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.

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)

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!

MPNs Gain Attention of Hematologists

In case you haven't heard, hundreds and hundreds of hematologists gathered in Atlanta, Georgia for the 2012 ASH (American Society of Hematology) conference this past week.

Until I was diagnosed with Polycythemia Vera, I had no inkling how complicated our blood is.  I also didn't realize how much 'we' (including the medical and scientific communities) don't know about about blood functions at the protein and molecular levels. 

It's a beautiful thing when blood functions properly.  But when it doesn't, well, it turns a person into a patient. 

Fortunately, we've got some very inquisitive, intelligent people studying many aspects of MPNs.  This year's conference featured several presentations on new learnings about Myeloproliferative Neoplasms (MPNs) -- from PV phenotypes to drugs that impact the disease processes and side effects like anemia.  

Here are a few helpful summaries of the MPN highlights from the conference.  The videos features Dr. Ruben Mesa of the Mayo Clinic, Scottsdale, Arizona.

Dr. Ruben Mesa summarizes MPN updates from ASH conference  
(updates on Polycythemia Vera and Ruxolitinib in Myelofibrosis patients)

In the next video, Dr. Mesa discusses the molecular abnormalities of MPNs (especially those with Polycythemia Vera):

Genetic Profiling Shows Promise for Customized Treatments

I want to learn more about this, as each fellow PV-er I meet seems to have a unique experience.  Varied severity of symptoms, responses to treatments, and outside factors make living with MPNs all the more frustrating.  Human beings want certainty, predictability, and a sense of control.  

It seems we are in the midst of the MPN learning curve.  At this point in human history, we can be grateful for the forward momentum of knowledge in this relatively obscure area of blood science.

Check out the ASH website.

Let's Play Medical Pinball!

Apparently, I'm a slow learner.  I believe that when things/people/events show up in one's life they present opportunities for introspection, learning and growth.  Sometimes it's to practice patience, listen better, explore other perspectives, remember to be grateful. 

The latest "opportunity" in my life is arthritis and chronic pain.  I've been dealing with increasingly frequent and more painful swelling in my hands and feet and arthritis in all my joints since April of this year.  (I posted pictures on an earlier post).  Some days I cannot get out of bed without assistance.  Seven months is a long time to live like the rusty Tin Man from Oz.

Not My Department
At first, the doctors put me back on prednisone (despite my previous horrible experience with it) because it is considered the "gold standard" for dealing with inflammation.  It helped for awhile, but never completely knocked out the inflammation and pain.  By August, the side effects of the prednisone outweighed any benefit.   Tests for Rheumatoid Arthritis and Lupus came back negative.  Whew!

All the while, my rheumatologist who treats me for Behcet's Disease was sympathetic to my plight but didn't have any answers.  This would be an extremely rare side effect of the Remicade treatments, so he ruled that out.  His hunch is that it is caused by the Polycythemia Vera disease process.  He prescribed Colchicine (now called Colcrys) and I've been taking it for two months with no relief.

My hematologist/oncologist believes it is rheumatological and not caused by the PV.
My internist said that because I am a "complicated case with two rare diseases."  The inflammation could be a result of one of the disease processes, a side effect of the Remicade infusions I get every 6 weeks, or something altogether new.  

Still Haven't Won this Game!

Living the Life of a Pinball
This fall, we "kicked it up a notch" and sought specialists at Emory University Hospital.
I've been wanting to change my treatment for Polycythemia Vera from Hydrea to Pegylated Interferon because my blood counts are difficult to manage, even with higher dosage of the Hydrea.

There is a non-randomized clinical trial through Emory that I might be able to join.  It appeals to me because the study will document the effects of peg-interferon on PV patients.  I find comfort in the notion that this illness has meaning if it contributes to greater knowledge for future patients.

Dr. Winton, the trial manager at Emory, requested I consult with their rheumatologist who is experienced treating Behcet's patients.  That doctor viewed my parts that were swollen at the time of the visit as well as photos I've kept of previous flares.  He concurred with my Kaiser rheumatologist that the inflammation process was not related to Behcet's, Remicade, or any other rheumatological disorder.   

With the mystery inflammation acting up, Dr. Winton is reluctant to have me start taking interferon.  Interferon can have many undesirable side effects;  it can trigger auto-immune responses.  I don't need gasoline on the fire!

