Although Not Life-Threatening, PFAPA Has Definitely Affected Isabel’s Life

My daughter Isabel, who will be six in July, suffers from PFAPA or Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome.

“This syndrome includes recurrent episodes of fever with aphthous stomatitis (mouth sores) and pharyngitis (sore throat with redness). Occasionally, there also may be exudate (white patches on the tonsils) and usually the lymph nodes in the neck are enlarged (adenitis). Episodes of fever start suddenly and last for 3-7 days. Fevers occur routinely every few weeks; often, families know the exact day when an episode will start. Some children have other symptoms like joint pain, abdominal pain, rash, headache, vomiting or diarrhea. Children are completely well between episodes.”

She started getting high fevers every two weeks at 18 months old. Her highest fever on record was 105.6F (and I know that is not the highest it can get). The episodes would last about one week.

At this point, we made the decision to get her out of daycare and to see if we could find out what was wrong with her. The diagnosis took nine months of regular doctor’s visits, journaling, sleepless nights, baths in the middle of the night to keep the fever down (because regular fever medications didn’t work, etc.). Even at that young age, she suffered from terrible mouth ulcers, joint pain, headaches, dark circles under her eyes and stomach pains.

After her diagnosis we tried several medications, but they only seemed to spread the episodes out, without providing a real “cure.”

We then tried Prednisone, which is a steroid, and it worked like magic for getting rid of her fever, but the idea of giving her this medication without any studies showing long-term use on young children was not an appealing option to us.

So, at the age of three, we opted for her to have a Tonsillectomy and Adenoidectomy, since the few studies on PFAPA showed great success in getting rid of the episodes with this procedure. The surgery seemed to work for a few months, and it provided some relief (long remission periods, fevers around 102F and shorter episodes), yet she continues to have these “feverless episodes” where she gets all the other symptoms (ulcers, headaches, dark circles under her eyes, joint pain, headaches and stomach pain).

At this point, we are about to try Prednisone again to see if it stops the episodes and to see if it might give her a break from these episodes (which now happen every four weeks.) While she has had a good childhood, these episodes sometimes prevent her from going to school or from other social activities.

I am sharing her story because I feel that growing up with PFAPA, while certainly not a life-threatening syndrome, does affect how we live our lives. We plan around the time when she’ll be having an episode, so we don’t travel or make plans for social events on those days. So PFAPA disrupts our lives enough that it is not fun. If I cannot do anything more for my daughter, promoting awareness is important. For that reason, a couple of years ago I created a Facebook group in Spanish, which currently has 47 members, to help those who do not speak English and cannot participate in the several support groups that already exists.

And just the other day, after taking her to the doctor again, trying to see what else we can do to help with these episodes, the doctor told me that thanks to my daughter’s case, she has been able to diagnose two kids suffering from PFAPA. So, while I’m still sad we have to go through this every month, I’m glad two families found answers.

Filed Under: Uncategorized

Tags:, , ,

Global Genes Comments

  1. Hi, I read your blog named “Although Not Life-Threatening, PFAPA Has Definitely Affected Isabel’s Life” like every week. Your story-telling style is awesome, keep doing what you’re doing! And if you are interested in other sites you can look my website about szexpartner kereső Thank you for experience! Have a great day my friend!

  2. In the early days of researching my daughter’s condition (an undiagnosed periodic fever syndrome without mouth sores), I only found literature on PFAPA. But after joining an autoinflammatory support group on Facebook, I learned there are dozens of other diseases that are very similar to PFAPA and commonly the initial diagnosis is that- but it changes after symptoms change and genetic testing is done. For anyone who hasn’t gone through the genetic testing, I recommend checking out the Autoinflammatory Alliance’s website here and looking carefully at the symptom list. While PFAPA is benign and can be outgrown, other conditions can be long-term and quite serious unless the right treatment is found. For us, we are still chipping away at what’s going on but we hope to get genetic testing done if my daughter’s flares return (she is having a break).

    As for causes of recurrent fevers that are of the infectious disease nature, I definitely recommend talking to your dr about testing or treating your child for it too just in case- commonly they are Epstein-Barr (aka mono), Bartonella (trench fever and cat scratch fever), Lyme Disease (Borrelia; the bulls-eye rash isn’t very common!) and its co-infections, tuberculosis (the great mimic), and rat bite/Haverhill Fever (from rodent exposure and is super hard to culture so dr’s usually put patients on a long course of penicillin- which we have just finished and I hope this is what it was!). I hope every one of you finds the diagnosis and treatment right for yourself or your child!

