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.

STEM CELL TRANSPLANT STEP 1: MEDICAL EVALUATION

DONOR NEWS!!

I learned this week that my life saving blood stem cell donor is a 22 year old man who lives outside the United States. He has agreed to the stem cell collection schedule my doctor proposed. How cool is that?! Young, healthy stem cells ~ it doesn't get any better.  As a patient, I am grateful that this young man agreed to take time out of his summer to save my life.  As a mother, I feel a sense of pride that this young person learned about the need for bone marrow/stem cell donors, joined the registry, and agreed to help a stranger in another country.  Both my kids say they hope they can do for someone what this young man is giving me. I get teary every time I think about this.

As people share wishes, prayers, and positive thoughts for my health, I ask that you include my selfless, nameless donor in those efforts. 

COUNTDOWN TO TRANSPLANT

We are ONE MONTH from my Re-birthday! The process begins with a thorough Medical Evaluation. Tomorrow (June 27, 2013) is my big test day.

7:00 am:   Pre-BMT labs, Vitals
   I was told they will draw the blood from my Power Port, so I take that to mean they need a lot of blood.

7:30 am:     Drink jug of Barium.  What a refreshing treat!

8:00 am:     Bone Marrow Biopsy   [need another 'tag line' for my rump!]

8:30 am:     Meet with Clinical Health Psychologist  (this one is most worrisome ~ what if they realize I'm a bit nuts?)

9:00 am:     Meet with Research

10:00 am:    Pulmonary Function Test w/DLCO

10:30 am:    Meet with Atlanta Blood Services re: my need for platelet donors

11:00 am:    Register for CT scans

11:30 am:    CT scan of Brain / Chest / Abdomen / Pelvis

1:00 pm:     Lunch (probably the highlight of my day)

1:30 pm:     Register for Echocardiogram/EKG

2:00 pm:     Echocardiogram

It is no secret that among my numerous quirks, I have 'text anxiety.' 
Whether it's my car's emissions test, the vision test to renew my driver's license, or weekly blood tests, I get a rush of the 'heebie jeebie jitters'. This may not yet be a recognized technical term, I think you know what I mean.  

To calm those jitters, I mindfully picture the test administrator in his/her underwear and focus on my breathing. 

In this situation, I recognize that all these tests will form a baseline of the health status of all my systems pre-transplant. My biggest threat to surviving the transplant is organ failure. The results of these tests enable the doctors to anticipate issues that may arise during chemo and post-transplant and plan accordingly.  

I will find out the results of all these tests on my Education Day, scheduled for July 10th.
As the song goes, the waiting is the hardest part.

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.

I Blame the Hemoglobin

There seems to be an inverse relationship between my Hemoglobin level and Anxiety level. Hemoglobin is a protein in the red blood cell that carries oxygen through the body. As the hemoglobin decreases, I become more easily annoyed -- with myself and everyone around me. I get the anemia double-whammy from the alpha thalassemia trait (red cells are smaller and oddly shaped) AND low hemoglobin count.  

The difference between me with hemoglobin at 12.0 and 9.5 is 'Marina the Upbeat, Can-do Gal' and 'Marina the Grouch.'  12.0 is fun to be around; 9.5 and below is best to avoid.

The filter between my thoughts and my voice has thinned over the course of prolonged illness. I used to be a pretty tactful person, able to smooth ruffled feathers and still get a point across. Active listening, patience, body language and good humor are reliable communication techniques. I've studied non-violent communication and practiced appreciative inquiry as a part of my spiritual growth and leadership development. 

Yet when the hemoglobin dips below 9.5, all bets are off. 
Active listening morphs into "what the heck were you thinking??"  
Patience evaporates in the midst of household procrastination (isn't it a good idea to save big clean-up projects for the hour before company is to arrive?). 
Humor is replaced with sarcasm: "of COURSE I know where your [insert any random item] is. Let me turn on my magical internal GPS device that tracks items I've never seen or used and find it for you."   

