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).