Ask the Experts:  Fellow Patients
Accessing the wisdom of my extended, international PV family, I shared pictures and queried whether anyone else has these issues.  Turns out, it's rare, but YES.  I was directed to explore Erythromelalgia (EM), another rare disease.  Some PV patients develop symptoms best described as EM.  It turns out that EM manifests in several shapes and forms.  It is a clinical diagnosis (the only test is for patients who have a genetic pre-disposition -- generally younger onset).  There are no tests for folks like me who may have "secondary EM." 

So, back to my doctors I go, armed with this new information.  "Not likely" is the response I get from all but my internist.    Still, I try a few of the treatments that work for some EM patients.  I've tried taking antihistamines (both H-1 and H-2 inhibitors), plain old aspirin, and colcrys.  All to no avail.

My Next Experiment: 
I am going to hold off on Remicade infusions (the next one is scheduled 3.5 weeks from now) and see if the inflammation resolves when the Remicade leaves my system.
Why shouldn't a very rare side effect occur in someone who appears to specialize in "rare" conditions?   Know I've got to keep fingers crossed that Behcet's stays away when the Remicade wears off.  There aren't many other treatment options available to me.

Let's Try Another Approach:  Acupuncture
Two weeks ago, I began seeing an acupuncturist (professionally, of course -- Robert approves!).  She determined that the primary cause of this painful inflammation is too much "heat" in my system.  My blood, in particular, carries too much heat; likely due to the toxins in my system.  

Toxins... really?  You mean all those chemo pills, warfarin, and host of other meds can be toxic?

She explained that the acupuncture process will take awhile for meaningful results because it is working against all the medications I put in my body every day.  [Note:  she is not encouraging me to leave Western medicine, nor claiming to have the cure for the PV or Behcet's]

Judge these non-retouched images at your own risk!  I'm pretty cranky these days. ;)

The needles don't hurt a bit!

Frustration Galore
The frustration has become overwhelming.  It conjures up a slew of non-productive questions in my mind:

How come so many intelligent, highly-trained doctors cannot determine the cause, or at least identify some treatment options for the inflammation?
Sometimes it feels like they can't wait for my office visit to time out.  It can't be easy for them to have no answers.

How much worse would it be if the physical symptoms of the pain could not be visibly observed?  
Even I think I'm making this crap up sometimes!

How many days of intense pain can a person reasonably endure?
The pain can consume one's thoughts and make it difficult to think of, let alone tackle, everyday life.  I'm taking low doses of Lortab (hydrocodone & tylenol) and have resisted stronger pain meds for fear of addiction.  Some patients are really struggling with this issue, on top of everything else.  

How much are these illnesses harming my wonderful kids and hubby?
Moms are supposed to protect their children, be strong, and set good examples for their kids.  "What's for dinner?" is a reasonable question;  it shouldn't be answered with "I don't know ~ go fix yourself something."

What would happen if I stopped taking all medications and let my body de-tox?
Not sure if my body could de-tox before the blood thickens, new clots form, strokes occur, and Behcet's sores take off again.

What the heck am I supposed to learn from all this?
Now this question prompts me to find the humor in this.
Stay tuned for the next post!

URL Pharma Price-Gouging of Colcrys Is a Pain in the Foot

When I had my first gout attacks as a result of my Polycythemia Vera, the doctor prescribed colchicine to provide relief.  Colchicine is a generic drug that is 1,500 years old (it comes from the seeds of the autumn crocus) and was used by the ancient Egyptians and others to reduce inflammation and other pains.   

In 2010, my $10 prescription co-pay priced the pills at $0.16 each.

Last month, my rheumatologist prescribed colchicine again -- this time for extremely painful and inflammed joints and tissue inflammation (erythromelalgia).  

Bring back the colchicine!

I went to pick up the medicine and the pharmacy tech asked, "Are you sure you want this Colcrys?  It's $328.75."

"What?  I want the generic version, colchicine.  I don't need the name brand version." was my reply.
She went back to the pharmacist to ask about this.

How could a single pill go from costing $0.16 to $5.48?   What the heck?  

Here's what I learned:

In 1962, the FDA began a drug testing and approval process to make sure that new medications on the market are safe, effective, and accompanied with helpful information for physicians, pharmacists, and patients.  

