Scleroderma, Now and in the Future: An Interview with My Mom
Scleroderma is a chronic autoimmune rheumatic disease in which the body attacks its own connective tissue. We’ve explored scleroderma in general and learned that it can cause much more damage than “hard skin.” It can cause damage to the lungs, heart, kidneys, esophagus, and gastrointestinal tract in patients who live with the disease.
My mom, who lives with scleroderma and lupus, has agreed to share her story with us today. I’m very glad that she’s willing to give one patient’s view on the disease and the effects it can have on the body and one’s future.
Please tell us about your diagnosis. When were you diagnosed with lupus? What were your early symptoms?
I noticed strange symptoms in fall 1987 after a serious bout with Rocky Mountain Spotted Fever. I began having unusual health issues in that with each illness something was left “broken.” First, my inner ear malfunctioned after the flu; then my pancreas quit regulating my insulin levels properly; and by 1994 I was having difficulty breathing during a trip to Colorado. Finally, I was diagnosed with systemic lupus in early 1995.
I was very lucky because Oklahoma held a lupus symposium twice yearly to educate lupus patients. It was perfect timing. At the spring symposium, the main speaker explained exactly what was going on with lupus. He said that when you’re young your thymus gland helps the body learn to recognize what is your body and what is not so that your immune system works against “invaders.” Somewhere along the road the body loses its ability to differentiate itself from virus, bacteria, etc. so that when one becomes ill, the body’s immune system is just as likely to attack itself causing damage. I thought, “what a simple explanation.”
How were you diagnosed with scleroderma?
I was under the care of a rheumatologist for years when my doctor diagnosed CREST syndrome. The name CREST was used to identify symptoms: Calcinosis in soft tissue, Raynaud’s syndrome, Esophogeal dismotility, Sclerodactyly, and Telangectasias. I was never told that CREST was associated with scleroderma because I only had the first two symptoms as part of the syndrome. I thought it was just another feature of the lupus. The first time I heard scleroderma as a diagnosis was during a doctor’s visit when my rheumatologist referred me to a scleroderma specialist. At that time, I really didn’t know what scleroderma symptoms were.
Last week we learned that there are many different types of scleroderma and that the designation CREST syndrome is no longer used. What form of the disease do you have?
I have limited cutaneous systemic sclerosis, a name which is not nearly as understandable as the acronym of CREST. Limited cutaneous systemic sclerosis is also sometimes referred to as Mixed Connective Tissue Disease. I am still learning about the disease and its different names.
Scleroderma can affect several different body parts and functions. What are your primary symptoms?
My symptoms occurred over time. My esophagus often didn’t push food to my stomach effectively causing me to choke on food, but I managed that by drinking liquid during meals. Next, I began having serious gastric reflux and was eventually diagnosed with gastric paresis, where the stomach quits “churning.” Because food stays in my stomach, I now have a problem with aspirating food into my lungs. More recently, I became very short of breath and was finally diagnosed with pulmonary arterial hypertension (PAH), the most serious of the conditions. With PAH, the cardiovascular structures in the lungs stiffen and narrow causing “back pressure” in the right side of the heart. The heart is eventually weakened from working too hard against the pressure causing heart failure.
Scleroderma is an incurable and progressive disease. Are there effective treatments which help with your symptoms?
While there are no drugs yet to stop progression of scleroderma, there are drugs to help with symptoms. Currently, I take Nexium to control acid production, Domperidone to help push food out of the stomach, Questran to control diarrhea, Spironolactone as a diuretic, and Revatio to increase the nitric oxide in my lungs, thereby opening the arteries more. Interestingly, Revatio is just another form of Viagra which helps open blood vessels.
Do you take prednisone for inflammation or any disease-modifying drug?
I took prednisone 15 years for my lupus, but I no longer take it since my lupus doctor became concerned after a research study showed bones could appear dense but were more brittle. When I asked my scleroderma specialist if there were any medications that would help control scleroderma, she said there are none. I participated in an NIH study in which I received pulmonary rehabilitation to determine how exercise affected everyday functioning and endurance. Part of the rehab included education, which I can’t overly stress, is critical to understand and better manage chronic disease.
What are your concerns for the future?
Right now, I’m concerned with quality of life. When I was first diagnosed with PAH, I missed a lot of work, seemed unable to do anything, and became very upset because I thought I would have to quit working and couldn’t afford to retire. But, the Revatio helped tremendously and I’m still working although I don’t have much energy left after work each day. This impacts my ability to accomplish house and yard work or just go to a movie. I spend my weekends resting so I can work during the week. I am saddened that I can’t play with my grandsons, ages 10, 8, and 4, as I used to because I tire quickly or just plain can do things anymore. I don’t travel much because I just can’t do the walking, especially up inclines and stairs.
The most serious threat is mortality. Studies have shown that those patients diagnosed with PAH, where the underlying cause is scleroderma, usually live 5-10 years after PAH diagnosis. This affects so many decisions at my age (61.) I was diagnosed with PAH two years ago in 2009.
Thank you very much for sharing your story, Mom. We learned so much from personal stories such as yours.
For More Information:
If you are interested in learning more about scleroderma and related diseases, make time to explore the enormous amount of medically referenced and documented information available from the International Scleroderma Network. The Scleroderma Foundation also offers detailed articles on various aspects of the disease.