PFAPA Syndrome

Pearce was just formally diagnosed with this on Tuesday.  PFAPA:  Periodic Fever, Aphthous Stomatis (mouth ulcers) Pharyngitis (sore throat with redness), Adenitis (enlarged lymph nodes in the neck) Syndrome.  Thankfully, it sounds worse than it is.  So, starting in March, Pearce started getting frequent fevers with no source of obvious infection...ears were always fine, no strep throat although his throat was always red, and no flu.  When the first fever happened, I didn't think much of it.  He had just gotten over a stomach bug and about 9 days later he came down with a high fever.  I thought maybe he wasn't quite over the bug.  I took him to the doctor and she did a strep test, but all came back normal.  Pearce's pediatrician said it's probably just a fever virus and to manage it with Tylenol and Motrin.  He was pretty miserable with fever for about 4 days and then finally, no fever.

 

  Then about 2 weeks later, he came down with another fever all of a sudden.  I took him to the doctor and again, all tests for ear infection, strep, and flu came back negative.  Again, he's just unlucky and picked up a little virus.  During this episode, I did end up taking him to the after hours clinic because at one point his fever reached 105!  They did the same tests because I was just convinced he had the flu because all he wanted to do was sleep, but nope, nothing.  

 About 3 weeks later, another high fever began.  Now Chris and I started to get worried.  I took him to the doctor again and was praying he would have strep throat...pretty awful when you are praying for that!  I just wanted some reason for the fevers!  Again, no obvious source of infection.  Our pediatrician agreed with me that this was starting to get strange.  I asked if we could do blood work to give us some answers, but she wanted to wait and see if another fever episode came  within the next month.  I was disappointed, but agreed.  It was then that she told me there are several periodic fever syndromes that we will test for if another fever happens again.  I had already googled "frequent fevers" and Periodic Fever Syndrome came up.  It seemed to fit Pearce, so I was pretty convinced that's what he had.  Three weeks later, he woke up with another fever.  I knew we were going to be doing blood work and I was so relieved!  Pearce's pediatrician agreed and off we went to the lab.  They had already applied numbing cream to his arms.  I was pretty nervous how Pearce would do.  I just told him a nurse needed to look at some of his blood to figure out why he was feeling so bad.  I held Pearce in my lap and the nurse was really fast.  He did not cry at all when she first put the needle in and started drawing blood.  I was so proud of him!  But for whatever reason, she had to draw more blood from the other arm.  This is when he started crying.  Poor little guy!  But he was still and it was over quick!  These blood tests were to rule out a bunch of things.  If they all came back negative then, we would go to a specialist.

 

 After a few days I got the results back and everything came back negative except for a marker of inflammation.  I made an appointment with the specialist but wasn't able to get in until 3 weeks later because I requested a specific doctor recommended to me by our pediatrician.  Chris went with us to this appointment.  Dr. Fernandez was so sweet to Pearce and very informative.  She went over his blood work we had done previously, went over the notes Pearce's pediatrician sent over, asked us lots of questions, and then did a quick exam of Pearce.  She said that her gut tells her Pearce has PFAPA Syndrome based on everything she has seen, but wanted to do some more blood work while he was fever free and also wanted to see him when he got his next fever.  Pearce did great...only cried when they took the bandage off that was holding the numbing cream on his arm....didn't cry when they stuck the needle in!  After a few days, we got the blood work back and it was consistent with PFAPA.  So now we just had to wait until Pearce got another fever and then we were to bring him back to Dr. Fernandez.

 

 
 Crazily enough, he went 6 weeks before he came down with another fever this past Saturday.  I was beginning to think that maybe he wouldn't get another fever again....but nope.  This episode was different in several ways.  First of all, he went 6 weeks between fevers.  Secondly, pretty much all day on Saturday, he complained that his neck was hurting...he had never told us that before.  This was the first sign we had that he was starting to get sick.  His lymph nodes on his neck were very swollen...about the size of an almond.  Sure enough, that night he woke up crying with a fever.  On Sunday, he just wanted to lay around and not do anything.  He complained about his tummy and neck hurting this day, too.  The other thing that was different is that his fever never got above 102 and only lasted 3 days.  I took him back to Dr. Fernandez like she had asked and she examined him and did find one small ulcer in his mouth.  After going over a few more things, she formally diagnosed him with having PFAPA.  So, this is what I know about it:
1.  Fevers come on quickly and are usually high and last about 4 days.  Pearce's fevers are usually 102-105.
2.  On average, the fevers come every 4-6 weeks, but there can be a lull where you go months without any fever...or they can become more frequent.
3.  He should grow out of it at some point in his childhood....usually between age 5-7.
4.  Besides Motrin and Tylenol, the only other treatments are removing the tonsils or steroids.  Don't want to do either, so Motrin it is!  The only way I would remotely consider the other two treatments is if the fevers become more frequent and last longer causing him to miss school/activities or if they somehow interfere with his growth.
5.  No more follow ups with Dr. Fernandez unless I feel the need.

 

Of course it breaks me heart to see Pearce feeling bad, but I'm just so thankful this thing is totally manageable and in the grand scheme of things, it really not that bad!