Our Journey through PFAPA – Family Friday

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

Our Journey through PFAPA – Family Friday

What is PFAPA? 

I’ll break it down..

PFAPA stands for (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) 

Periodic fever definition-is a group of rare autoinflammatory diseases characterized by inappropriate, uncontrolled, and often spontaneous signs and symptoms of inflammation.

Aphthous Stomatitis-is a common condition characterized by the repeated formation of non-contagious mouth ulcers (aphthae) in otherwise healthy individuals.

Pharyngitis- Acute pharyngitis is characterized by the rapid onset of sore throat and inflammation (with or without exudate meaning pus on the throat in white patches)

Adenitis- is a general term for an inflammation of a gland. Often it is used to refer to lymphadenitis which is the inflammation of a lymph node.

Put all of that together and you get PFAPA

PFAPA is a childhood syndrome that causes repeated episodes of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis generally every 3-6 weeks. In my kids it was every 3 weeks or less.

PFAPA is not contagious. You can’t catch it from someone who has the condition. 

The following was taken from the Cedar Sinai website. 

“The immune system’s job is to find and destroy things in the body that may cause harm. This includes bacteria and viruses. The immune system works to help keep you healthy. Inflammation is part of the immune system’s response. It causes cells of the immune system to become active. These cells make substances that signal the brain to raise body temperature and cause a fever. These signals also cause redness and swelling.

In some cases, this may happen without a good reason. This appears to happen in PFAPA. An abnormal activation of the immune system causes episodes of fever and other symptoms. PFAPA is one of a group of disorders that cause fevers due to abnormal inflammation. It’s the most common condition in this group.”

When you get sick you want a fever to kick in to raise body temperature to kill off bacteria. In PFAPA your body over reacts to everything so a fever kicks in when you don’t need it to. Basically your body thinks everything is a germ, or “normal germs” that don’t effect most of the population, your body overreacts to, causing fever, sore throat, mouth ulcers and swollen lymph nodes.

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

I took the following symptoms from the Cedar Sinai website here https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/p/pfapa-syndrome.html

PFAPA has several symptoms which include:

  • Fever
  • Mouth sores (aphthous stomatitis)
  • Sore throat with redness (pharyngitis)
  • Enlarged lymph nodes of the neck (adenitis)
  • White patches on the tonsils

Also less common but can coincide with the symptoms above are 

  • Headache
  • Joint pain
  • Rash
  • Belly (abdominal) pain
  • Nausea and vomiting
  • Diarrhea

For a parent, until diagnosis, PFAPA is a complete nightmare! 

(Allie suffering through a fever, we were desperately trying to lower it with meds and wet cloths.)

It is a childhood syndrome that causes repeated episodes of fever, and sore throat that are similar to strep throat, repeated every 3 weeks like clockwork. 

The problem is, it looks like and is treated like strep throat so unless your lucky enough to find a practitioner that happens to be familiar with it, you will most likely suffer through many years of unnecessary stress like we did.

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

See how it looks like strep? Gross sorry!! ?

You can read more about this condition here  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/p/pfapa-syndrome.html

Repeated episodes of high fever was the predominant way in which it presented itself in my kids, about EVERY 3 WEEKS FOR 10 YEARS!! In our family, 3 out of my 4 kids had it. My son and my two youngest girls. 

It took 10 years for us to receive a diagnosis, and therefore a real treatment, because PFAPA is rare and presents so similarly to strep throat that it’s usually just treated as such with antibiotics and a hope that they will get better. 

They do sort of…

In between episodes your child is completely normal, which is even more confusing until the diagnosis.

It was a LONG 10 years until we received a diagnosis, a diagnosis that happened by pure accident, on a weekend by a nurse practitioner at a doctors office that we don’t normally visit across town. 

I’m convinced it had to have been divine intervention, after many prayers and pleading, in order for us to receive some relief from the cycle that would never end. (More on our diagnosis story in a minute.)

