maybe some hope

Today, PorkChop had his very important appointment with a pediatric of Infectious Disease to try to pinpoint just what is going on with our little man. And I have to say that I was very impressed. Not only with how wonderfully everything went but with how willing this pediatrician was to work with us to find an answer.

We still might not have any concrete answers on what is going on in PorkChop's body but at least we are one step closer and have a good idea on what avenue to look towards.

big guy at his big appointment

The long and the short of the appointment, Dr F believes that Porker has PFAPA Syndrome or Periodic Fever, Aphthous Stomatitis (mouth ulcers), Pharyngitis (red throat), Adenitis (swollen adenoids). It is catergorized by a sudden high fever that comes in a pattern, usually lasting 3-5 days, ulcers in the mouth that develop around the second day, a red, irritated throat (but not strep) and swollen adenoids. He presented today, after an almost four day long high fever with the ulcers all over his right cheek (which I am surprised that we all missed in the ER since they were very noticeable today), swollen adenoids along his right jaw and a reddened throat.

Unfortunately, there is no definitive blood test for this auto-inflammatory disease. Diagnosis is made by a repeated visit with the same symptoms. And since these symptoms were never "looked for" during his earlier fevers, we  cannot say that they have all ran the same path. Now, it is unusual for a baby to develop this syndrome. Most times, it begins in children between the ages of 2-5 years. So what can I say? Either PorkChop doesn't have this condition or he is an over-achiever. And there are a few other periodic fevers that we can still look into but for the initial visit, this one definitely seems to fit the current bill.

Because of family history though and Porker's penchant for low urine output even on the best of days (and virtually non-existant on high fever days) means that something else could still be going on. So we also have a referral to have an abdominal ultrasound done on the little man in the soonish future. Basically, the doc wants them to take a look at his kidneys, spleen, liver, pancreas and gall bladder to make sure that everything looks ok. He doesn't expect this to change what he thinks is already going on but it will also rule in or out if 1. we are one step closer to a diagnosis of PFAPA and that the fevers are just leaving him quickly dehydrated 2. we find out that there is a kidney issue causing the fevers or 3. we find out that there are two totally separate conditions going on here.

I am not going to lie.... its scary hearing that your baby might have an auto-inflammatory disease. Even scarier knowing that there is a family history of auto-immune disorders. Its hard hearing that your baby might have a condition that has had no long term studies because its a relatively new finding. In most cases, the fevers end during the second decade of life but that doesn't mean that it always happens. PorkChop is lucky that his cycles, right now, appear to be every two months. One sign is a fever that repeats every 3-5 weeks. There is no "cure" for this but having a tonsillectomy "may" end the fevers. Thankfully, our doc is not about removing a healthy organ from a young toddler just to see if it works. He will not consider it unless the fevers become too much of a burden on PorkChop's life. Another option will be putting him on prednisone whenever a fever starts. It will shorten the life of the fever (yay) but can cause them to come closer together (boo).

So that's where we stand right now. No more appointments until another fever starts. Then we are to bring him in on the second or the third day to see if the other symptoms appear. After the ultrasound, if everything appears normal, we won't hear any more about it until the next fever appointment. If something does show up.... well, we will cross that bridge when we come to it.