Rosie, pictured with Dr. John Bradley, after her harrowing journey.
By Josh Baxt
Rosie Lopez is a gregarious 14-year-old: smart, well-spoken, energetic. She likes to move and was doing cartwheels in Jesmond Dene Park, near her Escondido home, when her left arm went out from under her. Compound fractures to her ulna and radius sent Rosie straight to the emergency room.
Though a little dirt had gotten in the wound, the course of treatment seemed straightforward: clean the area, set the bones. She wouldn’t be doing cartwheels for some time. But about a week later, something strange happened.
“I woke up and my vision was a little off,” says Rosie. “I couldn’t figure out quite what was wrong, but I knew something was different.”
The symptoms got worse. That evening, her voice seemed muffled and she had trouble breathing. When she tried to drink a glass of water, she couldn’t swallow. The family went back to the ER. It was getting harder for Rosie to breathe, and her parents were having trouble understanding her speech. She was run through a gauntlet of tests at two different hospitals, but none gave any definitive answers. After about 22 hours, she was transferred to Rady Children’s Hospital’s intensive care unit and hooked up to a breathing tube.
When You Hear Hoofbeats
There’s an old saying in medicine: “When you hear hoofbeats, think of horses, not zebras.” In other words, don’t lean toward an exotic diagnosis when the obvious may be staring you in the face.
Rosie was experiencing unusually rapid, progressive paralysis without a fever, which could mean several things. Physicians from different specialties collaborated closely to make a diagnosis. The most likely cause was myasthenia gravis, an autoimmune disease that causes muscle weakness. Guillain-Barré syndrome, another immune condition, also was considered. Still, the team was not entirely sure what was happening to Rosie. There were other possibilities, such as wound botulism, but that seemed like a long shot—there are only about four cases in the United States each year, and it usually strikes intravenous drug users.
Rosie’s paralysis was getting worse. She couldn’t speak while on a ventilator and was writing on a whiteboard. But even that was becoming difficult. Her arms weren’t working properly and she could barely see. Her brain was fine, but she was becoming increasingly isolated.
“It was really hard, because I talk a lot,” Rosie says. “I didn’t know how long it would be like that, how long I was going to be paralyzed and couldn’t do anything. I was just trapped inside myself.”
Though they weren’t 100 percent certain of the diagnosis, the Rady Children’s team began treating Rosie for myasthenia gravis.
The Zebra
While Rosie was being treated, Juan Chaparro, M.D., an infectious disease specialist at Rady Children’s and physician instructor in pediatrics at UC San Diego, was running down the botulism lead. Wound cultures were growing, increasing the likelihood that some infectious agent in the park’s soil was contributing to Rosie’s condition. When the myasthenia gravis therapy didn’t work, wound botulism became a more likely candidate.
“When she didn’t respond, the neurologists recognized it wasn’t what we thought,” says John Bradley, M.D., director of the Division of Infectious Diseases at Rady Children’s and professor and chief of the Division of Infectious Diseases at UC San Diego. “We go through the most likely candidates first. When we knock stuff off the top of the list, we start treating things farther down.”
Meanwhile, Dr. Chaparro had been in close contact with Eric McDonald, M.D., deputy public health offi cer for the County of San Diego and the Centers for Disease Control and Prevention (CDC) in Atlanta. The CDC was testing the wound cultures—Clostridium botulinum is so rare, local labs aren’t equipped to do so. Rosie’s parents weathered the process with amazing composure. Though they were growing increasingly concerned about her condition, they also were confident that the Rady Children’s team would come up with the right answer.
“Hundreds of friends and family members in different parts of the world were fasting and praying often for Rady Children’s team members and for Rosie. We felt reassured that decisions and actions would be guided so that they would be effective in helping Rosie,” says Oscar, Rosie’s father.
“The most unnerving part was the uncertainty,” says Susan Lopez, Rosie’s mother. “But I also had a sense of calm that everything would turn out all right.”
Though the diagnosis wasn’t certain, the arrows were pointing toward botulism. Given her condition and the relative safety of the treatment, the Rady Children’s team moved forward with botulism immune globulin therapy. After a few days, the CDC confirmed their diagnosis. “This was the first case of wound botulism I had ever seen in person,” says Dr. Chaparro.
Treating Rosie’s Botulism
Though botulism is rare, physicians at Rady Children’s still see it occasionally. In most cases, it’s infant botulism caused by tainted food, often honey. Clostridium botulinum generates the botulinum toxin, and to treat patients, physicians must address both the bacteria and the toxin. Dr. Chaparro explains that the treatment for wound botulism has two stages: first, removing the infected tissue and killing the bacteria producing the toxin; second, preventing the toxin from further weakening the patient. That’s where the immune globulin comes in. Antibodies bind to the
toxin and prevent it from attaching to the body’s neuromuscular junctions, or the interface where neurons and muscles connect.
In addition to the antitoxin, Rosie was given strong antibiotics to kill the bacteria. According to Dr. Bradley, “Rosie had the potential to have spores still in the wound that could germinate into growing organisms and produce more toxin,” so orthopedic surgeon Maya Pring, M.D., led several procedures to clean the wound.
Gradual Recovery
While immune globulin is effective, it’s also quite slow. The antibodies remove free toxin but can’t do anything about the botulinum that has already attached to the neuromuscular junctions. The toxin binds permanently, causing paralysis until the junctions regenerate, a process that can take weeks. Rosie was in the hospital for 77 days.
Throughout the ordeal, the family had an amazingly positive outlook. “The main lesson has been that, in life, there’s always going to be challenges, but those challenging moments are opportunities to learn and grow,” Susan says.
Rosie had exceeded expectations, getting out of bed a full month before her doctors predicted. She was happy to be recovering but chafed at hospital life. “They have so many rules to keep people safe. I just wanted to get up and move!” Still, she has been a good sport during rehabilitation.
“There’s a lot of pain associated with her injury and rehab,” says Dr. Bradley. “But she’s not a shrinking violet. If she knows it’s going to be best for her, she doesn’t complain.”
While Rosie’s radius has mostly healed, her ulna is still causing problems, and she has trouble gripping. Her Rady Children’s team is keeping a close eye on her progress—they’re concerned that lingering spores in her body could regenerate the disease, and she can’t stay on antibiotics forever. Eventually, they want to write an article about her case for a medical journal.
The Lopezes are feeling a lot of gratitude for the care Rosie received. Their confidence in the Rady Children’s team helped get them through weeks of uncertainty.
“I love the approach to try to involve the whole family, and allowing the parents to be partners in their children’s care,” Susan says. “That really made a difference to our family.”
Originally published in the Fall 2017 edition of Healthy Kids Magazine