any new news on PFAPA?

[tvoo]

My 19 month son has just been finally diagnosed with PFAPA.  After his 6th unexplained high fever in a year period, a new pediatric group has determined he may have this syndrome.  We have moved recently, but I took detailed notes since the first episode of the 5 day fevers.  He was always given antibiotics in the past. The 6th time, we did nothing but motrin and tylenol at the same time to keep him to 102.  It still lasted 5 days.  So, 3 weeks later (7th episode)when he gets it again, we did the prednazone for 3 days and his fever stopped within one day.  My questions are as follows:
I.  Could we have stopped the prednazone as soon as the fever is close to normal? (My son's manic moodiness was hard to take!)
2. Anyone have long term issues with either the steriod or Tagamet treatments? How do we choose what is best?
3. Anyone know of anyone tracking this syndrome to find out any patterns or treatment statistics? Is anyone doing a study? My son did not have a fever episode from August until March...why?
4. I have been told that these kids just stop getting the fevers.  Are their any parents out there who have lived through this and have advice and/or have any information on lasting medical issues in their children down the road.

[MWPT]

we also have PFAPA diagnosis after 1 year of looking for help.  similar sed/CRP as others.  ruled out TRAPS, FMF, HID thru testing. Steriod treatment provoked faster recurrence. Began 2 years of Cimetidine. Every summer (while not in school) we try an episode without cimetidine, to test if she's growing out of it.  I feel it makes her a bit drowsy, labile.  this summer stopped in July and fever didn't come back. Daugher is 6. has had fevers since 3. Had one epsidode of rash whcih concerned us, and now she has a fever from an ear infection.  (upside to PFAPA i found was that we almost never had a "typical cold" as it got "'cooked off")

[hope9705]

Hi,  we are quite lucky that our children have been diagnosed with Pfapa as when my son was getting diagnosed I was told that it could have been Leukemia which would have been devestating to me as I had already lost an 8yr old to a rare immune deficiency over 14yrs ago now.  It started when Luca was 6 months old he kept getting temp every 4 weeks without missing a beat his fevers would last up to 7 days with no break in between they told me to change from paracetamol to ibuprofen which did not make to much of a difference lucky enough we new a very good immunologist which was originally MY DAUGHTERS SPECIALIST.  We took him several times to the childrens hospital and it was there that we got to see the immunologist specialist one of the best in Australia after weeks of testing once a week for 10 weeks they the put it down to Pfapa he is now 34 months old he has gone for bigger gaps without fevers even though he has been having reaccuring ulcers in his mouth his fevers have seemed to get further apart and when he gets a fever he is given a dose of Prednisone and that is sufficient to get him through to the next hurdle I know he is in good hands the only thing is that his red cells are low and he gets extremely wingie and does not want to eat I beleive by reading that removal of the tonsils is the way to go he was given so much antibiotics that he never needed even now besides his fevers caused by his Pfapa he has not ever gotten sick but complains of being tired all the time which I beleive can be due to his iron defficiency.  Hope that eventually you will all be posting good news to the newly diagnosed as by the age of 5 I have been told is around the time the disease dissapears and give hope to people who don't really know what Pfapa is Good Luck to all.

[gracehouse]

