Saturday, December 25, 2010
The site requires registration (free) with an email address to view the full articles.
TIP #1 for patients: create a free email account that you use just for your health research (like you hopefully do for your on-line contests) to prevent too much junk filling up your personal email account.
TIP #2 for patients: just because something is in print, doesn't make it true (or true for you). Especially with these diseases, science is working hard to catch up.
Thrombosis Risk Rises in Women With Myeloproliferative Disorders - OncologySTAT
When you register with the website, you can select the type(s) of cancers you'd like more information about (including MPDs and rare cancers!).
Let me know if this is helpful for you.
Thursday, December 16, 2010
A real migraine makes me feel nauseous, sensitive to sound and light, dizzy, and the pain is so intense that I can't think straight. And it is all confined in this small space on top of my neck! I want to scream but the noise of my screaming would be annoying. When it happens in a public place (like at work), it's horrible.
And if others are around when a migraine strikes, I'm likely to use my less cultivated vocabulary to describe how I'm feeling and what they can do to help me.
An MRI of my brain shows several white spots that are consistent with migraine damage (no surprise to my brothers that I have brain damage) from days gone by.
We've spent this year trying various medications to keep them at bay. So far, the prednisone (steroid) I take for the Behcet's Disease works the best. But I can't stay on the steroid long term, as it is not good for bones (and I already have degenerative disk disease in my spine).
So now I've started Topiramate, a medicine for epilepsy which is also used for migraines. One of the side effects is loss of appetite. I'm hoping this counteracts the side effects of the steroid (munchie activator and fluid retention). We'll see.
The National Migraine Association's website has links to many sites and sources of great information for migraine sufferers. Check out the website: www.migraines.org
Friday, December 10, 2010
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:
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.
Wednesday, May 12, 2010
I have Polycythemia Vera, a myeloproliferative neoplasm (MPN) -- the stems cells in my bone marrow over-produce Red Blood Cells (RBCs). This thickens the blood and causes potential for clots and stroke. In addition, my blood has the Alphathalassemia Trait (the RBCs are smaller than normal and oddly shaped, so they don't transport hemoglobin efficiently). I am also iron-deficient (and can't take iron supplements, as iron stimulates RBC production) With these things working together, Anemia and the accompanying fatigue is part of my daily life.
My mom traveled with me so we could learn together (and she could keep a watchful eye on me -- to keep from over-doing it). I was so glad that she was there. Much of the cellular biology and mutation discussion was over my head, and she helped me make some sense of it. There was also a huge emotional component to being there that I hadn't anticipated.
I went to this symposium with several questions:
1. What has been learned about causation of MPNs?
Nothing more than the JAK-2 mutation, and even that does not occur with 100% of the patients.
2. What progress has been made in managing the effects of MPNs?
There is no substitute for eating healthy foods (grains, fruits and vegetables), daily exercise/movement - 30 minutes a day, and more rest than the average healthy person.
3. Is there a "cure" in the works?
No cure, but there are clinical trials of drugs to affect the JAK-2 protein.
4. Is there a correlation between the blood diseases and auto-immune diseases (because I was recently diagnosed with Behcet's Disease)?
No observed correlation between MPDs and auto-immune diseases. There are some patients with MDS who also have Behcet's Disease.
5. Is there another protocol I should be considering with my physicians?
As long as the Hydroxyurea is working, no need to go to the shots of Interferon or Pegasys.
6. What is the likelihood that I will get my "old self" back?
It's time to accept my "new normal" The more physically fit I can be, the better I will feel. But there is no cure or way to overcome the side effects of the disease process and medications to be able to resume the full-speed ahead pace of my past. AGGHHH!!!
7. If "no" to #6, is there a rate of disease progression that I might expect?
Each patient with an MPN progresses at a different rate. They are still collecting data to see if there are some trends. Because most MPN patients are diagnosed in their 60's, the life expectancy figures are not directly applicable to younger patients.
While I won't begin to do justice to the vast amount of information that was shared, I will share my "Aha" moments and some of the points I found most interesting.
Dr. Mesa brought together an impressive group of specialists who prepared their remarks in printed form so we could follow along. They took their Saturday and Sunday (which was also Mother's Day) as personal time to share their knowledge with approximately 160 participants -- mostly blood cancer patients and their spouse/caregiver.