While I'm not quite ready to appear on the Jerry Springer show, it seems like I'm trying out the role.  My gripes, rants, and sarcasm are not how I'd like to be remembered, especially by those who get the brunt of my mis-placed frustration ~ my loved ones. 
If life is my Advanced Placement exam, I'm clearly not ready for graduation.

I offered several "reasons" for my crankiness: 
*  teenagers are self-aborbed                     *  it's the anemia talking  
*  'no one' shares my sense of urgency         *  not enough oxygen to the brain   
*  we don't live in a barn                           *   fatigue causes frustration

The correct answer may be "all of the above PLUS fear"

When my blood levels are closer to normal, I feel good enough to keep busy with the kids, help with the marrow registry drives, and spend time with friends. I don't make time to contemplate the upcoming stem cell transplant.

When the need for a blood transfusion approaches, I am so fatigued (this is different from feeling 'tired') that I physically cannot do as much. Climbing the stairs sets my heart pounding and I rely on the handrail to rest. I spend time in bed or on the sofa with hopes of 'recharging' enough to complete a basic household task.

When I'm forced to be still, questions and thoughts about what lies ahead bubble up in my head. Will I get a matched unrelated donor?  Will it come through in time? 
What happens if the myelofibrosis progresses too fast to acute leukemia before a donor is found? 
What kinds of complications could happen during transplant process?  
My organs have endured a lot of stress the last 6 years with the blood clots and medications. Will I be strong enough to over come the challenges? 
How will my marriage change? What about Alex's senior year in high school ~ how will I keep track of him? What about Katrina's first year in college?  I've seen enough movies on the Hallmark channel to know that mom's illness can reek havoc on kids' academic performance.

These thoughts remind me that control is truly an illusion, albeit one of my favorites. The feelings of vulnerability and powerlessness over my future stir an inner rage. It is not my happy place. I've read enough pop-psychology books to know that anger is a mask for fear. No amount of scenario planning can sooth the ever-present frustration that lies beneath a seemingly smooth, confident veneer.

It seems that I can accept the uncertainties when I'm busy. I comfort myself by doing what I can to help others, be present with my family and friends. I embrace my positive outlook and an inexplicable but real sense of peace that everything will work out for the best. 

Beating the Odds Starts with a Swab

When I began this journey 3 months ago, I was pretty confident that one of my four siblings would be my match (a 10 for 10 match of HLA-types; it's not about matching blood type).  Each had a 25% chance, so wouldn't 1 of the 4 have to match?   That's not how probabilities work, I learned. 

I figured the Northern European ancestry on my mom's side would ensure several potential donors. Apparently our Greek ancestry is a bit more diverse than we thought.  

My family and friends immediately jumped on my challenge, determined to find me a donor. They have already run nine (9) Bone Marrow Donor registry drives ~ in Houston, Tempe, Grand Rapids, and four here in Georgia.

My mom even brought donor registry kits to her retirement party yesterday in Dallas ~ I believe she swabbed 32 new willing donors! 

556 new healthy people have joined the 

National Marrow Donor Registry 

because of these efforts!

Need for Marrow Donor Goes 'Viral'

My plight has hit social media!  While I've been public about my journey, I struggle with asking for help for myself ~ especially from perfect strangers.  

Friends convinced me that a Facebook page would help promote awareness and organize volunteers. I've seen similar pages (Genny's Hope Foundation and Help Nalini Now) that share a common goal:  to increase the number and diversity of healthy people registered to be bone marrow/blood stem cell donors. 

So now we have "Marina Needs Us" to promote the marrow donor drives and blood drives that are organized in my honor across the country. We'll see how helpful it is and how it changes organically as conditions change.

Donor drives have been done in partnership with AHEPA, Be the Match, and DKMS Delete Blood Cancer.  All the donor information feeds into the National Marrow Donor Program. 

