I have just received the first of six monthly cyclophosphamide infusion treatments (chemotherapy), hopefully to control my very aggressive scleroderma condition that was diagnosed in June 2001. My involvement with autoimmune disease started in the spring of 1998. Here's my story.
One morning, I bit into a fresh orange and nearly went through the ceiling in pain. My body shook for about twenty seconds and it felt as if someone stabbed me with a knife in the temple near my ear. For the next sixteen hours, I could not even take a teaspoon of water. My parotid gland bulged out like a golf ball. The next morning, things were pretty normal. I had no pain and I could eat anything. The gland was down to a slight swelling. I went to see my doctor. She confirmed it was the parotid gland and told me if it happened again to come right in without an appointment. She wanted to see what was happening when it happened.
The next attack took place about three weeks later on a Saturday evening. Naturally, by Monday morning, things were normal again except for a swollen parotid gland. Within six weeks I had three attacks and was referred to an ENT (ear, nose & throat) doctor. This ENT specialist ordered several tests and found I had calcified particles (stones) in the gland. In addition, the lining of the gland duct was very thin in several places indicating damage. My blood test showed very high ANA (antinuclear antibodies).
I had two options. Take a wait and see position or go for surgery. My doctor explained the dangers of surgery with possible damage to the nerves in that area. I knew I could not sit around and wait for another attack. I was afraid to eat, never knowing when I would get another flare-up.
Since I decided to go for the surgery, my doctor did not take a lip biopsy to confirm Sjögren's Syndrome, as the parotid gland would be biopsied. The tissue was sent to the University of Pittsburgh Medical Center for diagnosis (I live in Southern California). The results were startling. Not only did it confirm that I had Sjögren's but I was one of the 4 percent of Sjogren patients with 'parotid lymphoma' (Zone B cell - Malt type). After a total body CAT scan, they also found that I had lymphoma of the nasal pharynx.
My radiation oncologist was very optimistic about the results of treatment. I had six weeks of head and neck radiation (five times a week). Two weeks after starting treatment, my entire mouth blistered up. I could not eat or sip anything because of the pain. My doctor had the pharmacist prepare a lidocaine mixture. This would numb my mouth so I could take in some liquid. Food was out of the question. For the next month I lived on supplemental nutrient drinks (five a day). There were times when the pain was so severe, I would put a tablespoon of lidocaine mixture in my mouth and keep it there for two to three hours to give me relief. When I finally tried to eat some solid food, I had a soft scrambled egg. It tasted better then a hot fudge sundae with all the trimmings!
Other than my mouth and extreme fatigue, I was fine. The important thing is that I went into total remission. It is more than three years since I completed radiation and I am doing fine. Surgery was 100 percent successful. But after radiation, I completely lost my taste buds. Bananas were salty. All food tasted bitter. There were very few foods I could enjoy.
Although I was on Salagen (a drug to help create moisture in mouth and eyes), I still had a very dry mouth and needed fluid to help get the miserable food down. I could not tolerate fruit and vegetables, whether raw, steamed or canned. I continued to depend on two cans of supplement nutrient drinks daily and experimented with different foods to find anything tolerable.
When Evoxac appeared for treating dry mouth, I changed to the new drug and six weeks later, my taste buds returned to almost normal. I still cannot eat raw fruits and veggies, but steaming helps.
I was fine until January 2001, when I diagnosed myself with Raynaud's Phenomenon. I also found that when I walked up a flight of stairs, I could not talk. I had my annual physical checkup in May and reported this to my doctor. She looked at my fingers near my nails and said I had scleroderma. I could see nothing and thought she was nuts. I went home quite hysterical as I knew one person with a very severe case of scleroderma and I could not believe this was my future.
After doing my homework on the Internet and getting information from the Scleroderma Foundation, I realized that everyone was different and I must take hold of my emotions and do what has to be done.
My doctor had me take a chest x-ray which confirmed mild pulmonary fibrosis. She referred me to a pulmonologist and rheumatologist. In July 2001, a pulmonary function test indicated a 50 percent rate of diffusion, which the doctor considered mild. My blood test was negative. He said he must treat the symptoms and ignore the blood test. He said my case was very early and mild and I would do fine.
In October I caught a slight cold and started coughing. I had a routine appointment with my pulmonologist three weeks later and waited until then. He said I had acute bronchitis. I was treated for it, and my lungs cleared up in two to three weeks. However, I remained with the worse cough anyone could imagine. Sometimes, I could not stop for two to three hours. When I laid down, I was fine but as soon as I sat or stood up, the coughing would start again.
