PFAPA

Hi, my name is Adrienne. I am writing today to ask if anyone has a child with PFAPA? My youngest son who is 2 is pending a diagnosis of PFAPA. I am not even sure that this is what he has. We have been to the er several times for high fevers going all the way up to 107.2. They keep telling me its viral. He gets these fevers every six weeks usually to the day. The only other symptom besides the fever is sores in his mouth. His neutrophils seem to be low also. We were sent to a specialist who ruled out cyclic neutropenia. it isn't a thyroid issue either. His cbc's always seem to be abnormal. Can ANYONE shed some light on any of this?

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Adrienne, I feel your pain. I also have a 26 mo old who has been suffering from fevers monthly for the past 10 months. They have gotten up to 104 and last 3-5 days. This past October our pediatrician tried him on prednisone, mainly to see what his reaction was. If the fever wasn't resolved, it would more than likely have been a virus. I have never imagined a medicine could work so quickly! Within a half hour of him taking it, his fever was completely gone and he was back to normal. I did know a side effect of the prednisone was the fevers may come back sooner. He went exactly a month in November, took another dose and the next episode was only 3 wks apart. He took another dose. Well, we had another episode over the weekend- it had only been less than 2 wks. I took him in to the doc today who contacted a specialist at the Children's Hospital in Little Rock. I guess she is working with 2 children with the same thing and has been prescribing prednisone for 2 yrs. He mentioned a tonsilectomy as another option down the road but that it wasn't a 100% cure. I have been doing muuuuch research on this condition and tonsilectomy seems to be the best way to end the cycle. Some people in certain studies have some episodes come back a couple times a year. My concern is what exactly are the side effects of using prednisone consistently? For the month of December we will have had 2 doses. As a mother, I want to do whatever is quickest to make my baby better. I have to respect the doctors view also and not jump to anything too quickly. Have you come across any new findings?

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Hello,
Yes PFAPA is not wide known. BUT it is far more acknowledge now than when my daughter was first DX 7.5 years ago. DD was 2 years old and had been fevering almost since birth. We were blessed that I have a wonderful ped whom knew me well(i have a now 20 yr old also). She worked so hard to help DD. But it still took us 2 yrs before we saw a rheum @ Boston Children's he had just been at a conference were the 'guroo' of PFAPA presented.
Ask for sed rates and CRP(C- Reactive Protein ) levals to be drawn during fevers and also during wellness for comparision. The sed and CRP levals tend to be high during fevers. It is beleived that the fervs are the bodies response inflamtion as the SED and CRP markers are inflamtion markers. The ulcers, stomach pain, light sensetivity, leg pain, head aches etc can all be present. Not all children have all symptoms. Some start with some and decrease or increase. In my DD's case she is severe. Fevers run over 107 in 30 minutes or less, only controlled by steriods. She started at every 28 days as a newborn went to every 18 days then every 12 days for 6 days of steriods. So she was 'healthy' 6 days then it started all over again. At the age of 3 she had a T&A, for DD it brought her fevers to every 18 days and she had much better sleep at night. We were happy with this! Over time thanks to NIH's drug protcal the past 4 yrs she has been on Kineret and her fevers are roughly 6 a year. BUT she has major chronic inflamtion issues in her feet, legs, hand. She is on amny many meds and also does Reiki to help. The DX of PFAPA is a rule out DX, there are several KNOWN genetic fever syndromes which should be rules out. When DD was DX there was hardly anything out there on PFAPA, in the past several years there has been an explosion of DX's I always tell the newly DX to make sure the child is tested for all the known genetic diseases. Since a true DX of PFAPA really can not be given without ruling out the KNOWN diseases. Just my '2 cents'
Please feel free to ask any ?'s
Also here are some rescources out there:
yahoo......groups......PFAPA
yahoo....groups.......TRAPS
yahooo....grops........HIDS
Facebook.....PFAPA
Facebook...CAPS
Tracy

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Hang in there! My daughter is 14 and has had PFAPA for most of her life. Tonsillectomy did not work for my daughter, but it is still effective for many. My daughter's blood work always comes back normal. Typically, children outgrow this by puberty. My daughter hasn't, but the doctors still think she will. It is frustrating. You have to be careful with Prednisone. It often causes the episodes to occur more frequently and in our daughter's case only lessened an episode by a day. My daughter does see some benefit from daily doses of Tagamet. She used to have episodes every month now there every 2-3 months. My daughter's PFAPA isn't as severe as the commenter's child above, but is still a very frustrating disorder. Good luck.

