not a candidate for a clinical trial.
We need to prepare for a transplant:

• “Type” me
• Look for a match – my siblings, then www.bethematch.org
• Get insurance & local docs on board with this plan
• Stay as healthy as possible

I was hoping that a transplant could occur after Alexander graduates high school and heads out to college (mid-2014).  The doctors said that is highly unlikely.  There is a window of opportunity for successful transplants and weekly monitoring of my blood counts will inform us of the timing. That bums me out because I want to be fully present for his last year at home. He said, "Don't worry about me, mom. The sooner you get the transplant, the sooner you feel good enough to do all the things you want to do." 

As long as I make sure he has gas money... (wink, wink)

The Power of Expiration Dates

How would you live your life if you knew your expiration date?  
Would you live differently today if you knew you had 20 years?  10 years?  5 years?  1 year?

Now, I have no idea at this writing of the state of my health condition. I’m not going to borrow worry;  it will be what it will be. And besides, I could get hit by the proverbial bus or picked off by a random shooter before the disease calls 'time'.  I refuse to live in the state of fear, disease or no disease.

Some say they wouldn’t want to know, it would make them paralyzed to do anything.  

After a sudden illness five years ago that I wasn’t supposed to survive, I have a new perspective. I consider it a gift because it pulled me off the treadmill of life and forced me to learn how to be present instead of focusing on the future.   

This present circumstance is a reminder to ask the questions I like to avoid:  

1.  How do I want to be remembered?  

  




2.  What memories do I want to make to fuel me when I can't get out and do as much as I can now?

2012 Family Reunion

Fun w/Roman Street Performer

Visit with Theas Estelle, Kay & Angie

3.  What is keeping me from being the person I want to be and making the memories I crave?

Here are some steps I am working on this year:

Simplify, Simplify, Simplify
I have a well-known clutter problem. Paper is my arch-nemesis;  it is everywhere! 
But it is also difficult to part with certain things because 'someone' may need them 'sometime'. My neighbors call me when they need an odd kitchen gadget, super large mixing bowls, or platters.  The kids’ friends come over to borrow wigs, costumes, capes, and all sorts of art supplies for school projects. And then there are books – pick a topic and I’m likely to have at least one book on the subject (I’m not kidding – any topic).  To me, it is absolutely terrible to throw books away.  Who wants some books?

I still have a dozen boxes full of conference binders, books, and articles I authored when I was working.
News flash:  the Smithsonian has no interest in my 20 year adventure in creating nonprofits and developing affordable housing.  And shockingly, neither are my kids.  Ouch! Time for purge mode!

Attachment to Things
I am less attached to things. Don’t get me wrong, I still like nice things. But I don’t wish for much anymore.  For example, I love Waterford crystal.  My husband won several beautiful large bowls and vases in golf tournaments over the years.  We’ve purchased and received as gifts wine goblets, tumblers, and other pieces over the years.  They are beautiful and are rarely used.

I also have a collection of more than 300 cookbooks. I really enjoy reading them and purchased many along my travels. But truthfully, I only cook out of about a dozen of them. Today I’m more likely to go online for a recipe because I can print out the shopping list and have the nutritional value calculated for me.
Yet, I've hesitated to thin the herd because it would mean my collection wouldn't be as big.  Notice the irony -- thinking bigger is better… in the food department!
Who wants some cookbooks?

Use the Good Stuff
I’ve decided that as the “every day” items dwindle, due to a hungry garbage disposal, poorly loaded dishwasher, or clumsy food runner, I’m going to switch over to using our “good stuff.”

Like every good Greek-American girl, when I was engaged and preparing for marriage, I registered for “everyday” place settings, flatware, glasses, and serving pieces.  I also registered for “formal” place settings, silver utensils, crystal, and serving pieces.  These formal dining pieces are used approximately two times a year.  Since I’ve been married for 22 years, that’s less than 50 times, while the everyday pieces have been used thousands of times (and been replaced as needed).

What am I waiting for?  If I know my expiration date is coming up in a decade or two instead of 30-40 years, I’m going to enjoy what I’ve got.  The same goes for my shoes, bedtime attire, Parmagiano/Reggiano …

Demonstrate Gratitude Everyday
The practice of gratitude is a constant in my life.  It is a spiritual practice as I make note throughout the day and every night of the things, actions, and people for which I am grateful.  

When the illness became overwhelming and required me to quit working, I was furious.  I pushed as far as I could at my nonprofit and then with part-time consulting until my body and brain wore out.  My work provided psychological as well as financial income and I was accustomed to pushing through adversity.  98% of the time, I prevailed.  This was when I realized how much of my stressful yet wonderful life I took for granted.  I thrived on spinning plates at work, at home, and in community activities.

I am forever grateful that my two children are darn near perfect in health, spirit, and abilities.  I’m grateful that my husband is a loving, funny, and dedicated father and husband; he handles the new me with great care and good humor. My extended family has always been incredibly loving and supportive. I have some of the truest friends anyone could hope for.

Now I need to remind myself that even small things are worthy of gratitude.  Since I developed chronic illness and accompanying side effects such as extreme fatigue, foggy brain, bone and joint pain, muscle pain, migraines, and depression, I sometimes have to dig deep.  Hey!  I put my bra on all by myself! 

Some days, I’m grateful I can get out of bed without assistance, walk down the stairs, or sleep through the night.  Other times I’m grateful the medicine took away the pain; or I went an entire day without a headache.  

Since I’ve accepted the value of appreciating things that once seemed small, I’m taking the next step this year.  I am going to demonstrate my gratitude to others every day.  I want to be sure that people know what they mean to me, that I appreciate them, that they make a difference in the world.  Some gratitude will be expressed verbally, in writing, in person – who knows what I’ll come up with.  
But being grateful isn’t enough for me now;  I need to express it as I feel it.

Accentuate the Positive
I will also demonstrate gratitude by not piling on the negativity in the world – through social media, movies, the "news," gatherings, or other means.  I can be sarcastic and snarky with the best of them, and sometimes it feels like a good release (especially when I'm in pain or depressed).  But does it do anything to improve the human condition?

When I read comments to on-line articles and some facebook posts, I’m struck by the number of people who write things that they probably (hopefully) wouldn’t say to someone’s face. I'm guilty of it, too. It’s as if cynicism makes one feel superior. Not only do the Negative Neils and Nellys usually post opinions based on incomplete and sometimes absolute fiction, the result is raising their own and the readers’ anxiety, anger, and blood pressure.  Really?  Is this how you want to be remembered?  Showing your amygdala to the world?  

From now on, I'm working hard to moderate my expressions with the simple question:  will what I say/post/write help or hurt the situation or other person? 
The old adage, “if you don’t have something nice to say, keep your mouth shut.” Is my new mantra. Of course, I still like the southern version sometimes:  “If you don’t have something nice to say, come sit by me!”  (wink, wink)

People often don't remember what you said or what you did, but they always remember how you made them feel.  

This imperfect person is focusing on spreading love in hopes that it puts a bit more healing and motivation into the world.

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!