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.

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!

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!

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

Behcet's Disease Awareness Day --  May 20th

If you don't know about Behcet's, consider yourself lucky!

Learn more here:

American Behcet's Disease Association

Info on Behcet's Disease

The major BD symptoms include:  ulcers in the mouth, ulcers/lesions on the genitals, eye inflammation, arthritis, skin rash/acne.  

Other symptoms may include: skin lesions, bowel/intestinal inflammation and lesions, fatigue, meningitis, and cranial nerve palsies.  

Imagine waking up one morning with a small spot inside your mouth that feels a bit tingly (not the pleasurable sensation).  From the tingle an ulcer emerges.  It grows from a small dot to the size of a dime.  And the sore is painful when touched by food, a tooth, etc.  Often when one ulcer erupts, another is also in process.  The tongue, inside the cheeks, lower and upper lips, and gums are all favorite places for these pesky and painful sores.  Along with the sores, the inside of the mouth often becomes inflamed.  These are different from canker sores, which typically occur on the lips or outside the mouth.  


Whether there is one ulcer or a mouthful, the experience is quite painful.  It is painful to talk, chew, and lay your head down on a pillow.  Without treatment (typically the steroid prednisone), the ulcers grow and linger for three to four weeks.   I've gone through several tubes of Ora-jel and lots of "magic mouthwash"to numb the sores.  

   

But wait... it gets better:  imagine the same tingle or itch in the genital area.  The ulcers are particularly painful for women and men.  Sitting, walking, and urinating are painful when Behcet's erupts "down there."  Orajel helps numb these sores, and lidocaine/prilocaine work much better.   


During a flare, it is important to drink a lot of water and remain properly hydrated.  The natural tendency is to reduce drinking to reduce the need to eliminate (which is quite painful).


Many Behcet's patients also experience uveitis (inflammation in the eye).   I am grateful that my eyes have not been affected.  

Why Haven't You Heard of BD?
Behcet's (pronounced "beh-chet'") Disease is a rare, chronic inflammatory disorder.  The disease was named after the Turkish doctor who identified this illness, Dr. Hulusi Behcet.

Behcet's is relatively rare in the United States and Europe.  It's prevalence is 0.3 - 6.6 cases per 100,000 population.    It is estimated that there are approximately 15,000 people in the US living with Behcet's.

It is most prevalent in the Mediterranean region, the Middle East, and Far East (China and Japan).  It occurs there in approximately 1 in 10,000 people. Behcet's Disease is often called the "Silk Road Disease."

Considered an auto-immune disease, there are no clear answers as to what causes the body to attack itself in this way.  While it is associated with HLA-B51 allele, genetic testing has not progressed sufficiently to be conclusive.

Why is it difficult to diagnose? 
Several of the symptoms are seen in other diseases.  Other causes must be excluded, such as virus, bacterial, or sexually transmitted infections. There is not yet any identified protein, gene mutation, or other element that can easily confirm the diagnosis.

In addition, because there are so few cases in the US, most rheumatologists and other specialists do not have much experience with Behcet's.

The first rheumatologist I saw said he doubted I had Behcet's Disease because in his 25 years of practice he had only 5 patients with Behcet's Disease.  He knew that I also have Polycythemia Vera (a Myeloproliferative Neoplasm) which is also rare.  "It's highly unlikely one person would have such a constellation of rare diseases."  I decided that I needed a doctor with more experience treating the disease.


I am very fortunate to have found Dr. Andres Salazar with Kaiser Permanente in Georgia.  He did his Rheumatology Fellowship at Emory University Hospital & Emory Clinic under Dr. Jonathan Waltuck.  Dr. Waltuck has Behcet's patients and Dr. Salazar became familiar with this rare disease and various treatments.

The Behcet's ulcers made their presence known in me in the Fall of 2009.  In December, 2009 the ulcers were so plentiful and debilitatingly painful that I stayed home while my family went on a much-anticipated ski vacation.  While the family enjoyed the slopes, I got to meet an infectious disease doctor.
After ruling out all types of infections, he believed that I had Behcet's and referred me to a rheumatologist.
I was treated with prednisone, a corticosteroid, to hasten the healing.

For one year I took prednisone daily, varying the dosage to minimize the ulcer flares and shorten the duration when a flare occurred.  The side effects, however, were absolutely terrible.  Not only did I gain 40 pounds, I had insomnia, became sleep deprived, was very cranky, and forgot many important things.  All this combined made it impossible for me to work reliably.  I didn't like myself nor anyone else.

In January, 2011 I tapered off the prednisone and begged my doctors to let us try something else.

