Sometimes, it’s good news to hear that your kid doesn’t have a cold, but an obscure rare disease.
If it’s the twelfth “virus” that’s given him a fever of 105 every 28 days for the past year, the diagnosis of that obscure rare disease becomes a downright blessing.
This is the strange state that is being the parent of a child with PFAPA syndrome, a benign condition that brings about very high fevers for days with alarming regularity, and no obvious cause. (It also brings that interesting acronym, which stands for periodic fever, aphthous stomatitis, pharyngitis and adenitis. Try rattling that name off as a party trick for your nurse- and doctor-friends, who have likely never heard of it.)
Often, the child has mouth sores or a mild sore throat. But in other cases, the child merely has some slightly swollen glands: an indication that, yes, your child might be fighting something. But what that is is anybody’s guess for the parents for months, and very often, years. Otherwise, the child is happy, healthy and robust, and doesn’t even catch a smidge of illness when all the big kids around him are flu-ridden and miserable.
It just doesn’t make sense. And you become certain you’ve gone insane.
My trip to this twilight zone started innocently enough, with what was certainly a late bout of roseola in the two-year-old. For six to seven days, he had a fever above 103 and 104 – all the time — and no other symptoms. We didn’t medicate him when he seemed reasonably content and playful to help him fight off this apparent mystery virus. We brought him to the pediatrician twice, and she found nothing but swollen neck glands.
Every morning for about seven days, my husband and I would expectantly check him for a red rash on his torso, the rash that gives the infant condition roseola its nickname, baby measles. The fever ended. The rash never came. But the fever did again, 28 days later. As did my newfound fears: Meningitis? Could it be meningitis? Is he septic? Quick, let’s check him for cuts! Can Kawasaki disease be cyclical?
And is yet another medical staff member going to tell me “some kids just get high fevers with every illness”? Really? And always with no other symptoms? Am I being punked by pediatric residents?
He surged to 105.8 one day, which resulted in my running with him — yes, running like a lunatic, with him precariously placed in a broken-down stroller — to the nearby regional children’s hospital. Verdict? “It’s just a virus.” It was another six-day fever that started high, stayed high, and wasn’t going down much with acetaminophen.
Twenty-eight days later, we were on vacation in Hawaii, and the bizarre mystery fevers were a distant memory, until 56 days later (yes, that is indeed 28 times 2). And 28 days after that, a crazy-high fever spiked to the 105s again.
Twenty-eight days later, same cycle, same pattern. And so on, and so on, and so on. For the next 13 months.
The local children’s hospital’s urgent care clinic became our new haunt on many weekends. Most of the time, we were sent home with the diagnosis of “a virus” and the advice to keep doing what we had been doing – which of course, seemed to always lead to a fever of 105.8. (It also led to two horrible urethral catheterizations, since the feverish and cranky boy steadfastly refused to pee in any cup for anyone.
When your kid spikes major fevers with no explanation, the doctors need to check for a urinary tract infection. Even, apparently, when the kid has this pattern every damned month and never has a positive test for a UTI. I just consider us lucky that the physicians never got around to coming at us with paperwork for a spinal tap. Every month.)
Finally, after two summer fevers, during which I knew exactly what day his fever would go to the 105s, I started demanding answers; I stopped wishing it would all stop happening.
I asked his excellent pediatrician if the fevers were a concern to her. “Well, it’s not like he’s getting a fever every month,” she remarked. Um, yep, it is.
That’s when I went back over every e-mail about a missed playdate, every begging request to kind friends to pick up my older child at school, every darn monthly letter explaining to my sick child’s co-op preschool class that I would be missing my required classroom day yet again, and did anyone want to sub for me for, say, the seventh time?
That was when we knew for sure that we were on to something. It was as if we were tracking a menstrual cycle. Every 28 to 32 days, he got a visit from the fever fairy. It was not in our heads. After a quick but traumatic blood draw at our pediatrician’s office to look for signs of cyclic neutropenia, a very rare but serious disorder of the white blood cells, we were given a referral to the children’s hospital’s infectious disease unit.
By that time, from my many Google searches for “recurrent fever,” I knew we were likely dealing with PFAPA, and that it is considered harmless and is not infectious, but the few postings online from other frantic parents told me that the only people who really knew a whit about periodic fevers were infectious disease specialists and rheumatologists.
And, this, for a disease that is neither rheumatological nor infectious: A no man’s land of childhood conditions.
The day of the big appointment, I came armed with my sloppy but detailed handwritten diary, and discussed the history at length with a resident. And then the ID doctor came in. She was the person I had longed to speak to for more than a year. Her greeting: “This is not a virus.”
I have since read others’ accounts, and this is the most gratifying thing a parent can hear after months or even years of uncertainty and late-night fears spurred by a borderline delirious child in your bed, screaming about shadows again.
We have not yet opted for the current first line of defense –one or two small doses of the powerful steroid prednisone given at the start of a fever — but already we are resting easier. We know it’s not meningitis, or a raging infection, or cancer. Or a virus. And expensive genetic screening has almost certainly ruled out any possibility that my son could be dealing with another rare but more serious periodic fever syndrome, such as familial Mediterranean fever or TRAPS (formerly known as familial Hibernian fever).
PFAPA is certainly not fun, but in almost every case, it does go away, say doctors, usually by the time the child reaches the double digits in age. (It generally starts in early childhood, between the ages of two and five.) With a tonsillectomy, it can go away even faster. Since the precise mechanism that triggers the fever is unknown, it’s still a mystery why removal of the tonsils and sometimes adenoids seems to work. But it seems that in 80 percent of cases or more, it does.
In the case of my son’s specialist, at least, the course of action is usually to try prednisone first, then daily cimetidine (yes, Tagamet), then finally to seek a surgical option, which is, of course, more invasive, but has had good results for many kids. Is there a chance this disease is arising because young children used to have tonsils removed routinely in alarming numbers, and now it’s a discouraged procedure? No one knows, and there are very few doctors studying this.
It is known that PFAPA was once a disorder believed to exist in a smattering of children when it was first identified in the 1980s, as Marshall’s syndrome. Today, there are more than 400 parents who are active on the Facebook page PFAPA Child. Of course, not every family is aware of the page, or even aware that their child is suffering from such a condition, so it seems there are quite a lot of us out there in the United States alone.
After the diagnosis, a 105 was just a sign that we needed to back up the ibuprofen given five hours before with some acetaminophen, not a sign that we needed to get to a hospital at break-neck speed. I learned to appreciate the little things, like the beautifully smooth operation of a brand-new oral syringe for liquid ibuprofen.
And my time-tested ability to measure out 1.5 teaspoons with my eyes closed. And to not even blink when I read 105.8 on a thermometer. It’s just another month in the life of a PFAPA parent.
Previously posted on my children’s health blog, stickersandlollipops.com