Periodic fever, aphthous stomatitis, pharyngitis and adenitis

For those of you that have been following our story for a little while, you are already aware of the situation with Emmett and his recurring fevers. For those new to my blog, here is a snip-it of background. Basically, Emmett has there recurring bouts of high fever. They last about 7-10 days and usually have no cause.  After the first few days we always take him in to be seen….just to be on the safe side.

Emmett’s pediatrician has referred Emmett to immunology at Akron Children’s Hospital, where he sees Dr. Nancy Wasserbauer, DO.   Dr. Nancy Wasserbauer, DO sees Emmett and Gavin both. She’s a fantastic doctor and really takes the time to explain everything to us. She evens returns our calls herself.

Because of Gavin recent, diagnosis of Primary immunodeficiency (she said this is what we are calling it for now), she ran the same battery of tests on Emmett to make sure he was dealing with the same issues. As it turns out, thankfully Emmett’s immune system seems to be okay, at least for now. This week we had a follow up appointment with Dr. Nancy Wasserbauer, DO, in order to form a plan of attack and discuss our options. One of the things we pointed out was Emmett’s enlarged lymph nodes in his neck. They have been that way for as long as we can remember.

We have had it checked out many times and no one ever thought it was anything to worry about. However, Dr. Nancy Wasserbauer, DO said she thinks that they are actually the cause of the fevers, even though they never appeared to be connected. Based on the tests we have done to date and her physical examination she thinks that Emmett has something called Periodic fever, aphthous stomatitis, pharyngitis and adenitis or periodic fever aphthous pharyngitis and cervical adenopathy. We’ll call it PFAPA for short.

According to Wikipedia, this is how this disorder is defined:

PFAPA syndrome is a medical condition, typically starting in young children, in which high fever occurs periodically at intervals of about 3–5 weeks, frequently accompanied by aphthous ulcers, pharyngitis and/or adenitis. The syndrome was described in 1987 and named two years later.

PFAPA is frequently discussed together with other periodic fever syndromes, but it is unknown whether the cause is primarily genetic or due to an initial infection.

So what do we do next?

Well, for starters, the next time he spikes a fever we have to give him Prednisone. The first day he spikes his fever, we give him one dose of Prednisone in the morning and one dose of Prednisone at night. This should break the fever in a few hours. Not entirely sure why this works so I’ll have to do some more research to find out. Basically, if the Prednisone breaks the fever then that confirms the diagnosis….at least as I understand it.

However, she said that if the Prednisone doesn’t break the fever then we are dealing with something else entirely. She said that there are other possibilities but they are far more unpleasant and we just aren’t going to go there just yet.

I’m not really sure what to even say anymore. As far as these fevers go, they typically happen at least once a month. The problem, aside from the obvious, is that Emmett misses way to much school. He can’t go to school with a fever…right? He also misses therapies and other important appointments. In fact, part of his vaccine schedule is off simply because he’s running fevers all the time and they don’t vaccinate when he’s running a fever. The fevers can be managed with Advil or something like that but that treats the symptom and not the problem, if that makes sense.

The addition of a new doctor

Dr. Nancy Wasserbauer, DO has referred Emmett to an ear, nose and throat doctor or ENT for short. Our first appointment is at Akron Children’s Hospital, on September 1st. Actually, this is more of a return of a doctor as apposed to the adding of a new one. Emmett has already been to the ENT several times and even had tubes put into his ears for the chronic ear infections. Now that I think about it, we are actually changing ENT’s, so I guess we are adding a new doctor. The ENT will evaluate Emmett’s condition and help us to plan the next step.

Dr. Nancy Wasserbauer, DO said that because of the frequency of the fevers and the length of time they have been occurring the ENT may opt to remove Emmett’s tonsils and adenoids. The thought of Emmett having surgery makes me feel sick to my stomach. I know it could always be worse but any time your child may have to go under the knife, it’s hard to image it being any worse. Either way we have a much better idea of what’s going on September 1st. According to Dr. Nancy Wasserbauer, DO and the research I’ve done already, most kids will out grow this. The problem is that this often gets worse before it gets better. Dr. Nancy Wasserbauer, DO said that because Emmett’s fevers last for so long already they will likely increase in frequency or duration to the point where they are happening every other week.

So for now, we are once again left in the dark. Hopefully, this will turn out to not get anymore complicated and we can get Emmett back up any running, so to speak, ASAP. He needs to be in school and he shouldn’t have to be sick all the time.