Now I get Remicade infusions every six weeks and take prednisone at the first sign of a BD flare.
Remicade (infliximib) is an immuno-suppresant drug used to treat rheumatoid and psoriatic arthritis and Crohn's disease.  While it can lose its effectiveness on some patients, I'm hopeful this will provide relief for as long as possible.  Some of the other treatment options are not available to me because of the polycythemia vera (my bone marrow over-produces red blood cells).

So far, so good.

Rare Disease Day - Around the World

On the last day of February, people around the world living with rare diseases or disorders shine a light on the rare diseases they deal with every day.

We see pink ribbons on everything from car magnets to KFC buckets (yes, really!) to increase awareness about breast cancer.   Rubber wristbands became the rage after the "Live Strong" campaign from Lance Armstrong's foundation got media attention.  

Rare diseases do not get the same attention - or funding - because they are, well, rare.  Without enough patients to comprise a big enough "market" it is difficult to get a strong message heard amidst all the chatter.   Did I mention that it's also difficult for people with the same rare condition to find each other, much less gather the energy and resources to organize huge public awareness campaigns?

The beauty of Rare Disease Day is that it opens up opportunities for new conversations at all levels around the world.  This is the fourth year of RDD in the United States.

So what makes a disease or disorder "rare"?  
The US definition is a disease or disorder that affects fewer than 200,000 people (as defined by the Orphan Drug Act of 1983).   With this definition, there are approximately 7,000 rare diseases active in the US.  Almost 30 million Americans (10%) are living with a rare disease.

The European definition of a "rare disease or disorder" is one that affects fewer than 1 in 2,000 people.

Rare diseases/disorders are often life-threatening or chronically debilitating.  Many of these diseases tend to change over time, degenerating the patient's quality of life and ability to work, live independently, and enjoy many of life's simple pleasures.   Some people are asymptomatic for years before the disease progresses.

Rare diseases/disorders are difficult to diagnose because they share symptoms common to other ailments and the symptoms also may vary from one patient to another.   Many patients are first misdiagnosed and live with inadequate treatment until the true disease state is discovered.

"Rare, but Strong Together"

My diagnosis of Polycythemia Vera, a Myeloproliferative Neoplasm (MPN), was given in the fall of 2009 -- two years AFTER the triple vein thromboses which almost took my life.  At the time of the thromboses and ensuing ischemic bowel, peritonitis, and organ distress, I was diagnosed with Factor XII and Protein C deficiencies.   It took another "close call" event to occur before I received the full diagnosis and began appropriate treatment in 2009.

The diagnosis of Behcet's Disease, an auto-immune illness, was made after a lot of pain and many tests by my internist, gynecologist, and infectious disease doctor in 2009-2010.  The first rheumatologist said he didn't believe one patient could have two rare diseases.  This one continues to be a real roller coaster, but my rheumatologist is excellent about keeping up on the latest (albeit limited) literature, studies, and practices concerning Behcet's.

What's a NORD?
I encourage anyone with a Rare Disease to register with the National Organization for Rare Diseases (NORD) or EURORDIS (Rare Diseases in Europe).   When you register, you can opt to be notified to participate in on-line surveys, clinical trials, and learn when new findings about the disease are made available.  It's free and it's a way to contribute to better science.

National Organization for Rare Diseases

Top Ten Travel Tips for Folks with Chronic Illness

I learn something new each time I venture out with Polly and Behcet’s.  While I get the “You don’t look sick” comment all the time, I’ve learned that the physical limitations are real.  If I don’t listen to my body and pace myself, I will pay for it handsomely with several days in bed or worse.

At the same time, it is important for many of us (especially those with children) to keep up strong appearances as much as possible.  Kids are in tune with our actions even more than what we say.  “Oh, I’m great, really!” doesn’t convince my teenagers anymore.   But if I get out of the house and cheer him on at tennis or volunteer with my daughter’s theatre production, then they feel more certain.

TIP #1:     Rest up A LOT before a trip.  Seriously!  Lay low;  essential errands only.   Let your body get as calm as possible before upsetting it with travel, time zone changes, new foods, beverages, etc.

TIP #2:     Take LOTS of photos, no matter how long you are out and about.  Take pictures of cool buildings, crowds, bridges, flowers, street signs, billboards, people… whatever you see.  They don’t all have to go in a photo album (does anyone make these anymore?).  If you are a facebooker or email your friends, share the pics.  It gives the impression you are out and about a lot longer than you actually are.
I did this during a 3 day trip to Venice and people thought I was all over the place.  The truth?  I had one meal each day and spent 6 hours one day “touristing.”   Probably spent a total of 12 hours “out and about” over the 3 days, but no one would have guessed from all the photos I shared on facebook.

