Posted: Aug 6, 08 8:50am
It's great to see you here in the AF discussion group. Also known as desmoid tumor, AF is a rare genetic syndrome that causes the connective tissue in the body to grow into spindle cells that attack and destroy surrounding tissue. Desmoid tumors are considered a subtype of sarcoma and malignant in the sense that they cause extensive destruction of surrounding tissue. The tumors were once thought to be very slow-growing, but new evidence, and my personal experience, prove that desmoids can grow quite rapidly. Most desmoid tumors occur in the abdomen, followed by the legs, and the neck and face. They can lead to physical debilitation, limb amputation, and eventually death. There is no cure and the disease is not well understood by physicians, so it's very important for a person with AF to become knowledgeable about the disease and take a proactive role in his or her course of treatment. Current treatments include radiation therapy, surgery, chemotherapy, acetic acid injections, and radiofrequency ablation, among others.
My tumor first appeared in my brachial plexus (right shoulder/neck area) in 1993, and after radiation therapy, surgeries, and chemotherapy, it is now on my spine at the neck and growing into my chest. I am literally one inch away from becoming a quadriplegic, should the tumor infiltrate my spinal chord. If it reaches my lungs or heart, I will likely die because the tumor is inoperable. I have already lost most of my thyroid gland and my right carotid artery (yes, you can live with just one) along with most of the muscles and nerves in the right side of my neck and shoulder. Radiation therapy kept me tumor-free from 1993 to 2005, when I had a recurrence. I was told by doctors at UCSF to save additional radiation as an absolute last resort. I was on inpatient chemotherapy for 3 months, which was unsuccessful, then Arimidex/Sulindac, which caused a severe allergic reaction and had to be discontinued. I've been on a relatively new chemo drug called Gleevec for the past year, and it seems to be keeping the tumor stable.
You can find a lot of information at the Desmoid Tumor Research Foundation (www.dtrf.org), and you can read my story at www.kelvins-page.blogspot.com.
There is also a group in France called SOS Desmoide (http://www.sos-desmoide.asso.fr/) whose "aims are to break patients' isolation, collect and circulate knowledge about the disease, and stimulate research."
Kelvin Lynch
