Karen Worthy shares her experience with SJS/TEN.
Last winter, Karen Worthy, Assistant Dean for Undergraduate Studies, thought she had the flu. Fever, cough, and muscle aches were all symptoms she experienced with the flu the previous year, except this was very different. As the day progressed, she began having other worrying signs that indicated not only a possible illness but a medical emergency.
During her assessment with a nurse practitioner at a local clinic, she presented with a fever of 104°F, and her face was beginning to swell. An emergency was unfolding, and time could be the difference between life and death.
Dr. Worthy was transported by ambulance to a local hospital, where her condition deteriorated rapidly. Her face was becoming more edematous (swollen), and her lips and skin began to blister. Due to the risk for airway closure, she was also facing the likelihood of intubation. Yet, as they worked on emergency treatment options, there was one thing she and her health care team had yet to figure out: What exactly were they treating?
“The doctors did not immediately recognize what the medical condition was but knew it was atypical and life-threatening. They quickly formed an interdisciplinary team including specialists from Intensive Care, Emergency Medicine, Dermatology, and Internal Medicine to care for me and my rare condition.” - Dr. Worthy
Ultimately, it was the dermatologist who diagnosed her condition as Stevens-Johnson
syndrome diagnosis proved to be a crucial step in prescribing the proper treatment
as quickly as possible. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis
(TEN) are rare diseases, affecting 1 to 2 per million people each year. SJS/TEN are
degenerative skin disorders characterized by severe inflammation, redness, and skin
shedding or sloughing. These diseases are similar, normally only differentiating in
the percentage of the total body surface area affected, with SJS involving less than
10% and TEN more than 30%. However, some cross-over between 10-30% can be diagnosed
as either SJS or TEN. Both usually occur as a reaction to a “trigger,” the most common
triggers being medications, such as sulfur or seizure medications. However, in Dr.
Worthy’s case, neither of those medications were the culprit. They can only guess
at the cause now, but it could have been something as simple as a pain reliever and
fever reducer she had taken just two days before her hospitalization. While this may
or may not have been Dr. Worthy’s trigger, every patient is different, and all experiences
with SJS/TEN are unique with variations in severity, complexity, and recovery.
These skin disorders leave patients susceptible to infection, multi-organ system failure, and other severe complications, which is why individuals affected by SJS or TEN should be treated at a multi-disciplinary burn center. Dr. Worthy was immediately transferred to the Joseph M. Still Burn Center at Doctors Hospital in Augusta, GA.
"Medical Director, Dr. Fred Mullins, was the first person I talked to when I arrived at the Burn Center," said Dr. Worthy's daughter, Dr. Ja'Pel Sumpter, a gynecologist in North Carolina. "He brought me into a room and told me the prognosis and exactly what to expect. He was amazing, and any time I had questions, he was always more than willing to discuss them. You could tell from his humble disposition and compassion he was made for this job. There aren't many people who can build such a rapport the way he could with his patients and their families. My mother always said, 'In life, but especially in health care, people don't care how much you know until they know how much you care.' He got me through the initial shock of everything," she shares.
Dr. Sumpter had never been to Augusta and only had an overnight bag. She was alone in a strange city, and after getting her mother admitted, by the time she left the Burn Center to look for a place to stay, it was 3:00 a.m. That is when the Burn Foundation of America intervened. They offered her a room in the Chavis House across the street from the hospital, which offers free lodging and daily meals for family members and loved ones of patients in the Burn Center during the extent of their stay and recovery period.
"The Chavis House was an amazing resource to have for people like myself who have no local family and are there alone. I quickly started building relationships with the other family members. We stayed in the house together, prayed together, and we would check on each other daily to make sure everyone was okay. We became each other's support system. It was a very trying time, but it also rebuilt my faith in humanity," says Dr. Sumpter.
Dr. Worthy and her daughter were in Augusta for a month, and Dr. Worthy experienced extensive physical and medical changes. Her outward appearance and her overall health changed as SJS/TEN ran its course. "SJS/TEN causes the body to go through a complete regeneration, and you can't stop that process. My skin sloughed off on several body areas, and I had to have two skin graft surgeries. My nails fell off, and I became legally blind in my right eye. I was intubated and on the ventilator for approximately two weeks to protect my airway," she says.
In addition, her shoulder-length hair had to be shaved in the process. Her skin both looked and felt different, with a new sensitivity to direct sunlight and her eyesight deteriorated. But none of that mattered to her. She saw a different person on the outside when she looked in the mirror but knew what made her who she was and what she stood for remained the same. She was grateful and appreciative of every day.
"I know who I am, and I know the journey I have traveled. These physical aesthetics are not important to me. My family and I will move forward from today. Every day is like the first day of the rest of my life. It's all a blessing; I am blessed!" - Dr. Worthy
Dr. Sumpter says that her mother was a trooper and very motivated. Once she was extubated, they got her out of bed that same day. Because of her nursing background, knowledge, and skills, she knew the importance of ambulation as foundational for beginning her recovery process.
No matter the trials, Dr. Worthy is steadfast in her faith in God, her love of family and friends, her positive spirit, and her nurse’s intuition. She feels it was the process she had to go through, and nothing could stop or alter that process. “Through God’s unwavering grace, the prayers and love of my family and friends, and the skill and knowledge of my medical team, today my skin has regenerated, my vision is 20/20 without corrective lenses, and I walk over a mile a day. I do not take credit for my progress—I give all the glory to My God Almighty,” she says.
To this day, her providers still do not know what caused her condition. Dr. Worthy shares, "I've had people ask, 'Well, you must have asked God a million times why you?' Never. Not once did I ever ask God, 'Why me?' I asked Him, 'Why not me?' I know everything happens for a reason, and this challenge only strengthened my faith in God."
Last year seems like ages ago now that Dr. Worthy has resumed her role at the college. As a nurse and a professor educating future nurses, she feels she is in the right place and profession. She is so proud and thankful for all the nurses who were and still are a part of her SJS/TEN journey.
Dr. Worthy says, “I would not have chosen a different profession. Nursing is embedded in the core of my heart and soul. I am passionate about nursing, education, and my students as we educate future nurse leaders, stewards of the community, and future researchers. We are phenomenal providers and a vital part of the interdisciplinary healthcare team. My family and I could not have asked for a better experience during such an unexpected, vulnerable time. As nurses, their compassionate prayers along with genuine acts of kindness and caring enhanced a positive outcome. We are the heart of health care!”
Adapted from the Burn and Reconstructive Centers of
America Patient Stories