Myeloproliferative Neoplasms Conference - Day 1 Highlights

More than 200 cancer patients, supporters, and physicians/researchers gathered at Mayo Clinic Scottsdale this morning at 8 am to begin a lively conference about the state of knowledge and treatment of the Myeloproliferative Neoplasms -- Essential Thrombocythemia, Polycythemia Vera, and Myelo Fibrosis.

Dr. Ruben Mesa opened the session and welcomed everyone and reminds us that this conference is because of Joyce Niblack.  Her husband Bob carries on her work through the CMPD Education Foundation with this Biennial Conference.

It will take some time to prepare proper notes from the conference, as the presenters had visual presentations and talked quickly.  When I get all the information together, I will post a complete written report.

Here are a few nuggets from today:

The hematocrit that feels best for you is where you should be.   While some people feel good at 42 or 45, others may need it lower to feel good, like 38 - 40.  Don't let your doctor get stuck on a prescribed number if it doesn't feel good for you.

Don't worry about tracking your JAK2 allele burden over time;  we don't know that it has any clinical relevance.  All the drugs today inhibit the JAK2.

JAK2 inhibitors are unlikely to eliminate the disease.

Whole exome sequencing to identifyMPN alleles and gene sequencing is becoming more affordable.
Soon there will be a lot of data; they will be able to learn to identify what genes cause the transformation to leukemia.

In your genes:
2 copies of JAK2 => PV
1 copy of JAK2 => ET


Future in drug therapies:  combine drugs in rational combinations to facilitate next level of clinical trials

Prolonged use of interferon can reverse fibrosis in the bone marrow (does not cure the disease).

Help for Pruritis (itchy skin):

• JAK2 inhibitors are good.
• Interferon is better than HU.
• UV narrow band B works 70% of time (see dermatologist)
• Weight wraps (prednisone in a wrap) (see dermatologist)
• Aprepitant, an anti-emetic, works on the Substance P

Formication:  the sensation of ants crawling on your body.

Hematopoietic Cell Transplantation (HCT):
The only treatment that has potential for a cure for MPNs is the stem cell transplant.

Pegasys (Pegylated Interferon) is more potent than regular Interferon-a.  The dose is not so important as the duration.  The effect is probably due to your own biologic abilities.

Standard practice regarding Phlebotomies:  no more than 4 per year.

If you are going to get a splenectomy, go to one of the half dozen surgeons who have done the procedure on MPN patients.  Removing the spleen has real risks:  clots in the area;  5-6% mortality (similar to open heart surgery).

When to have another Bone Marrow Biopsy (BMB)?  only if concerned the diseased has progressed to Myelofibrosis.


That's all for now!

Calling All Folks Living with ET, PV, & MF

MPN Upate:
The 6th Biennial Joyce Niblack Memorial Conference on Myeloproliferative Neoplasms kicked off today with a great luncheon at Alchemy restaurant in Fountain Hills, Arizona.  Bob Niblack ordered perfect weather so we could enjoy an outdoor buffet and meet others in the journey with MPNs.  

There are many smiling faces;  this has to be the friendliest group of blood cancer patients!
It is so refreshing to meet others who "don't look sick" on the outside yet have so much going on in the bloodstream.  

I am attending the conference with my mom, Eileen, and together we'll be recording notes of the presentations for the next two days.  I'll then post them and share with others who cannot be here.  They will also be helpful for later reference.  Photos will come when I get them uploaded.

Stay tuned for the latest in MPN news brought to you by the CMPD Education Foundation & Mayo Clinic, Scottsdale.
Cheers!

Fa La La La Hum Bug!

Now that Christmas is over, I’ve got to get this off my chest:  I’m glad it’s done.   All I wanted for Christmas was a “Fast Forward” button for the month of December.    Yep.  Call me  Grinch, Scrooge, Grumpy, whomever you like.    I know it’s not politically correct.   As I went about the usual December activities with a cold and a Behcet’s flare-up, I secretly wanted to escape from all the holiday frivolity.  