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Julia, how long did your daughter have the fevers before they did the tonsilectomy? My doctor also mentioned that most children out grow this, but I don't think either my son or I can do this for another 10 yrs! I can't even imagine how hard it would be for a 14 yr old. The fevers can be so debilitating, has she missed much school? That is another of my concerns for the future. At this rate my son will be missing a week of school every month! Is Tagament a steroid also?

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She had been having fevers about 2 years when she had her tonsillectomy. At the time, she had not been diagnosed with PFAPA. You asked about Tagamet. No, it is not a steroid and it is about as safe as you can get. It is used as an acid reflux drug in most people. It is only by coincidence that doctors found out it sometimes helps prevent PFAPA episodes. It doesn't help everyone, but it has reduced my daughter's frequency episodes from every month to every 2-3 months. For a few years it was every 4 months, but this last year they have increased in frequency.
We homeschool. I don't know how she could do regular school. Normally, she is an over acheiver, but this year she is behind in a couple of courses due to the PFAPA episodes. My daughter's case doesn't appear to be the norm, so don't worry. Your child will probably outgrow it sooner. It is discouraging, but my daughter's attitude is great and I do remind myself that it could be so much worse. PFAPA is for the most part a benign condition. It is terrible during an episode, but she is otherwise healthy with no organ damage or grim prognosis. Reading other peoples' disease posts on NORD helps remind me of how blessed she is.

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Hi, Everyone!
I'm so glad I've found you! I was beginning to think I was the only one with a child with PFAPA. I was looking for support groups about this and someone to talk to.
My son is now 6, and was diagnosed with PFAPA in 2010. After MONTHS of steady, timed, recurrent fevers...his Pediatrician finally realized that I was right and something wasn't right here. After 2 rounds of bloodwork, urinalysis, and rehydration in the hospital, we were sent to a Pediatric Rheumatologist out of Penn State Hershey Medical Center. He said that my son appears to have PFAPA, after eliminating other factors and possibilities for now.

I don't know anyone else in my area with this, or anyone else period with this. I go through this every 4-6 weeks like clockwork usually. I actually thought that we might be out of the woods there for a little while. He went several months with no fever...but it came back 3 weeks ago...and he just started another episode on Monday. So, today is day 3 of this episode. The highest his fever has gotten is 104.7...but usually it remains in the 103's.

Does anyone know of any possible treatments that aren't prescription...such as herbal or natural? Is there anything that you do to get these fevers down when they appear? We've been doing the usual, Motrin, baths, popsicles, etc...
They are sending him for more bloodwork when this episode is finished. Apparently, there are things in the blood that are only visible within 24 hours after the episode is finished...so hopefully we can get some more answers.
I look forward to talking with you all!

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Also, about the Tagament question.
I was informed by my Dr. that it basically lessens the episodes a little. For example, if their normal is 104, the Tagament might make it only come to 103. If it normally lasts 6 days, the Tagament might make it only last 4 days, and etc. Personally, I haven't been able to find many articles about Tagament linked to PFAPA...so I haven't found many side effects. The only one I've heard about is a possibility that it has been linked to slight breast enlargement in older boys. That's what my Dr. told me at least. But liek I said, I haven't been able to find any other side effects linked to PFAPA...

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I'm wondering what people do about school and how much school kids with pfapa miss? Does anyone have a 504 plan for their child which would allow them have more "sick" days without considering them truent?

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To the person who asked about school, my daughter is homeschooled. I see no way she could go to a traditional school. She is in high school and it would be very difficult.

As for nonprescription remedies, we have yet to find anything that has been effective.

Also, I ran across a study that was just published in April about a promising new treatment.
http://www.news-medical.net/news/20110411/Scientists-identify-new-treatment -to-combat-PFAPA-syndrome-in-children.aspx

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One of the alternative medicine that is working for my daughter is Upper Cervical Chiropractic care...It has been more than 5 months that my daughter has been fever free...google ' pfapa stopped' and read my blog.

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Hi guys, quick question re your kids and PFAPA...

Do you all experience what I call 'post fever syndrome'... i.e. 2-4 days of extremely poor behaviour post the fever, mostly due to exacerbated tiredness and an inability to control behaviour? This for us is harder work than the fever and was interested in others experience... Our daughter is amazingly well behaved and an incredibly good listener, however when in 'post fever' things change considerably and we're embarrassed to take her anywhere for a few days until she has had time to recover. It usually takes as least 3 days after the fever until behaviour starts to adjust again. Typical post fever includes extreme tiredness, shouting, rudeness, difficulty to get back onto food, etc.. etc...

