For these, I am thankful

For trust & hope

For Joy
And the beauty in each day

For Larry

Who has wooed me

with lilies

For Retreats
On Star and Appledore
& New Harbor, Maine

For Music
That soothes
comforts
lifts my spirit
opens my heart
moves my body

For Books
To fall into
fall in love with
& read again
& some again

A Good Samaratin? I guess it depends on your perspective

Strange weather in New Hampshire. The the northern tier -- what we call the 'North Country', is under a flood watch this evening. But a "heavy snow" warning begins for them at 4 AM through to 9 PM on Friday. Such is the diverse beauty of New England.

On the coast today, it was dark and gloomy with frequent and periodic heavy rain most of the day. Temperatures hovered close to the 60 degree mark -- a little warm for the season.

I had a salon appointment after work and arrived just in time for another burst of hard rains. Parking as close to the door as possible, I opened my umbrella and made for the door.

A young mom was walking toward me from the mall pushing a carriage with a toddler. She had no cover from the rain.

I offered to cover her with my umbrella and stay with her as she walked to her automobile.

A pretty ordinary scenario? Don't you think?

Well, the young woman only reluctantly allowed my assistance. I was prepared to stay with her and cover the child while she opened the doors and lifted the child into the car. But I was quite summarily dismissed. And I realized only later as I walked away that she was suspicious of the assistance.

So I wondered: what must your life be like if you cannot trust a 5 foot, 63 year old grandmother, who looks like this:

Oh. You caught me. I wasn't wearing my crown.

Behcet's can be lonely place

I don't know one single person who shares this malady.

In fact, its prevalence in the US is about 1 in half a million people.

Tonight, however, I joined an internet 'chat' in-progress, for people with BD and very quickly felt affirmed and less lonely.

In truth, there were less than 10 people involved in the 'chat' but those ten represented an amazing geography: one participant is from Greece; the moderator is from Northern Ireland; and several others are from the New York, Virginia, New Jersey and I think Wisconsin; and there were a few others whose location I didn't learn.

'Chat' participants were warm and welcoming. Not unlike what I have come to expect from the CLL Forum and CLLC Friends: generous, friendly, encouraging, intelligent.

One of the interesting aspects of the nearly 2-hour online event was the voluntary translation going on. A woman from New Jersey was translating from English to Greek and Greek to English for the gentleman from Greece.

This was not her job. She is one who shares Behcet's and I feel certain she joined the 'chat' for her own personal reason.

Yet it was just done.

Because it was needed.

Because she was there and had the skills.

I'll Be Seeing You

July 22, 1945

Germany

Dearest,

I am on my way back to camp. I'm almost there . . . . . I've been in (this) place for two days . . . . getting lots of rest and good food.

How are you, darling . . . . And Patty?

As for me, I am in the best of health.

I haven't heard anything about going home but it won't be too long now, I hope.

Write often, darling.

I love you with all my heart.

And hope to see you soon.

Love and Kisses, Bob

PS.
Give Patty a big kiss for me.

I love you, Dot.

Below the body of this short letter is the admonition to:

REPLY BY V-MAIL

They were so young, those WWII warriors. He was only 24 when he wrote this letter. They were married in 1942 but there wasn't much time for them to be together, to know each other. The war was on; he was married in his uniform.

My father and his two brothers, Norman and Richard, fought the 'good war'. His father, Noe Pierre, was a casualty of WWI and spent most of his adult life in and out of veteran's hospitals suffering the effects of mustard gas. Noe was a staunch advocate for returning soldiers of the WWII and worked tirelessly for them when he was able. He died when I was ten but I have such clear memories of him in the lead automobile heading up the Memorial Day Parade in our Massachusetts town. Military and patriotism were important themes in his household.

During the war years, my mother and I lived with my paternal grandparents. I was the first grandchild and doted on and probably spoiled by my mother, grandparents and youngest uncle, Richard, who was still at home when I was born. I'm told that he would gather me into his arms, take me into his bedroom, prop me on pillows and tell all about his latest 'girl' and his date for the evening.

I recall a room that functioned as an office for my grandfather with a large desk and world globe. My mother said that he also had a large map of the world over his desk on which he plotted the advance of the allied forces. He had a bald, shiney head and I loved the way it smelled. I never smelled that fragrance again.

Hormonal Imbalance

We got tickets for the Four Bitchin Babes this Saturday evening at the Music Hall in Portsmouth -- a show we really looked forward to as we hadn't seen them for a few years. Their new show called, Hormonal Imbalance, was a near-sell-out. By the time we decided to go there were only great single seats left. We took the singles reasoning that after 20+ years we could actually be apart that long.
Hurrican Noel was rushing up the east coast from North Carolina on its way to the Canadien Maritimes. Reports and storm warnings called for all-day rain, high winds and rough seas. It was a blustery night but by the time we were leaving for the show, the storm had been down-graded to a tropical storm (another indication of global warming? seems late for us to be getting a tropical storm!)

I felt it was a great night to curl up with a book and a cup of tea rather than brave the wind and rain from parking garage to Music Hall. But we had already paid for the tickets; we went to the show which was, indeed, a full house with a pleasantly appreciative audience (mainly women).

This foursome is not the original Four Babes; only Sally Fingerett (upper left in the photo) was an original Babe with Christine Lavin, Patty Larkin -- and one other, whose name escapes me at the moment. But these four babes can sing. And write. They are called 'cross-over folk' artists. Their harmony is supreme. They give funny, poignant, heartfelt, high-energy performances.