In 2006, the FDA began the "Unapproved Drug Initiative" to get old medicines that have not gone through the FDA approval process off the market.  These old drugs were about 2% of the prescriptions written at that time.  The intent is good:  protect us from bad drugs and potential dangerous side effects and interactions of medications. 

It turns out that in 2009, URL Pharma agreed to take Colchicine through the FDA's review and approval process.  In return, it gets three (3) years of exclusive rights to sell the Colcrys for gout and seven (7) years to sell for Familial Mediterranean Fever (through the Orphan Drug Act).  

It also can price it however it chooses.  The FDA does not regulate pricing of drugs.
But how much is too much???   
I believe increasing the price by 34 times higher is beyond greedy... it is downright cruel to people who are ill and in significant pain.

So here's the practical rub:  

The study with 1,000 patients conducted by URL Pharma met FDA requirements, and it did not provide any new insights about the drug, contraindications, or interactions that were previously unknown to the medical community.

We are no safer today with colchicine (aka Colcrys) than before URL Pharma came along.  What was a generic drug (and quite affordable) has become a brand-name drug owned and sold in a monopolistic, predatory manner.

A rheumatologist told me that in a few years, it will go back to the $0.16 per pill.

I double-checked with my rheumatologist about this medicine, given my sticker shock.  
We decided that I would bite the bullet and get the colcrys.

Today, I went for my refill and learned that the price dropped a whopping $4.00 for 60 pills.  So this month, each pill costs me $5.41.  That's still predatory pricing in my book.

This drug that costs $5.41 per pill used to cost me $0.16 per pill.

The pharmacy tech that dispensed the Colcrys to me said, "Wow!  I couldn't afford to take this medicine if I had to.  You're lucky you can afford it."

I asked him if he sees a lot of patients who leave prescriptions at the counter because they are too expensive.  "Several times a day," he replied.  "Some people ask for a week's worth of a prescription because that's all they can afford this week or this month."  


His words really hit home... I am indeed lucky.  

I am married to a wonderful guy who has a great job with excellent health insurance coverage.  I have not been able to work in a meaningful way since struck by illness and that has taken a huge toll on me mentally and emotionally.  (We miss the earnings in our household budget, too).

If dear hubby lost his job and health insurance, I would be among the millions of "Un-insurables" with serious pre-existing conditions. 

The State of Georgia defers to the federal government's High Risk Insurance Program for people like me.  To qualify for the federal program, I would have to be uninsured for 6 months.  I'm pretty sure we wouldn't be able to afford 6 months of specialist visits and all my medications (we'd likely make too much money to qualify for patient assistance programs).  I would likely experience thromboses again and maybe a stroke out before I could access the medications again.

It's no wonder that medical bills are the #1 cause of personal bankruptcies in the United States.  People will do anything they can to ease the pain of their loved ones.

My parents taught us this maxim:  Just because you can, doesn't mean you should. 

This concept applies to many aspects of life, including price gouging.  

Imagine a world where people, corporations, organizations, and governments adhered to this!

Feeling Hot, Hot, Hot

Just when I think I'm managing my "new normal" something unexpected and significant interrupts my well-being.  My hubby asks, "when are the locusts arriving?"

This past spring, my toes, feet, fingers, and hands began to behave in a most unusual way:  they would turn red, swell up, and itch like crazy.  It starts with a tingle, then the tissue would rise before my eyes.  Remember those pieces of cardboard that turn into sponges when dipped in water?  

The first couple of months, the swelling would occur in one limb for a couple of days and then disappear, only to "re-emerge" in another extremity.  It was unpredictable and painful.

I was put back on prednisone because it's considered "the gold standard" for inflammation.  I was in so much pain that I agreed to take it despite my previous experience (a full-year of me becoming an irritable, annoying, and extremely overweight insomniac).  I was desperate!  
I tried it for 3 months;  at first it seemed to reduce the swelling, but after a couple of months it made no difference. We had to try something else.

I share these pics with the following disclaimers:  Yes, I know that I need a pedicure.  Yes, my feet could easily make Time magazine's "Ugliest feet in America" award.    

The foot tissue swells, itches & burns.

