PFAPA and my little girl

After over a year of taking Abbie to the doctor every month when she went through this crazy fever cycles that sometimes reached 104° we finally got a diagnosis in August. Finally having a diagnosis has been such a relief but at the same time I finally had to admit that there really is something wrong with her.

What is PFAPA?

PFAPA is Periodic Fever, Apithous Stomatitis, Pharyngitis and Adenitis. When people ask me what PFAPA is I always say "Period fever something something something." Basically it means that I can expect a fever once a month lasting five days that gets really high. She sometimes complains of her mouth hurting because of mouth sores (which I still haven't been able to find) or of stomach pain or of her legs hurting. It tends to run in families. But the symptoms of these fever syndromes are so vague that it is rarely diagnosed and is sometimes misdiagnosed as MONO. I can tell when a fever is coming on because Abbie gets cranky and clingy and sleepy. She takes wonderful long naps but then the fever hits and we spend nights soaking her in the tub and I have to hold her all day. Did I mention she will have this into her teens. Basically baby PMS that she will grow out of just in time for the real PMS. Isn't she lucky. And you know what? No one knows what causes these fevers. They are just a result of her autoimmune system being triggered by some inflammation resulting in a fever.

What can be done?

Bwhahahaha....that's funny. Advil. Coffee for mom. Hugs. Get used to being sleep deprived and living as a zombie.  No seriously. There are four medical options. Are you ready for this. Number one: Motrin given every 8 hours with Tylenol in between as needed for spikes. Luke warm baths to bring down fever. Number 2: A single dose of steroid given when the fever starts usually makes the fever go away; however, the next attack may come as much as two weeks sooner. So basically a dose of strong steroids twice a month during her growing years. Number 3: Medications such as cimetidine and colchicine. The problem with this is that I couldn't find a pharmacy to fill Abbie's prescription One said they couldn't get it any more. Another said their supplier would no longer fill the liquid version. And he told me that because of the horrible side effects he would recommend the steroids over this option. I called my pediatrician's office to see if they could find a different prescription that might be available and they never even called me back. So I thought I would be destined to ride out the fevers until her next appointment. Number 4: Having a tonsillectomy sometimes cures the fever but her specialist said that he would prefer her to wait until she is older to have this done and I don't want her to undergo major surgery unless it is really going to work. Some cases have the fever to go only to return a year later. Sometimes it doesn't affect the fever at all. All of these options just seem so bleak. Here I am with this beautiful, smart, funny little girl and when she starts school she will be missing all these days (medically excused, but still) every month without anything I can do about it.

What did I do?

So my mom just happened to be listening to Dr. Oz. She is this huge healthy/ natural health nut and she loves him because he looks for natural ways to make us healthier. He was talking about tart cherry juice and all the wonderful things it does for arthritis, pain relief, inflammation, trouble sleeping through the night, and reduce heart disease because of the antioxidants it contains. Dr. Oz explains that an enzyme causes inflammation which causes pain or in Abbie's case, fever. The tart cherry juice blocks the enzyme, whatever that enzyme may be. He recommends having it with your dinner. What I have done is mix one teaspoon of tart cherry juice with a glass of water and give it to Abbie just before she goes to bed (because she is always asking for something to drink before she goes to bed). I've been doing this for three months with surprising results. The past three fevers have been very mild and have lasted only 2-3 days. This past month I didn't even give it to her religiously and we haven't had a fever lasting more than two days.

What did Abbie's doctor think?

You never go against your doctor, right? I don't know. A member of my family committed suicide after following doctor's orders on a drug that had a known side effect of depression and even when he told his doctor about his concerns the doctor told him to stay on it. Do doctors always know everything and what is best for you? I'll leave that up to you. In this case, Abbie's specialist liked my choice for Abbie. I told him about the roadblocks and how her pediatrician had not worked with me. He wants to look more into tart cherry juice and wants me to keep track of how it affects her future fevers. She goes back in three months. He said if this is anecdotal then we will try a different drug, but if it is working and she is responding then we should continue.

More info on PFAPA:
http://www.rarediseases.org/
http://www.nomidalliance.org/index.php
http://www.clinicaltrials.gov/ct2/show/NCT00001373?term=periodic+fever&rank=1
http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/pfapa.asp
https://www.facebook.com/groups/pfapachild/