Did you know that the body of medical knowledge doubles every 36 months?
The doctors presenting specialized in the various categories of blood cancers:
- Leukemias (acute, chronic, and myeloproliferative neoplasms -- ET, PV, MF)
- Lymphomas (Hodgkins and non Hodgkins)
- Myeloma (MDS, others)
Blood is an "organ" made up of both cells and liquid plasma.
Dr. Joseph Mikhael provided the analogy of the 3 types of wine (red/white/rose).
The Red Cells carry Oxygen (like trucks, they pick up oxygen from the lungs and deliver to the organs).
The White Cells are the immune system (like an army, they protect the body from invaders)
The Platelets help with clotting (like an ambulance -- they are very social, first responders to the scene to begin clots to stop the damage).
Blood is created in the bone marrow (the factory) from stem cells. If the factory is occupied with disease, then all the cell lines may be affected. If the factory is busy making one kind of cell (in my case with Polycythemia Vera -- red blood cells), the others will not be made in adequate amounts.
Problems with the blood occur when the bone marrow ceases to function properly -- either decrease in production or over production of cells, and when collections of cells occur in the lymph nodes, spleen, or other organs.
Why are these blood diseases considered the big "C" word - Cancer?
Cancer is defined as identical, uncontrolled cell growth.
When the body's normal balance that allows cells to grow in the right amounts for the right period of time is disrupted, cancers grow. This includes solid tissue (i.e., breast, colon, lung) and blood cells.
The "double whammy" of blood cancers is that they are the cells meant to protect you. He used the analogy of a crime committed by a citizen vs. a crime committed by a law enforcement officer.
What caused the Polycythemia Vera?
I didn't get any satisfactory answers. We know that I have tested JAK-2 positive -- meaning that the JAK-2 protein has mutated which tells the stem cells to over-produce Red Blood Cells. But we don't know why the mutation occurred.
Almost all blood cancers have no known cause. Possible contributors include the environment, diet, stress, smoking, toxins, and radiation.
Management of MPNs
The standard protocols for managing Essential Thrombocythemia, Polycythemia Vera, and Myelofibrosis (the main MPNs) remain phlebotomies, Hydroxyurea, Interferon, and Pegasys (pegylated interferon).
Given the options available, I am satisfied with my current course of Hydroxyurea. The daily interferon shots provide flu-like symptoms (not an option for me) and the Pegasys shots are done weekly and are very expensive. I am not yet in a mental place where I can give myself injections and don't want to ask that of my family at this time.
Because these cancers are so rare, the big drug companies don't see a big enough market for much R&D. That is why the treatments are adapted with drugs that have more generalized effects (and additional side effects).
There are some clinical trials with new medicines that appear to work at the molecular level on the JAK-2 protein. Not viewed as a "cure" but as a better management opportunity. These trials are being conducted with patients with Myelofibrosis (MF), as that is the next progression of the disease process. There is some promise that a couple of drugs may make it to market in the next 3-5 years for MF, and then could be demonstrated valuable for patients with PV.
I had the opportunity to speak with Dr. Claire Harrison, Consultant Haematologist with Guy's and St. Thomas' Hospital in London, England. She specializes in Polycythemia Vera and provided some great information. Among her PV patients, she has one who also has Behcet's Disease. She said that the fatigue is unquestioned given the PV, Alphathalassemia Trait, and Behcet's. She encouraged me to walk and exercise as much as I can to keep my heart and bones as strong as possible. The steroids for Behcet's make me a jittery tired person, so I need to be mindful of that.
Dr. Harrison's main presentations were on patient adherence to their medication protocols. She pointed out many myths we hold regarding which patients will adhere to their medication regime over time and why.
Specifically, those of us with chronic diseases who require long-term use of medications -- we can get tired of "taking all these meds" and if we feel okay, decide that we will stop taking them for awhile. We have our own underlying belief system about drugs and we get inputs from people all around us.
Dr. Brent Bauer of Mayo Clinic presented information on Complementary & Alternative Medicine.
