Introduction

We are a family of four (plus two furry puppies) who recently moved to Louisiana from Alabama. Our son was born May of 2012 and in April of 2013 we found out we were pregnant with our daughter! She was born a month early, November 2013, and we moved in December 2013 so my husband could start his new job with his family near. We have been very busy this last year, but are so grateful for all of our blessings.

This blog is really for me to document what we go through on a monthly basis with our son, Rowan, as well as get some of the feelings/questions out that I have running through my mind. I am hoping that it helps other families through their journey with PFAPA, and brings all of us together to help find a way to make life easier for our sweet babies.

PFAPA= Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome.
From what I have read, they named it based on the symptoms because no one knows what causes it or where it comes from. It is a rare syndrome that includes fever episodes, every 4 weeks or so, which last from 3-7 days, and in our case include: mouth sores, sore throat with redness and enlarged lymph nodes. Rowan also experiences severe stomach pain, joint pain (which causes clumsiness = lots of scrapes and bruises), groin pain (which keeps him from being able to sit on hard surfaces for too long), loss of appetite (which causes weight loss/very slow weight gain) among other symptoms. We are one of the "classic cases" being that Rowan has all of the main symptoms that make up the syndrome.

Our journey began when he was probably 10 months or so. We didn't really see a pattern until after we moved and he was around 18 months. I remember taking him to the doctor in Alabama for fevers and mouth sores. They would always run a strep test (because his throat was red), and it ALWAYS came back negative. "Another virus" or "Hand, Foot, Mouth." I was a first time mother, but because I had been a special education teacher, I knew that kids could get sick a lot the first couple of years, so I tried not to stress out too much over it (yeah, right!). The week we moved, Rowan had one of his highest fevers- 104.5. We were in this new house, with no furniture, sleeping in pack n plays and on blow up mattresses, with a brand new baby (a preemie), and one very sick little boy. I didn't even know where the hospitals were yet! After giving him Motrin and stripping him down, the fever started to go down a bit. I already set up an appointment for my daughter at our new Pediatricians office, so I was able to call them in the morning to see if I could bring him in. (Of course I called our old peds office that night to see what to do. I didn't get a call back until 3 hours later- some mess up in the after hours clinic or something). Our new ped. told us that it was just a virus (after a negative strep test and taking blood), and that some kids just tend to run higher temps when sick. SO we took him home to wait it out. A month later, another fever. Again, took him to the dr. They did another strep test and took more blood. Another virus. Each month we would go through the same drill. Things just didn't seem to be adding up. We never went anywhere, being that we were in a new city and didn't know where anything was, or have any friends yet. I guess I could see a toddler getting a virus each month if in MDO or a daycare, but Rowan stayed home with me. AND no one else ever got sick.
Of course I turned to the internet and started searching recurrent fevers. I found PFAPA and it sounded just like our sweet Rowan. Expressing my concerns to his doctor and getting nothing in return except the "crazy first time mother look," I decided to call Rowan's previous pediatrician to see if I could get a referral to a specialist. I needed to see if this might be what he had. I was told to call and schedule an appointment with a Pediatric Infectious Disease Specialist- cause that doesn't sound scary at all- at Tulane. After making many many many phone calls, I finally was able to get an appointment (without a physicians referral) for June 17th.
I found that one of the most important parts to getting a diagnosis was to record each episode, along with the symptoms, so that the doctor would be able to see the pattern. I called and requested medical records from both pediatricians offices, along with a copy of all the blood work they had taken over the course of Rowan's life. I went through and highlighted each visit that involved the symptoms of PFAPA so that the PIDS would be able to see all of the info we had easily. I went back and recorded every date I could remember and could find based on his records, and was armed with all of this information when it was time for our appointment.
We met with Dr. Schieffelin on the 17th, and for the first time I didn't feel like I was going crazy. He was so nice and knowledgeable. After listening to us and EVERYTHING we had to say, he said it sounded like a classic case of PFAPA. Since we researched this on the internet, it was familiar to us, and was more of a confirmation of what we already knew. He said that he wanted some blood work done during Rowans next episode and then some done while he was in-between episodes so that he could compare them. He also wanted to give Rowan a does of Prednisone during one episode, and if it ended it, then that would be the real confirmation that it was PFAPA.
We went home feeling like we were armed with new information and had a direction to go in. We were ready for his June fever (which should occur around the end of the month/beginning of July).