June 8, 2000, was one of the low points of my life. I had just been diagnosed with pulmonary fibrosis, and tears coursed down my cheeks as I left my doctor's office. All I could think was how short my life would be, of all the things I still wanted to do, and of all the friends and relatives I would miss. I was wallowing in self-pity.
The next few months were filled with myriad visits to various specialists for MRIs, lung biopsies, x-rays, and more. Despite the strong advice of all the experts about the importance of beginning treatment as soon as possible, I was not given any medication.
After I regained a bit of my equilibrium, my sister (who is a computer guru) and I began an intensive search on the Internet to learn as much as possible about pulmonary fibrosis and any promising medications. At first, we zeroed in on a drug that appeared quite promising, but we later learned that clinical trials of the drug had been disappointing.Ultimately, I came across an article about an Austrian doctor who had conducted some very promising phase one clinical trials of interferon gamma and prednisone. (Webmaster update: The INSPIRE study of Actimmune for treating idiopathic pulmonary fibrosis (IPF) was stopped because an interim analysis showed that patients with IPF who received Actimmune did not benefit. FDA 03-05-07.)
In September 2000, I learned that additional wide-scale testing was starting in the United States. However, the trials were being done on a double-blind basis; meaning that even if I was qualified to participate in the trial, I could still end up taking a placebo rather than the actual drug! I elected to go off protocol, taking exactly the prescribed medication used in the study.
We shopped around for an inexpensive source of the interferon. My sister even went to Mexico to locate a supplier. The least expensive supplier we could find was one thousand dollars per week! At the time, no one would reimburse me for this horrendous expense but, as I saw it, I either take it or die. I was forced to sell some assets and I assumed a large debt, but it turned out to be worth every penny I spent. Now there is a chance that health insurance will start paying for this treatment.
In August 2000, I purchased an inexpensive oximeter to measure my blood oxygen saturation. Now able to monitor my pulmonary performance after commencing the treatment injections administered by my wife, there was a marked improvement in my condition. Since that time, my condition has stabilized and remained more or less unchanged for about a year.
During the past year, I have found I can participate in many activities without the use of supplemental oxygen. For instance, I can canoe, fly fish, travel by plane, hike on level ground, and dig clams. With four liters per minute of supplemental oxygen, I can bike, hike the Appalachian Trail, exercise in the gym, and generally do modestly strenuous activities. I also learned that working in cold weather uses much more oxygen than working in warm weather. Altitude has a strong bearing on blood oxygen saturation. For every one thousand- foot increase in elevation, oxygen saturation decreases by about one percent. Oxygen saturation, as measured in the finger, does not start declining until thirty or forty seconds after the activity that triggered the decline begins.
It takes the body an appreciable amount of time to accommodate a new set of conditions. For example, one cannot sit in a car for any period of time, exit the car, and start walking and expect the blood oxygen content to remain stable. It will drop until the heart and lungs adapt to the new set of conditions, in this case, walking. Breathing in through the nose and exhaling through the mouth, as opposed to both inhaling and exhaling through the nose, is very desirable; it can increase blood oxygen saturation by as much as two percent.
In the past fifteen months, I have learned a lot, and I am still learning. Most importantly, I have learned to fight pulmonary fibrosis without giving up all the activities I once enjoyed. My pulmonary performance is better now than it was in July 2000. I do not think I will be dying soon!
I plan to go snowboarding this winter and to go camping and fishing in Baja next spring. Life still stretches out before me. I think that you can also make similar strides, if you are determined to do so. Do not give up!
New email address needed 08-06-09 SLE
Old Email Prefix: swflybob
Story posted 8-6-01
Story edited VH1: JTD 9-9-03
Webmaster update note added 03-21-07 SLE
Story Artist: Shelley Ensz
Story Editor V1: Judith Devlin
Voices of Scleroderma Volume 1
The story on this page is featured in the top-notch Voices of Scleroderma Volume 1 which features articles by top scleroderma experts, including Dr. Joseph Korn, as well as 100 true patient and caregiver stories from this website! The book delivers excellent information and support for scleroderma patients and caregivers. It's a great way to inform family and friends about scleroderma, too!
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.