In my last post, I said something about how difficulties 'add flavor to life'... well, I'm done with flavor and am ready for some good old fashioned gruel!
This past week is over and done with and I am thrilled that we are out of it! Last week brought another fever cycle for Emmett, sinus surgery and recovery for Scott, croup for Garrett, colds for Owen and Teagan and I, and the flu for everyone... bring on the gruel and slop and every other bland food possible, we're done with flavor!
In the past few months I have been talking with other families who are dealing with the same PFAPA diagnosis we are and have learned some from them and drawn a lot of strength from reading their accounts and their struggles and their triumphs, so I thought it might be time that I give back a little too, just in case there is another mom out there pulling her hair out trying to figure out how far off base her PFAPA kid is from other ones. So this entry is going to be mostly about Emmy's walk down PFAPA Lane so far, so not many pictures, and nothing really funny, really long, but it gets it down in writing for me and others out there.
Emmett started having these cycles when he was 5 months old. He would get terribly high fevers that were from 102-105, with no other symptoms. We took him to the Doctor for three months each time because he was having febrile seizures with them and having a little guy with a temp of 104 for days on end was really terrifying! After being reassured by our doctor that it was roseola, and the rash 'would come', or having tons of blood drawn to find out he was as healthy as a horse (with a high fever), or being looked at cross-eyed and patted on the head like I was a new mom, one doctor suggested I keep a calendar of them for six months and then come back in and we could talk.
So I kept meticulous notes - checked temps every 2 hours, recorded what meds I gave and how he reacted to them. Any suspicious symptoms, behavior changes, life style changes, any possible allergy concerns that could have triggered them... you name it, I wrote it down. His schedule was a 5-6 day long fever cycle followed by 3 weeks of health and then a 3 day fever cycle and then 4 weeks of health. They were like clockwork and were pretty easy to chart future cycles. He had seizures on both the short and the long cycles and was miserable for both.
Six months later we went back in to the doctor, he took half a glance at it and referred us to an infectious disease specialist.
One month (and another cycle) later we saw Dr. 'O' and he sat down and briefly looked through my notes, but asked a lot of questions to weed us out (in his words) from the worried parents pile and see if we were in the actual problem pile. He was really kind and polite about it, and explained everything he was thinking. He said he narrowed it down to 6 things, with PFAPA being the thing he thought most likely. But it isn't something you can test for, you have to rule all the others out and narrow it down to this.
PFAPA, or Marshall's Syndrome, stands for periodic fever with aphthous stomatis, pharyngitis, and adenitis (you see why they call is PFAPA). In short, it is a fever that comes on a regular schedule - usually 4-6 weeks apart - and sometimes comes with mouth ulcer, sore throat, or tummy issues.
He also explained the idea of a seizure threshold to us. He said everyone has their own seizure threshold, some are higher than others. For those kids who have febrile seizures, theirs are lower than most, so any number of triggers can bring a seizure. It's not just a quick jump in temperature, it could be going from dark to light, change in routine, stress, exhaustion...anything. So for Emmy, he had had seizures at 99 and at 104, so it made sense to me that it wasn't just quick jumping that made him seize.
He told us that there were three different options for dealing with PFAPA. Option one was a daily dose of Tagament - usually a stomach medicine, but for some reason it seems to shorten the length of the fever (but also shorten the time between them as well). Option two was to take 2 doses of steroids at the beginning of the fever and that stops that round of fevers (it has an 75-80% success rate, but it also had the possible side effect of shortening the time between). And option three was to have the tonsils and adenoids removed (no one knows why this one works, but it has an 80% success rate). His preference was option 2 and said that he wouldn't advise number three without trying the steroids to 'prove' that it was PFAPA. So we (begrudgingly) filled the steroid Rx to try one round of it and see if would work. (Our preference was to take the tonsils and adenoids out... I worried about the long term effect of monthly doses of steroids until his body finally outgrew the syndrome (which averages to be about mid teens).
We did one round and it seemed to stop that fever. It was a shorter fever than normal, so we took it as a 'yes' for PFAPA. We scheduled the surgery and had to go through two more fevers in October and November before the surgery, which meant more seizures, more worry, and more (in my mind) brain melting! His fever, by this point, were consecutively hitting 105 and 106, and I was really worried. (Although the ER doctor told us not to worry until the fever hit 107 or his seizures lasted 15+ minutes... but 106 isn't too far off from 107, and his seizures were from 10-13 minutes each, so I think my worry was a little founded!)
