Ashleigh came to the Emergency Center at Johns Hopkins All Children’s Hospital in late September after a fever that wouldn't break. She had not been feeling well for days, and the morning she came in, she began vomiting and developed a body rash.
“I’ve always said that if she were ever sick, that this is the one place that I would go,” says Ashleigh’s mom, Katie.
Until that life-changing day, Ashleigh had been a very healthy child. But In the Emergency Center, the 8-year-old girl started having seizures. Her illness quickly became a mystery as her blood work, cat scan and spinal tap weren’t providing definitive answers. At this point, she was admitted to the pediatric intensive care unit (PICU).
“When she was admitted to the PICU, she was very lethargic and difficult to arouse, with a very concerning neurological exam,” explains Thomas A. Nakagawa, M.D., FAAP, FCCM, chief of the Division of Critical Care Medicine and director of the PICU at Johns Hopkins All Children’s. “Additionally, she had extremely high protein levels in her cerebral spinal fluid after she had a spinal tap done to look for infection in her brain.”
The protein being really high generally means the white matter that connects parts of the brain is breaking down or it could be related to an infection, but the cerebral spinal fluid lab results were not characteristic of a child with meningitis.
“She was clearly encephalopathic, which means her brain function was abnormal, which could be transient or permanent,” Nakagawa says.
Nakagawa suspected Ashleigh had acute disseminated encephalomyelitis (ADEM). Ashleigh’s condition continued to deteriorate through the day with more seizures. She required placement of a tube and a ventilator to help her breathe. She slipped into a coma and there was concern that pressures in her brain had increased to life-threatening levels.
“Ashleigh was literally dying in front of us,” Nakagawa says.
The team instituted more aggressive therapy, which allowed them to reduce the brain swelling. This gave enough time for neurosurgeon George Jallo, M.D., medical director of the Johns Hopkins All Children's Institute for Brain Protection Sciences, to place a monitoring device into Ashleigh’s head using a small drill. Her brain was experiencing 10 times the amount of pressure compared to that of a normal brain. ADEM was likely her body’s extreme response to a viral infection. Keeping Ashleigh alive hour by hour became the goal.
“The virus stimulates something in the body and how that virus reacts in the body is dependent upon our immune system, and everybody reacts differently,” Nakagawa says. “There’s no specific medicine or surgery that can cure ADEM. Sometimes plasmaphoresis (which helps clean the blood), steroids, or immunoglobulin are used, but for the most part, supportive treatment is required.
“The beauty of having that monitor that Dr. Jallo put in to measure pressure in the brain was to see the response to our therapy over the course of a few hours,” Nakagawa continues. “The ability to sit with our medical and surgical colleagues and talk about these cases and to work together is a commitment to our investment in children’s health, and making sure they get the best care possible in this institution.”
Caring for Ashleigh took the collaboration of multiple clinical staff including other PICU doctors, nurses, respiratory therapists and pharmacists, as well as the support from the hospital’s infectious disease specialists, neurosurgeons, neurologists and palliative care team.
“Dr. Nakagawa is amazing,” Katie says. “Everyone in the PICU is fantastic. They never once hesitated to ask or answer a question or take 30 minutes to sit down with you and explain even though they probably explained it 50 times already.”
Slowly but surely, Ashleigh’s body fought off the virus and her brain swelling decreased. She was able to go home right before her ninth birthday and the holiday season after spending more than a month in the hospital.
“It’s still very emotional—the scariest thing I’ve ever been through for sure,” Katie says. “We came very close to losing her quite a few times. Dr. Nakagawa and his team saved her life multiple times.”
“The fact that she just woke up and is walking and talking with minimal deficits is just truly amazing,” Nakagawa says. “It’s such a privilege to work with my colleagues in the PICU and the many specialists at Johns Hopkins All Children's. They are really great doctors, nurses, respiratory therapists, and so many other support staff that work so hard to provide the best care possible for patients and families. These are the cases that we reflect back on and they allow us to keep coming back doing this job day after day.”