The Babes gave a nice nod to the men in the audience giving them lots of kudos for bravery! It was a female, night-out for sure.

Their patter and their tunes really poked fun at female angst. One of my favorites was, The Boob Fairy Didn't Come For Me, and poked fun at women who are never satisfied with their hair, face, hips, legs, butts, lips, ears, and-you-name-it-we-probably-hate-it!

Another tune that brought the house down was based on 30,000 pounds of bananas -- but the truck driver in this tune was carrying 30,000 pounds of VIAGRA -- which, on impact, got into the town water supply.

These Babes are on tour together and individually.

I enjoyed the foursome; I would hear each one on her own as well.

Check them out here. You can play their video and listen to tracks from the show's CD.

Life is too short for drama and petty things,
so kiss slowly, laugh insanely, love truly

and forgive quickly.
(Unknown)

Life is too short for drama: I have witnessed argument after argument on the CLL boards (ACOR and the Forum) and I just cannot understand the motivation, the rationale for such behavior. The newest board, the CLLC Friends, was started nearly a year ago because of the defection of several members who had a falling out with other members. Now, another member of the Forum is threatening to leave because she feels her opinions cannot be shared without sarcasm or rancor.

Life is too short for drama and petty things: After all, what is being shared is information ~~ information that we all need to know. Other times what is shared is opinion and/or personal experience. But whatever they are, they are just words on the screen. They can so easily be 'erased' if found offensive, upsetting or frustrating. One has the ability to just walk away in a very real sense. So why not? What does motivate people to harangue over small details and personal opinion or interpretation?

We need to forgive quickly: We, the members, are all the same. Our great equalizer is the disease we share. But that just doesn't seem sufficient of itself to keep people from hurting one another.

Last summer when I was hungry for information about CLL, I found the CLL Forum. Actually, I started looking for information even before I had a diagnosis, when all I had was a phrase (small clonal population in bone marrow and an appointment to see a hematologist in Boston. Recently, I heard someone say they had a degree from Google U. which is my first choice when I want to know something. So, I googled "small clonal population" and up popped a list that included many references to CLL. It was DrM. who gave me the diagnosis; it was also DrM. who advised against using the internet as an information source on CLL saying that the information on the web was "old" and "frightening". I didn't heed his advice.

I quickly became a member of the Forum, a big step for me as I had never done any kind of on-line bulletin board or chat. I lurked awhile and then took the plunge and what a response! I felt wrapped around by the warmth and friendliness of strangers ~ ~ and I learned about my disease, I began to feel less alone. Over the months, I saw and experienced the wonderful generosity of spirit as members shared their personal stories, offered advice and made suggestions. There is a great wealth of knowledge among its membership and it freely shared. One feels almost daily to be on the cusp of what is new and current in CLL. The present disagreement is about the efficacy of alternative treatments or holistic interventions and whether there is sufficient science (data) to support their use. Clearly, I just don't understand why we just cannot leave our members to their own discretion. Let those who are interested in such topics expound on them in the thread that is allocated to it. If one is not interested, one needed visit the thread.

At the ACOR site, that is not as easily done. I continue to visit ACOR but I feel less connected to it; it feels somehow less personal. For me, it is not as compelling as the Forum. And, there are a lot of rules about what is kosher to post and this stiffles what could be worthwhile and important discussion. ACOR members seem even more given to argument ... which seem to devolve into free-for-alls as folks weigh-in their opinions.

I continue to be a frequent visitor to ACOR because of the posts by Dr. Terry Hamblin whose great gift of knowledge and experience are generously shared. Now I scan topic headings and authors and read only what is of interest to me at that moment ~~ unless it is an answer by Dr. Hamblin or one of my favorite authors. And then I leave the site. Finish my internet or other computer business and walk away. And get on with my life and the business of staying healthy.

This level of angst about small things just cannot be conducive to a healthy body and mind.

What to do?

If you wish to make anything grow you must understand it

NIGHT GARDENING

A friend recommended

Night Gardening

E.L. Swann, Hyperion, 1999.

E.L. Swann is a pen-name for Kathryn Lasky, well-known children's author.

Well she didn't just recommend it; she brought me her copy saying she just knew I'd love it. IT was given to her by another friend for the same reason. I read it once and just fell into the novel. I reveled in it's power and fell in love with the characters, Maggie and Tristan. I wept.

The novel is just filled to the brim with magical prose and lyrical narrative. For example:

". . . . Kind of half yearning and half -

well, half like he was on the edge of a dream, a very old dream.

"A very old dream," Maggie repeated softly. "How do you mean?"

""I'm not sure. you know how some people spend a life just searching

for something and don't really know what for exactly,

but they know that somewhere out there it is really there,

if they can just get to it.

Well, that is how it was with him."

And now, having finished a second reading, the book is just as satisfying, just as delicious.

I recommend this quietly moving short novel to you.

Surfing the web to find out more about E.L. Swann and the novel, I read that she had the story in mind for a long time and that the catalyst for the story was the "Secret Garden".

The novel's structure is built around quoatations from gardening books. These begin each chapter. Japanese gardens and contemplative gardens are recurrant themes in the citations. The first chapter opens with this quotation which I believe is the very essence of the novel.

If you wish to make anything grow you must understand it,

and you must understand it in a very real sense.