Some use SignUpGenius, a free on-line volunteer organization site for community organizations to post volunteer opportunities. We learn as we go.

Goal #1:   Register 1,080 New Potential Donors 

Since I always work best with a goal, I set my sights on adding 1,080 donors to the registry. Why 1,080?  Because 1 in 540 people on the registry end up donating their blood stem cells to a patient like me, I'd like to pay it forward to cover a matching donor for me and one more patient. 


I continue to be humbled when I see neighbors, friends, friends of friends, and parents & siblings of my kids' friends volunteering to work these donor drives, get swabbed, and talk it up in support of our efforts to increase the donor pool.  

Helpful hint:  students can earn volunteer / community service hours for their assistance with these efforts.


Goal #2:    Dispel the Myths

I readily admit that I didn't know anything about bone marrow transplants until I met people with various MPNs (myeloproliferative neoplasms) that progressed to burn-out the bone marrow. 

What's worse that no information?  BAD information!

A Friend In Deed for a Friend In Need
None of this happens in a vacuum, and I certainly have not done much to make these drives happen.

Lynn Agnes, fearless friend

I must take a moment to openly thank Lynn Agnes for taking on this challenge of finding my match. We haven't seen each other in quite some time, yet Lynn showed up to help with our big drive at Peachtree Ridge High School on May 10th. Lynn researched my illness, the transplant process, and how marrow donor matching is done. During the drive at my kids' high school, she began brainstorming and calling people to schedule more drives.

Lynn secured several drive locations in a matter of days:  Gwinnett Chamber of Commerce, TPC Sugarloaf Country Club, Gwinnett Medical Center, Gwinnett Technical College, downtown Duluth (Georgia), and the Annunciation Greek Orthodox Church in Atlanta.  And she has more activities in the works. 

Lynn inspires me and my family with her bold tenacity. She is teaching my kids how to take action when there is an urgent need.  We know that her efforts will save lives.
Thank you, Lynn!

A Port for Weary Veins

Port of Pireaus, Greece

When I think of a Port, I first imagine a place near land for boats to find shelter from rough seas or load and off-load goods and passengers.  

The second thing I imagine is a glass filled with a dark, heavy, rich dessert wine made in Portugal.

This coming Wednesday, I will experience another kind of port - up close and personal. I'm having a port installed in my chest. But it is for loading something other than libations.

When I went in for my second transfusion of Red Blood Cells (RBCs), the nurses told me it was time to get a port installed. My good veins for IVs are shot due to phlebotomies and Remicade infusions over the last few years. There is scar tissue and some veins have flattened out. Other veins are covered by lipomas (benign fatty tumors) on my arms and legs (fat isn't limited to cellulite, my friends!).  

The port installation is an out-patient procedure with local anesthetic. I do hope they will give me something to calm my nerves, because watching people run tubing through my chest will trigger some of my natural anxiety.

IV Educational Moment:
In preparing for this, I've learned that there are three types of intra-venous (IV) catheter mechanisms: Ports, PICCS, and Lines. Each is used for different medical purposes.

Ports:  Ports are funky-looking things that are implanted just below the skin's surface on the upper chest. A tube connected to the port is fed through a central vein towards the heart. Ports can stay in ones body for years if necessary. Ports are good for chemotherapy, transfusions, and delivering medication and other necessities. A special needle is required to connect to the port. Ports have lower infection risk because there are fewer points of entry to the body than the tubes from PICCs and Lines.

How Does A Port Work?


PICCs:   Peripherally Inserted Central Catheter 
I had one of these when I had the whopper hospitalization in 2007. They can draw blood, give medication and liquid nutrition without sticking me every time. A purple tube went from my right arm into a vein that led to my heart. I was unconscious when it was installed, but I do remember that when the nurse pulled it out it was no big deal.
PICC lines are not used for chemotherapy. 