He said that since most cancer patients use some CAMs, the physicians need to take that into account and discuss with their patients. While some can be dangerous, many others are helpful with Quality of Life issues such as stress management, fatigue, mental focus. Among those cited as helpful to patients:
- Art therapy
- Massage therapy
- Music therapy
- Prayer, spiritual practices
- T'ai Chi
In fact, Mayo offers acupuncture, Massage, Meditation, Music therapy, and Yoga because the science has supported their efficacy.
Other CAMs like herbal remedies (like mistletoe, cat's claw, and sangre de drago) have been shown in studies to actually cause proliferation of the blood cancers.
Isn't it amazing how many people offer their non-medical advice? Here's a few that have been shared with me:
- A friend of a friend tried this herbal remedy and is completely cured!
- It's all in your head -- read The Secret and you can call the cure to your body!
- Try this special vitamin -- it's guaranteed to work.
- Pouring all those dangerous chemicals in your body is bound to make you worse, not better.
While we may politely dismiss these well-meaning suggestions, a veil of doubt settles into the subconscious. After all, I secretly want to will this illness away and return to my former self.
One thing I hear from many other MPN patients is the issue of fatigue. Many doctors dismiss this symptom, leaving the patients to feel dis-believed and unsupported. Dr. Mesa offered that most doctors don't ask about fatigue because they don't have a solution. Doctors like questions for which they have answers!
He spoke about VO2 max and studies that demonstrate the burden of fatigue and quality of life with blood cancers. He also gently reminded us of the vicious cycle we must work out from under:
I'm tired and don't feel like exercising --> don't exercise --> lose muscle mass, lung capacity --> susceptible to other illness --> feel more tired. lather, rinse, repeat.
If we keep moving, we are less likely to lose ground with VO2 max, will heal quicker from regular occurring illnesses, and are less likely to get pneumonia. Our goals are to optimize flexibility, heart & lung function, and muscle tone. These will help us improve strength, improve sleep, and reduce fatigue. He suggested we try walking, weights or other resistance work, and yoga.
He reminded us that those with PV and ET are at risk of heart attack and stroke -- so we need to get moving!
One of my favorite quotes from the session:
"Research shows that optimism has many health benefits. It can boost your immune function, reduce stress, lower blood pressure, relieve pain, and improve sleep. Being optimistic also may help you feel more creative, make better decisions, and communicate more effectively."
-- Teresa Pipe, Director of Nursing Research at Mayo Clinic, Arizona
Coming to Terms with the Illness
C.S. Lewis wrote: "You do not have a soul. You ARE a soul. You have a body."
Dali Lama: "We cannot give what we do not have."
Monday, April 12, 2010
After sleeping in a bit, Robert, Katrina, and Alexander took the train to Versailles. Since I had visited Versailles with Robert 20 years ago, I didn't feel the need to go again (how much does the history change, after all?).
Through the magic of the internet, I reconnected with a college friend, Eric Stevenson, who lives in Paris (and recently got dual US/French citizenship). Eric was the student body president at the University of Arizona back in the day and he is simply one of the most hospitable, kind, and interesting people I've ever met. He is planning his second trip around the world, inspired largely through his experience as a passenger on the US Airways plane that Captain Sully successfully landed on the Hudson River. Photographs of him on the left wing of the plane and media interviews were everywhere.
I met up with Eric at Le Tuillerie Jardin and caught up on 25 years of living. We had a very Parisian lunch and traveled into some multi-cultural neighborhoods. The people of Paris are quite friendly, contrary to some stereotypes perpetuated in the U.S. Eric pointed out that the custom in France is to greet someone with "bon jour" before diving into a request, question, or statement. American visitors often launch into their question or request without saying hello. That throws the locals off, understandably. I saw it occur many times once Eric pointed this out to me. WE, the Americans, are often unintentionally rude. Imagine that!
Robert and the kids caught up with us in the late afternoon and we had a happy hour in Eric's wonderful home on the 25th floor near the Seine. The skyline views were breathtaking. Eric shared binoculars with the kids and challenged them to identify all the major features of Paris.
We then went to a creperie where we enjoyed savory crepes or dinner followed by dessert crepes. The kids were in heaven and we had fun all around.
That night, we packed up and prepared for a quick flight to Madrid. We are so excited to see my sister Chrisa, her husband Jaime Valero, and their beautiful children. Bon soir!