I don’t begrudge anyone who feels jolly – I had that twinkle in my eye for many years.  I get it.  I really do.   And for the first time, I have some compassion for the Bah Humbug crowd.   

This isn’t a complete swing of the pendulum from Holiday HO HO to Heck NO.  

I’m not against celebration.  It’s not anger, resentment, or loneliness.  I’m just not feeling it. 

But just because I lack enthusiasm doesn’t mean that I place a judgment on another’s fervor for the holidays.   I’m not into that polar argument nonsense.

I remember getting sick after winter final exams – college kids often come home and crash for a couple of days before getting into the holiday spirit.  The opportunity to catch up with friends and go out on the town was enough to distract a young mind from any holiday funk.

As I got older, a winter cold might take hold, often accompanied with a case of melancholy.   There is a seasonal heaviness in my heart that is difficult to explain – a desire to nestle in at home, shed a few tears for no apparent reason, think deeply and introspectively and nap.    This has become an annual ritual for the last ten years or so.

Before the onset of Polycythemia Vera and Behcet’s Disease, I thought I could outsmart the seasonal depression by planning activities to keep my brain and body distracted.   It seemed to work. 

But holidays with chronic illnesses is a whole new ballgame, and I’m still learning the rules.   The strictest rule seems to be:  the ill body is not as elastic as the healthy body.  Translation:  you cannot push yourself as far as you were once able.    The force of will cannot over come the limitations of a weakened body.   

That “one last errand” can trigger  exhaustion that forces early retirement to bed and cancellation of later plans.  A medication forgotten can result in severe pain or blood levels dangerously thrown off balance.   Every day has to have Plan B & C contingencies based on health levels at the time.

If you invite me to an event, please do not be offended if I cannot attend.  I don’t want to dress up and pretend to feel good.  Just getting psyched up to get ready is exhausting.  I don’t want people to see me looking like I do now – “moon face,” skin discolorations, and weight gain from steroids and other medicines.   It’s hard for me to stand for extended periods of time.  Sometimes I lose my balance (when I haven’t had any alcohol) and my short-term memory isn’t as reliable as it used to be.   I’m self-conscious and uncomfortable in my own skin. 

  

Do not mistake this for becoming anti-social.  My methods of interactions are changing.

I find myself keeping up with people through social networking sites like facebook and LinkedIn.  I enjoy seeing what family, friends, and acquaintances are up to in the comfort of my own home. 

This holiday season required more conscious energy than ever to keep up enthusiastic appearances for my kids.  Shouldn’t the enthusiasm naturally emerge?  It always has before.   Perhaps the reclusive tendency is more a means of self-preservation than holiday resistance.

I now have sympathy for the Bah Humbugs.  They each have their own stories, just as I have mine.  Rather than judge them, I silently or directly wish them a Happy New Year.  

MPD Foundation's latest Symposium & Other Good News

Those of us living with rare bone marrow-based blood cancers (myeloproliferative neoplasms) can find helpful information and support through the MPD Foundation (www.mpdfoundation.org).

Based in Chicago, the MPD Foundation sponsors patient-physician symposia across the United States, stimulates research for new treatments (and eventually cures), and fosters collaboration in many ways. I'm a big fan of the organization -- not only its mission, but the people who make it happen. By website and by phone, the MPD Foundation was helpful and reassuring when I was first diagnosed with polycythemia vera.

On October 25th, the MPD Foundation hosted a patient educational symposium at the University of California San Diego Moores Cancer Center in La Jolla, California. They brought in experts from leading institutions to share the latest in the range of research, clinical trials, the patient experience.

One of the experts is a patient who went through a bone marrow transplant and now lives with chronic Graft vs. Host Disease. You can read more and view the symposium video on the MPD website.

This month, the MPD Foundation represented us patients at the American Society of Hematology meeting. Because the MPDs (I guess we need to get accustomed to calling them MPNs) are rare compared to all the other blood cancers, their presence reminds practitioners that PV, ET, and MF patients are in their communities. They also make practitioners aware of local MPD support groups across the country.