Russel Page, The Education of a Gardener

Maggie Flaherty Welles, only child of Irish immigrants marries into a Boston Brahmin family. She was a bright, bold, red-headed young woman who was completely taken over by the material power of Adams Welles and the entire Welles family. But for all the life and energy that she brings to the marriage, it simmers away in the alcoholic haze of her husband. Now at 61, a widow with one son and a daughter who both carry on their father's failures and weakness for alcohol, she has suffered a stroke that paralyzes her left side. Maggie, who was once an avid gardener, now painfully compares her lifeless body to her derelict garden. She only half-heartedly gives herself to her various therapies and one feels she is retreating from life. A particularly painful moment for Maggie (and for the reader) is her realization that the stroke has caused very little change in her life. In fact, she realizes, her life had been deadened by a kind of paralysis during her long, dry marriage to Adams.

The spark in the novel comes in the shape of Tristan Mallory, a silver-haired landscape gardener who is at work re-building the garden of Maggie's nearest neighbor with whom she shares a garden wall. Unaware, each is watching the other:

"Once an avid gardener, Maggie is intrigued by the activity. Tristan Mallory, the silver-haired, blue-eyed architect, recognizes a kindred soul and returns her interest. Far from being repulsed by her stroke-impaired appearance, Tristan sees Maggie as a person of rare inner beauty. They begin to meet at night to restore Maggie's garden, which has withered from the combination of Maggie's financial and physical limitations. " (Susan Scribner)

It is in their budding relationship that Maggie begins to heal, to feel life moving through her again.

"Night Gardening skillfully portrays a fleeting but powerful romance between these two mature characters . . . . . in its brief pages, Maggie's personality comes alive . . . . (Susan Scribner)

The romance is powerful and all too brief. I wanted it to go on and on.

I N F J People

PERSONALITY TYPING

I was reading the CLL Forum this evening and was intrigued by one of the posts. It was a link to a self-administered an on-line personality test not unlike the Myers-Briggs based in Jungian theory. Several of the members had taken the test and reported their results.

Once, a long time ago, in a professional team-building setting, I was given the test and then promptly forgot the results. Ever since I have been slightly envious of those folks who blithely recite their profile.

The results of tonight's test are: Introvert. Intuitive. Feeler. Judger (INFj)

Life as an Introvert, Intuitive, Feeler, Judger
People of this type tend to be:

creative, original, and independent;

thoughtful, warm, and sensitive;

global thinkers with great passion for their unique vision;

cautious, deliberate, and planful;

organized, productive, and decisive;

reserved and polite.

I have no idea whether and how much the results of a Myers-Briggs test can change over time and if these results are anything like the previous ones.

I found the following at www.personalitytype.com:

"The most important thing to INFJs is their ideas, and being faithful to their vision.

According to this type, Introverted Intuition with Feeling, the direction of my energy is 'internal'; I suposedly put a premium on information from the 'internal' or imaginative world; I tend to make decisions based on emotions; and I organize life events and act according to plans.

More mundane analyses of this type of personality include, among other things:

* not good with money

* dislikes conflict and criticism

* has high expectations of herself and others

* is a perfectionist.

Hope ~~ perches in the soul

HOPE

Hope is the thing with feathers

That perches in the soul,

And sings the tune ~~ without the words,

And never stops at all,

And sweetest in the gale is heard;

And sore must be the storm

That could abash the little bird

That kept so many warm.

I've heard it in the chillest land,

And on the strangest sea;

Yet, never, in extremity,

It asked a crumb of me.

Behcet's 101

Evidence of Behcet's Disease has existed for centuries. But it was not 'discovered' until 1937 when Turkish skin specialist, Professor Hulusi Behcet, published a report describing patients with recurrent mouth ulcers, genital ulcers and eye inflammations that the combination became known as 'Behcet's Disease'. Since this original description, however, people have come to believe that the disease may be associated with many other symptoms (gastrointestinal, pulmonary, cardiac, central nervous system ~~ in fact, wherever inflammation of the blood vessels occurs).

Causes for Behçet's are unknown; it is not known if there is just one cause. Whatever the cause, the result is that affected tissues become inflamed. Small blood vessels, particularly, become inflamed (this is known as vasculitis) and this can cause symptoms in many different parts of the body.

It is possible that a specific virus or bacterium may be the cause of the syndrome, but no specific infection has yet been clearly identified.

Most writers state with certainty that Behçet's is an autoimmune disease in which the immune system attacks the body's own tissues, but for others this is not yet certain; the disease is different from other autoimmune diseases in some aspects.

There is no evidence that Behçet's syndrome can be passed on to other people. It is not associated with cancer, or with a specific diet, or with any particular lifestyle (past or present). Most people with the syndrome will live a normal lifespan.
How common is Behçet's syndrome?
Behçet's syndrome is rare in the UK -- probably about 2,000 people are affected. In the US there may be 15,000 people affected by Behcet's.

The condition is more common in Mediterranean countries, Turkey, the Middle East, Japan and south-east Asia. Because Behçet's syndrome is more common in these regions, the illness has been called the Silk Route Disease, after the ancient trade route for silk which ran through these areas.
Behçet's can occur in most ethnic groups, and it is still unknown to what extent ethnic background increases or reduces risk. Behçet's occurs in males and females, and can affect all age groups, although it usually develops in the 20s and 30s. Some writers/researchers have said that males are more seriously/aggressively affected than males.

It is a long-term (chronic) problem which sometimes becomes less troublesome in later life.

Mouth ulcers occur in 97% of patients with Behcet's.

Genital ulceration and skin lesions occur in 75% or more of patients.

Brain (neurologic) disease occurs in less than 20% of patients.

Inflammation inside the eye occurs in up to 75 % of patients.

