By the time most patients receive the diagnosis of the bacterial infection Lemierre syndrome, they are extremely ill in the intensive care unit.
But thanks to Dr. Shankar Upadhyayula, an infectious disease specialist at Akron Children’s Hospital, honor student Amanda Gamble had an early diagnosis and a quick recovery and is now back at Hudson High School completing her senior year.
Amanda’s symptoms started on Aug. 20, around the first day of school. She had a sore throat and neck pain for a couple of days that kept getting worse, along with fever and tiredness. Her mother, Cheryl took her to her pediatrician where she had a negative strep test.
According to Dr. Shankar, as Amanda affectionately calls him, patients with Lemierre’s syndrome usually present well into the disease state with blood clots in the jugular vein that often spread through the blood to the lungs and heart.
Untreated, this could lead to life-threatening sepsis.
“From the time my pediatrician called Dr. Shankar with the description of my symptoms – neck pain, fever, sore throat – he was on top of it,” Amanda said.
As Amanda continued to get worse, her pediatrician advised her mom to bring her to Akron Children’s ER.
Dr. Shankar was the infectious disease specialist on-call and recommended she be tested for mono and the Lemierre’s syndrome bacterial culprit, Fusobacterium necrophorum.
Both tests were negative, but the latter test needed 3 days to have the definitive culture results.
At home, her fever rose to 105 degrees and her symptoms kept worsening.
“That next morning, Amanda was touching me to get my attention because she couldn’t talk,” said Cheryl. “She had a severe pain in her left cheek and her neck was swollen.”
Dr. Shankar recommended a CT scan. It showed a small clot in Amanda’s cheek vein, but no clot in the jugular vein. So he sent Amanda home again after giving her IV fluids and antibiotic injections.
Before they had the bacterial culture back, Dr. Shankar suspected Amanda had early-stage Lemierre’s. On Aug. 24 the culture results confirmed this and Dr. Shankar admitted Amanda.
He treated her with a combination of IV antibiotics since patients fighting Lemierre’s can develop additional infections such as strep.
Several days later, when the swelling in her face and fever had gone down, Amanda was ready to go home.
As she and her mom waited for home instructions for her IV PIC line, Amanda began having chest pains and difficulty breathing.
Dr. Shankar immediately ordered a chest X-ray to rule out pneumonia.
“The X-ray showed loss of volume on the right lower lung with pleural effusion (extra fluid),” Dr. Shankar said. “This was unusual because generally this happens when clots from the jugular break off into the lung – with Amanda, she didn’t have the jugular clot.”
He offered her the options of either draining the fluid off her lungs or just watching her carefully. Amanda chose the latter and a second chest X-ray confirmed that the lung findings were worse.
Because she had very little symptoms of pneumonia, Dr. Shankar released her with home IV antibiotics and close follow-up in 2 days. He considered her case milder since she was treated so early and the infection didn’t spread to the jugular vein.
A week later, Amanda was back at school for half days. It took her a while to get her strength back, but with the help of her family and teachers, she caught up quickly.
“I learned that Lemierre’s syndrome is kind of a ‘forgotten disease’ which prior to antibiotics was fatal,” Amanda said. “I felt so fortunate to have had Dr. Shankar on my side – if they would have caught it later, who knows . . .”
Lemierre syndrome on the rise
Although Lemierre syndrome vanished with the advent of antibiotics, it’s been making a coming back in recent years. Dr. Shankar said he has seen 4 to 5 cases in the last 4 to 5 months.
It often is overlooked because it usually occurs in young, otherwise healthy adults.
Amanda and Cheryl learned how lucky they were to have Dr. Shankar and Akron Children’s Hospital close to home.
“They were very thorough, the nurses couldn’t do enough for us,” Cheryl said. “Dr. Shankar was in to see us every day, he was in touch with colleagues to try to get answers for us and explained everything that was going on.”
“I just loved him,” Amanda added. “He was so good to me, my mom and dad – what a great doctor!”