PFAPA Syndrome and Outpatient Surgery

Before I finally begin talking about Will's Children's evaluation, I wanted to tell you about a significant piece of Will's medical history. Around 14 months old Will started to get sick as if he had a cold without real cold symptoms. He was also teething and cutting those teeth really bothered him. He was always covered in drool up until around 2-1/2 when the last ones FINALLY came in. I thought the kid would never stop teething. It was just the normal time frame, but it seemed much longer.

So he started to get sick with a mystery illness. He would get a high fever for a few days and be really unhappy. I remember him worming along the floor with his butt in the air, drool pouring out of his mouth and whining. I would take him to the doctor and he would tell me it's a viral illness. "Has he been sick recently?" "Uh no, not really." It seemed like his drooling would get worse around this time and everyone always blamed it on teething, including myself. With his sensory issues, being sick is a different ordeal for Will than it is for your average kid. He won't just lay down and rest and watch TV. He doesn't know what to do with himself. He doesn't want to be held; he doesn't want to be put down; he doesn't want to eat a thing; he sleeps restlessly.

As time went on these fevers and pediatrician visits were becoming more regular, like once a month. It is very hard to examine Will, even back when he was a tiny guy. The doctor would take a quick look in his ears and see nothing. He would try to get a good look at his throat during all the screaming and squirming. He might see a slight redness back there. Will would present with a high-grade fever, up to 104 to 105. He scared me to death. I would be up all night with him making sure he wasn't having a febrile seizure or possibly just die in his bed. Pretty dramatic, but that's how it was. He would wake up many times in the night with the fever and I would give him Tylenol or Motrin around the clock. It would bring the fever down a tad and he would sleep some. His neck also got very tight feeling and you could see his glands bulging out. After 3 days the fever would disappear and he was normal again... until the next time. Towards the end of this illness he was getting the fever exactly every 21 days. I would mark the calendar to make sure I didn't schedule anything during this time, and sure enough, the fever would come.

Finally 6 months into this or so the doctor dug into his volumes of knowledge and told me about a condition called PFAPA syndrome. This stands for periodic fever, aphthous stomatitis, pharyngitis and adenitis. The doc had only known of one other child in their practice who had this. He wanted us to go see an infectious disease doctor and get this whole workup done. He also fatefully and thankfully sent us to an ENT right here in town. I read several stories online about how a tonsilectomy and adeniodectomy had a 90% cure rate for this peculiar condition. It started somewhere in the tonsils and caused the fever. I really don't know why it is cyclic. There honestly isn't very much information about this online.

The ENT agreed to do a T&A and threw in some PE tubes (due to his speech delay) for the full-plate special. I didn't even bother taking Will down to Children's to see Infectious Disease. I just wanted to get those tonsils out of there and wanted him to be better. The feeling of helplessness was overwhelming. I wanted to help my boy so bad. It was so hard to watch him with these fevers, caterpillaring across the floor, flopping around, drooling. Not to mention I also really wanted to get some regular sleep again. I hadn't slept through the night since my mid-pregnancy with him. If you know me, you know I need lots of sleep to be happy. I can't get by on 6 or 7 hours. I'm talking 8 at the least, 9 at the most perfect. I'm still working on this goal becoming a regular kinda thing.

Anywho, after suffering straight through the holidays with fevers while waiting for the doctor to be back in the office and have an opening for surgery, Will had his outpatient surgery at 21 months old. He did amazingly and bounced back quick. It was great! I watched the calendar and waited for his next fever due date... and it didn't happen! He is 18 months out from surgery now and no high fever has ever again occurred. This awful thing is behind us now and I hope to never see it ever again.

Can you imagine all these fevers might have stunted his early development? They didn't help anyway. I held onto hope for a good year that he would start catching up. He hasn't yet, but that's okay.

Why do all these things keep happening to Will? Is it somehow connected?