She needed pediatric specialists to combat a rare medication reaction.
When Sydney Marble’s medical condition took a dramatic turn for the worse it happened very quickly.
In November 2009, Sydney developed what seemed like the flu. Her symptoms, however, progressed from a mild fever and cough into gasping for breath and spitting up mucus. But it was a rash of dark circles covering the 4-year-old’s chest and back that most alarmed doctors at the hospital in Hampton where her parents took her initially. When the doctors there couldn’t diagnose Sydney’s strange illness, they told Renatta and Craig Marble that their daughter needed to go immediately to the emergency room at CHKD.
“They told us something really wasn’t right – this was not a normal flu or pneumonia,” Renatta recalls. “That’s when things started to move very fast. In the ambulance and then in the hospital, I was watching my daughter deteriorate right before my eyes. She looked horrible. I know now that the right doctors got to her just in time.”
The blisters in Sydney’s throat and mouth and on her skin were becoming open wounds as they sped to CHKD. As frightening as it was to her parents, the dramatically developing symptoms were the key to her quick diagnosis when she arrived at CHKD. Pediatric intensivists there immediately realized Sydney’s condition was a severe case of Stevens-Johnson syndrome, also called SJS, a rare disorder in which skin and mucus membranes react severely to a particular medication or infection.
In Sydney’s case, the trigger could have been an antibiotic prescribed to her days before she got to CHKD. Antibiotics in combination with other medications, such as ones taken for flu symptoms, are known triggers for SJS.
Pediatric critical care specialist Santa Johnston explains that symptoms can include facial swelling, hives, skin pain, rash and blisters, all sometimes preceded by flu-like illness. “The top layer of skin actually blisters, dies and sheds off,” Dr. Johnston said. “In some cases, like Sydney’s, the worst damage occurs in the mouth, nose and eyes, which can interfere with breathing, swallowing and eyesight.”
Sydney had a severe type of SJS that affected about 30 percent of her body surface area. “Serious complications can include infection in the bloodstream or of fluid surrounding the brain, vision loss, organ damage and permanent skin disfiguration,” Dr. Johnston said.
Since the skin acts as a barrier to germs getting into the bloodstream and causing infection, patients are at serious risk for infection when 30 percent of the skin’s surface is shed. The mortality risk for Sydney’s type of SJS is between 10 and 15 percent.
“SJS, like many other diseases in children, requires a variety of specialists to maximize the child’s best chance of doing well,” Dr. Johnston said. “It truly does take a village, and we have a really good village at CHKD.”
For Sydney, that meant doctors, nurses and specialists from critical care, infectious disease, ophthalmology, dermatology, physical therapy and nutrition throughout her 17-day stay at CHKD.
Sydney got much worse before she got better, her mother recalls. “After she arrived at CHKD, the blisters in her mouth and throat made breathing such a struggle that doctors had to insert a breathing tube and sedate her to avoid further injury to her airway.”
She was given medication that relieved her pain. Doctors and nurses kept her hydrated with intravenous fluids, regularly cleaned out her breathing tube and kept her open sores clean and covered with antibiotic cream and, in some areas, gauze.
They were particularly careful to keep her eyes and eyelids medicated to avoid excessive scar tissue from forming.
“We treat these patients like they have a serious burn,” said Dr. Laura Sass, a pediatric infectious disease specialist at CHKD. “It’s intensive supportive care; there’s no particular medication to slow the progression of the disease. We concentrate on getting the patient through the worst of it without medical complications and keeping them as healthy as possible as they heal.”
Renatta and Craig took turns staying at the bedside of the youngest of their five children. “Sydney shed skin and tissue in her eyes and mouth – including her taste buds, which had to grow back – and from the bottoms of her hands and feet,” Renatta recalls. “Blisters covered her from her face down to her legs. Her lips swelled and her eyes could open only in slits. There were days when Sydney couldn’t see at all. “Dr. Santa warned us she’d be afraid, and sure enough, her first words were, ‘Mommy, I’m scared,’” Renatta added.
But every day, Sydney got a little better. Her lips and mouth were among the last areas to heal, which made eating difficult. “The inside of her mouth was still pretty raw, so everything felt and tasted funny to her,” Craig Marble said.
Sydney’s team of caregivers kept her on intravenous feeding as a nutritionist encouraged her to eat by using a sticker chart to celebrate every bite and swallow. Slowly, with the help of a numbing “magic mouthwash,” Sydney graduated from whole milk to small bites of chicken nuggets and ice cream.
“The CHKD staff was just so kind to her,” her mother said. “The last thing that had to happen before we could go home was getting her to eat, and they had just the right skills to get a 4-year-old to eat.”
A year later, Sydney is a healthy 5-year-old who likes to color, participate in church activities and practice writing words she has learned in her kindergarten class. Bouncing around her home in a cheerleading outfit and a pair of wings from her Halloween bumblebee costume, she is outgoing with visitors. Asked about her hospital experience, she said, “I just remember when I had to take a poke,” referring to her numerous blood draws. Did she have a favorite doctor or nurse? She responds enthusiastically, “I had lots.”
Sydney still has some minor skin discoloration on her body, cheeks and lips, but doctors say that should fade over time. She has a little scar tissue remaining at the back of one eye, but her mother says it hasn’t harmed her vision. Sydney does wear glasses, but so do many others in her family. She will avoid amoxicillin and the painkiller ibuprofen, another medication she took shortly before her diagnosis, as a precaution to guard against the unlikely return of SJS.
“This is a hypersensitivity reaction to drug exposure, not really an allergy,” Dr. Johnston said. “Is it possible this could happen again? Yes. But SJS is relatively uncommon, so it is unlikely it will happen again to Sydney.”
Drs. Johnston and Sass practice with Children’s Specialty Group PLLC at CHKD.
This story was featured in the first quarter 2011 issue of KidStuff, a publication of Children's Hospital of The King's Daughters. Click to read more patients' stories.