Both the small and large blood vessels (the vascular system) can be affected.

People who have vascular involvement are at an increased risk of a blood clot in a vein.

The vessels of the central nervous system brain, spinal cord, or heart may be affected.

Everyone has a story

I love a good story and can't resist developing my own stories about the people I see out and about in the world; I wonder, "What's their story? Are they happy? What are their motivations? Passions? Secrets?"
I love novels; I fall in love with characters and never want good stories to end. I create stories with in my work with paper, fabric, fibers and beads, with ephemera, vintage lace, found objects and books and create 4x6 mailable art, fabric journals and altered books with narrative foundations (www.studionotesandmusings.blogspot.com).

(Left, collage, 4x6 mailable art originally uploaded by Pat Spiller 2005.

True Stories. I have been reading stories about real people living with chronic lymphocytic leukemia (CLL). When I was diagnosed with CLL in 2006, I started surfing the web with a hunger for information about adult-onset leukemia. That was when I discovered the CLL Forum. It was just under a year old and started by people who are living with CLL. There are well over 1000 members scattered around the globe, a brave and hearty community of people generous with their wisdom and support and open and candid with their personal stories.
But lately, I started scouting out information about Behcet's Disease. There doesn't seem to be a lot of first person narratives on the web perhaps due to the fact that so few Americans have this disease but I did find a story written by a woman who tells of a 17-year search for a diagnosis. Her story is more common than you might imagine. And, it echoes my own.


My story is continually evolving since my diagnosis is new but I share a 20-year frustration with that woman of going from doctor to doctor to yet a another specialist and coming up empty handed. For me, the mouth sores came first. They apeared suddenly and painfully, lasted about 3 weeks, disappeared only to make another appearance again the next month. I was around 40 and menopause was suggested as a catalyst. Alergies to food, another. Every doctor I saw had a different theory, a new treatment. Every treatment worked.....for a little while. They were refractory to every treatment; they still are.
(Left, original art uploaded by Pat Spiller from an altered book entitled, "Singing With A Full Voice", 2006).


Then came gastrointestinal issues and more docs and no answers. No doc linked the two symptoms even tho' 97% of all people with Behcet's Disease suffer from mouth ulcers and quite a high percent deal with GI issues. But no one made the connection. Now, of course, I understand that Behcet's is difficult to diagnose; some of its characteristic features resemble other conditions (for example, Crohn's Disease has both apthous ulcers and GI issues). And I have recently learned that it can take years for all the symptoms of Behcet's to manifest so that a certain diagnosis can be made.
Moreover, this disease has a historical prevalence along the old Silk Route. In countries like Turkey and Japan the prevalence is very high but negligable in the UK. Some docs asked about my ethnic heritage or said "it" looked like Behcet's but probably isn't because I am not Middle Eastern, Mediterranean or Jewish.
New Symptoms
About 4 years ago, fatigue became a frequent visitor; it would hit with vengeance, suddenly and without warning I'd be down for the count for days. I could literally sleep for 12 or 15 hours at a stretch. And the mouth ulcers got larger, more painful and lasted longer. I was afraid I had some sort oral cancer. I panicked. My primary care physician referred me to a specialist credentialed in 3 areas, rheumatology, immunology and alergies. He tested and tested; sent me to various and sundry other specialists. He suggested lupus. Polymialgia rheumatica. Somebody's Granulomatosis. Behcet's. And others. But nothing clear and definitive. He treated symptoms and used colchicine. Methotraxate. Medrol. And other poisins. With no positive benefit.
And More New Symptoms
While I was treated by this new doc, he discovered that I had an elevated protein in my blood -- some sort 'small clonal population'. He sent me to a hem/onc who watched it for a while and then discharged me as fully healthy! But within weeks, though, I was hospitalized for a severe anemia ~~ hemoglobin 5 and hematocrit 15 ~~ (normal references in that lab are Hemaglobin 11-15 and hematocrit 34-46). I was transfused with 3 liters of packed red blood cells and treated with 80mgs of prednisone/day and the search began to find a cause for the anemia. Four months later, I had titrated the prednisone down to 5 mgs/day with the added benefit of no mouth sores, no GI issues! That remission lasted for nearly 8 months leading docs to believe the symptoms were somehow related to or caused by the CLL and a dysregulated immune system. My hematologist at Dartmouth Hitchcock said that one of the mysteries of CLL center on whether the CLL causes the dysregulated immune system or the dysregulated immune system causes the cancer.
In the last 5 months, we saw the CLL as not only stable but a very placid dragon, indeed. But the other autoimmune problems of mouth sores and GI problems were raging on and I began to experience skin eruptions on my thigh (another classic Behcet's symptom, I later learned), forearm and cheek. My skin was biopsied; I was referred to a new doc, an immunologist/researcher.

You know the old saw? if it walks like a duck and talks like a duck,

it probably is a duck.

It is a duck. It looked like a duck before this but other docs were unwilling to be wrong or unwilling to take a risk. My new doc says he is not afraid to be wrong. And not afraid to admit he was wrong.

But this is not the 'rest of the story' (a la Paul Harvey). The story is still unfolding. I don't know what the Behcet's will do next. Having declared itself to be an oral, GI and cutaneous type of disease, maybe it won't evolve to the lungs, heart, central nervous system and eyes.

And since I don't know enough to be pessimistic (who said that),

I can only

Original art uploaded by Pat Spiller

from an altered book entitled, Singing With A Full Voice, 2006

Vintage paper, collaged/painted vintage lace, Lumiere paints

Amid the whirlwind

When the world around you is in a whirl
where do you go to find your center?

