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.

What's in a name?

My name written in Chinese.
Patricia.

I have been struggling with the names of things lately. Especially medical names. And this has led me on a small journey of discovery. The process began when my hematologist said, 'Don't look for peers in this disease. Your CLL is atypical.' I didn't ask follow-up questions then but later her remark began to peak my interest and I wondered in what ways it was different.

Well, one certain difference is that my diagnosis came from the severe anemia that hospitalized me in July and was later termed autoimmune hemolytic anemia (AIHA). Only about 10% of people with CLL present initially with AIHA although about 30% of those with CLL are at risk for it throughout the course of their disease.

I have a bundle of autoimmune issues that are non-hematologic but hematologists at Beth Israel and Dartmouth medical center have said these are 'probably' related to the CLL which 'corrupts' the immune system. But in reading on the web, I came across something written by Dr. Hamblin of England, one of the international lights on this disease, who wrote that non-hematologic autoimmune complications are rare in CLL.

The diagnostic report from Beth Israel stated that I was 'hypogammaglobulinemic' -- low immunoglobulins. For me, the IgA is low: only 47 (normal reference is 70-640). More reading on the internet yielded this: 'hypogammaglobulinemia is associated with complications in the progress of the (CLL) disease". For sure, it explains why I have that 'bundle of autoimmune issues' like large multiple painful mouth sores and chronic intestinal problems.

"AHIA and hypogammaglobulin do occur in the mutated subset, and in our experience hemalytic anemia maybe the presenting feature of low-count, stage 0 disease." Kay et al, 2002

In Boston, a flow cytometry test was done and my cells were found to be ZAP70 positive. However, there are many false negatives and false positives in this test has procedures have not been standardized or as David Arenson (www.clldiary.blogspot.com) says, the 'kinks' have not been worked out yet. Most experts agreee that the only laboratory where one is certain to get an accurate reading is UCSD.

Another test, the B2M in July was 2 and in March it was 3.5. Some experts believe that anything over 2 heralds a more aggressive disease. Other experts say a number of 4 is the indicator for a more aggressive disease. Others disagree. Still others say it not indicative of CLL at all. But there it is, increasing.

All winter long, I have experienced frequent and episodic fevers that run from 99.5 to 100 with no alteration in the white blood count. I have night sweats (but not drenching) and day sweats occassionally and episodically. And fatigue that can put me in bed for one or several days at a time. All three are 'B-symptoms'.

But the real ringer is that my CBC reports stay within normal ranges in term of white blood count and absolute lymphocyte counts. Typically, one would expect to see rising counts. Over the last eight months since diagnosis of AIHA, there has been consistent evidence on the CBC (LDH and reticulocyte counts) that I am still hemolyzing red blood cells -- but the balance is holding and I have been off steroids since October.

"The lymphocytes in the blood are part of the total lymphocyte mass in the body; they circulate through the bone marrow, spleen and lymph nodes. In CLL, they have derived from a single abnormal lymphocyte and have taken many years to reach a number that can be detected. The basic defect is a failure to die." Hamblin, 2007

Yesterday, I posed THE question to my local hematologist/oncologist in Portsmouth: could there have been a mistake in the diagnosis of CLL? He agreed that my disease didn't present in a consistent way and mentioned the possibility of "SLL". He wondered out loud whether I should have a re-take of the ZAP70 test and another bone marrow biposy.

I also posed the question to Dr. Hamblin on the CLLC Friends board; he mentioned "SLL".
I laid out my scenario to the membership of the CLL Forum; and several members mentioned "SLL".

SLL: small lymphocytic lymphoma. Not a leukemia because the disease is in the marrow and the nodes. It is the same disease biologically as CLL, treatment protocols are the same. But it is different in that it is not seen in the peripheral blood.

This begins to answer some of my questions.
Of course it also poses a few more.
Does the CLL/SLL difference matter?
Is the natural course of the disease the same or different?
Should I look for a second opinion from one of the 'experts'?
Which one?

Singing with a full voice

Watching the moon at dawn
Solitary
Mid-sky.
I knew myself completely
No part left out.