My husband and I spent many, many, many, scary sleepless nights, up every few hours rotating medications on schedule, setting alarms to wake and administer them, to try and keep their fevers under control. Many nights of 2am sponge baths to a feverish child trying to fight their body for control from their raging fevers.

Many exhausted mornings, wondering why this kept happening? 

What were we missing? 

When would this stop?

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

Each episode of PFAPA lasts an entire week. So you can imagine when my two youngest, who are 18 months apart, started showing symptoms and rotating every 3 weeks with a fever, sometimes overlapping, how difficult and discouraged we were as parents.

My friends kids didn’t get this sick and they were crawling on dirty bathroom floors, I couldn’t even visit the grocery store without my child becoming incredibly ill. Even with wiping down shopping carts with disinfectant and keeping a ginormous bottle of sanitizer in my car, being religious about it’s use. My friends thought I was CRAZY and looking back, if I hadn’t been experiencing what I was, watching from the outside, I may have thought I was crazy too. 

I became OBSESSED with cleanliness. Wiping door knobs, spraying light switches, cleaning bathrooms, kitchens and dishes… excessively, thinking it had to be my fault somehow… something I was missing, and I was going to fix it! It didn’t matter if I was exhausted from fevers and cleaning, I was going to keep my house so clean that theres no way it would be my fault that my kids ended up sick with a fever. 

It was futile, really. My house wasn’t dirty. However, it was still many years before I would figure that out and know that it wasn’t my fault. I could have used hospital cleaners and kept my house sanitized from top to bottom, which I did for several years, and my kids would still end up with fevers and sore throats constantly! Every. Three. Weeks. 

Even if we stayed home and never left the house to contract a single germ, my kids would end up feverish, lethargic and sick.

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

With two kids on that schedule you can imagine that I had a sick kid, pretty much all the time. We visited the doctor so often that I knew everyone in the doctors office pretty well. We used our insurance so much and I was so grateful to have it.

I remember one particular terrible month where we spent $650 in copays and prescriptions. Think about that, at visits being $25 per copay and most medications at $10 a piece.  That’s a lot of doctor visits and medications. 

I feel terrible about the antibiotics I religiously gave my kids, thinking it was making them better, all the while it was damaging their intestines causing other problems that we would have to deal with later.

At one point, when my son was 4 years old, his throat swelled almost completely shut. We took him to the emergency room where they diagnosed him with a peritonsillar abscess and sent us home with an antibiotic. After 3 days,  he still wasn’t feeling better, I took him into our pediatrician where she panicked when she saw him. She gave him a shot of steroids, and two different antibiotics, then we scheduled his tonsillectomy. It was a month before we could get his tonsils removed. So he stayed on antibiotics for an entire month, thinking it was caused from strep, only it wasn’t. 

My son did have his tonsils and adenoids removed and the episodes stopped. It was hard on him and us at the time, but so worth it!

So, fast forward several years, when my two youngest daughters were born and started showing the same symptoms, we waited out the 3 years before they will remove tonsils, and after 3 years we begged the doctors to remove them. 

They wouldn’t recommend us for a referral. 

Only because they didn’t have enough strep diagnosis’s to warrant the removal and weren’t familiar with PFAPA. The problem being, we would bring them in with throats so awful that even with a negative quick strep test, sometimes no test at all, they would always treat them with antibiotics. Their throats were so bad that they usually wouldn’t send us home without one. Occasionally, we would go in and they would test negative, so no antibiotics were given, only to be back in a couple days because their fevers were still off the charts. Then thinking the test was wrong, antibiotics were usually given, and because we would have been about 3 days into the week of fever, after 3 days of antibiotics, they would get better. When really the fever episode was just half over. 

My kids fevers were 102* at the lowest,  and the highest range for my two youngest, who were effected more harshly by PFAPA, were usually between 104-106*! 

106*

You can imagine the panicked emergency room visit that followed when my daughters temperature topped that at 106.5 one night.

