Stevens-Johnson Syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, potentially fatal allergic reaction usually triggered by certain medications or infections, in which layers of skin slough off of the affected patients as a result of cell death and sores on the mucous membranes. The disease can cause eye damage, which can lead to blindness.
In Donna Emley’s case, the trigger was the two Tylenol she took for muscle aches at her home in Fort Wayne, Indiana, prior to a planned trip to Kentucky.
When she contracted SJS/TEN in June 2015, she had been taking no more than four pain relievers per month off and on for about the last two years. In other words, very infrequently, said Emley’s husband, Dennis.
“We are not exactly sure why this time it triggered a reaction, but were told it is possible to happen even if you have taken a drug in the past without reaction,” he said.
When a 104-degree fever, blistering wounds and blurred vision sent Emley to the Medical Center of Bowling Green, she was fortunate to been seen by Vanderbilt-trained ophthalmologist Marc Moore, M.D. Recognizing the severity of her reaction, he recommended she go to Vanderbilt University Medical Center.
That decision saved her eyesight…and her life.
People affected with SJS/TEN are usually hospitalized in a burn unit as their wounds mimic burns, and these units are best equipped to provide the critical care needed to treat them.
Emley was admitted to Vanderbilt’s burn unit on Friday, June 19. Blair Summit, M.D, assistant professor of Plastic Surgery and medical director of the Vanderbilt Regional Burn Center, and his team worked on her through the night, administering hydrotherapy baths, wound care and antibiotics. An ophthalmologist was called in immediately to assess the damage to her eyes.
“A SJS/TEN reaction from acetaminophen or Tylenol is extremely rare,” said Summit. “We almost never see it. Donna had a very severe case, as severe as we see, with burn-like wounds over 40 percent of her body.”
Summit said SJS/TEN reactions like Emley’s carry a mortality rate as high as 80-90 percent.
“These patients need specialized care. It requires a team effort from the burn team, Dermatology, Internal Medicine, Ophthalmology and Infectious Diseases,” he said.
Emley says the first 48 hours at Vanderbilt were a blur.
“Because I couldn’t see well, I just had to trust what was happening and everything that was being done to me,” she said. “I didn’t second guess it. I had to believe in the people around me and what they were doing to save my life and my eyesight.
“I felt like everything was under control. I knew that in the moment they were doing everything that needed to be done. At the time, I didn’t think about what the aftermath would be—what it would be like once we got through this part.”
During her initial hospitalization, Emley was unrecognizable. The top half of her body was covered in open wounds and scabs, her body puffy with inflammation, her eyes swollen and sewn shut. She spent a month in the hospital. When her wounds healed sufficiently, she was moved from the burn unit to a step-down unit, and the focus shifted from keeping her alive to saving her eyes.
SJS/TEN caused the ocular surface of her eyes to slough off. She had severe inflammation. The scarring from this could lead to complete loss of vision. To save Emley’s eyes, she needed a specific intervention. Sahar Kohanim, M.D., assistant professor of Ophthalmology and Visual Sciences, oversaw this novel procedure.
“We transplanted amniotic membranes onto Donna’s eyes. These membranes help promote healing, decrease inflammation and minimize scarring of the eye,” said Kohanim. “This is a newer treatment. In the past, without this type of treatment, patients like Donna would have gone blind.”
The amniotic membranes would give them time to heal and the chance to recover.
Over several weeks, her wounds healed, leaving behind new deep red skin. She was slowly beginning to look like herself again.
“One of the doctors told me, ‘You don’t look like the same person you did two weeks ago,’” Emley recalls. “We’d go for a walk in the ICU and some of the nurses would say, ‘Wow, look at your skin. It looks so much different.’ I didn’t really think about how I looked until people started telling me.”
Emley was discharged from the hospital on July 17, 2015. She still needed specialized eye care as well as a feeding tube to eat because her mouth and throat were healing from the blisters.
The Emleys stayed in Mt. Juliet, Tennessee, with longtime friends for a week so they could check in with their care team and receive a few more eye treatments before heading back to Fort Wayne. Emley’s long-term follow-up eye care was arranged with a cornea specialist closer to home, with short-term appointments also scheduled at Vanderbilt.
Emley said she still has some healing to do, but has come a long way compared to where she was when she was in the hospital. The feeding tube has been removed, and her vision and eye comfort has greatly improved, though she is still very sensitive to light. In late December 2015, Emley began the process to be fitted for PROSE (prosthetic replacement of the ocular surface ecosystem) contact lenses, which will improve her vision and healing, and diminish light sensitivity.
She knows she’s lucky to be alive.
“If something happens that doesn’t appear normal, don’t put off determining if it’s serious or not. I’m blessed to be alive and my outcome could have been dramatically different if I didn’t get help when I did and where I did. I’ve always believed when it comes to health issues to be my own personal advocate, and also advocate for those I love. I know without a doubt that the excellent care I received made all the difference, and feel fortunate to be able to share my story with the hope that others will learn from everything I experienced.”