This image is a page from my first altered book. I worked on it last summer when lack of energy and focus kept me from doing so many things. This project was perfect. It allowed me to play in a new medium, with tiny bits and pieces at a time. It had no heavy lifting. It offered lots of scope for the imagination. It was a very forgiving project.

In October, I entered the completed altered book which I called, "Singing with a full voice" in a small art show; it was on display until January '07. And to my delight, when it came home, there was clear evidence that the book had been read and enjoyed by lots of visitors to the show.

"Last night it did not seem
as tho' today it would be raining."




In preparing this page,
I collaged antique lace
Glued over it and into every little crevice
Painted may layers over the glue
Cut shapes
Created a unique & entirely new surface ~
No longer delicate lace but
Durable
Strong

Trying to sort it out

When I look through the notes I made last summer, I see how difficult it was to sort out what was happening to my body. I kept wondering: "How could one disease kill off red blood cells and make too many lymphocytes (white blood cells) at the same time?"

At the end of June, I saw my primary care physician (PCP) with complaints of fatigue. Several blood tests were ordered. But when they were done, the phlebotomist neglected to do the most important one, the CBC (complete blood count). This was not noticed for several days while I was continuing to spiral down in energy and waiting for a call from the doctor's office. Finally, a frantic technician called to inform me of their error and ask that I come in asap for the test. About four hours later, another frantic call came from the PCP's office to 'go immediately to the emergency room'. I did.

July 5 - admitted to hospital

July 6 - transfused with 3 units of packed red blood cells; Hemoglobin was 6 and Hematocrit was 16; I saw the hospitalist at 5 PM who spoke to Larry and I about hemolytic anemia and the need for further tests. He said they don't know what has caused the anemia. They started giving me 80 mgs of prednisone once a day in the morning, folic acid and pepcid.

"Autoimmune disease happens when a confused and corrupted immune system starts attacking perfectly good cells, something that should not happen under normal conditions. Roughly, 11% of CLL patients with advanced disease will develop full blown autoimmune hemolytic anemia (AIHA) but as many as 30% of CLL patients may be at risk for developing it. In simple terms, their immune systems start attacking red blood cells, and if these crucial oxygen carriers are killed off faster than they can be replaced by the bone marrow, the result is anemia, low hemoglobin levels and low hematocrit. Anemia is a quality of life issue. Low red blood cell counts and low hemoglobin lead to poor oxygen supply to the tissues, which in turn lead to fatigue." (CLL Topics)

July 7 - CT scan and bone marrow biopsy
July 9 - discharged from hospital with the caveat that that is going to be a long-term thing. Hemoglobin, 11 and Hematocrit, 29
July 13 - fatigue, pressure on chest, heart pounding, nausea, difficulty sleeping. Letter from Anthem Blue Cross - saying hospitalization was "not medically necessary" - denied
July 15 - exhausted, shakey, constant headache; but energy level a little better in early evening
July 17 - Hematocrit 30; reduced prednisone to 60 mgs. Sleepless - awake most of the night and finally fell asleep at 6 AM
July 19 - Human Resources put me on Family Leave
July 20 - we had our appointment at Beth Israel in Boston and learned the definitive diagnosis was CLL

"Chronic Lymphotic Leukemia (CLL) is a slowly progressive form of cancer, characterized by an increased number of B lymphocytes, of the white blood cells and bone marrow. Blood cells are normally produced in a controlled way, but in CLL the process gets out of control. The lymphocytes multiply too quickly and live too long, so there are too many of them circulating in the blood. These Leukemia lymphocytes look normal, but are in fact not fully developed and do not work properly. Over a period of time the abnormal cells replace the normal white cells, red cells land platelets in the bone marrow. It is the most prevalent form of leukemia and occurs predominantly in patients over 55 years.

