If you’ve followed along with my Instagram stories the last year you may have noticed me mention Maia’s chronic fevers. She gets them roughly every 8 weeks and they last 3-5 days. I started to really keep track of them in March of this year, and also started asking the pediatrician if something more could be going on. To be fair I had also been watching a lot of House around this same time which I was worried was skewing my judgement so the conversation with my pediatrician went more like this:
Me: Have I been watching too much House or should we start being more concerned about these fevers?
Pediatrician: Well quite honestly, both.
Ironically after this conversation the episode of House I watched was on Familial Mediterranean Syndrome, which is another type of periodic fever syndrome. Being of Mediterranean descent on both sides I was a little concerned about this. But I digress…
From March to August we had 4 fever episodes, each came with temps between 101-103 degrees. The only symptoms Maia has during all these episodes are being tired, cranky, achy, and no appetite. There was never vomit or cold symptoms, her rapid streps and urine cultures were always negative too. Essentially we had ruled out other things that could cause fevers.
Our pediatrician had ordered blood work to look at markers for infection and inflammation. We decided it would be best to go during another fever episode. So in August we went, and the same day we received results indicating elevated white blood cells and CRP (c reactive protein, an indicator of inflammation). At that point he decided it was time for a referral and said it was a toss up between Infectious Disease and Rheumatology. He ended up sending the referral to the Pediatric Infectious Disease because he thought we would be seen sooner. We actually were able to get in for our appointment 2 weeks after the referral.
We met with the pediatric infectious disease doctor and she went over a huge list of questions, we worked from head to toe essentially. We went over all the episode, past medical history, her birth, etc. After the questions and a quick look at Maia she stepped out of the room, which I assumed was to look everything over and plan next steps. When she came back she gave us a diagnoses of PFAPA or Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome. This was exactly the diagnosis I was expecting, it was just reassuring to have someone with an actual medical degree confirm it; because my google medical degree had already came to this conclusion I had already looked at some of the treatment and prognosis information. I knew she would eventually grow out of this and I knew I didn’t really want to treat with steroids. Another reassurance of this diagnosis was that it wasn’t just another unknown virus causing her to have another fever. There was some real frustration in bringing her to the doctors so often with these lingering fevers and being told “it’s viral, it will run its course, this is what happens with kids in daycare.”
So what exactly is PFAPA? The American College of Rheumatology’s definition is: “This syndrome includes recurrent episodes of fever with aphthous stomatitis (mouth sores) and pharyngitis (sore throat with redness). Occasionally, there also may be exudate (white patches on the tonsils) and usually the lymph nodes in the neck are enlarged (adenitis). Episodes of fever start suddenly and last for three – seven days. Fevers occur routinely every few weeks (usually between three -six weeks); often, families know the exact day when an episode will start. Some children have other symptoms like joint pain, rash, abdominal pain, headache, vomiting or diarrhea. Children are completely well between episodes.”
In research I’ve done our onset to diagnoses was quicker than usual. I will attribute this to my google medical degree and my pediatrician likely getting sick of seeing us so often, but in all seriousness I advocated for Maia despite my nagging concern I was only one that saw these fevers are an issue. Because we had been to the pediatrician for most of the recent fevers or at least spoken to the office they were well documented too. Also we had ruled lots of things out in other fever episodes and Aria never got sick after Maia so there more evidence that she didn’t just have a virus.
Moving forward we will just continue to ride this fever wave. We were given new dosing for ibuprofen that should hopefully help reduce the fevers, as they previously didn’t go down or stay down during episodes. If they do become more frequent we may look into removing tonsils and adenoids as this has been show to reduce episodes. The biggest reassurance with this diagnosis is she isn’t contagious during these fevers so as we reach school age, if she feels up to it she could still go to school during episodes. Also now that we have a diagnosis I will be trying to keep better track of what leads up to these episodes in hopes we can eliminate some triggers.
Although this is a pretty personal post I felt compelled to share about this journey because until we were experiencing this I had never heard of periodic fever syndromes or auto-inflammatory diseases. I think because it’s something we don’t really hear about that may be why many others onset to diagnosis takes years. Even if just one person sees this and can relate or help advocate or their child, that’s one less person feeling frustrated, confused, lost, and even a little crazy about their child getting fevers so often.