  3. Joanne Pearson says:

    My daughter is 26, and had these exact same symptoms that started at 12 months old. all cultures would be negative , and temp greater than 105-106. she would be listless , large lymph nodes and whimper in pain. she was so sad.
    Now her daughter has had it since she was a year old, but we have a Name for it now and can use the steroids to treat the first sighn of fever. The funny thing is she is so healthy all in between episodes. I am just glad to know that I was not a crazy person when my daughter was little

  4. My 6 year old grandson has been diagnosed and my poor son is a single parent. I watch him have to leave work and my grandson miss school. He had one last Thursday and another tonight Tuesday. No child should have to have these fevers and mouth sores bloody noses and headaches. The steroid lessens the duration but they come way more frequently. Help

  5. Was your daughter tested for Epstein Barr Virus? My son just turned 3 and has had 8 months of episodes. My dr suggested a periodic fever last month but sent us for blood work and his blood work showed that he had had mono somewhat recently but wasn’t currently positive for it. He is currently having another flare with fever of up to 105 and I find it hard to believe that mono can come back once a month for 8 months! Thank you for your time!

  6. I just cried when I found this site I thought I was going crazy, no one believed me. My now 6yr. grandson has been going thru this for 8 months now the doctors are scheduling to remove the tonsils. I fear it wont work I hate any surgery. I would also like to find this support group or blog. His fevers reach 104 or higher, falling behind in school, I cant get real answers from any doctor, strep test come back neg. all the time. Fevers come every month like clock work.

  7. My son was finally diagnosed at age 5 with PFAPA. His rheumatologist put him on a daily dose of Cimetidine, which you can get over the counter as Tagamet. He said they don’t completely understand why, but this medication reduces the number of fevers. Since my son was put on it, he has gone from having a fever every three weeks to stretches of more than seven months without a fever.

  8. I am from India have twins a boy n a girl .of these two my daughter keeps on getting fever every 10 to 15 tell u the truth I am becoming a nervous wreck always counting days praying to God and c
    Continuously hoping this time she won’t or should get

  9. Fletcher Magill says:

    Our daughter suffers from PFAPA, too. We noticed her having repetitive fevers and aches within about 6 weeks of her first year of school (4 years 8 months). Initially we thought she was in contact with other kids and generally just catching the flu etc. These fevers and aches continually happened over the next number of months. We tried to eliminate all allergies and tried various diet changes and homeopathy alternatives. She had missed a fair amount of school and fearing that she would be repeating her first year we continually visited our family doctor, whereby off chance one had said she’s got PFAPA, “I’ve seen it before, give her the steroid (Pedrisone).” The steroid nullified the symptoms but would basically nullify anything. We didn’t want her taking steroids every 2,3,4 or 5 weeks for her childhood, It concerned us what the steroids could do. It has been a frustrating 12 months since due to the lack of recently qualified specialised doctors and surgeons in our area. Misdiagnosis, underdiagnosis and a long surgery waiting time to have tonsils and adenoids scraped out.
    About 4 months ago she had the tonsillectomy performed, hoping that would have ‘cured’ the symptoms. It did not ‘fix’ the problem but has reduced the severity and frequency of the episodes. She is sore and unwell for about 36hrs at a time now (compared to 3 to 4 days) but she notices bearable to increasing unbearable stomach pains about 2 days prior to the fever initiating.
    It’s warming to know that these fevers and pains will hopefully near cease by adulthood. Until then, as parents, we plan and organise to enjoy the times that our daughter is ‘feeling awesome’ and take care and have ‘down time’ when she’s ‘not so awesome’

    If other parents in the same situation can let me know what to expect over the next years, please let me know.

  10. My son was diagnosed yesterday with PFAPA. The sad part is, I had to take the suggestion to my doctor myself because I was still getting the brush off every month I brought him in with “its just a virus”. Finally, after an ER doctor gave him prednisone and within 2 hours all symptoms went away, my pediatrician believed me. Moms and Dads, you know your kids best! If you feel like PFAPA might be what’s going on, bring it to your doc’s attention. And, don’t forget, TRAPS is similar to PFAPA but genetic. If a tonsillectomy does not drastically improve PFAPA, beg for the genetic testing for TRAPs (and other similar auto-inflammatory diseases). TRAPS is not present at birth. It is a protein folding that occurs later on and responds the same as PFAPA with steroid treatment, but does not respond to tonsillectomy. Also research HIDS if the child has had rashes since birth.