"Many cases of CLL are detect by routine blood tests in persons with no symptoms, however, patients may have enlarged lymph nodes, enlarged liver and spleen, fatigue, bone pain, abnormal bruising, excessive sweating, loss of appetite, weight loss, re-occurring infections and lack of energy. CLL affects a type of lymphocyte called the B lymphocytes and causes suppression of the immune system, failure of the bone marrow, and infiltration of malignant cells into organs. Although CLL starts in the bone marrow, it can spread to the blood, lymph nodes, spleen, liver, central nervous system and other organs. It does not usually form a solid mass or tumor." (CLL Support Association - Patient Support and Education for CLL)

July 21 - nausea, dizzness, shakey, tired; slept from 11 to 2 but didn't get up until 4; intestinal discomfort
July 22 - read David Arenson's blog (http://www.clldiary.blogspot.com) and found his citation of "The Anatomy of Hope" by Jerome Groopman; Larry got it from our town library

How different are my blood test results from normal in terms of lymphocytes? Platelets?
How does the autoimmune hemolytic anemia (AHIA) affect diagnosing of CLL? Staging of CLL?
Was a Coombs test for AIHA done?
Does AIHA complicate CLL? How?
During this watch and wait period, what symptoms are there? What should I be watching for?
If I have an ordinary infection, virus etc, which doctor will treat it? PCP? Hem/Onc?

July 24 - Hematocrit 29; decreased prednisone by 10 mg; Phone call from Amy: Victoria is coming - my only & favorite & wonderful granddaughter is coming for a visit.

July26 - I have been home from the hospital fro 16 days. Seven of those days were notable for the fatigue. During some of that time, I could sometimes get a little energy for my studio in short bursts; I had lots of creative ideas.

Then came the prednisone reduction ------- and then the name, Leukemia. Double whamy! Withdrawal from the prednisone is impossible; it feels like a sucker punch to take medicine to relieve a health condition only to find that the stuff compromises one's daily well-being. Now, I have no energy -- for creativity or for doing much of anything. I am unable to focus on anything long enough to do it: want to do it -- plan to do it. In the first days home, my creative juices were strong; now I feel empty. My head wants to work but I am unable to translate it to action.

July 27 - I am blessed with wonderful people in my life.

"Happiness is the spiritual experience

of living very minute with love, grace and gratitude"

Denis Waitley, Psychologist, American, 21st century

A Girl-Warrior in the World of CLL

The Girl Warrior Begins

Seven months. Not long in a cosmic sense. Long enough emotionally to have unearthed some truths. About myself. Long enough to have learned a little about this disease.

In my early reading, I came across the question of whether to tell anyone about the diagnosis. Some said never tell your employer. Clearly, others disclosed to family and friends, only. I thought about this issue for a bit and made several decisions.

First, I would tell my employer and those co-workers to whom I share personal stories. I decided that I would tell three or four friends in my social circle. I told my daughter and siblings. These were good decisions as the out pouring of love and care and concern has been quite wonderful. I understand why in some work environments, one would chose to remain silent in fear that others may not think one up to the task. My work environment is a small private school for students with physical and developmental disabilities. The executive director has created a warm and loving environment not only for students but for staff as well. I have not regretted this decision.

Telling my daughter was perhaps the most difficult of all. Amy and I are all that is left of my original nuclear family: my son Jonathan died when Amy was six and my husband died when she was only twelve. I knew the words 'cancer' or 'leukemia' would be difficult to hear and process.

I chose not tell my mother. My family has experienced a lot of illness, particularly cancer. My brother had testicular cancer in his thirties and prostate cancer in his fifties; one sister had a soft tissue sarcoma (intestinal) and just last year another sister had something called a schwanoma. I just could not tell my mother! If the CLL is indeed 'indolent' I may not need treatment for a number of years so I reasoned that perhaps she just needn't know.

My mother is worrier. She never stops worrying about us even though we now range from 44 to 46, 50 to 52, and 60 to 62, we are still her children. I wanted to spare her the angst of knowing and not understanding and not being able to 'do' anything.

But during this winter when B-symptoms appeared with some frequency and I was learning to accept the diagnosis -- to say it to myself: "this is my life, now" "this is not going away" "I have cancer". And I realized that withholding important information from someone you love changes the nature of the relationship.

Clearly the withholding didn't come into focus as a problem until I was able to know (in my bones) and assimilate the 'word' and its meaning to me and my life. Once I moved into that realm of understanding, the disconnect between my mother and I became very uncomfortable for me.

And so a new decision is made. Larry and I will visit my mother this weekend and during our time together, I will disclose my new reality as a "Girl Warrior" in the CLL World. My sister, Susan and her husband Rich, will be with us and this is comforting.