PFAPA

Since November 2011 Ben has been running a fever every 4 weeks.  It took a few months before I noticed that there was a pattern, but found it odd that it was always gone within 48 hours and he had no other complaints.  Often during these fevers he would complain about his neck hurting and not want to turn his head.  In March I took him to see a pediatrician (his was on vacation) who said his throat and tonsils were very red and inflamed, but the strep test was negative.  Also, the lymph nodes in his neck were swollen which is why he didn't want to turn his head.  The next day he was fine again.  May comes and we finally get to see his regular doctor.  Unfortunately his fever was over a weekend so by Monday all I could do was explain and show her my documentation.  She ran blood work that came back normal.  So, now we wait until the June fever.  I spent a lot of time reading things online trying to figure something out.  Then one day Ben told me he had an ulcer in his mouth.  He told me that he got them a lot.  I threw that into Google with the monthly fever, the swollen lymph nodes, tonsils and throat and after more reading I discover PFAPA.  He could be the poster child.  We go the the doctor in June and I immediately ask if he could have it and with a big smile on her face she shows me her list of diagnoses that we were trying to eliminate and there it was.  I told her that he had never mentioned the ulcers before and that was what helped to me figure it out faster and with no evasive testing.  The trick was to do blood work during the fever to be certain.  In July he started running the fever on a Friday night so we headed to the lab first thing on Saturday.  When the results came back a nurse called and told be to come in to discuss the results.  He definitely has it, but luckily it is mild.

PFAPA is a rare autoimmune disorder that has no known cause or treatment, but is shown to be hereditary.  The fevers happen every 28 days give or take 2.  The average fever lasts 5-7 days and Ben's are 48 hours or slightly less.  Fevers can range from 100 -106 and his are typically under 102.  All of his numbers from the lab work show him at the low end of every range.  This is something he should outgrow between 10 and 12 years old.  It is not contagious and if he is turning his neck then you would probably never know anything was wrong.  He eats and plays normally over the 48 hour periods.