Do you find it in a sunset

or waking to a glorious sunrise?

Is it in the brush of summer petals?

Or the trickle of rain down the window?

Do you have a spot that is just your own?

A place where you go to read, to dream, to think.

Perhaps it's there.

Original art uploaded by Pat Spiller
from an altered book in progress, 2007

In September, Larry and I took the Uncle Oscar from Rye Harbor and made for the Isles of Shoals for our almost-annual Mid-Week Retreat on Star Island.

All summer long, there are weekend and week-long conferences on Star. But our favorite is Mid-Week II, typically held during the final week before the entire island is buttoned up for the winter. At a usual summer conference on Star, there is a lot of action, families doing, learning, playing. But at Mid-Week II, there are few activities: none planned, none mandatory. Breakfast, lunch, and dinner. Naps. Rocking on the wide veranda in the old fashioned chairs. Reading. Dreaming or just looking on this island world with a soft gaze.

So, we retreated once again to the tiny island that could be miles and miles away but isn't; it could be in another century, but isn't. On Star, there are no roads, no automobiles save the one utility truck that runs from the dock to the hotel with supplies. There are no newspapers, no radio or TV, no telephones or computers. It is peaceful and quiet. The Atlantic laps the shore. Lobster boats working or a sail boat doing its water ballet. Thats all. There are no street lights and when evening falls the island is dark; coastline across the water looks like a necklace of jewels.

Each day on Star begins and ends with Chapel. A welcoming, peaceful, place of communion. Each time I leave, it is with a lighter heart. In the evening, along about 8:30, folks assemble on the porch to receive their lighted lanterns. We walk single file, silently up the little hill behind the Oceanic Hotel to the Chapel. The music beckons. Each person enters the tiny chapel and hangs his lantern on the wall and the room is soon flooded with a soft light. We sing. We listen. We pray. We commune in peaceful silence.

I find my center there.

"We learn as much from illness as health, as much from sorrow as from joy." Pearl S. Buck

Today is one of those glorious October days that tourists come to New England to experience.

Last week we had visitors from Michigan and Ohio here to celebrate Larry's dad's 92nd birthday. It rained.

Today 'tho is bright, sunny with lots of autumnal color.

Yellow. Orange. And red.

After finishing a little desk work, I set out on a morning walk to the beach. Fallen leaves rustling under foot always remind me of childhood. Gay chrysanthumums offer a multi-colored-show in my neighbors gardens.

The ocean surface sparkles. I can see seven miles out from Jenness Beach to the Isles of Shoals. I see a freigher anchored by the Shoals waiting for the tides to change so she can come up the Piscataqua River to Portsmouth Harbor.

Star and Appledore Islands are standing up so clearly on the horizon I can almost knock on the front door of the Oceanic Hotel.

Larry and I were on Star Island in September for a "mid-week retreat ~~~ just before the island was buttoned up for winter.

As I walk, I am reminded of something I learned from Dr. Christiane Northrup about 15 years ago.

How to have a healthy adrenal system.
Dr. Christiane Northrup founded Woman to Woman, an OBG-YN practice in Yarmouth Maine. She was my physician a number of years ago. At that time, she mailed out newsletters to her patients and in one them, she wrote about 4 principles that can give you a healthy, less stressed adrenal system.

Here they are.

Say them to yourself when you feel stressed, worried or anxious.

You know how easy it is to second-guess yourself.

Well, of saying,

"I wish I had"

"If only I had done" say to yourself:

"Whatever I did today, is exactly what had to be done."
"When it begins is always the right time."
"When it's over, it's over."
"Whoever show up are exactly the right people."

Ahhhhhhh. Can't you just feel the release, the letting go of tension. Ahhhhhhh.

Cooking is one of the activities that give me an "ahhhhhhhhhh" kind-of feeling, too. At the end of the work day, it is a creative transition into evening ~~ a letting go of the unfinished business of the day. I love the planning, assembling, mixing and tasting; I am in love with the fragrance of cooking that fills the kitchen.

Today, I am cooking an autumn-weather dinner. After the heat of summer and lots of outdoor grilling, in autumn I always look forward to savoring slow-cooked meals from the oven. The mingling aroma of garlic and onion simmering with wine and fresh herbs is such a treat for the senses. On today's menu is a Daube Provencal with small white onions, mushrooms, carrots, potatoes and beef, thyme, red wine, saffron and tiny bit of tomato ~~~ it all works to create a deep mahogony sauce for the tender beef. Daube Provencal? ~~ a fancy name for beef stew. I'll make biscuits, too, I think.

And, for dessert, a homemade pumpkin pie. Ahhhhhhhhhhhhhhhhhhhhhhhhhhh.

Treatments for Behcet's

There is no cure for Behçet's disease. Treatment for Behcet's focuses on reducing discomfort and preventing serious complications. Effective treatment, however, results in longer durations between Behcet's 'flares'. Treatments are both topical and oral, used singly or in combination to pain and discomfort. Oral treatments include rinses, gels, pastes, ointments and creams usually containing corticosteroids to reduce inflammation and/or other anti-inflammatory drugs to suppress the overactive immune system, and relieve symptoms.

The treatment of Behçet's syndrome depends on the severity and the location of its manifestations in individual patients; below are commonly prescribed medications.

