Search Results

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?”.

Late February 2005, I developed symptoms of fever, headache, and nasal congestion. To alleviate my symptoms, I decided to take different over the counter cold medications. Several weeks later, I developed a variety of symptoms including hives, severe itching, and a red and very hot rash. The itch across my body was so tremendous that my wife took me to the emergency room. The physician in the ER diagnosed me with an allergic reaction to my cold medications and prescribed me with oral corticosteroids to help bring down the severity of the itch and alleviate my symptoms. This medication was of no aid, as my itching and fever persisted. I also began to develop other symptoms such as general malaise, and painful sores across the inside of my mouth and body. I was not the same person. The sores in my mouth made it so difficult to swallow, that I resorted to consuming fluids through a straw. With the aid of our family doctor, we were soon were arranged a consult with Dr. Willoughby from Guelph who diagnosed me with Stevens Johnson Syndrome. Dr. Willoughby took swab cultures of the blisters and provided me with antibiotics for the infections I have developed. She suggested that I stay at home, rest in bed, and continue to have adequate fluid intake. We discussed the possibility of being hospitalized, but she recommended I stay home as the chances of obtaining an infection from the hospital would be greater. After a couple months, the hair on my body began to grow back, the wounds and sores began to heal with healthy skin, and the sores in my mouth were gone, allowing me to eat again. I slowly developed my strength after regaining the ability to eat. I have thankfully not had any recurrence of these symptoms since that incident as I don’t take any medications at this time. I am very thankful that my doctor first recognized these symptoms in its early stages.

I was a completely healthy individual until I got the flu vaccine in October 2019. I was tired and a little achy the next couple of days after but did not think much of it. Three days after receiving the vaccine, my eyes swelled shut. I went into the local emergency clinic and it was suggested that I had a contact allergic reaction to something. I was given IV steroids and IV antihistamines and sent home. The next day, I developed a fever and sores in my mouth. After getting assessed again at the emergency clinic, I was sent home to recover. They thought I just had a cold or flu. I kept getting progressively worse after I went home. More sores developed in my mouth, so much so that I was unable to eat. I had no energy and was in bed all day. I went back to the emergency clinic and was sent home again even before getting assessed as they said there was a 5 hour wait time and I was told I just needed fluids and rest. The next day, I started getting rashes on my torso. After 5 days from initial symptoms, and 4 emergency visits, they finally admitted me to the hospital. I was diagnosed with SJS by the admitting internal medicine doctor and was in the hospital for 10 days. During my stay, none of the doctors or nurses have ever heard of SJS and all they could do for me was ease the discomfort and pain that I was in as there is no actual treatment/cure for SJS. Today, I am mostly recovered except for my eyes and mouth. I have no tear function so they are extremely dry and sensitive to light. I’ve had 3 corneal ulcers because of this, and I am still waiting for the ulcers to heal so that I can pursue other treatments that will ease the dryness. My mouth is healed; however, they are still extremely dry. It definitely has been an emotional journey and I am grateful to have found this SJS group.

The impact of Stevens Johnson Syndrome affects each victim differently depending on the severity and duration of the illness. Here’s one victims’ story who asserts she escaped the destructive course of SJS with relatively little damage. It all started with feeling flushed and slightly feverish… “I must be coming down with a cold” were my thoughts. Fast forward a few hours and now my eyes were starting to burn, really badly. I could only keep them open for a few seconds at a time before having to close them for some kind of relief. Now my body temperature is beginning to act very, very strangely. I would be freezing cold one minute, cover up with a blanket and be burning hot the next minute. Not just hot – burning hot. That night was the worst I have ever experienced. My body temperature was raging out of control and I could barely open my eyes for the pain. The next morning I awoke with what looked like a heat rash all over my body. Although concerned, I thought it normal since my body temperature had been so hot the night before. As the morning continued, my health continued to plummet, and the rash became increasingly purple and hive-like. It was the craziest rash I had ever seen. My husband and son were planning a trip to leave for the day so I decided I would go back to bed to try to sleep off whatever was going on. As my husband was just about to head out the door, he turned to look at me and said “You know what – you really don’t look well and I want to check something on-line before I leave you.” Two minutes into his search online regarding side-effects of the medication I was taking there it was – “Show me your tongue” he said to me with a look of fear on his face. As I stuck out my tongue, his faced became very pale and the next words to exit his mouth were: “GET IN THE CAR NOW!!!!” The next 24 hours were a blur of needles, shots of adrenaline, bags of cortisone, Benadryl, and very concerned faces of nurses and doctors that were caring for me. Although the rash and my vital signs had stabilized the hospital staffs were very unsure as to what to do next since the rash was not going away… Today, nine months later. My eyesight has deteriorated; I have chronic fatigue, chronic pain, memory and concentration issues, and difficulty coping with stress and anxiety, sun and heat sensitivity. My skin that once felt like sandpaper for months almost feels normal now. Despite all this and in spite of my bad luck, I consider myself to be extremely luck. I suffered a rare case of SJS that was caught in the early stages of development. My skin did not develop painful burn blisters, I was not blinded, my internal organs are still functioning properly. My hair only fell out a little. I’m alive. Compared to the path of destruction that SJS can create, I managed to escape with relatively little damage.

A Research titled: – STUDIES ON GENETICS is currently been conducted by: Dr. Bruce Carleton Chair, Division of Translational Therapeutics Faculty of Medicine University of British Columbia As per Dr. Carleton, the central theme of his research program, Pharmaceutical Outcomes and Policy Innovations (POPi), is the study of drug therapy with the goal of improving human health and quality of life. He is interested in developing models for evaluating drug effectiveness, medication use models designed to improve patient health, and effective surveillance systems to improve the safe use of medication. He also has an interest in the clinical management of patients who experience serious adverse drug reactions. You can learn more about Dr. Carleton’s research program by emailing him at the email address listed below. FOR DETAILS ABOUT THIS RESEARCH PROJECT: Please email: bcarleton@popi.ubc.ca Survivors and family members of Stevens-Johnson Syndrome & Toxic Epidermal Necrolysis are encouraged to get involved. #genetics #translationaltherapeutics