Recovered!
Hi all,
I wanted to share my son's story of overcoming PFAPA in the hope that it may help someone's child, who suffers from the same. Here is our story:
My son's recurring fevers started at 6 months of age. Initially the fevers, occurring every 4 to 6 weeks like clock work along with swollen adenoids were his only symptom. No runny nose, cough infection or anything else. The fevers lasted 5 days and did not respond to tylenol and motrin. They would peak at 40'C every 5 hours. We were in despair, my husband was missing so much work, and we could do nothing for our sons suffering! At just over 1 year old he started to have a febrile seizure once during every episode. We were in the ER every month with no help. In August 2008 he had two seizures during one fever episode and spent 5 days in the Calgary Children's Hospital where he had a host of blood work and tests done, along with a brain scan. We were discharged with no answers,  only a referral to a pediatrian. At this time I began to do as much reading as I could and learned about PFAPA and it's various treatments but our pediatrician had never heard of it and wouldn't even entertain the idea that he might have it. The pediatrician sent us to an infectious disease specialist who diagnosed him with PFAPA, after just coming back from a conference on it and learning about it for the first time. We were very reluctant to start steriod treatment, and opted to go the route of adenoidectomy, although at younger than 2 our son was not yet old enough. So we prepared ourselves to wait.
Now for the good news! At around the same time as the diagnosis, we were contacted by an Upper Cervical Chiropractor in Calgary who had heard of our son's case and asked if he could see him. I was hesitant at first, not even knowing what "Upper Cervical" was and whether a chiropractor could possibly cause more damage than good. However we went ahead... The Dr. explained to us that Upper Cervical chiropractors specialize in the treatment of the very top bone in the spinal column, which encases the brain stem, called the atlas. They take very precise and careful measurements to assess whether it is subluxated (out of place). After performing x-rays on Benjamin it became apparent the his atlas was rotated and shifted over to one side, putting definite pressure on his brain stem. They also found on that very first day that the temperature on one side of the base of his skull was 1'C hotter than the other, another indicator of pressure on the brain stem. The day after his first adjustment, the difference in temperature was less than 0.25'C. Now for the best news! Treatments started at 2 to 3 times per week. The first month, he developed a fever, but it was entirely less severe and lasted only 2 days. He had a very minor seizure. The next fever was 8 weeks later, very mild and manageable with Motrin and no seizure. Then nothing for 3 months. Then a mild fever, but after a visit with the chiropractor, the fever ended after 30 hours. And now we have been fever and seizure free for 5 months! My son is himself again. He only goes to see the chiropractor once a month just to check up and they never adjust him unless there is a need. He loves to visit "Dr. Dave" and we have been so encouraged! If anyone has questions please let me know. I hope this brings encouragment!

[dani1456]

I am sorry to hear about your son. I am going through the same thing with my daughter. She fits the criteria for this PFAPA. The only things she does not have are the mouth sores and her fevers actually come more often.  These symptoms started when she was 11 months old. She would have high fevers about 102-105 (Lasted anywhere from 3-5 days), nausea, and severe headache. The doctors would always say, "It's just a virus, it will go away." It never did… they would always admit her and treat her with some strong IV antibiotic.  She went through extensive testing for everything. We have seen so many infectious disease doctors, and family practice doctors; all of them checked her for all different types of viruses, cancers, bacterial infections, and her immune system. They never found anything. All of her labs were the same she had a High White Blood Cell count it was always between 12 and 25, A High SED RATE that was usually around 33 and a High CRP that was between 1.1 and 5.9. She gets very constipated, whiney, and I think a very low-grade fever 2 days before the High fever attack. When she turned, two we noticed that her fevers were coming more often like every 14 days like clockwork. Sense her fevers started coming every 14 days the doctors referred us to Primary Children's Medical center. They repeated all the labs, plus some… They referred us to an infectious disease doctor now that her fevers are coming every 12 days like clockwork, and they still did not know what it could be. Again they said "It's just a virus, don’t worry." And they sent us on our way. We could not sit here and watch our little daughter suffer. Therefore, we did some home research and came across a website that had PFAPA information. After reading this, we printed it off and made an appointment the next day with her primary care doctor. We went over all the information with him and we decided to try the only option that we read to diagnose this PFAPA. We gave her a dose of prednisone (steroid) at the first sign of the fever. It did not completely take the fever away, so we gave her another dose the next day. In addition, the fevers diminished. We also gave her a ½ a tsp. of tegument four times a day. The last infectious disease doctor had us get her blood drawn once every week for 4 weeks. Each time we did it, the labs came back normal.  Now she is 28 months old and we found a rheumatoid immunologist doctor in Cleveland Ohio that specializes in PFAPA.  We called him and explained what has been going on with her and he referred us to one of his colleagues in Salt Lake City. We are currently waiting for the doctor to call us back with the appointment.  Does any of this information match your son? I would love to hear from other parents suffering from this PFAPA… Thanks for spending time to read this…  My email is ***@****com…  

[Dr. Kokil Mathur]

Hi
Thanks for writing to the forum!
I am sorry to hear about your son. I am providing you with a link that will help you: http://www.pfapa.net/
The site is still under construction but you can enroll your son with PFAPA registry and gather more information about studies and research in this field.
Hope this helps. Do let me know if there is any thing else and keep me posted. Take care!