Stubborn, strong, resilient, annoyingly positive and ridiculously outspoken; all adjectives people have used to describe me. Are these compliments? It depends who you ask. I do know these characteristics have helped me through my darkest and brightest days living with scleroderma.
In 1985, after two years of misdiagnosing my symptoms, I was diagnosed with scleroderma at the ripe old age of ten. This was long before you could Google anything you wanted to learn about on the Internet. Growing up, all I knew about scleroderma was what my mom had told me, which was that my skin was tighter than most people’s. She did not tell me that I would develop telangiectasias all over my body, that my limbs would become mangled and deformed, or that my fingers and elbows would leak calcium. I discovered these cool party tricks for myself.
In 1993, while home on spring vacation my freshman year of college, I had the same battery of tests run that had always been done every six months since my scleroderma diagnosis. It was determined that my lung capacity had been significantly reduced and there was some sclerosis found on my lungs. The doctor suggested that I drop out of college immediately so that I could undergo intensive treatment to try to stop further progression of the disease.
Had I just been steamrolled by a truck? What was this doctor talking about? I wasn’t sick! I just had tighter skin than everybody else, right? Within the hour, I was sitting alone in my internist’s office as she explained, “There is a possibility that your scleroderma is getting worse and beginning to affect your internal organs. This could lead to sclerosis of the esophagus, lungs, heart and liver. Lisa, this would significantly impact your ability to eat and breathe, and could also affect your kidney function.”
“Well, what does that really mean? Could I die from scleroderma? People don’t die from scleroderma….do they?”
“While new medications are being explored all the time, most people with systemic scleroderma do not survive more than seven years due to the toll the disease takes on all of their major organs.”
Tears trickled down my face. Through the shock, I managed to stand up and get back to my car, where I sat and processed this confusing news. I collected myself and headed straight to the public library. Trembling, I entered the word “scleroderma” into the computer and waited for the results. I found a whopping seven articles on the subject and quickly retrieved each one from the microfiche archives.
Every article depicted scleroderma as a horrifying disease where people looked completely mangled, lost their hair, needed oxygen tanks to breathe, experienced total kidney failure, and couldn’t fit a toothbrush in their mouths. Of course, some of the articles did mention that there were varying degrees of scleroderma and some experimental treatments, but this did not apply to me. I was going to get progressively worse, look like a walking skeleton, and ultimately die. I would never get married, have children, be a teacher, travel, or enjoy life in a normal capacity. I thought I might hyperventilate. The train to doomsville was departing and I was ready to hop aboard.
The next day, we went to see another doctor who calmly told my mother and me that, although my test results did show that the disease had affected me internally, he did not think I needed to drop out of school and head for the nearest hospital. Rather, he would put me on a new medication, Penicillamine, to see if that would prevent progression of my disease. We would run all the tests again in six months and see if my organs had continued to sclerose or if they remained stable.
After six months, my tests revealed that my condition had not improved, but it had not worsened either. This was an excellent sign. I would continue to be tested semi-annually and as long as I remained stable, there was no reason to be alarmed.
Six more months passed, I was still stable. A year, two years, three years… thirteen years, and I remained stable. I married my incredible husband and became a teacher. My condition went unchanged through my first pregnancy, the birth of my son, and my second pregnancy.
In 2006, I suffered catastrophic complications after the healthy birth of my daughter and remained in the hospital for 218 days after her birth. We will never know how large a role my scleroderma played in what happened. I lost my colon and spleen, underwent seven major surgeries, had two tracheotomies, experienced severe ICU psychosis, and was temporarily paralyzed due to extreme deconditioning. More than once during my hospital stay, my family was told my chances of survival were slim.
I spent more than nine months in physical, occupational, respiratory, and speech therapy. With the support of hundreds of friends, family and medical professionals, I learned to walk, talk, eat, and independently breathe again. My children are now eleven and eight. They are the sun, moon, and stars in my sky. I am thankful to report that my scleroderma is once again stable.
My journey as a scleroderma patient has spanned three decades. I am grateful to spend every day living and working as a mother, wife, sister, daughter, friend, and elementary school teacher. When I was nineteen, I grasped the gravity of this disease. It took me a while to realize that living as if you are dying is not really living, it’s just not dying.
I have been asked by many how I always keep a positive outlook in the face of this illness. It’s simple: I don’t. I’ve taken a swim in the pity pool and have played the “why me” game more times than I can count. There have been times in my life when scleroderma has brought me to my knees. Times when the pain was so unbearable, I thought I could not persevere. Here’s the thing though, we are only given one life, one body, and a finite amount of time to live on this beautiful planet. How we choose to spend our time is up to us. We are the authors of our own life story.
We can view life as a series of disconnected events strung together by time and space. We can dwell on our misfortunes and keep a running tally of all the injustices we have endured—that’s a great recipe for a life of boredom, depression, and loneliness. We all need and deserve to take a dip in the pity pool once in a while, but let’s not spend our lives in the pool training for a misery marathon. I believe there is a better option.
As scleroderma patients, can we fight for awareness, research, and a cure? Can we come together and pledge to rage against this disease and not back down until a cure is found? I know we can! We are a resilient group of thick-skinned individuals (pun intended) who will not relent. Scleroderma sucks, but living with scleroderma should not. Let’s all commit to being stubborn, strong, resilient, annoyingly positive, and ridiculously outspoken! It is up to us to determine our future and ensure it is bright.
– Written by Lisa Helfand