Lines:
There are 2 types of lines, tunneled and not tunneled. Tunneled catheters / lines are used for chemo, blood draws, delivery of nutrition, medication, etc. They are not for long term use.

An on-line friend shared this brief summary of these 3 types of IV catheter approaches.


I'll post a photo or two of Marina's Port (sounds nautical, doesn't it?!) on Thursday or Friday.

Blood Stem Cell Transplants in the 21st Century

When I first heard about bone marrow transplants, I envisioned the donor getting drilled in the hips for bone marrow extraction (like the bone marrow biopsy and aspiration) and then a surgeon drilling into my large bones to implant the donor marrow. I am happy to say that I was way wrong!


In most cases, blood stem cells are harvested from the donor through a process called Apheresis. It is like donating plasma (some of you may remember college students doing this for weekend money).   

Over the years, the stem cells that get transplanted have many names:  

bone marrow, stem cells, hematopoietic stem cells, peripheral blood stem cells, and pluripotent stem cells. These are the stem cells that become red blood cells, white blood cells, and platelets. 

What's Involved in the Transplant Process

For me:    Patient and her caregiver must live near the transplant center for 6 months.
The week leading up to the transplant, I get blasted with chemo that will kill all the cells in my bone marrow (among other things) and also receive immune-suppressants to prevent my body from rejecting the stem cells.  I’ll lose my hair, get mouth sores, feel like crap…

For my donor: The week leading up to the transplant, my donor will have labs updated (to ensure no new infections) and get a growth hormone shot each day for 5 days.  Then will have blood drawn out of one arm; the stem cells are separated in a bag for me, and the blood is returned to the donor in the other arm.  Generally takes 4-6 hours. S/he will likely feel fatigued for the next couple of days. 

For me: I will receive the donor’s stem cells by IV.  Then I sit and wait for those brave stem cells to engraft in my bones and begin producing blood (takes 2-3 weeks).
I’ll also continue to receive anti-rejection medications for the first 6 months post-transplant.

At day 100: a bone marrow biopsy will be done to see if the transplant is successful.

Major Risks of SCT

Transplants for secondary Myelofibrosis are very different than transplants for other leukemias and much more intense than organ transplants.

The risks are highest in the first year of transplant.
1. Risk of Graft Vs. Host Disease: 30-50% with sibling donor.  Of those 20% will not be responsive to steroids (aka steroid refactory).  MF patients have more GVHD than other leukemia patients.
2. Risk of Death: 15-20% within the first year.  Organ failure is most common cause.
3. Relapse Rate: 20% within the first 1-3 years
4. Graft vs. MF effect:  treated with donor lymphocyte transfusions

These are odds I'm confident I'll beat.

How We Chose the Transplant Center for Me

After an absolutely wonderful escape to Sicily, Robert and I returned home ready to tackle the next big adventure.  Where should I go for the stem cell transplant?  Who will be my donor?

Robert, Mom, and I met with Dr. Lawrence Morris of Bone and Marrow Transplant Group of Georgia in Atlanta. This group is under contract with our health insurance provider.

Here's what we learned:

About the Blood and Marrow Transplant Group & Facilities at Northside

Dr. Lawrence Morris came into our appointment well-versed in my condition and situation. Right off the bat he noted that I must be tired of hearing doctors say that I have an unusual and complicated case. 

The transplant group did 186 transplants in 2012 and their success rate exceeds the "Confidence Interval" (the expected success rate is determined for each center after controlling for the diseases transplanted, age of patients, co-morbidities, etc.). He said only 10 centers in the US exceeded their confidence interval. 

This center is well-within the top 10% of transplant centers in terms of scale. It's not a mega-center (like MDAnderson, City of Hope, Hutchinson, or Sloan-Kettering...)

