Cook Children’s saves teen from rare infection
Briannah Nichole Buckley’s ordeal began with a small red purplish bump on her shin. A little over a month later, her leg had to be amputated.
The growth was first thought to be a staph infection but even antibiotics didn’t work very well.
The irritation would improve a bit, then worsen gradually. By the end of July, 2011, the bump had increased to about the size of a silver dollar. Her parents and doctor were puzzled. On Aug. 3, she was sent to the Cook Children’s Emergency department by a local pediatrician, because she was not improving with treatment.
The experts at Cook Children’s immediately began searching for answers, but found frustration. In the meantime, the anxiety of Briannah’s parents, Shawn Buckley and Brandi Teplicek, grew. What was happening to their daughter?
“It was devastating,” Teplicek said. “We didn’t know which way we were going. Nobody knew how to treat it or what it was. We didn’t know how it was going to turn out. We just knew it wouldn’t go away.”
The Infectious Disease team at Cook Children’s, led by Donald Murphey, M.D., thought they solved this more than month-long mystery when they initially discovered the common bacterium, pseudomonas aeruginosa.
But the rash came back, bigger and stronger than ever. Something strange was happening to Briannah and with each passing day, the rash got worse and was slowly spreading in her shin. Signs of a fungus were seen in this area, but it did not grow in the Cook Children’s lab.
Dr. Murphey is a well respected and nationally known ID doctor and with the help of a team of experts at the Cook Children’s Medical Center’s state of the art genetic testing was used to identify the cause of this infection as a very rare germ similar to a fungus and also to an algae.
Jim Dunn, M.D., the medical director of Microbiology at Cook Children’s, enlisted the help of an outside lab to take part of one of the biopsies of the tissue and use DNA sequencing to find the culprit. The doctors, nurses, pharmacy and therapists could then focus on treating this extremely rare infection specifically. This germ is called pythium insidiosum. This is so rare that only a handful of cases have been seen in the United States. This is a different germ than any others causing infection in humans. It often does not respond to treatment with antibiotics. It does respond to surgically removing the infected tissue.
“The doctor who told us, didn’t even want to tell me because she was afraid we would Google it,” Teplicek said.
How long did it take before they went to the Internet? “Immediately,” Buckley said. What they found on their search was frightening. Human infections are rare, but when they do occur, the consequences are severe and often fatal.
The infection was spreading. Briannah’s left leg looked like a gruesome, special effect in a horror movie. Her skin below her knee and in the front of her leg was gone. You could see bones and tendons. Doctors made the life-saving decision, the only option was amputation.
Briannah is autistic and her parents say that may have been a blessing for her during this whole ordeal. Because of her autism, Briannah has a reduced response to pain, allowing her to better cope with the 10 surgeries she received during her nearly three-month stay at Cook Children’s. The surgeries at first were a desperate measure to save her leg, the final surgery was to save her life.
“She helped us make the decision,” Teplicek said. “The night before the amputation, Briannah told us she was done. To take it away. It was a hard decision, but it was the best decision. It was the only decision.”
Today, doctors believe that stagnant water is a cause of pythium insidiosum, and other dangerous bacterium. Briannah’s parents both want to use their experience to warn other parents about the dangers of stagnant water and the harm it can cause. They believe that Briannah was playing in a creek or puddle near her home to contract the bacterium. The family also wants to raise awareness of pythium insidiosum to increase research and find a cure to a disease that while rare is seeing more cases in humans globally.
“I call it an invisible monster,” Buckley said. “You can’t just do a blood test and say you got this. Surgeons can’t see it while in surgery. That was the hardest part for them. I feel like it’s my responsibility now because it is such a rare thing to let people know about it and warn them about the dangers of stagnant water.”
Briannah’s parents feel like they owe it to their daughter to let their voices and be heard and both of them marvel at their child’s bravery through all of this. Her dad said that the night before the surgery, her uncle, a pastor, gathered everyone around the room to say a prayer. Briannah startled everyone when she said no to her uncle praying. But they smiled when she demanded she lead the prayer. She prayed for her surgeons and her family. Then chastised everyone for crying.
Surgeons amputated Briannah’s left leg 15 centimeters above her knee on Oct. 5. For the 17 doctors and countless man hours put in by her doctors, the medical team at Cook Children’s finally believe they’ve ended this ordeal for Briannah.
“Brianna is a beautiful, joyful girl who has been through a trying ordeal, along with her very supportive family,” Dr. Murphey said. “We hope she is cured of this devastating infection. We tried everything possible to cure this infection short of amputation, but there was no lasting benefit from multiple treatments. This was a very difficult and emotional situation for all of the family and staff involved. She has done very well with amputation, without it she would not have survived this infection. The challenge of finding this rare germ and caring for her shows the strengths of the team here at Cook Children’s Medical Center. I am happy that we have Brianna safe and healthy.”
A few days after the surgery, Briannah sits in her wheelchair entertaining her nurses. She has a Halloween doll that shakes as it sings Elvis songs. Briannah wiggles in her chair, singing along and then laughs. In her room, known as the ‘happy place’ because Briannah doesn’t allow anyone to get sad, her parents are tearful talking about everything their daughter has gone through lately. Then her dad looks out and sees her giggling. He smiles too.
Finally, after months of mystery and anguish, everyone can smile.