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

We gave Allie, my youngest, Tylenol, which she threw most of it up en route to the hospital. Having experienced high temperatures topping 104* with my other kids and being treated by doctors like I was crazy or that there was no way it was possible, I made my husband stop by the store and pick up a new thermometer and take pictures of the readings on the way, so they would believe me. 

And just like before, I got the pat on the head and sent home. 

Discouraging things were said like, those thermometers are not reliable. (What?!) 

What was I supposed to use then?

Or is this your first? You must not know that little kids just get sick a lot. 

ON and ON like that….

When they took her temperature at the hospital it had lowered to 104* thinking that some of the tylenol stayed in her system to lower it slightly. But even on medications it wouldn’t lower any more than 102*

Needless to say, it was a frustrating, long, disheartening and difficult several years…

Then one weekend, there were MANY, we had to visit the doctor for one of my sick girls. The only office open was across town, with a nurse practitioner we didn’t know. Thinking it was strep, and wanting to get the antibiotics started so my daughter would be able to get back to school on Monday, we headed into the office like we had many times before.

During the visit they tested her for strep and it was negative, so they were going to send us home without an antibiotic, and only a steroid to help the swelling in her throat go down because it was so bad!

I’m not sure what happened but this time I started crying while explaining our situation. Pleading with her not to send us out empty handed because we would just be back in a few days. I said “I know it’s negative, but please give us an antibiotic” and continued on with my reasoning as to why I couldn’t go home empty handed.

This time, this lady listened. I had begged doctors to test my kids immune systems, only to be met with scoffs and an air of “you don’t know what you’re talking about or what you’re asking for” or my favorite “you must be a hypochondriac” type attitude.

After listening she said, “have you ever hear of PFAPA?” 

We hadn’t. 

So she explained it, sent us home with the instruction of using the steroid for a day, and if my child was better in 24 hours, symptom free with the sore throat and fever suddenly gone, then we might want to explore treatment for that…

…Which is tonsil removal. Though not always 100%.

We did what she said and in 24 hours our daughter was completely healed. Not ready to remove her tonsils yet we waited for another round just to maker sure. Sure enough 3 weeks later and another fever and terrible puss filled sore throat, we told our pediatrician our experience with the on call nurse practitioner before. They sent us home with another steroid, and like magic, my child was healed within 24 hours. 

The problem with treating PFAPA with steroid is the episodes get closer together, more frequent and intense with each steroid treatment. 

Which is exactly what happened to us.

Luckily, our doctor documented the problem this time and we were able to have my youngest daughter’s tonsils removed which solved the problem.

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com
Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

Shortly after my second youngest daughters tonsils were removed.

Our Journey Through PFAPA – Family Friday - CraftyWildflower.com
Our Journey Through PFAPA – Family Friday - CraftyWildflower.com

We didn’t visit the doctor for almost 2 years after their removal.

Something I hadn’t ever done, and my oldest was now 16.

This is what everyone else experiences. This is heaven I thought!! ??????

And it is! My kids are rarely sick these days. We struggle with other things like allergies and asthma but the fevers and sore throats completely stopped. 

No more night time alarms for medication, no more lethargic and constantly sick kids, no more medicine and worry. 

There was hope!

If you or someone you know is struggling with PFAPA or showing symptoms, please seek out a physician who can help you. Don’t suffer like we did. Don’t be afraid to “shop around” for the right doctor who will listen to you. Keep good records. Write down symptoms and fevers on calendars for your own records to take into a doctor. 

Don’t be afraid of tonsil removal. I had 3 of my 4 kids tonsils removed, and it was the best, life changing thing we have ever done. 

It improved their quality of life 100%

Even my youngest, who deals with Ehlers-danlos syndrome (more on that diagnosis another day) had hers removed. She stayed in the hospital overnight but recovered fully and grew at an alarming rate after we removed her tonsils. She went from pale and sick to healthy, growing and happy!

If anyone is going through this and has questions, please feel free to send me an email at support@craftywildflower.com

I’ll help answer any questions if I can.

Here’s to health and happiness!! 

Have a Happy Day!!

XOXO

Susan

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