Colchicine can minimize recurrent ulcerations, treat mucous membrane and skin symptoms in Behcet's. Joint inflammation can require non-steroidal anti-inflammatory drugs (such as ibuprofen and others) or oral steroids. Colchicine and oral and injectable cortisone are used for inflammation involving the joints, eyes, skin, and brain. (interesting: a research study in Turkey showed that it is more effective in males than females.) And it is not without some pretty nasty side-effects.

Sulfasalazine has been effective in some patients for arthritis.

Prednisone is a corticosteroid used to reduce pain and inflammation throughout the body for people with severe joint pain, skin sores, eye disease, or central nervous system symptoms and is seful in early stages of disease and for acute severe flares but are of limited use for long-term management of central nervous system and serious eye complications.

Immunosuppressive drugs help control an overactive immune system which occurs in Behçet's disease, reduce inflammation throughout the body, and can lessen the number of disease flares. Doctors may use them when a patient has eye disease or central nervous system involvement with the Behcet's; they also have serious side effects. Doctors may use one or more of the following drugs depending on specific symptoms: Azathioprine (Imuran) is used to suppress the immune system it is now used for people with Behçet's disease to treat uveitis and other uncontrolled disease manifestations. This medicine can upset the stomach and may reduce production of new blood cells by the bone marrow. Chlorambucil is used to treat uveitis and meningoencephalitis. Cyclophosphamide is referred to as a cytotoxic drug. Unfortunately, normal cells also are affected, and this results in serious side effects. Cytoxan also suppresses the immune system and is also referred to as immunosuppressive. Cyclosporine when used by patients with Behçet's disease reduces uveitis and uncontrolled disease in other organs.

Catching Up

My last post was more than 3 months ago just prior to an appointment with Dr. Bengston at Dartmouth Hitchcock for the CLL.

During the previous months, there had been no changes in the CLL ~~ at least in terms of peripheral blood reports: WBC in normal ranges; no lymphocytosis; no lymphadenopathy; no enlarged spleen or liver. So, I thought, "do I really have CLL?"

The CLL dragon is so quiet but why am I plagued with mouth ulcers, gastrointestinal and skin issues ~~ all sequella that disappeared with high doses of prednisone for the autoimmune hemolytic anemia in 2006 and from which I had a relatively long remission.

Dr. Bengston listened respectfully to my concerns and questions. She ordered a second flow cytometry test (a diagnostic test). The first was done at Beth Israel in Boston in the summer of 2006. The 'flow' was done before I left Dartmouth Hitchcock Medical Center that day in June. In addition, she made a referral to an immunologist/rheumatologist,also at Dartmouth Hitchcock. Dr. William Rigby is a professor at the medical school, a researcher who only sees a very few patients and he reserves the right of refusal if those who are referred to him don't meet his research interests. I was accepted as a patient; an appointment was set for mid-July.

Several weeks later, I received a phone call from Dr. Bengston with results of the flow cytometry: the CLL has gone below detectable levels in the peripheral blood. Amazing.

AMAZING.

In email correspondance with Dr. Terry Hamblin (UK), he said these remissions can occur in early stage CLL when patients have been treated for autoimmune hemolytic anemia. The result is that the prednisone knocks the CLL back.

But that is just part of my summer saga. On July 16, I had my first appointment with Dr. Rigby. I went expectently to the meeting, hopeful that he would find answers for the pesky autoimmune issues that just won't quit. I confess: I expected him to say that the autoimmune problems were the result of a dysregulated immune system caused by the CLL. But that is not what he said. Not at all. He actually gave these autoimmune issues a name. A diagnosis called Behcet's Disease. A rare autoimmune disorder under the general category of 'vasculitis' that causes inflammation of blood vessels both large and small througout the body.

There is no cure.

And this is what July and August felt like. A roller coaster of emotions. From that day in July at Beth Israel hearing 'CLL' for the first time. Learning the vocabularly of leukemia. Surfing the web and beginning to understand concepts I thought I'd never need. Discovering the CLL Forum and CLLC Friends and meeting wonderful people on the web who also live with this dragon of CLL. Feeling their support.

And now, in the middle of autumn, I've seen Dr. Rigby three times and each time feel my great good fortune in having this bright, attentive, caring man on my side. And here I am again: learning the language of Behcet's. A new vocabulary. Surfing the web for new treatments. New science. New support.

What a strange year.

And here's some skinny on Behcet's Disease. Rare (only 15,000 people affected in the US). A multi-system disease.

The symptoms I experience with are: oral (97% of people with Behcet’s have mouth sores), gastrointestinal, joint pain (especially wrists), and more recently skin eruptions. I have lots of body temp fluctuations and spike low-grade fevers. Since starting medication in July, severe fatigue has improved. I have lots of day/night sweats and fevers.

The doc says that my immune system behaves as though ‘the Russians are coming to Hampton Beach’; all peace time activities have been halted to ready for this massive invasion. And for the first time this week, I could actually ‘see’ this analogy. When my lab work was done it showed lymphocytes in the peripheral blood had ‘tanked’ at 9% (normal reference in that lab is 15-45) and is indicative of an ‘exhausted immune system’~~~~~my immune system thinks I am the Russians !!

MOST COMMON SYMPTOMS AND SIGNSOF BEHCET'S DISEASE
Behçet's disease is common in the Middle East, Asia, and Japan. It is rare in the United States. In Middle Eastern and Asian countries, the disease affects more men than women. In the United States, it affects more women than men.

Behçet's disease tends to develop in people in their 20's or 30's, but people of all ages can develop this disease.