TIP #3:     Don’t be shy about using a cane or other assistive device.  There are a lot of good looking canes and walking sticks on the market now, so don’t be shy!  It serves many purposes:
1.   Gives you something reliable to lean on when covering unfamiliar areas, especially stairs and cobblestone areas.
2.   Enables you to stay out longer.
3.   Keeps your hands off public railings and other things that people touch with who-knows-what.
4.   Signals others that you may need a bit of patience or kindness.
I used my cane in Venice when I did the walk-about with some of the other wives and simply explained that I have chronic conditions that make me fatigued and a bit unsteady;  the cane helps me keep up with everyone else.  I know I wouldn’t have lasted the 6 hour day without it.

TIP #4:     Establish an Opt-In Policy with your travel companions.   Set the expectation that you are content to hang out at or near the hotel while your family/friends do major sightseeing.  You will go with them when you feel like it.  This takes pressure off of everyone.  The others don’t have to limit their plans (e.g., that might take too long and mom will need a rest;  can’t do that because mom can’t climb to the top of the lighthouse).   It also takes pressure off of you from having to ‘suck it up and go’ when you really don’t feel good enough.
When we planned our spring break trip, we explained to the kids that mom is not going to go on many of the adventures.  In fact, we’ll assume that she is NOT going unless she tells us that she IS going to join us.   I get to hear all their stories when we connect at the end of the day – sometimes we meet at a restaurant, sometimes they come back to the hotel.

TIP #5:     Be adventurous on your own!   While the others are out and about, you need not stay in your hotel room.  Get some fresh air and wander outside a bit.  Find a café or park nearby and do some good people-watching.
I love to see people in their natural habitat.  Each new city is like a human zoo to me.  Do they make eye contact / smile / greet passersby?  Are they taking in their environment or wrapped up in their own mental worlds?  Do they smoke?  Do they litter?  Do they spit on the sidewalk?  Are they talking on cellphones or bluetooths?  Do they eat while walking or stop and eat?  Are there a lot of dogs on leashes?  Small dogs or large?   Are there babies in strollers – pushed by moms or nannies?  What type of shoes do the men and women wear on the street?  What are the locals wearing versus the tourists – can you tell the difference?     Is there graffiti?  Posters on walls – for entertainment, political statements, advertisements?

TIP #6:     Plan ahead and check out excursions or sites that are easily accessible.   You may inquire:  are there benches to sit?  Can I get water if needed?  Are there ramps or elevators instead of stairs?  The internet is a wonderful resource (and so is your public library);  you can find out about interesting places to visit that are accessible to you ahead of your arrival.  You may choose to see some things while your companions do other adventures.  It will provide great dinner conversation later!

TIP #7:     Keep some of each of your medications with you at all times.  Don’t leave all them at the hotel/cruise ship or wherever you are staying at night.  You never know what might lead you off the planned schedule, and it is good to have them handy.  This includes a pain reliever and bandages for foot blisters or minor cuts (just in case).

TIP #8:     Keep a list of all your medications, dosages, and times of day for each in your wallet (and near your passport).   This is important for those who travel with you.  Should something happen to you, the handy list will relieve some stress of trying to recall all your meds.

TIP #9:     Keep a list of the diseases, ailments, ALLERGIES, health issues in your wallet (with your medicine information).   It is helpful for emergency personnel to know if you are diabetic, hypertensive, on blood thinner, allergic to medications, etc.

TIP #10:   Bring healthy snacks with you.  Particularly if you have food allergies (like gluten, nuts, lactose, etc.), it is wise to bring some dry food bars and snacks that you can access if the right kind of meal isn’t available when you are hungry.
I find that breakfast bars are my salvation.  I need to eat something with my morning medication cocktail, and I’m hardly “dining room ready” when I take the meds.  Those breakfast bars come in very handy!

Please share your favorite tips and I'll add them to the list.  Safe travels!

Cheers!

Here are some more tips from fellow MPN and Behcet's Travelers:

TIP #11:    Remember to bring your physician contact information with you, along with your health insurance information.

TIP # 12:   Keep a set of all your medical information (Tips 8, 9, & 11) together for easy reference.  Keep it in an envelope marked "Jane Doe's Medical Information" along with a copy of your passport, drivers license or other photo identification.  Again, if you are this organized, you will most likely never need to share this information.  But imagine if something happened to you... having all your info in one handy place would make treatment so much smoother!

TIP #13:   Group tours are a great way to travel.  Once you choose a destination, look into tour packages.  Someone else plans the itinerary and arranges the logistics, you can ride in comfortable buses and learn from knowledgeable tour guides.  

TIP #14:   Pack spare underwear in your carry-on bag.  You never know when you and your luggage will be separated for a day or two.  While you can live in the same street clothes for more than a day, a fresh pair of undies makes life a bit more pleasant.

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.  

The Beauty of a Competent, Caring Physician

My kids have heard me say many times, "Never underestimate the power of your smile.  It may be the one nice thing someone receives today."

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?