To physicians, I say "Never underestimate the power of your intentional listening. It may be the relief your patient needs when there is no cure for what ails them."
I recently had two such pleasant experiences in one week with doctors I had not met before. After 20 days of a Behcet's ulcer that was proving to be more resilient than its host (me), I got an emergency appointment with Dr. Giardina, a gynecologist with Kaiser Permanente who was kind and sympathetic. He admitted he hadn't seen a Behcet's case before, but he was familiar with the disease (that it is an auto-immune disease and very rare). He provided some pain relieving creme and antibiotic ointment to tide me over until my appointment with a rheumatologist two days later. It's the same "good stuff" they use on baby boys when they are circumcised.
That Thursday, I met with Dr. Andres Salazar, a rheumatologist with Kaiser Permanente. Fortunately, he is familiar with Behcet's Disease and reviewed my case before he entered the exam room.
[side note: This was an important visit for me because not only was it my first appointment with him, but my family was leaving for a Spring Break holiday the next day. My husband and I weren't sure if I would be able to make the long trip comfortably since the Behcet's wasn't healing. I was prepared to venture on, but dear hubby wanted the doc's opinion.]
I took prednisone daily for the first year to manage the flare-ups, but the side effects proved hazardous to my physical, emotional, mental, and marital health. Since I stopped taking the prednisone in January, the flares have been more frequent and take longer to heal. This, he says, further confirms the Behcet's diagnosis. In addition, I now have arthritis in my hands, hips, and ankles. All I need is the dowager hump and I'll be walking like Quasimodo!
Dr. Salazar also explained that the meds that are most effective for Behcet's also affect the bone marrow. Since the Polycythemia Vera is a bone marrow malignancy, we need to tread lightly on this. He is going to confer with Dr. Kondapaneni, my hem/onc about the next course of treatment.
I am just one year into the disease, so there is still a good deal to learn about which of my systems are affected by Behcet's. He ordered a lot more blood work and we'll reconvene in a few weeks. I've also got to go to an ophthalmalogist to be checked out for uveitis; while I doubt there is any involvement in the eyes, I do have some vision issues, so we need to check it out.
Did either of these doctors solve my problem? No. But they listened to my concerns (I felt heard), recognized the illness (I'm not crazy, making this up), acknowledged and treated the pain (they understood this hurts like hell), and continued probing for more information by asking me questions and doing more lab work (willing to gather more information to determine next course of treatment).
I left my appointment with Dr. Salazar feeling better than I had felt in weeks simply because he displayed competence and thoughtfulness during my appointment.
Living with chronic illnesses can be a part-time or full-time job (depending on the type and severity). We get to live with the symptoms, manage multiple medications & their side effects, endure the funky things the illnesses throw our way, go to doctor's appointments, lab visits, deal with insurance companies, etc.
When we are fortunate to find physicians who treat the patient and his/her illness with attentiveness and compassion, it's amazing how much better we feel.
In my daily practice of gratitude, I am thankful for medical professionals who remember that their patients are people -- with names, lives, families, histories, hopes, and desires. It's challenging, I'm sure. Doctors don't see us patients when we are at our best. And they have a host of pressures outside their 15-20 minute appointment with each patient.
When you work with physicians or other medical professionals who are competent and kind, let them know you appreciate them. Don't we all like to feel the love?
Thursday, April 08, 2010
While we´ve tried to be intentional with this all along, the urgency has increased since my close call with the long dirt nap in October, 2007. Now, living with polycythemia vera and Behcet´s disease, I am reminded every day that each healthy moment is a gift that should not be wasted.
Robert cashes in frequent flyer miles and hotel points for our spring break trips. This is a nice family perq for a job that requires about 50% travel. We want to expose K & A to the big, beautiful world beyond their hometown. So off to Paris and Madrid we go!
Alex wants to spend his Euros on croissants and Katrina wants to eat crepes every day (she would also like a pair of fancy stilettos, but we´ll have to see about that).
We arrived in Paris on Saturday, April 3rd and took a taxi to the Hilton Arc de Triomphe. None of us slept much on the plane, so we took a nap before heading out to explore the town. It was a rainy day, yet the streets were full of people.
Here we are at the Arc de Triomphe. We quickly decided that the Eiffel Tower would be our ¨where´s Waldo¨ of Paris. We took pics every time we could see the Eiffel Tower in the background.