In other news...

I've seen a few queries on various MPD listservs and posts (and I asked this myself when I was diagnosed with Polycythemia Vera one year ago):

Is PV/ET/MF considered cancer?
The question may not seem important, but it is for several reasons:
1. health insurance and disability insurance coverages are impacted based on illness codes.
2. naming "it" helps you come to terms with what's going on in your body.
3. it simplifies the explanation to the casual inquirer.

And now it's official! You've got a Cancer.
The World Health Organization (WHO -- not to be confused with "The Who") officially reclassified the myeloproliferative "disorders" as "neoplasms" (meaning "malignancies" or the dreaded "cancer").

This reclassification will help patients who need the chemotherapies to survive get the coverage they need from their health insurance. Believe it or not, all insurers are not up to speed on indicators for polycythemia vera, essential thrombocythemia, and primary myelofibrosis; current standard treatments; and promising off-label treatments.

Fighting a disease and fighting a bureaucracy at the same time is doubly exhausting!

The good news is that we can be living testimony that "Cancer" does not set an immediate expiration date. While there is yet no cure, we can live with these pesky diseases for quite some time as long as we take special care of and listen to our bodies.


There is still some conflicting information on the web:

www.cancer.org (American Cancer Society) still refers to MPNs as "chronic disorders and diseases"

www.lls.org (Leukemia & Lymphoma Society) refers to them as "clonal diseases" but the fact sheets are in the leukemia category.

Funny / True Story:  

Someone actually called me a few days after we had a conversation about my friend "Polly" and said, "Hey! I've got great news for you! I looked up polycythemia on the ACS website and it's a disorder, not a cancer!"

It was then I was reminded that words hold emotions for us all. While the word didn't change a single thing about my condition, this person felt a lot better. What should I say? I'm so happy for you? We hadn't spent a lot of time on the "C" word, so I was a bit surprised. I explained that I wasn't angling for the cancer label. The shift from "disorder" to "neoplasm" was fairly recent and because the MPDs don't afflict a large number of people, the news might not have reached the ACS webmaster yet.

Sometimes it just is what it is...

Hematology Experts Raise Hope and Spirits!

I remember a day when Katrina was a little girl working intently on one of her self-initiated projects. I asked her a question and she said, "Mommy, I just don't know. My mind is full right now!" She put her fingers on her temples for emphasis. Not only was that foreshadowing of the intense personality that was developing, she taught me a phrase I find myself using a lot lately.

My mind is full! And I feel affirmed and understood -- two great gifts today.

This morning, Robert and I went to our much anticipated appointment with Dr. Elliott Winton, a hematologist at Emory's Winship Cancer Institute in Decatur, Georgia. (http://cancer.emory.edu/)

After researching as much as I could on-line, connecting with other patients living with Polycythemia Vera and other Myelo Proliferative Neoplasms, I couldn't wait to sit with someone who literally studies and thinks about these rare blood cancers on a daily basis. Yes, I finally said the "c" word; the science world has raised this blood disease group from a "disorder" to "neoplasm" which means malignancy. That's okay -- I can live with it. I call it "Polly." The personification helps me focus my frustration on this "thing" that has a lot of control over my life.

Dr. Winton studied my medical history of the last two years (sent over by Kaiser Permanente) as well as information Emory kept on file from tests I had there in 2003 (which I had long forgotten). He noted that there was evidence of an MPN in 2003 when my bloodwork showed high platelets and low hemoglobin. At that time, he said, they wouldn't have made the diagnosis, as the breakthrough scientific discovery of the JAK-2 protein gene mutation didn't occur until 2005.

In October, 2007 I experienced thrombosis (clots) of the portal, splenic, and superior mesentary veins. Those clots caused a cascade of additional health issues which I've described in posts on this site from around that time. Dr. Winton explained that these types of clots in the gut are major indicators of an MPN, but because they occur so rarely, the JAK-2 test sometimes isn't ordered and the diagnosis is missed. I have been on an anti-coagulation regimen of warfarin (coumadin) ever since. This treatment has staved off more clots; it does nothing to address the root source of the problem.

