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!

A Few Quick Thoughts from the MPN Patient Symposium

I just got back home from the whirlwind trip to New York City for the 6th International Patient MPN Symposium sponsored by the MPN Research Foundation and the Cancer Research & Treatment Fund.  The day was filled with fascinating speakers (several of whom were new to me) and each of them taught me something new.

I'll organize the 25 pages of notes later, but I thought I'd tempt you with these nuggets. :)

Cool Terms I Picked up at the MPN Patient Symposium

Somatic mutation:  a change in a gene that occurs after birth (you aren’t born that way)

Molecular mimicry:  some genes mimic others

Epigenome:  the new area for research

We are all entitled to our own opinions, but not our own facts.

JAK2 and Exon 12 and TET2 – a few of many mutations under exploration

Genomic Instability:  affects the micro environment and what happens with MPNs

Specialized Niche Cell:  cells that are highly specialized (example:  Endothelial cells)

Endothelial cells:   lining of blood vessels;  they also instruct other cells and broadcast directions throughout the body.  They also instruct organ regeneration (eg, liver and lungs). 

Inductive angiogenesis:   uh, i forgot already!

Proliferative angiogenesis:   forgot this one, too!

Role of vascular niche cells in self-renewal of stem and tumor cells:

The micro environment that are created by the mutations have huge impact on what happens. 

Angiocrine Factors                

Niche cell:  activate endothelial cells

Leukemic initiating cells – they expand the vasculature in the bone marrow.

Factoids and A-Ha’s! 

• Platelet-Leukocyte aggregates and micro-particles promote thrombosis.  The different kinds of cells can act together to promote thrombosis. 

• We need drugs that target the endothelial cells.   Endothelial cells expand hematopoietic stem and progenitor cells by angiocrine expression of Notch ligands.

• The first hematopoiesis occurs with the endothelium. 

• The leukemia from MPNs is completely different from the normal leukemias.  We need different treatments because the biology is different.

• The Chronic Myeloid Leukemia is triggered by the Philadelphia chromosome, so it is regarded as a different disease process from MPNs.

• JAK2 is not a “switch” that can be flipped on or off; it has at least 10 different mutations, which cause different disease results in patients.

JAK2:  The One Mutation, Three Diseases problem.

Why do patients develop ET, PV, or PMF?

Level of JAK2 allele burden determine ET, PV or PMF – more so in men than women

How much is the disease versus the host predisposition to develop an MPN?

Are there other cooperating somatic mutations?

• Despite all the advances, we still have a long way to go – do not be misled by where we are.

• For those of us taking warfarin (Coumadin), Dr. Weksler said that the newer anti-coagulants are “not there yet”.  Some patients have tried Pradaxa and gone back to warfarin.  Pradaxa must be taken twice a day consistently and this is problematic for many patients.  She believes that we’ll have a better alternative in a few years.

• Much of the knowledge gained and progress made has occurred because patients before us and among us have shared their tissue samples, DNA, and whole selves for testing and clinical trials.

There was so much more covered.  I hope this tempts you to watch the video when it is available online at the MPN Research Foundation

Stay tuned!

peace,

Marina

Bone Marrow Biopsy in the Twilight

Talking with other people about the bone marrow biopsy experience brought back memories of women comparing childbirth stories. Everyone has their personal story, and thoughts on how it "should" go. There seems to be a continuum of preferences, from "all natural" to "wake me when it's over."

Since I have a pretty high pain tolerance and don't like anesthesia, I was inclined to just have the local numbing where the needle would be inserted. I decided research this a bit. So I turned to the definitive resource, YouTube. Believe it or not, some people have had their BMBs videotaped! After seeing a couple of patients (with the local anesthetic) cry out in pain and yell at their doctors to "hurry it up!" I decided to go with the "twilight anesthesia."

I decided to find some levity in this upcoming procedure. So I asked my daughter to draw me some "rumper stickers" the night before the big poke. On my right cheek, she wrote "Private Property" and on the left she wrote "Bad 2 the Bone."

When the nurses got me situated on the CT platform, they both seemed surprised when they saw the rump notices. "Let's not tell the doctor -- he'll be surprised!" one of them said to the other. Apparently this does not happen all the time.

The next thing I knew, the twilight juice was flowing and while I think I remember being awake during the procedure, I truly do not know. I could feel some pressure, but it was not at all painful.

It was a long day... we arrived at 9 a.m. for the 11 a.m. procedure. Because of delays in the hospital, my BMB didn't take place until after 1 p.m. After the two hour "observation" period, I was allowed to go home around 4 p.m.

Once we got home, I zonked out for a few more hours.

Now, we must wait for the results -- they should start coming in next week.

The lovenox shots resume again (thanks to Robert and Katrina for shooting me in the belly) as I add the rat poison (warfarin/coumadin) back to my daily diet.

What's that saying? Better living through chemistry.

Yippee!

Bone Marrow Biopsy -- take 1

My bone marrow biopsy is now scheduled for next Thursday at St. Joseph's in Atlanta. From that we will learn how damaged the bone marrow is. It will be a good point and time reference. Hopefully, they will karyotype the chromosomes so we can learn more about the particulars of my disease process. It will also provide info that the docs can use to determine if I might be a candidate for clinical trials as they become available. These myeloproliferative neoplasms (MPNs) are still not well-understood.

I've received great encouragement from fellow MPNers through the MPDChat group, so I'm really not nervous about the procedure. The most challenging part will be getting up and arriving at the hospital without my usual dose of caffeine (I am NOT a morning person).

Here's a little ditty I wrote to the tune of "Tomorrow" from Annie the musical.

Ode to the Bone Marrow Biopsy:

The needle will draw
bone marrow
Bet your bony bottom
that tomorrow
Won't be fun

Just thinkin' about
bone marrow
Crunching sounds and
aspirating needle
Hope I'm numb

When I'm stuck in the rump
Feel the thump

Hear the grinding

I just say Hurry Up

BMB

On me...

I have no doubt
bone marrow
will tell me to hang on
'til tomorrow
Tests delayed
Bone marrow! Bone marrow!
I love ya, bone marrow
You're keepin'
the MPNs
at bay