Behçet's disease is an autoimmune disease that results from damage to blood vessels throughout the body, particularly veins. In an autoimmune disease, the immune system attacks and harms the bodies' own tissues.

The exact cause of Behçet's disease is unknown. Most symptoms of the disease are caused by vasculitis (an inflammation of the blood vessels). Inflammation is a characteristic reaction of the body to injury or disease and is marked by four signs: swelling, redness, heat, and pain. Doctors think that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful "foreign" substances, such as germs, that enter the body. In an autoimmune reaction, the immune system mistakenly attacks and harms the body's own tissues.

Behçet's disease is not contagious; it is not spread from one person to another.
Behçet's disease affects each person differently. The four most common symptoms (as listed) are mouth sores, genital sores, inflammation inside of the eye, and skin problems. Inflammation inside of the eye (uveitis, retinitis, and iritis) occurs in more that half of those with Behçet's disease and can cause blurred vision, pain, and redness. Other symptoms may include arthritis, blood clots, and inflammation in the central nervous system and digestive organs. Behcet’s disease has the ability to involve blood vessels of nearly all sizes and types, ranging from small arteries to large ones, and involving veins as well as arteries. Because of the diversity of blood vessels it affects, manifestations of Behcet’s may occur at many sites throughout the body.

However, the disease does seem to target certain organs and tissues; these are described below:
Eye: Behcet’s may cause either anterior uveitis (inflammation in the front of the eye) or posterior uveitis (inflammation in the back of the eye), and sometimes causes both at the same time. Anterior uveitis results in pain, blurry vision, light sensitivity, tearing, or redness of the eye. Posterior uveitis may be more dangerous and vision–threatening because it often causes fewer symptoms while damaging a crucial part of the eye — the retina.

Mouth: Painful sores in the mouth called “aphthous ulcers” (known as oral aphthosis [af-THO-sis] and aphthous stomatitis) affect almost all patients with Behçet's disease. Individual sores or ulcers are usually identical to canker sores, which are common in many people. These sores are usually a result of minor trauma. They are often the first symptom that a person notices and may occur long before any other symptoms appear. However, the lesions are more numerous, more frequent, and often larger and more painful. Aphthous ulcers can be found on the lips, tongue, and inside of the cheek. Aphthous ulcers may occur singly or in clusters, but occur in virtually all patients with Behcet’s. The sores usually have a red border and several may appear at the same time. They may be painful and can make eating difficult. Mouth sores go away in 10 to 14 days but often come back. Small sores usually heal without scarring, but larger sores may scar.

Gastrointestinal: Behçet's disease causes inflammation and ulceration (sores) throughout the digestive tract that are identical to the aphthous lesions in the mouth and genital area.
Ulcerations may occur anywhere in the gastrointestinal tract from the mouth to the anus. This leads to abdominal pain, diarrhea, and/or bleeding. Because these symptoms are very similar to symptoms of other diseases of the digestive tract, such as ulcerative colitis and Crohn's disease, careful evaluation is essential to rule out these other diseases.

Skin : Skin problems are a common symptom of Behçet's disease. Skin sores often look red or resemble pus-filled bumps or a bruise. The sores are red and raised, and typically appear on the legs and on the upper torso. In some people, sores or lesions may appear when the skin is scratched or pricked. When doctors suspect that a person has Behçet's disease, they may perform a pathergy test, in which they prick the skin with a small needle; 1 to 2 days after the test, people with Behçet's disease may develop a red bump where the doctor pricked the skin. However, only half of the Behçet's patients in Middle Eastern countries and Japan have this reaction. It is less commonly observed in patients from the United States, but if this reaction occurs, then Behçet's disease is likely. Pustular skin lesions that resemble acne, but can occur nearly anywhere on the body. This rash is sometimes called “folliculitis”. Skin lesions called erythema nodosum: red, tender nodules that usually occur on the legs and ankles but also appear sometimes on the face, neck, or arms. Unlike erythema nodosum associated with other diseases (which heal without scars), the lesions of Behcet’s disease frequently ulcerate.

Lungs : Aneurysms (outpouchings of blood vessel walls, caused by inflammation) of arteries in the lungs, rupture of which may lead to massive lung hemorrhage.

Joints: Arthritis or “arthralgias” which is inflammation of the joints, occurs in more than half of all patients with Behçet's disease. Arthritis causes pain, swelling, and stiffness in the joints, especially in the knees, ankles, wrists, and elbows. Arthritis that results from Behçet's disease usually lasts a few weeks and does not cause permanent damage to the joints.

Brain : Behçet's disease affects the central nervous system in about 23 percent of all patients with the disease in the United States. The central nervous system includes the brain and spinal cord. Its function is to process information and coordinate thinking, behavior, sensation, and movement. Behçet's disease can cause inflammation of the brain and the thin membrane that covers and protects the brain and spinal cord. This condition is called meningoencephalitis. People with meningoencephalitis may have fever, headache, stiff neck, and difficulty coordinating movement, and should report any of these symptoms to their doctor immediately. If this condition is left untreated, a stroke (blockage or rupture of blood vessels in the brain) can result. Central nervous system involvement is one of the most dangerous manifestations of Behcet’s. The disease tends to involve the “white matter” portion of the brain and brainstem, and may lead to headaches, confusion, strokes, personality changes, and (rarely) dementia. Behcet’s may also involve the protective layers around the brain (the meninges), leading to meningitis. Because the meningitis of Behcet's disease is not associated with any known infection, it is often referred to as “aseptic” meningitis.

