Wednesday, January 2, 2013

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/

14 comments:

  1. I have an inflammatory auto immune disorder that caused a neurological disorder. (Lucky me, my central nervous system shut down...)

    I take tart cherry (the pills tho) daily and it has SOOO helped my inflammation levels. My inflammatory panel lab values used to be off the chart... I also did 2 years of daily high dose steroids, and a year on methotrexate (a chemotherapy drug) to shut my immune system down--basically like giving a computer a hard reboot. All those helped, but the cherry I believe as well as following *somewhat tho not religiously* an antiinflammatory diet has been what has helped the most. I've still been disabled, but my pain levels are manageable now and my ankles no longer look like they belong on elephants. :)

    I hope they find something that will CURE your baby girl. How heart wrenching for a mama.

    Until then, I will pray for her if you don't mind.

    I found your blog looking up things about the PUL diapers for BVB fabrics... I am enjoying it. Thanks!

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  2. Interesting! Thanks for the tart cherry juice tip. My three-year-old son has PFAPA.

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  3. Hello, my daughter might have PFAPA, She is 5, she has been getting a fever every month now since January. We went and seen an ID specialist last month with not too many answers given. I was wondering if you are still giving your daughter the tart cherry juice and if it still continues to decrease the amount of time she has her episode?

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    1. Yes, She hasn't had a fever all summer. She started coming down with one Sunday night, but it has been very mild. She loves it mixed with fruit juice. It also helps her to sleep well. I give it to her right after dinner.

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  4. Hi, my son is 2½ and has PFAPA. I am altso wondering if you still give your daughter the tart cherry juice?

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    1. Yes, She hasn't had a fever all summer. She started coming down with one Sunday night, but it has been very mild. She loves it mixed with fruit juice. It also helps her to sleep well. I give it to her right after dinner.

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  5. Hi, do you use concentrated or unconcentrated form for your daughter? Where can I find dosage recommendations? Also, how long had she been taking it when you started to notice improvement? Can't thank you enough for posting this. My 6yo w/PFAPA and I are excited and hopeful about this possible help!

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    1. We use the concentrated form and simply went by Dr. Oz's recommendation of taking a tablespoon at dinner. Sometimes Abbie refuses to take it without mixing it with juice because it is strong and she now dislikes cherries, but her fevers are down to only two days.

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  6. May I ask WHERE you buy your tart cherry juice? My 4 year old was recently diagnosed with PFAPA and is taking prednisone at the onset of an episode, but since taking it, her episodes have become as frequent as every 14 days. I would love to try something else for her before we attempt surgery :(

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    1. Grocery stores that have the health food section usually have it, but I just order mine off of Drugstore.com. My mom keeps this brand at her house for Abbie: http://www.drugstore.com/dynamic-health-organic-certified-tart-cherry-juice/qxp399162?catid=183303 I bought this one because it is cheaper. http://www.drugstore.com/dynamic-health-100-pure-organic-juice-concentrate-tart-cherry/qxp412882?catid=183303 I don't know that the different brands make that much of a difference.

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  7. For those of you looking for tart cherry juice and know how strong it is, I just found these chewable.....might be a solution if taste is a problem: http://www.drugstore.com/enzymatic-therapy-tart-cherry-ultra-chewables/qxp404835?catid=183303

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  8. Just stumbled across your blog in Googling PFAPA and tart cherry. I'm just about to order the chewables on Amazon. Wondering, how is your daughter doing with them? Will she take them? My 3y/o with PFAPA is pretty picky. Also, how many chewables are your giving her? TIA and thanks so much for sharing your story and making things a little easier for the rest of us stumbling through this.

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  9. My daughter loves the chewables and wants more. I just give her one of the chewables. It's so much easier to get her to take than the liquid! Good luck!

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