My name is Kathleen, and I am from Washington, USA, just north of Portland, Oregon. I had my first reaction to Steven Johnson Syndrome (SJS) in 1965 at the age of 6, however, I was not formally diagnosed with this disease until much later. Instead, I was told that I had a skin disease which was a form of “erythema multiform”, an infection that causes a rash. SJS was extremely rare back then, and so it took a while for me to be diagnosed.
It all started with what my mom thought was a simple cold. My doctor visited me at home and gave me penicillin, which only made things worse. By the next day, my mouth developed sores which increasingly became worse; meanwhile my body became covered in red welts. The doctor returned but he could not diagnose the problem, so he called an ambulance to transport me to the hospital. I spent the next three months in quarantine in the burn unit with no one knowing what was wrong. I shed parts of my skin each time a nurse would come in to rotate me on the bed. Throughout my hospital stay, my only solace was my mother who came and read to me every night. Thankfully, after a while my skin started to regrow.
After this, I began to experience problems with my eyes. My eyelids started to grow and fuse together, and the doctors had to cut the lids open. My eyes were in constant pain from the lights and even the air. I spent the next 6 months being home schooled and avoiding light as far as possible. My ophthalmologist tried for years to help me, but finally suggested that I go to Boston Massachusetts General eye clinic in Boston, Massachusetts.
Finally, a few years after having this disease, I was diagnosed with SJS. I was now 10 years old. At that time, I was told that only 5% of cases survived and that I was a lucky one. I disagreed with that.
The pain in my eyes was constant. I had severe light sensitivity, dry eyes, no tear ducts, and pain as the lashes grew inward scratching my corneas. At that time, we lived in Rhode Island, USA and for years, my mom would take me by bus every Wednesday to the Eye Clinic. At the clinic, I was seen by the medical students as “Patient X”. I tried all kinds of experimental solutions to simply improve my comfort level as well as having my eyelashes removed each time. When I was 17 (after getting my learners permit to drive), my local doctor said that my vision and light sensitivity were at such a poor level that I was legally blind. It was devastating! At the time, I was living on my own and went into a depression for a very long time.
I eventually met someone at my local Commission for the Blind who helped me understand what my life could be like despite my disability. Nevertheless, I went to attend college, had a baby that I raised well, and eventually got a guide dog. Life was getting better once I accepted the condition with which I must live. However, my vision would just keep getting worse with my eye lashes scratching my corneas. Then my life changed…
After 25 years of being a guinea pig at the Boston Massachusetts General with Dr Dolman, he referred me to the Boston Foundation for Sight. I thanked God that day since I was accepted into the trial program for the scleral lens that Dr. Perry Rosenthal was developing. My life changed so much that first fitting although it took months to finally be able to wear the device. But it was so worth the wait. Now, my light sensitivity is nearly non-existent. There was even a good side effect. My vision improved dramatically! I am still legally blind, but I can now read with the aid of magnification and no longer need a guide dog.
I have been wearing the scleral lens for 25 years with many upgrades and was recently fitted for my 89th lens. I still cannot remove my own eye lashes and need the doctor to do it every 6 weeks. However, at this stage in my life, I have decided to take all my experience and use it to help others with low or no vision. I believe am making a difference to many others by helping them to adapt to their new lives. Now, I am a “Sight Loss Instructor” for adults who have recently lost their vision due to various reasons. I am also a Program Director for an Adult Summer Adventure Retreat.
Besides my eyes, my lungs have also been affected. I was told for years that my breathing problems were due to being in poor shape. I was also treated for asthma before, and now I am being treated for Chronic obstructive pulmonary disease (COPD). I have never smoked in my life, yet my lung operates at 63% capacity. I have other medical issues as well, but to date no medical personnel have been able to definitively say if these too are side effects of SJS.
SJS is a painful experience and I find it so hard to believe that it still affects so many people of all ages. I am, and always have been an independent person. But when my eyes are hurting, all I want to do is to hide under a blanket. I have tried but cannot pull my own lashes apart and with the Covid-19 pandemic, a visit to the doctors is nearly impossible. Still, I am hopeful that things will improve as time goes by. Finally, although I have been an SJS survivor for over 50 years, at times I look towards the heavens and wonder, “Why me, Lord?”.