Genitals: Genital sores affect more than half of all people with Behçet's disease. The sores look similar to the mouth sores and may be painful. After several outbreaks, they may cause scarring.

Ebb & Flow

There is a natural ebb and flow to this disease.

I know this intellectually and I celebrate the ebbing.

Days with no fatigue.

No autoimmune issues.

No b-symptoms.

But then WHAM, the dragon hits again.

On the good days, I nearly forget that CLL is lurking.

The recent three weeks have been difficult with all the associated issues that a disregulated autoimmune system bring:

fatigue,

gastroenteritis,

migratory dermatitis,

persistent, painful mouth sores.

Tomorrow I see Dr. Bengston at Dartmouth Hitchcock Medical Center. Copies of the excel spread sheet that I use to document autoimmune and b-symptoms was mailed a week ago along with a list of questions for discussion at our meeting. I also proposed that we look at returning to the high dose immunosuppressants used last year for AIHA. One side benefit of that treatment was a knocking back of the autoimmune sequella for about about eight months.

Like The Lillies in The Fields

I love June.

The cool mornings,

the warmth of the noon-day sun

comforting breezes

the riotous color of spring gardens. . .

Our little side garden is just popping now.

Today our very first poppy appeared, tall, blood red and beautiful

surrounded by 3 foot Asiatics that will be next to unfurl their petals.

The little garden is hardy:

it survived the unseasonal winter warmth

and late cold of May that was followed by lots of hard rain.

It survived

persevered to thrive:

first, the daffodils

grape hyacynth and tulips

now poppies and lillies.

I want to be more like the flowers in my garden.

Withstanding the unexpected

Celebrating days of strength and energy.

I want to learn to accept the days of fatigue and fever

mouth sores skin eruptions intestinal pain

when I haven't the energy to work, to play, to cook or to eat.

It doesn’t matter why something happens.

It doesn’t matter why something happens.

Why you got cancer or why you lost your hair
or why you got into a car accident.

What matters is what you do with it.
What matters is how you use it.
What matters is getting your life back.

And since all you have to work with is who you are
and what you’re given,
you might at well use them.

We are more than our hair, our faces, our bodies, our relationships.
But we will never know that
until we understand the images
that keep us from remembering ourselves.

When they are recognized, they dissolve.
And when they dissolve,
we become that much freer
to be the vastness of who we are.

Geneen Roth

CLL? SLL?

Over the last 6 or 8 weeks, I had been asking questions such as: What is a small clonal population and what does that mean? Why are my counts (WBC & ALC) in the normal range? Without lymphocytosis, can this be CLL? If not, then what is it?"

I requested quantitative reports from Beth Israel Deaconnes Medical Center in Boston where the diagnosis was done last summer. They were waiting for me when I returned from Canada and I discovered some answers to some questions:

• The bone marrow biopsy/aspirate showed 33% lymphocytes -- that answers the "small clonal population" question as according to MD Anderson, "lymphocytes >30% are diagnostic for CLL.
• The flow cytometry studies showed the "presence of a Kappa restricted CD20 dim, CD23+, CD5 B-cell population that is immunophenotypically consistent with involvement by chronic lymphocytic leukemia/small cell lymphocytic lymphoma."
• CD38 expression is not detected (and that is a good thing!!!!)
• CD5+ B-cells express ZAP-70 (not a good thing ---- but ---- it could be a lab error)
• Molecular cytogenetic - four DNA probes -- all in normal ranges (another good thing)

So here is what I now understand: CLL and SLL are biologically and morphologically the same disease but present differently: CLL in the blood and SLL in the lymphatic system.

Scenes from the Ontario CLL Conference

Larry and I first heard about the Ontario CLL conference in the deep of winter and immediately decided to attend. It was to be the first patient-run meeting on CLL. The conference was the brain-child of Deb Baker who was joined in the effort by Elizabeth Locatelo; they gathered friends and cohorts into working groups, solicited funding and support; successfully approached world-class physicians and researchers in the field of blood malignancies; and negotiated space in a five-star hotel/spa and conference center.

And they were on.....and running.

Deb Baker told me that she was dreaming in black & white
but that Elizabeth Locatello was dreaming in full color!

When opening day of the conference came, there were over 250 participants registered. The meetings were chaired by Dr. Ronan Foley and international presenters included: Dr. John Byrd, OSU/Columbus, Ohio; Dr. Terry Hamblin, England; Dr. Susan Leclair, UMASS Medical School/Dartmouth. In addition, prominent medical professionals from the Canadian scene were there including: Dr. Graeham Fraser, Dr. Sagar.

These lovelies await the traveler as s/he enters the White Oaks. There are four of them each one representing a season. At night pillars of candle light flicker in tall glass vessels around them.

The White Oaks Resort is elegant & luxurious.

The conference meeting space was more than comfortable. Hotel staff were attentive. The facility sported squash courts, tennis courts, fitness rooms, a pool and a full-service spa, as well as a four-star dining room. Surprisingly, there are statuary, interesting pieces of art and some exceptional pieces of art-furniture here and there.

Yes. These really are chairs. With matching glass tables. Positioned in front of mirrored walls and scattered on landings throughout the hotel.

But more than gaining knowledge and acquiring state-of-the-art information on CLL research and clinical trials was the opportunity to feel part of something......to feel less alone......to share the 'dragon' with others who alos live in its grip.

I have been home from Canada for five days. I returned very tired and just didn't have the energy to get these images ready and my thoughts in order. I have yet to begin the task of re-reading the material I took from the conference. That is next on my agenda.