Let's talk about PFAPA

When Jack was very, very young, he started to be plagued by recurrent fevers with incredible periodicity. Every four - five weeks, he would get a really high fever (~103 - 104) which lasted for five days straight. It was easily treatable with children's Motrin but came back immediately at the five hour post-med mark. His tonsils were enormous, like giant boulders moving towards each other, and the glands just under his ears towards his throat were regularly enlarged. But his throat never hurt, and he was never contagious. The fever would arrive, he'd lose his appetite for the duration, and then everything would go back to normal. Until the next bout. For at least a year, doctors told me he kept catching random viruses. But no kid catches a virus with the exact same presentation, month after month like clockwork. We saw specialists, we changed pediatricians, J was missing a week of school of each month (thank goodness it was only  nursery versus elementary!). Finally, finally, during a random weekend trip to the pediatrician, we saw an elderly doc as our usual physician was off call. This wizened man who'd obviously seen years of everything under the sun suggested we try Cimetidine, aka Tagamet. It's an over-the-counter histamine blocker that prevents stomach acid production but docs have found that it also boosts the immune system. He seemed to recall it linked to successful preventive treatment of PFAPA, or Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome.

Believe me, no one can ever recall what PFAPA is an acronym for so your main take-aways should be: recurrent fever with clockwork regularity perhaps accompanied by mouth sores (the aphthous stomatitis, aka ulcers), sore and red throat (also giant tonsils) and adenitis (enlarged glands in the neck). Jack never had an aphthous ulcer but, as I mentioned, had the fevers and adenitis. In fact, his tonsils are now just huge all the time, and I have to remind physicians not to fret about their size.

The Cimetidine was an incredibly benign wonder drug. After starting it - a half-teaspoon twice a day- Jack was never sick again. For three years. Until he contracted a regular old virus just before his 7th birthday. And then it was gone, and he's been healthy as a horse ever since. If you think I felt immense relief, you're right. He hasn't taken the Cimetidine in years (kids very often grown out of PFAPA). And I have never missed that horrid PFAPA.

Until today. When I was grateful that our pediatrician agreed that Oliver might very well have a later-onset of it. Little mister has missed a week of school each of the past three months. This is awful for him, sucky for me, and in crass terms, really flipping expensive when you think of the cost of his schooling. The fevers are awful, huge spiking heat fests that make him shake and vomit and feel truly deathly. He doesn't have the adenitis but does get an aphthous ulcer here and there and his tonsils grow pus and get red and ache. 20 minutes after he swallows some Motrin, he is regular Ol, jumping, dancing and carrying on happily. The loss of appetite is still there, but otherwise he is fine and not contagious.

It has been a real challenge for me, with each boy, to convince our doctors (who are fabulous, and I love them) to let us try Cimetidine. PFAPA is rare, and the original suggestion was for us to use steroids as treatment. Uh, scary. So I'm really glad I pushed and studied and asked around, and I'm terribly hopeful that this will be the magic pill for Oliver too because three weeks in three months absolutely blows.

If your child demonstrates symptoms like these but most noticeably the febrile regularity, start tracking the fevers and associated symptomology. The periodicity is key; I can't emphasize that enough. Ask about Cimetidine as a first step versus steroids or a tonsillectomy. The latter might ultimately be necessary, but why not try a basic heartburn med first?!

Helpful links include:

The American College of Rheumatology: PFAPA

Wikipedia's entry on PFAPA

Basic but utterly clear info about PFAPA on Nomid Alliance