Anyways, we did the surgery in the middle of November when he was 22 months old, and he didn't have any fevers for all of December. It was a great month! He had been having cycles for 17 months straight, and we finally had a fever free month.
January came, and so did a 7 day cycle. High fever, no other symptoms until day 7 when he started with a runny nose and a little cough. I prayed it was just a cold, but I think it was more like a fever cycle that led into a cold because his immune system was overwhelmed.
So I charted it on his calendar and made note of when I thought another cycle might come in 4-5 weeks, and waited with crossed fingers. Last week was the 'perhaps' week, and sure enough, on Monday a fever popped up.
Monday - 102, no other symptoms, regular attitude, regular eating, Tylenol/Advil every 4 hours with no real temp change in between.
Tuesday - stayed 102 all day until the night and temp popped up to 104.8 and up, no other symptoms, slowed attitude, clingy, regular eating, drinking a lot of water to cool off, Tylenol/Advil every 4 hours, possible seizure at night - he froze up for a few minutes in the bed and then was more weak than normal and loopy eyed, I was half asleep during it and didn't zone back in until the end when I noticed he was stiff, hard night sleeping. Gave him one dose of his steroid to stop the fever cycle but it had no effect and the label said to not give if near sick people and his brother has croup so I didn't give the second dose.
Wednesday - fever stayed between 104 today and was up to 105.9, Tylenol never touched it at all and Advil brought it down to low 104's/high 103's, not eating, little drinking, clingy, wouldn't be alone, drowsy eyed all day, took 4 naps, terrible night sleeping
Thursday - fever stayed around 105 all day with very little variations, fever meds didn't work but we still did every 4 hours, his nose started running today and was a constant bloody nose/runny nose mix all day and night, no eating, little drinking (bought some Ensure for him and he drank about 4 oz of it), no energy, no complaining just lethargic, slight cough at times, very little diaper output which means dehydration is setting in, we called some members of our church over to give him a blessing and his fever broke for 4 hours so he could sleep then the fever returned just as high.
Friday - fever continued to stay above 105 and bloody nose continued all day. No eating, no drinking, very little movement, very lethargic, slept/rested 80% of the day, had to be held at all times, 1 wet diaper all day/night, slight dehydration, terrible night sleeping between fever and cough.
Saturday - fever went down to 104 for the day, cough is here at night, runny/bloody nose still here, no energy, drank about 3 oz liquid today, no food, loopy eyes, needed to be held all day
Sunday - fever stayed around 104, nothing to eat, drank maybe 4 oz of liquid, very lethargic and sleepy, felt miserable all day, slept/rested most of the day, fever meds not helping, bloody/runny nose continued, cough sounds wet and deep
Monday - We took him to the doctor who suspected RSV and sent us to the hospital for testing (results were negative for RSV but positive for influenza A - there are no meds to help) doctor said to up fever med to every three hours instead of every four, fever came down to 101-102 on it's own, he started nibbling on foods today, drank 6 oz of liquid, still little energy and clingy, loopy eyes, but smiling a little more so he seems to be feeling a little better.
Tuesday - fever is staying low 101-ish, continued cough and runny nose, bloody nose stopped, acting more like he has a cold than in a fever cycle, has more energy, eating and drinking about half his healthy normal, awake for his normal amount of the day with only one nap, giggly and happy kid again.
So now we are done with this cycle. We thought the surgery would be the end of his cycles, and the two he's had since then are still sort of suspect because he also caught a germ towards the end of each cycle. We are expecting the next one around the end of March - just in time for my birthday... maybe I'll get a nice birthday gift and he won't get one!
Scott came home from work early and stopped by the church to pick up the two little boys from me and that let me sit and relax and enjoy the evening. It was another fun pack meeting for everyone, and Garrett did his usual by staying late to help clean up and vacuuming the whole hall. He will opening tell whoever is cleaning, that he should be doing it rather than them, because it is a scout's duty to always be helpful. I love how he takes this scouting thing so seriously!
This was made for me by my buddy, Wendi. She made one of these for herself for her scouting experience with her three sons, and now I am just as lucky as she was and have one for my scouting experience with my three sons! I love how it's Cubby colors on one side and Scouting colors on the other! The three pins Garrett has already earned already have a home in their proper place!
Aren't those adorable? We found them walking down the hallway towards the school room and I was able to be prepared for the powder covered room that awaited me... thanks for the heads up Emmerson!
Granny gave him some great footed pj's with bears on them, an Aunt Jen gave him a push car that he loves. He has a favorite book about bears, so the jammies were a great match for him!