What are your experiences?

Background
Our daughter is 3.5 years old and has had fevers since birth and has since been diagnosed with PFAPA (partly as my wife is a paediatrician so was very observant from a young age and was aware of the syndrome). Everything else ruled out and responds to prednisolone with hours, but if we give the steroids it brings fevers back every 10-14 days instead of 3-4 week intervals. We stopped the steroids after a couple of months due to concerns of the cumulative dose over the year.

We've just been trying out 'Colchicine' - she is seen at Great Ormond Street and the consultant there has seen some positive results from this. We had eight weeks without a fever (daily dose, no side effects, treatment for gout! small tablet we crush with juice in the morning) but then the fevers have come back again three times with their normal recurrence and severity.

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My daughter, who is now 15, might be able to help shed some light on what your 3.5 year old is feeling. After her episode ends, she is still very tired and a bit irritable for a couple of days. We have also learned that before an episode begins, she is very tired the day before as well.

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Hi Julia, thanks for your reply.
Yes we have noticed our daughter is tired and goes off food the day before. She also goes mottled in her face and cheeks, sometimes over her body too. We can predict when it is coming by both the timing and this 'pre-fever' episode.
She then doesn't usually eat anything during her fever except for drinking loads of milk, albeit this has got better in the last year as she has become a bit older. We then really struggle to get decent food into her, particularly where we've become desperate and she hasn't eaten for a few days and everything is a fight. I guess the mixture of tiredness and being 3 isn't the best combination.
Would love to hear from your daughter. Thanks again for responding.

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My daughter was diagnosed with PFAPA in May. She is four and a half and has had recurrent fever approximately every 5 weeks since she was 14 months old. 
I knew when an episode was due to start as she would come into my bedroom in the night for two nights before the fever starts.  She gets a high temperature and tired. Her lips go very dry, she has strange smelling breath and ulcers in her mouth.  She stops eating and just lays on the sofa all day.  She is sometimes sick, usually in the night though and after i have given her paracetamol.  After a few days, she gets pains in her fingers and legs. Sometimes she complains of a sore throat.
I would take my daughter to the G.P and each time they would say to me "it's a virus". I came to hate hearing those words and many times, would end up not taking her.  After lots of worrying and knowing after so many fevers that something wasn't right, I asked to be referred to a specialist.  I got my appointment at the hospital and the doctor did blood tests,an ultrasound and urine tests and told me there was nothing wrong.  A few weeks later, I received a letter from the immunologist, Dr Vijaydurai, asking if we would go and see him.  Anyway, he ran more test and took all my daughters history.  A few months later, we went back for an appointment with a paediatric immunologist from Manchester, Dr Hughes and they finally diagnosed PFAPA.  My daughter had her tonsils out nearly two weeks ago and started with a high fever 4 days later.  I thought it may be a post op infection so I took her back to the hospital.  We stayed in hospital for 5 nights and for three of those days, my daughter had a temperature of 102-106 which just wouldn't come down.  It turns out it wasn't a post op infection but another PFAPA episode. I am hoping now that the episodes might become less frequent especially after the tonsillectomy but with her having an episode after 4 days, I am not very hopeful!!
Anyway, at least at last we have a diagnosis and won't have to listen to "it's a virus" again :).  
My daughter also gets very moody and naughty for two or three days after her fever.  She is very rude, fights with her sister and can sometimes be aggressive towards me.  Normally she is very polite and wouldn't dream of lashing out at me. I find it hard not to shout at her as being up in the night with her and all the stress of her being ill has, by that time, taken it out of me! 
It is comforting to know there are other people out there that are going through the same things as me.  
My worry now is that she's due to start school in September and I don't want her to be missing a week every month.  Hopefully her tonsillectomy will reduce the frequency of her episodes.

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Hey JessLucy's Mum,
How utterly disappointing about the tonsillectomy. It's just exhausting and 'post fever' is so hard on everyone.
We recently went back to Great Ormond Street and they were somewhat encouraged by the initial response to the Colchicine, despite the fevers coming back again. As such, we've doubled the dose and so far we've not had a fever in 7 weeks. It's definitely doing something, but only time will tell.
She had a week when the fever was due, when she was completely exhausted and we had 'post fever' without the fever but no fever. Recently it's been exhausting as we've a new baby as well, so we're all shattered.

