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

When Mom's Chemo Brain meets Teenage Brain

May should be named National Stress Month.  
For adults who live with chronic illness AND have school-age kids in the house, it becomes crazy time.
Your calendar is not your own.  Every note or email from the school contains an important deadline or event that must be tended to immediately.  The best laid plans can be turned upside down with one announcement.

My dear kids (ages 17 and 16)  are in high school, so May is the time for AP exams, End of Course Tests, final exams, and final projects.  There are also sports tournaments, awards banquets, honors night, musical production, and final meetings of various school clubs.  Thank you's for all their teachers and coaches all must be purchased and readied for the last day of school.  

Granted, my kids did all the heavy-lifting.  They are bright, talented young people and put in the effort to achieve their successes.  They know that they are responsible for learning about all their deadlines and managing their time appropriately.   Even with periodic reminders, tasks are forgotten until the last minute.  Then panic sets in.  It is predictable.  I shouldn't be surprised.  I shouldn't take it personally.  They have teenage brains.

What Exactly is a "Teenage Brain"?   
Recent neuroscience research shows that the human brain undergoes a massive overhaul between the ages of 12 and 25 years.  It's like a massive re-wiring and new network made up of axons and dendrites and neurons.  The connectivity and insulation improves, enabling the brain to process information quicker, recall old information, and influence the decision-making process.

At the same time, the brain matures first in the back part of the brain (responsible for basic functions like movement, vision, basic processing) then to the top and front regions (which handle more complicated functions like memory, decision making, planning, and creativity).    All these changes make for an awkward time for teens and the adults who love them.  Teens have their moments of mature reasoning coupled with impulses, self-interest, emotional outbursts, and attraction to risky behaviors.

Teenage brains are also most susceptible to brain chemicals like dopamine (a neurotransmitter that helps in learning patterns and making decisions) and oxytocin (which makes social connections more rewarding).   The teens/twenty-somethings can assess risks as well as adults.  Yet they often value the perceived rewards higher than do adults.  

Who are you and what have you done with my sweet child?
What the heck were you thinking?
What do you mean you don't remember?

When the teen answers, "I don't know," she speaks her truth.
That's when I have to take a deep breath.

What is Chemo Brain?
Patients will likely give you a different answer than many physicians.  Because it hasn't been extensively studied, some doctors are reluctant to give the phenomena much credence.

Persons who are undergoing certain chemo therapies for cancers, seizures, or migraines may experience memory lapses of routine items; difficulty recalling names, places, or dates;  difficulty in concentrating on a task or conversation;  difficulty in multi-tasking;  becoming easily distracted.

We typically realize something is wrong before others around us notice, and we try to compensate in various ways.  For example, I made sure EVERYTHING was put on my calendar on my computer and phone.  The problem was that I would forget to check the calendar every night to plan for the next day.  I missed appointments simply because I didn't look at my calendar.   I went to the pharmacy to pick up medicine that I had picked up the day before.  This is completely frustrating and demoralizing.

My dear kids know that mom's brain doesn't function as well as it used to (beyond the usual 'getting older' forgetfulness).  Whether my forgetfulness, distractedness, and inability to multi-task are due to the chronic illnesses or the medications, it doesn't really matter.  I need the medications to remain as healthy as possible.  Some days or weeks I have much more clarity of thought and function than others;  I feel much more like my true self.  But when the subtle changes occur and I forget important things, we call it "Foggy Brain" or "Chemo Brain."   That's their cue.

When Teenage Brain meets Chemo Brain, the stress level rises.
The teens know if they need something from me, it must be written on the family calendar in the kitchen.  Telling me something once in passing or when I'm about to go to bed does not count.  
Still, they rarely remember to write it down.  I shouldn't be surprised;  they don't remember where they put the car keys, the important notebook with their final project, or their ballet/tennis/running shoes.
Side note:  Apparently moms are expected to have the "Find It" gene;  when mom doesn't know where something is that she doesn't use or hasn't seen, dear teen gets indignant.  Really?!

So this May, a few things fell through the cracks of our fractured organization system.   They got frustrated when I didn't remind them of something and I got frustrated when they told me about something at the last minute.   When the stress got too much and a Behcet's flare and gout attack made me slow down, we all managed to stop, take a breath, and work as a team.

Teenage brains are very capable of compassion for others when it's most needed.  And a healthy sense of humor helps, too.

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.