I also began taking iron pills again after the surgery to address the anemia that resulted from blood loss from surgery. Guess what? That triggered more red blood cell production -- a natural process, just aggressive for someone with PV.

I never regained my full steam after the surgery and within a year, had to quit my job at The IMPACT! Group. I simply didn't have the stamina to meet the needs of the organization. Little did I know that my bone marrow was working over time. I was tired all the time and couldn't push through it.

By the spring and summer of 2009, I noticed my toes and fingers turned purple intermittently. The doctors shrugged this off and were unconcerned. By July I was itchy and in August, my left ankle began to swell, turned red/purple, and became painful. I went to the doctor in September; a wonderful internist ran some blood work and called me the next day to let me know that there are some concerns (my hematocrit was almost 70) and I needed to see the hematologist immediately. My blood pressure also went through the roof.

We started with twice weekly phlebotomies and then added Hydroxyurea (HU) chemo pills to bring the hematocrit level to a healthy range.

Dr. Winton shared that a hematocrit that high is rare, even for someone with an MPN; that it is amazing that I didn't have a stroke or worse. I knew it was serious and I knew I was scared; I didn't know that it was my second "close call" in two years!

Dr. Winton provided a clear and interesting lesson in the types of blood cells (can you name them? T-cells, B-cells, Red Blood Cells, Granulocytes, Monocytes, and Platelets) and how they are made in the bone marrow, differentiate, and eventually die (apoptosis).

He explained that the JAK-2 mutation that has occurred in me presently affects the proliferation of the stem cells that create red blood cells (that's the polycythemia vera type of MPN).

Note to reader: If you read this and there are inaccuracies or errors, they are due to my limited memory and poor note-taking. They are not to be attributed to Dr. Winton. I did not run this by him before hitting "publish post." Mea culpa in advance.

The clinical problems created by Polycythemia Vera can include:

1. Clotting & bleeding which can trigger strokes, heart attacks, etc.

This is treated with anticoagulation medications (I'm on warfarin/coumadin blood thinner because I had the major thrombotic event in October 2007). There are supposed to be some better drugs coming on the market in the next couple of years (yeay!) Other patients who have not had blood clots take "baby" aspirin -- 81 mg/day. This occurs in about 1/3 of the patients.

2. Myelofibrosis -- the PV also increases production of collagen in the bone marrow and extra medulary hematapoeisis (when blood is created in the spleen and/or liver).

This would be treated by decreasing the JAK2 clone size.

3. Acute Leukemia -- well, this is just not a good thing; very limited time on the planet. This happens in 3-5% of the patients.

So... How do we decrease the clone size?

Thankfully, there are scientists all across the globe working on this.

Right now, the only known cure is a Stem Cell Transplant.

Emory does "mini transplants" where some of the patient's stem cells remain, but receives many more from the donor.

That requires typing my cells and those of potential donors (siblings are most likely matches). It would require a 1 month hospital stay.

The downside? For a person of my age, there is a 15% mortality rate.

So this is not a wise option at this stage of the disease. I will pursue more information on this option as a Plan C or D, as it is better to prepare early rather than wait for an acute situation and have everyone scrambling.

The second option is Interferon. This chemo treatment slows the myelofibrosis, but the most common side effect is feeling like you have the flu all the time. No thanks!

There is a pegylated version (I think they use Roferon instead of Interferon) that requires just one injection a week and the side effects are minimal for most patients with the dosage appropriate for them. There is a Phase II trial coming up for this in about 6 months.

Most insurance does not pay for this yet, so some patients try to get into trials.

The third option is Hydroxyurea (HU) or Hydrea. This 40 year old chemotherapy slows the proliferation of the red blood cells. I am currently taking 500 mg of HU daily. The dosage level at 1,000 proved to be too much for me (resulted in the most awful sores in inconvenient places). There are other side effects of the HU that are annoying but bearable.