See the difference between the swollen right foot & the unaffected left foot?

Side view of both feet.

The inflammation seems to bounce around from foot to hand to foot to arm.  When my feet are inflamed, walking is extremely painful.  

Now, take a look at these ever-attractive hands:

Ring finger swells into hand.

 

Top of hand swollen, but not red.  Weird!

Hand & fingers so swollen I can't hold anything.

Right hand swollen & thumb burning red.

    

Right hand & joints swollen and sore.

When both hands are inflamed, major ouch!

In addition to the tissue inflammation, the joints became involved.  When the joints in my hands, knees, and feet are involved, my mobility is severely limited.  

Thus began a six month journey to find out what the heck is going on and what can be done to make it stop.  

I was tested for Rheumatoid Arthritis (because the inflammation had a symmetrical pattern on the extremities) and that came back negative.  Gout was also ruled out.

My rheumatologist confirmed that these symptoms are not at all related to the Behcet's Disease (auto-immune).  My Behcet's symptoms are well-managed with regular infusions of Remicade.  I also learned from others living with Behcet's that this is not one of their experiences.  

Through the great world wide web of support groups for people living with Polycythemia Vera and other MPNs, I was encouraged to pursue a line of questioning for Erythromelalgia (EM).   I brought the information (and photos) to my internist for consideration.  His research confirmed that these symptoms can occur as a result of the PV.  

The Erythromelalgia Association (www.erythromelalgia.org) provided some helpful information and links to other resources, including EM patient groups.  Once again, here's  a rare, odd chronic health condition that has no clear treatment.  What works for some doesn't work for others.  They confirmed that the trial and error path I'm on is par for the course.  

At the doc's suggestion, I apply lidocaine patches to the areas at the earliest sign of inflammation.  This helps to mitigate a full-blown inflammation for the area covered, but sometimes the swelling occurs above or below the patch.  I've also been taking antihistamines (both H1 and H2 blockers) and Colcrys (the now-expensive colchicine, an anti-inflammatory med) all to no avail.

After five weeks of continual swelling of hands, knees, and feet, I was ready to jump.

One day when my hands were swollen, I drove past a Chinese foot massage shop and decided to check it out.  Foot reflexology can be relaxing at worst, and curative at best.  Using my smart phone, I checked out the reviews of the foot massage place.  I decided to give it a try.

I must say, after the one-hour reflexology massage, I was relaxed and in less pain.  I went back the next day for more.  It didn't cure, but it does feel good!

When I showed these photos to my niece who is a chiropractor and acupuncturist in California, she said that they are signs that I have too much heat in my system.  I decided to find an acupuncturist in my area who could help me with this. 

I had my first appointments last week with Leena Sikand-Cook of Healthy Body and Soul clinic in Lawrenceville.  Leena explained that Chinese medicine is about keeping the body in balance.  She read my pulses and indicated that my blood has too much heat.  

Here are a couple of pics my friend took of me during my second appointment.

Please ignore all my chins!

These needles don't hurt a bit - really!

She also pricked the tips of my fingers and toes to get a few drops of blood to escape.  I'm going to give this some time to see if it will provide some relief.
Stay tuned...

What Happened to the Little Engine That Could?

Invisible Illness Awareness Week

September 10-16, 2012

invisibleillnessweek.com

Kind. Empathetic. Nurturing. Positive. Funny.  These are some adjectives that describe me.   I've always championed causes of the underdog and I have the resume and references to prove it.  Yet I am embarrassed to admit that until I became ill with Polycythemia Vera, I was not attuned to the suffering of millions of people who live their lives with no obvious outward sign of the pain and uncertainty they manage every day.  

My parents raised me with a strong feminist work ethic:  I had the same potential for achievement as my brothers.  I believed that if I tried hard enough, I could achieve most anything.  Persistence, stubbornness, and a bit of naiveté had served me well over the years, thus reinforcing those beliefs.  The power of my will and effort could overcome all obstacles.  That was my truth.  Until October 14, 2007.

Mid-Life's School of Hard Knocks has given me a new perspective.  I'm coming up on 5 years of a second chance at life.  Living these years has humbled me, frightened me, angered me, and depressed me.  I've been afraid to accept my 'new normal' for fear that it is akin to giving in to the diseases.  I don't want to be a victim so I try to deny what is happening in my body.  Yet the blood work doesn't lie.  The symptoms persist.  The side effects of the treatments are better than the diseases untreated.  I believe that if I keep digging into research and brainstorming the 'right' questions, cures will be found and I can get my wonderful life back.

