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.