The fourth and most exciting option is on the horizon: JAK-2 Inhibitors.

There are several drugs in the pipeline that are a few years away from being ready for prime time.

So what is next for me?

1. Pay close attention to my hemaglobin level (must stay below 42).

2. Pay close attention to my INR (coagulation ratio) -- must stay between 2.0 and 3.0

3. Get a Bone Marrow Biopsy -- to gauge the progression of the disease process and Type my bone marrow for possible stem cell transplant in the future.

4. Additional tests (like D-Dimer) to gather enough information on my situation for the experts to determine what clinical trials might be appropriate for me.

5. See if my blood pressure can be stable in these conditions without the nadolol.

If you've read this far, I hope you can see how good it feels to be affirmed and have a plan of action. I'm in it to win it! It is humbling to be in this situation at a time when scientific discoveries are happening in this rare blood disease area.

My experience with Polycythemia Vera will be added to the international data base of patients and contribute to future study and hopefully discoveries. (I'm not worried about privacy. By this point, I'm essentially uninsurable in the US with these significant pre-existing conditions. If Robert lost his employment with a large company, we'd be in a sticky wicket. But I'm not borrowing any more trouble right now, thank you!)

You know I don't like roller coaster rides. But after my session with Dr. Winton, I feel like I can handle a few more bumpy rides -- with a reasonable plan and little help from my friends.

Polly kept me from traveling with Robert and the kids to Beaver Creek, CO for a couple of days skiing this week. My family is the greatest and I'm so glad that we didn't let Polly "win" by completely spoiling the family trip. Polly also brought some good friends to me to pass the time in my family's absence. Gratitude is a gift one gives to oneself. I am grateful!

Looking for the Bright Side of Life's Challenges

This time of year has always caused more angst than joy for me. More obligations in work, family, and social aspects of life in a compressed time period (not to mention when the nights are longer -- less daylight) would tip me into the "winter blues."

This Christmas season, living with Polycythemia Vera (www.mpdfoundation.org) and its lovely side effects, I'm forced to re-set expectations and focus on getting and maintaining a reasonable level of healthiness. And guess what? The earth is still rotating on its axis even though I'm doing a fraction of what I've done in past years.

Searching for the bright side of living with a chronic, invisible illness, I've found a few:

1. Being immunosuppressed is a great reason to avoid crowded shopping areas and people you don't care to see. Getting a bargain is not worth getting a virus. And most people do not wash their hands enough!

2. Fatigue/exhaustion during the day provides opportunities for mandatory "cat naps." Ahhh... the oft-misunderstood delight of a brief respite during the day!

3. Family and friends are impressed with your accomplishments that previously would have barely attracted notice. So glad you came to that meeting! Dinner was great, mom! Thanks for making that extra trip when I forgot my project at home...

4. Someone else (like a wonderful teenage daughter) will do a lot of the gift wrapping that caused me great last-minute stress in previous years.

5. You get to know all the lab techs, nurses, and physicians with weekly/bi-weekly visits. You are not just another nameless co-pay in the health care industrial complex.

6. "Chemo brain" is a great excuse for forgetfulness. What was I going to tell you? Why did I open this drawer? Didn't I tell you ...?

7. While you don't get preferred parking spots, you can "fake it" pretty well and decide how public you want the illness to be. Since you don't look "sick" people don't assume the worst and bombard you with knowing looks of pity or fear.

8. You appreciate the "good" days so much more than ever before -- being able to go places, work, be an active parent and spouse, be a friend... No more taking healthy life for granted!

9. Side effects are just another way of forcing you to to pay attention to the present. Annoying? Painful? Frustrating? Yes! Yes! Yes! Living through this in the moment, step by step, is part of the journey.

10. Chronic illness is like a loofah sponge -- it forces you to shed the flakes from your life. Life is too short and time is too precious to spend on people / activities / thoughts that do not contribute positively.