If you haven't got the Dr to try it out, I definitely think it's worth it. It's one tiny tablet crushed with juice in the morning. Also get them to do a blood test checking for both CRP and Amyloid levels. CRP will be normal i.e. no infection, yet, if anything like our daughter's, the Amyloid levels very high, our daughters were in the mid 50's (usually would be under 10) even when she doesn't have her fever. We have to do a blood test now in the middle of a fever to see how high it spikes to, then another one out of the fever after a few months on the Colchicine.

As far as I understand, Amyloid is a marker in the blood which effectively shows the level of activity of the immune system, so it's like her immune system is incredibly overactive and always turned on and then periodically tries to either fight a non-existent infection or responds massively to any tiny infection. I think this also helps to explain why she is so over exhausted all the time as her immune system is so busy.

I think Colchicine can gently, over time, reduce this activity and Amyloid levels can be used to monitor the effect of the colchicine. There's no side-effects other than some loose stool, so its introduced slowly, and the dose is tiny, but there are no side-effects like steroids.

Definitely worth trying - get a referral or advice from the Rhematology team at Great Ormond Street if you're in the UK. Hope this helps.

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Thankyou very much CJCJ for your response, it's so strange feeling like I knew nothing about what was wrong with my daughter for years and in the past few months with diagnosis and operation and the info off you!! It's a bit overwhelming all this advice, in a good way!! I have a follow up with a doctor from the hospital in 6 weeks and will be definitely bringing up the points you mentioned about Colchicine and the blood tests.  I read on here about the steroids and for me, it is not something I would want my daughter to have.  I do worry about tablets with there side effects but the Colchicine doesn't seem that bad from what you've said. If the episodes don't decrease, I would definitely think about it.
I also have two younger children, a one year old and a two and a half year old.  I was told that it is very rare that siblings are also affected. I seriously could not cope with my younger two having it too!! It's been going on for so long now that it's just a way of life, any improvement would be brilliant, especially now she is due to start school.  I was told that there are only 2 children in the North west (I live in Lancashire) that have Been diagnosed with PFAPA so it is strange to be talking to you :).   Congratulations on your new baby, thanks again for your advice x x 

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The steroids are worth trying just to prove it is PFAPA - basically it stops the fever within about an hour or two. It's amazing. We get about 24 hours of tiredness, but nothing as serious as during a normal fever.
The problem with the steroids are that the episodes then come back much more regularly. Our daughter's came back every 10 days. So then we were giving such a high dosage of steroids (we tried it for 3 months) that we decided to stop. It's also very dangerous to be on steroids when there's chicken pox flying around and you can't have normal childhood vaccinations as well as serious problems with growth etc... in young children.
Anyhow, great to be in touch. I'll PM you if you want to keep in touch would be great to hear how things go with your daughter.

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Uggh! After reading all of this I am certain my son has Periodic fever syndrome. He has had recurrent fevers, stomach pain, vomiting, diarrhea, mouth sores, joint pain esp knees. Recent attack fever got to 107 and I thought i was going to have a stroke. Poor thing is only 3 1/2 and has been hospitalized 3 times. Last summer he was hospitalized and twice this summer 3 wks apart, but same symptoms. I was given the virus run around for 3 yrs from previous pediatrician. I switched pediatricians and now have one that wants to get to the bottom of this, but she seemed to quickly dismiss Periodic Fever. What should I do? The clincher is I am almost 100 percent this is what it is especially b/c I met my husband when he was 19 and thought he was so weird b/c he would have similar episodes 3 times/year. My husband had episodes into his twenties often getting apthous ulcers in his mouth/tonsils. He had a tonsillectomy in his early 20's and was very blessed that episodes subsided. Think he has only had 1 every year or two now (not completely gone but not as severe). PLEASE< PLEASE advise me what specialist I should see. We are in Chicago area if anyone has a recommendation. I do find it discouraging that not much seems to help, but at this stage a diagnosis seems helpful!

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Hiya "sickkid" I'm sorry I can't help you, I live in UK so really don't know how it would work for you. I asked my GP to refer me to the hospital and saw a paediatric doctor at the hospital who then got in touch with an immunologist who then worked with a paediatric immunologist who diagnosed my daughter. I feel I was very lucky to get a diagnosis. PFAPA is not well known and I have met lots of people working in the medical profession who have never heard of it. Hope you get sorted x x

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Here's the links I mentioned that are useful info sheets to explain it to others e.g. teachers:

http://www.printo.it/pediatric-rheumatology/information/UK/pdf/13_10_PFAPA% 20_UK.pdf

http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditi ons/pfapa.asp

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