I now assume that everyone has something that is a hidden personal challenge.  From this assumption, I try to enter interactions with a little more compassion.  I call upon patience when encountering someone may be a bit rude or disagreeable.  I don't condone rudeness, of course, but I also don't let it ruin my day.  After all, I know what prednisone did to me for one year!

I know that it is possible to be functionally 'normal' while masking chronic illness.  I know how exhilarating yet exhausting it is to be 'on' for work and family.  And I know that I still have a lot to learn about the effects of invisible illness on the patient, care givers, family, friends, employers, and community.  

The Invisible Illness Week organizers challenged us to answer the following meme.  Please share your reaction to this.

30 THINGS ABOUT MY INVISIBLE ILLNESS YOU MAY NOT KNOW

1. The invisible illness I live with is:  Polycythemia Vera, Behcet's Disease, and Migraines
2. I was diagnosed with it in the year:  PV in 2009;  Behcet's in 2010; Migraines 1983
3. But I had symptoms since:  PV since 2006; Behcet's since 2009; Migraines since 1981
4. The biggest adjustment I’ve had to make is: finding peace with limited energy and the inability to work regularly.
5. Most people assume: that I am healthy and fine because I have a happy, positive personality.
6. The hardest part about mornings are: waking up and moving through the aches and pains.
7. My favorite medical TV show is:  House
8. A gadget I couldn’t live without is: my iPhone
9. The hardest part about nights are: chronic insomnia
10. Each day I take 14 pills & vitamins. (No comments, please)
11. Regarding alternative treatments I: am open to them, but cautious of drug interactions.
12. If I had to choose between an invisible illness or visible I would choose: the invisible because I can "pass" as normal and not attract unwanted attention.
13. Regarding working and career:  I desperately miss my career and earning potential.
14. People would be surprised to know:  that I have withdrawn from many community and social functions because I don't like answering the questions, "are you well yet?" and "what are you doing these days?"
15. The hardest thing to accept about my new reality has been: feeling vulnerable and powerless about my future since there are no cures
16. Something I never thought I could do with my illness that I did was:  travel to Europe with my family
17. The commercials about my illness: do not exist because PV and Behcet's are so rare.
18. Something I really miss doing since I was diagnosed is:  being a consistent, vibrant part of my community
19. It was really hard to have to give up:  wine with dinner
20. A new hobby I have taken up since my diagnosis is: knitting (learned from YouTube videos)
21. If I could have one day of feeling normal again I would:  tour wine country and have lots of great sex with my husband
22. My illness has taught me:  that we take good health for granted.  Science and medicine have not kept pace with all the things that can go wrong in the human body.
23. Want to know a secret? One thing people say that gets under my skin is:  "You don't look sick" and "It's God's plan"
24. But I love it when people:  ask honest questions about the illnesses and listen
25. My favorite motto, scripture, quote that gets me through tough times is:  It could always be worse!
26. When someone is diagnosed I’d like to tell them:  It is scary as hell in the beginning; don't believe everything you read on the internet; there is a wonderful support community on-line  ready to welcome you to this special "club"; it gets better.
27. Something that has surprised me about living with an illness is: how many people are living with invisible illnesses
28. The nicest thing someone did for me when I wasn’t feeling well was:  two gal pals came to my house when I couldn't travel with my family.  We talked (they did most of the talking) and laughed and I momentarily forgot the pain.  They treated me as a full friend, not a fragile sick being.
29. I’m involved with Invisible Illness Week because:  I can use my voice to share my experience to dispel some myths, educate the currently healthy, and offer support and hope to others living with chronic, invisible illnesses.
30. The fact that you read this list makes me feel:  validated and grateful that you are curious to learn.

[Since September is also Blood Cancer Awareness month, I encourage you to learn about the Myeloproliferative Neoplasms family.  I have Polycythemia Vera, a MPN.  My bone marrow over-produces red blood cells (and sometimes platelets), causing thick blood and dangerous thromboses.]   MPN Research Foundation