So there's my Top Ten benefits of living with a chronic illness.
Please add others ... I'm interested to learn from you, too!

Sending best wishes for a Happy Hannakah, Merry Christmas, Sublime Solstice, Happy Kwanzaa, and all other celebrations you enjoy this time of year.

with peace and love,
marina

She's Drivin' Miss Daisy

Those of you driving around Duluth should stay alert for a new driver -- my Katrina has her permit and is now driving me around town! She is very alert and has the best posture I've ever seen behind the wheel. My mom gave her some driving lessons in the Arena parking lot last month; that's when she learned about the invisible brake on the passenger side floor board.

It is a real gift to be able to share this new chapter with Katrina. I can say with certainty that November is going to be a much better month than were September and October from a health perspective. I am so happy to report that the phlebotomies and HU meds are working. The hematocrit is down to 41 (where it should be) and I am feeling like my old self again. The platelets have creeped up a little high (to 499), but this is part of the balancing act that I'll be managing with polycythemia vera.

I've just started a new project for GSTAND -- we're looking for best practices across the state where local jurisdictions are working with nonprofits to implement the Neighborhood Stabilization Program funds. I look forward to learning about some good successes in reclaiming neighborhoods harmed by concentrations of foreclosures. Stay tuned!

It's official -- I do have a brain

Today was the only day this week without a medical appointment, but I did receive two bits of news.
First, Kaiser approved a referral to an MPD specialist at Emory University Hospital -- I'm very happy to be able to get the perspective of someone who works with a lot of patients with a myeloproliferative disorder.

The second news came in a phone call from my internist. The MRI & MRA done on my head and neck on Monday showed no clots (great news). But it does show white matter changes which could indicate demyelinating disease (multiple sclerosis). She referred me to a neurologist and I've got an appointment for December 2nd.

Hopefully we'll get some better insight to the headaches and blotchy vision that continues to disrupt my daily activities.

My blood pressure is under control and this provides great relief. EKG is normal and I'll continue to calibrate the blood pressure meds with my internist as the hematocrit improves with the phlebotomies and hydrea.

Tomorrow I'll be at Emory for a nuclear medicine test of the red blood cells.
Friday is phlebotomy day -- this will set me up for a great weekend!

Happy Halloween!

We've Got a Plan and We're Working It

Robert and I met with my hematologist on Tuesday and established a course of action.
Since the HGB and hematocrit levels aren't falling very quickly, I'm going to continue with 2 phlebotomies a week and begin a regimine of 500 mg of hydrea (hydroxyurea or HU) daily.

My blood pressure is still too high (165/110) so I went back to the internist to check the dosage of the BP meds. Those have been adjusted, so it should be improving soon.

So today is my first day of the chemo and blood pressure meds with the warfarin (blood thinner) -- makes me feel old to have a handful of pills in the morning!

I am still easily tired and my vision is annoyingly blotchy -- this is most likely due to the high blood pressure I am told.

I attended the World Class Leaders reception for Leadership Gwinnett this evening -- It was so great to focus on something other than this illness!

The phases of dealing with a life altering illness are much better read about than experienced. It is tough to strike a healthy balance between reading everything one can find on the illness, treatments, side effects and clinical trials and work assignments and family life. All three must be squeezed between naps, which are no longer voluntary.

Last week, I felt an incredible sense of relief that there is a "name" and real cause for the extreme fatigue and other symptoms that have been gaining steam in recent months. It's not all in my head; I'm not a hypochondriac... funny how the self-doubts can mount when there are no answers.

After the relief came the overwhelming desire to fully understand the disease -- what EXACTLY is it, how does it happen, what happens next... mix one diagnosis with others and what do they mean?

Right now, I'm okay with developing a series of questions. Each answer seems to lead to three more questions.

I am so GRATEFUL that we have gifted humans who choose to study the sciences -- and never underestimate the importance stem cell research has on all our lives. The results of their persistent curiousity is already giving me more time.

Better living through chemistry!

peace,
m