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!

Yippee! It's Time for Another BMB

I never thought I would look forward to getting another bone marrow biopsy & aspiration, but today I am eager for new information.  The first BMB was done in March, 2010.  It will be interesting to see how my busy bone marrow has changed in almost 3 years. 

Why a BMB Now?
In 2012, the first few months were the best I've had in a few years.  The Remicade was keeping the Behcet's flares at bay and Polly was managed well with hydroxyurea.  
In April, I began getting all sorts of odd inflammation in my hands and feet (shared in earlier posts).  At the same time, my hemoglobin and hematocrit were climbing high and we had to increase the hydroxyurea dosing and add phlebotomies.  Now that I've been off of the Remicade for over two months, the inflammation is greatly reduced.  It's still here, but the disabling pain is gone.  In the fall, the hemoglobin and hematocrit started falling, even after decreasing the hydroxyurea dosage.  Hgb was 9.3 and Hct was 30.4 on Christmas Eve.  No wonder I get so tired so fast.  Something is not right.  
The Diagnosis Information on the referral says:  Neoplasm Uncertain Behavior Polycythemia Vera.  

Homework for a BMB - Yuck!

Since I need to take Lovenox injections for a few days leading up to the procedure, I've talked my daughter Katrina and my friend Kimberly into pinching my gut and shooting me up.  I know I'm a wimp, but for some reason I just can't give myself an injection.  Yes, this from one who gets blood drawn at least monthly and has had many phlebotomies over the years.  Katrina is doing a fine job standing in for her dad (he's in Germany this week).

I've been taking warfarin (aka Coumadin) since the life changing thromboses of my mesentary, portal, and splenic veins in 2007.  The clots are still there and fortunately the body has an incredible way of creating new pathways to circulate blood.  

To prepare for any invasive procedure, we have to adjust my blood's ability to clot to prevent excessive bleeding during or after the procedure.  

Then, after the procedure, it's more Lovenox and warfarin until the blood returns to a safe "thin-ness"  (It's not really thinner, it's just slipperier to slow down clotting).


The BMB Procedure

image from University of Chicago Medicine website

It's a really straight-forward procedure.  The doctor injects some numbing medicine at the rump site and the anesthesiologist gives me some twilight meds so I can be awake but not really care that someone is drilling into my tailbone (posterior iliac crest). 

Thanks to one of the most authoritative resources, YouTube, I showed Katrina and Alex some BMBs that patients had recorded and posted.  The numbing of the bone is really painful.  Some go through the procedure without the twilight (conscious) anesthesia.  I thought about it... for a nanosecond.  I've been dealing with so much chronic pain that if I have a chance at some legal relief, I'm all in!  I may have "chemo brain" but I haven't totally lost it. I gave up the martyr crown a long time ago.  And thank goodness I have great health insurance!

When Katrina saw the one where the doctor said, "Oops!" a couple of time, she started to become real sympathetic.  

This Year's "Rumper Sticker"
I want the medical staff to see me as a person, not a procedure.  Thanks to the creativity of many friends on Facebook, I got lots of suggestions for what my daughter would write on my rump.  Last time, she wrote "Private Property" and "Bad 2 the Bone" above each cheek.  Some of this year's top suggestions:

• You Break It, You Buy It
• Objects in rear are closer than they appear
• Left        Right
• You'd better buy me dinner first
• Warning -- Blast Zone

Here's the winner:

We'll see how the doctor reacts!