Saturday, March 20, 2010
The initial report from the Bone Marrow Biopsy seems to confirm most of what we already know about my condition, with a few more details and hundred dollar words. The summary diagnosis includes:
- The bone marrow core is hypercellular with estimated cellularity approaching 90 with trilineage hyperplasia.
- Blood shows leukocytosis and erythrocytosis.
- No significant increase in myelofibrosis. (This is GREAT news. YEAYYY!!!)
- Iron stores absent. (helps explain the fatigue)
- Hypochromic microcytic anemia. (helps explain the fatigue)
The myeloid to erythroid ratio is 1:2. There does appear to be progressive maturation of the myeloid, erythroid, and megakaryocytic cell lines. The megakaryocytes are increased. There is hypolobation of the nucleus. Red cells and granulocytic cells also appear to be increased. However, there appears to be progressive maturation without a significant increase in blast forms.
The blood shows leukocytosis and erythrocytosis; however the MCV is low and the RDW is high. The red cell line shows anisocytosis and is hypochromic (low iron).
The microcytic anemia could be due to the alpha thalassemia and/or iron deficiency.
Bone Marrow Item: Units Reference Range
Blasts 4.2 (H) (0.1 – 1.7)
Bands 6.8 (L) (9.4 – 15.4)
Rubriblasts 3.0 (H) (0.1 – 1.1)
Prorubricytes 6.0 (H) (0.4 – 2.4)
Rubricytes 35.2 (H) (13.1 – 30.1)
Metarubricytes 4.2 (H) (0.3 – 3.7)
Total Erythroid 48.4 (H) (15.0 – 36.2)
Lymphocytes 8.3 (L) (8.6 – 23.8)
15.0 (L) (19.0 – 45.0)
M/E Ratio 0.8:1 (L) (1.1 – 3.5)
Other tests take longer to process, so we'll know more later.
I'm back on the Hydroxyurea (HU) now to keep the hematocrit level under control.
The fatigue continues, and my optimism is on the rise. I'm certainly interested in hearing from other MPNers who will share their thoughts on these test results.
Blessings and peace to you,
Friday, March 05, 2010
Thursday, February 25, 2010
Here's a little ditty I wrote to the tune of "Tomorrow" from Annie the musical.
Bet your bony bottom
Won't be fun
Crunching sounds and
Hope I'm numb
When I'm stuck in the rump
Feel the thump
will tell me to hang on
Bone marrow! Bone marrow!
I love ya, bone marrow
Sunday, January 24, 2010
The Style section of The Washington Post runs a weekly humor contest called the "Style Invitational." (www.washingtonpost.com) Each week is a new challenge. One of the most famous is neologisms create new words). The instructions: Alter any word by adding, subtracting, or changing one letter; then supply a new definition. Here are some winners:
14. Dopeler effect: The tendency of stupid ideas to seem smarter whenn they come at you rapidly.
15. Arachnoleptic fit (n.): The frantic dance performed just after you've accidentally walked through a spider web.
16. Beelzebug (n.): Satan in the form of a mosquito, that gets into your bedroom at three in the morning and cannot be cast out.
17. Caterpallor ( n.): The color you turn after finding half a worm in the fruit you're eating.
NEW DEFINITIONS: Alternate meanings to real words.
2. flabbergasted (adj.): appalled by discovering how much weight one has gained.
3. abdicate (v.): to give up all hope of ever having a flat stomach.
4. esplanade (v.): to attempt an explanation while drunk.
5. willy-nilly (adj.): impotent.
6. negligent (adj.): absentmindedly answering the door when wearing only a nightgown.
7. lymph (v.): to walk with a lisp.
8. gargoyle (n.): olive-flavored mouthwash.
9. flatulence (n.): emergency vehicle that picks up someone who has been run over by a steamroller.
10. balderdash (n.): a rapidly receding hairline.
11. testicle (n.): a humorous question on an exam.
12. rectitude (n.): the formal, dignified bearing adopted by proctologists.
13. pokemon (n.): a Rastafarian proctologist.
14. oyster (n.): a person who sprinkles his conversation with Yiddishisms.
15. circumvent (n.): an opening in the front of boxer shorts worn by Jewish men.