  11. my daughter is 6 yrs old and just been diagnosed, although shes had frequent bouts of tonsillitis the doctor has only now informed us of pfapa, i am now frantically searching the internet for answers. has anyone tried removing dairy and high processed foods from their diet??? as this is an inflammatory illness i am going to remove all high inflammatory foods before i consider surgery…. this is an awful illness and effects every aspect of my daughters and our families lives, fingers crossed that more research is made in to this and some kind of cure is found.

  12. My now 19 year old son had PFAPA, it was awful trying to get a diagnosis. Every 28 days, (I could basically preset a doctors appointment) he would present with all the symptoms listed, he saw his pediatrician monthly, every time they would test him for strep, then we would be sent to the ER at one point they almost did a spinal tap, but he was crying so hard he would not be still enough. Finally, we got a referral to Children’s hospital in Seattle for rheumatology. The doctor there had just written a paper on PFAPA and diagnosed him almost immediately. He was 2 1/2 at the time and had been having these fevers for almost a year. I always wondered if I should have asked for a baseline MRI as he has been slightly delayed in school. I can’t help but wonder if fevers as high as 106 without seizures can cause damage.

  13. This post as well as all the comments have been SO helpful!! We have been seeking out the answers to my 4 yr old daughters mystery illness. After many pediatric visits as well as specialist visits we are now moving forward with genetic testing to rule out and diagnose what particular pfs she has. I am curious as to what specialist everyone has had the best luck with? We have been reffered to immunologist, rheumatologist and neurologist..and assuming we have to get a tonsillectomy that would be a ENT? Is that right? Just curious what specialist has been the most helpful to everyone else.

  14. Hello, My son has had PFAPA for 18 years. He is currently in college and the episodes have decreased to about once or twice per year. When he was young it was every 28 days and fevers as high as 107. After a long involved process and pressing doctors for a real reason. He was finally diagnosed with PFAPA. He got dark circles under his eyes, ulcers in his mouth, very bad headaches and his legs hurt badly. Currently he takes prednisone 40 mg on the onset and 40 mg the next day. This type of dose seems to really work. Dylan started with this condition when he was about six months old.

  15. Janine Morgan says:

    My daughter is now 14 1/2 years old. She started being sick in preschool..approximately 3 years old. She was always sick. I thought it was something she was catching at preschool, but no other children were sick. It got to the point that I was saying “Mckenzie is sick AGAIN”. My friends even pointed it out to me. I started keeping a record of her fever episodes. Turned out it was happening every 21-28 days. She had it all….high fever, ulcers in her mouth, swollen glands and malaise. Took her to pediatrician (which we were no stranger to) and told her how desperate I was and showed her my record. She agreed. She even tested her for juvenile rheumatoid arthritis! Luckily that was negative. She then called Duke University Hospital to get me an appointment. The doctor she spoke to on the phone was able to diagnose her over the phone! PFAPA!! We started with the prednisone which seemed to lessen the the fever to a couple of days, but the episodes were still 21-28 days. Then she started real school and was missing a lot of days. With my research I found that removing the tonsils and adenoids could help. My pediatrician was on board! She was 9 when we did the surgery and she has not been sick since then…other than colds or an occasional stomach bug. I know surgery is invasive and scary, but it helped her and ME! Just thought I would add my two cents worth because I believe doctors are unaware of this syndrome. Like I said surgery is scary, but taking prednisone and antibiotics when they are not necessary is scary also. Good luck to all!

  16. How do i find this blog on facebook please?

  17. My daughter is 22, and she was diagnosed when she was 10 years old. She had tonsils and adenoids out, and it helped tremendously. She never had mouth sores or joint pain… Mainly throat swelling and extremely high fevers (105 degrees typically). It took several years and many doctors to finally get diagnosed. Her earliest fevers were around age 2 1/2. She still gets high fevers 2 or 3 times per year and had just learned to live with it. Much better than every 28 days like she was before surgery!

  18. Thank you for posting so we can all support each other. My daughter, now 9, has suffered for many years with PFAPA. A year ago she had her tonsils and adenoids removed, and that seemed to help for many months. Before surgery, she had missed 35 days of school and has struggled with her learning, reading, and writing. She had the high fevers, headaches, mouth sores, joint pain, nausea and vomiting. Now, post surgery 14 months, she continues with mouth sores, lower fevers, joint pain, headaches, and nausea with tummy aches. The biggest difference is that the fevers are not as high. Praying that this school year will be a healthy one and praying for relief for all those families dealing with this condition as well as other illnesses.

Speak Your Mind