PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) is an autoinflammatory childhood syndrome characterized by recurring high fevers on a predictable schedule (typically every 6 weeks), accompanied by swollen lymph nodes, sore throat, and mouth sores, with negative standard infection tests; it responds well to steroids (resolving symptoms within 12 hours) and can be cured with tonsillectomy (approximately 80% success rate), making it important for parents to recognize the pattern and consult a doctor if their child experiences repetitive high fevers on a schedule.
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My 2-year-old spiked to 106 fever, and then it kept happening exactly every 6 weeks. And it lasted for a good 7 days.
Had to give Tylenol and Motrin to get it to come down. His throat was really red and swollen with white patches. However, everything they tested him for was negative, and he was miserable. We chalked it up to some kind of virus. And then, 6 weeks later, the same exact thing happened. We started joking that we knew when he was going to get sick, and then we realized we could literally set a calendar to when he was going to get sick. As a peace nurse, I couldn't figure out what's wrong with my child.
My pediatrician wasn't sure. She eventually referred us to ENT, said, "He has PFAPA." PFAPA. Let me see if I can remember it. P periodic, F fever, A aphthous stomatitis, P pharyngitis, A adenitis. Basically, it's thought to be an overreaction of his immune system.
It's considered auto inflammatory. His inflammatory response is probably reacting to normal bacteria that we all have in our throats, but he was getting really sick from this. So, ENT said, "Next time he gets sick, try a dose of steroids." Went ahead and gave us a prescription. Now, of course, the trick here is recognizing, "Is this a normal virus, or is it PFAPA?" Because you don't want to give steroids if it's a normal virus, because that's going to suppress the immune system. But we got really, really good at identifying the difference. Sure enough, we gave him the steroids, and literally within 12 hours, he was completely back to normal. Now, this continued for about a year.
However, we could just give him the steroids when it happened, and we could keep it under control. But he eventually seemed to grow out of it until he hit kindergarten, and he got strep, flu, and COVID literally back-to-back. And it seemed to activate something in his immune system, and he started getting PFAPA, but this time it was back with a vengeance. Literally every every weeks.
We knew within 12 hours of when he was going to get sick. We were scheduling our events around when we knew he was going to get sick. He was crying because he was always so miserable even though we could get it to pass quickly with the steroids. The fever would still shoot up really high and he was missing out on activity. So, we decided to go back and talk to ENT. He said, "There is no cure, but the only treatment is a tonsillectomy and it does have a pretty good treatment rate about 80%." We weighed the pros and cons and decided at this point this was interfering with his daily life every 2 weeks getting really, really sick. So, we did it and that was 2 years ago and he has not had a single episode. So, if your child is getting repetitive high fevers on a schedule, usually swollen lymph nodes, really sore, swollen, red throat, sometimes mouth sores, talk to your doctor to figure out if maybe it's PFAPA.
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