My doctor said I had severe trauma to my system and it could take three to four months to clear up. It made sense, but I was very sick all winter. I noticed a warning label on Evoxac to be careful about taking this drug if you have pulmonary problems. I took myself off the drug. Within 48 hours, my coughing stopped. I assume Evoxac was causing a buildup of mucus in my lungs. Now, my mouth is much drier, but at least I do not cough. Warnings that comes with these drugs indicate that in some cases with pulmonary problems you should not take Salagen or Evoxac.
Prior to getting bronchitis, I had been swimming a half mile three times a week and working out with three pound weights three times a week. Within a short period of time, I could barely walk twenty-five feet without being short of breath. In February 2002, I attended a Sjögren's Syndrome Foundation support meeting in San Diego. Dr. Robert Fox, a nationally recognized rheumatologist, and an authority on Sjögren's, was the guest speaker. I had the opportunity to speak to him about my problem and he was most gracious to me. He told me I must take a high resolution chest CAT-scan to see what's really going on in the lungs and an echocardiogram to see if I have pulmonary hypertension.
The results were good and bad. No hypertension, but my lungs were much worse. The fibrosis was very advanced, with only a 33 percent diffusion. In approximately eight months, I went down from 50 percent to 33 percent. This should have taken many years, not months. In addition, all the lymph nodes in my chest were double the size as compared to the scan taken in 1998. Naturally lymphoma was suspected as I had history of the condition. A Mediastinoscopy was performed (surgery that allows doctor to view middle of chest and remove lymph nodes between lungs to test them for cancer or infection). Every lymph node visible to the surgeon was removed and biopsied. Good news: no Lymphoma! It was the scleroderma that enlarged the nodes.
During these months, my hands swelled and turned red. My skin thickened. I could feel my face tightening with red spots starting to appear. I have been losing my hair on a daily basis. I developed microstomia (small mouth). Bits of food get caught in my throat. My GERD is getting a little worse. I suffered from fatigue because of Sjögren's, but now it's worse.
I have control of my dental problems by being very conscious of dental care. I do not eat unless I can brush my teeth afterwards. I give my teeth a fluoride treatment daily. I floss twice a day (difficult because of my small mouth). My husband claims I will wear out my teeth. My dentist says I'm doing great.
My rheumatologist knew how he wanted to treat me, but he discussed the situation with Dr. Robert Fox and my original oncologist. They both agreed with him on taking very aggressive steps. Together with Prednisone and Actonel to prevent bone loss, I am receiving the chemotherapy by intravenous (IV) once a month over a six month period.
Once again, I did my homework on the Internet and found wonderful reports on this treatment. I found a report by researchers at John Hopkins Oncology Center in 1998 in treating autoimmune disorders with chemotherapy. The results were very exciting. The reports talk about remission or at least partial remission. The Hopkins scientists talked about reprogramming the immune system with this treatment. I know mental attitude is 80 percent of the battle. After seeing these reports, I find myself on "cloud 9", looking forward to six months of treatment, and hopefully going into some sort of remission and getting a new lease on life.
My thoughts went to the latest medical technology. I told myself if modern research will help me, the least I can do is try to also help myself. Where one month ago, I could not walk twenty-five feet, I am now walking one mile every day (in 30 minutes). I have a lot to be thankful for. I may have been through a lot these last few years, but I have also been very lucky. It could have been much worse. I am not currently in pain. I am not in a wheelchair. I am not bedridden.
My husband and I celebrated our 50th wedding anniversary, one month prior to my developing bronchitis. I was in wonderful health for the party and enjoyed it thoroughly. I am very upbeat about the chemotherapy I have just started getting. I just know it will help. I have taken control of my life and I just know there is a wonderful future for me.
New email address needed 08-06-09 SLE
Old Email Prefix: dorthy.t.smith
Story submitted 5-16-02
Story posted 5-26-02
Email address updated 6-7-03
Story Artist: Shelley Ensz
Story Editor: Judith Devlin
Radiation and Scleroderma
The story on this page is featured in our book, Voices of Scleroderma Volume 2! ISN.
SCLERO.ORG was the world's leading nonprofit for trustworthy research, support, education and awareness for scleroderma and related illnesses from 1998 to 2021. It was a grassroots movement from the original Scleroderma from A to Z web site, which was founded by Shelley Ensz. We were a 501(c)(3) U.S.-based public charitable foundation. We closed this web site and our nonprofit agency in April 2021.