Transplant Protocol
The transplant process would be managed mostly on an out-patient basis. This is very different from the Mayo process (5-6 weeks in the hospital). The chemo and the transplant would take place in their out-patient transfusion center. About one week after the transplant, I'd be admitted to the Transplant floor at Northside for 10-20 days, depending on how the engraftment is going, my vitals, any signs of rejection, etc. After release from the hospital, I will still go to the out-patient center daily and then every other day for check-ups. He said that at any point in the process, I could be admitted immediately to the hospital's transplant unit should that be necessary. 

They have better outcomes with the out-patient process, and I'd rather barf in my own toilet anyway. 

About My Situation

Dr. Morris explained the reasons why a stem cell transplant (SCT) is appropriate for me and the additional risks that I face. The Behcet's auto-immune issue is a bit of a wild-card. The portal vein thrombosis is also an issue; since my body has created new varices to deliver blood from the liver, we need to look and see if a similar thing has occurred in the esophagus (treatment would be required).

Transplants due to Myelofibrosis

He also identified the issues specific to transplants due to myelofibrosis: there is additional stress on the body due to years of medications, fibrosis in the bone marrow, and concern for stress on spleen, liver, heart, and lungs. That was a huge piece of helpful information for me because Dr. Fauble at Mayo said that there are unique post-transplant issues for MF patients but didn't specify what they are.

Given the stress on my body, he expects that the preparation regimen will be something between a full-in ablative chemo and the reduced intensity protocol.

Decisions, Decisions

After our Mayo and Northside appointments, Mom, Robert and I talked about the big, burning question: where to have the transplant. We want to go to the center that will have the best chance for a favorable outcome. 

The primary considerations: quality of care/experience;  quality of life for me & family;  insurance/financial considerations.

In the end, we are in agreement to have the transplant here in Atlanta.

Here's our rationale: 

Quality of Care/Experience:

• We looked at outcomes data and experience at Northside, Mayo-Scottsdale, MDAnderson, and others. Since there are very few transplants for MF, the disease specific data is hard to get. None of them do more than 2-3 per year for myelofibrosis. 
• Northside does enough transplants and participates in research; it's not a side business. It also does a great job - the docs have outcomes that exceed expectations and the transplant unit is state-of-the art.  
• They know the risks and issues associated with my particular situation. 

I don't believe we are compromising the care by staying here.  This group would be my local post-transplant support regardless of where the procedure is done. 

Quality of Life: 

• I really want to be close to Alexander during his Senior year in college. Whether I'm in the hospital, an apartment near the hospital or at home, he will be able to come visit regularly.
• Robert can do his job and he won't have to choose whether to stay with Alex on weekends or fly to see me somewhere. 
• Staying local will also help me stay out of the depression zone because I won't feel as isolated from my loved ones. I do better keeping my game face on when I've got good reasons to do so. 
• The out-patient protocol appeals to me a great deal. I recall going stir-crazy the last few days of my 2 week hospital stay back in 2007. Being confined for weeks is not appealing to me at all.    
• We have many friends who are willing to form a 'care team' to help out as the journey continues (it will be a marathon, not a spring). 

Insurance/Financial:

• I was initially inclined to go to Mayo-Scottsdale (as you all know). After looking at the data and meeting with the Northside doc, we'd have an uphill battle to get insurance to pay out of network.  I don't think we can make a compelling case that I'll get superior care elsewhere based on the data. 
• I don't want the added stress of insurance and financial burdens.
• Kaiser Permanente has excellent coverage if we do the procedure here. 

I'm glad we got the diagnosis and recommendations from Mayo first. Not only did it give us time for the info to settle into our minds and hearts, it gave me time to do more research, both data and anecdotal, before meeting with the folks that our insurance will cover.

We ran through several scenarios and the biggest question: If things go poorly here at Northside, is there going to be any shoulda/woulda/coulda discussions? 

There really isn't a stand-out, slam dunk transplant center that we dismissed. So we are in agreement.

So on we go... no regrets!

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

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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)

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!