Searching the medical literature for answers, Licameli found two small case studies in Europe, where doctors diagnosed PFAPA, removed the tonsils and the children got better, and decided to try this approach. It worked, with an immediate cessation of his daughters fever cycles (shes now almost 6), and other doctors began referring patients to him.
The hallmark of PFAFA is high fevers of a cyclical nature parents can often look at their calendars and predict what day the fevers will arrive. There are typically no other symptoms. Some children have sore throat, swollen glands and small mouth ulcers, but these symptoms arent very specific and can be very subtle.
On the face of it, it doesnt seem like a surgical problem, Licameli says. The tonsils and adenoids appear normal, even when you study them pathologically, yet surgery is immediately curative.
Before undergoing surgery, patients are evaluated for any rheumatologic or infectious etiologies that can also cause cyclical fever.
So what causes this condition, and why does surgery work? Licameli suspects the tonsils may harbor a chronic indolent infection to which the immune system is hypersensitive. He and his colleagues at Childrens including specialists in infectious disease, rheumatology and immunology -- plan to pursue these questions further.
In the meantime, they hope that increased physician awareness will allow children struggling with this condition to be offered surgery sooner.
|Contact: Keri Stedman|
Children's Hospital Boston