Friday, November 13, 2009

PFAPA


I took Zachary to the Doctor today. He goes to Valley Children's Hospital (Children's Hospital Central California) every 6 months for a check up because 2 years ago he was diagnosed with PFAPA Syndrome. I started noticing right after his 3rd birthday that he was getting a fever every month with no other symptoms (that I could tell) so I started keeping track of them and after a year and a half, he was officially diagnosed by his pediatrician. His pediatrician had only recently heard of this syndrome and referred us to a doctor who specializes in Immunology and Rheumatology.
Dr. Wright (http://www.childrenscentralcal.org/OurDoctors/Pages/dwright.aspx) is quite familiar with PFAPA and has five other patients with this syndrome.

Now that Zachary is in school and sports, I have had to try to explain this 'illness' more and more. I usually tell his teachers/coaches/school nurse that Zachary will seem very tired and complain of being cold. He will either have a fever or one will be coming on when these symptoms occur. His fevers occur about every 8-12 weeks and he is NOT contagious.

Before we found something to relieve his fever and the symptoms that accompany it, the fevers would last 3-5 days. Now that we give him a single dose of Prednisolone, the fever is gone within 2 hours.

Today I spent some time searching the Internet for a good description of PFAPA and the following is what I found:

Periodic fever, Aphthous-stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome

This syndrome includes recurrent episodes of fever with aphthous-stomatitis (mouth sores), pharyngitis (sore throat with redness and sometimes a throat that has a white covering – exudate - like that seen in a throat with streptococcal infection.

PFAPA affects children in early childhood, usually starting at age two to four years. Episodes usually decrease in frequency and resolve after the age of 10 years. This disease was recognized for the first time in 1987 and was called Marshall’s.

How common is it?
The frequency of PFAPA is not known, but the disease appears to be more common than generally appreciated.

What causes PFAPA?
The answer to this question is not yet known. No gene defect has yet to be found in PFAPA, although in some cases more than one family member has the disease. No infectious cause has been found in PFAPA, thus it is not a contagious disease. It is clear that the inflammatory process is activated during episodes but it is not clear why it is triggered.

What are the main symptoms?
The main symptoms are episodic fevers, accompanied by a sore throat, mouth ulcers, or enlarged cervical lymph nodes (glands in the neck, an important part of the immune system).

The episodes of fever start abruptly and last for three to seven days.

During episodes, the child looks very ill and complains about at least one of the three symptoms mentioned above.

The episodes of fever recur every few weeks and often families know the exact day when an attack will start. On the day the fever starts the child will feel a little ill before the attack and the family knows an attack is about to start.

Not all children have all symptoms, especially mouth sores. Some children have other symptoms like joint pain, abdominal pain, headache, vomiting or diarrhea.

How is PFAPA diagnosed?
There are no laboratory tests, or imaging procedures, specific for diagnosing PFAPA. The disease will be diagnosed based on the results of a physical examination and other symptoms. Inflammatory blood tests like the white blood cell count, erythrocyte sedimentation rate and the C-reactive protein are increased during attacks. Before the diagnosis is confirmed, it is important to exclude all other diseases that may present with similar symptoms (especially a streptococcal throat). The dramatic response to treatment (see below) also helps diagnose PFAPA.

How is PFAPA treated?
There is no specific treatment to cure PFAPA. The aim of treatment is to control symptoms during the episodes of fever, to shorten the duration of episodes, and in some children to prevent attacks from occurring.

In most children, the disease will resolve by itself without treatment, usually after the age of 10 years.

The fever does not usually respond well to Tylenol or nonsteroidal anti-inflammatory drugs.

A single dose of steroids (usually prednisone), given when symptoms first appear, has been shown to shorten an episode and sometimes even end the episode. However, the interval between episodes may also be shortened with this treatment, and the next episode may occur earlier than expected.

In some patients using cimetidine (a medicine that is used to treat stomach ulcers) may prevents attacks from occurring. In patients with very frequent attacks, a tonsillectomy (removing the tonsils by surgery) may be considered.

What is the outcome and course of PFAPA?
The disease may last for several years. Over time, the intervals between the episodes will increase and usually after the age of 10 years resolve by itself. Children with PFAPA continue to grow and develop normally.

http://my.clevelandclinic.org/disorders/periodic_fever_syndrome/rheumatology_overview.aspx The Cleveland Clinic © 1995-2009. All Rights Reserved. 9500 Euclid Avenue, Cleveland, Ohio 44195 800.223.2273 TTY 216.444.0261

11 comments:

Allie said...

Very informative! I think that you need to print this out and give it to all Zachary's teachers and coaches to help them understand it better. Along with the prednisone, I recommend lots of kisses and cuddles ;)

Cindy M said...

Wow, your research is very impressive. And, by the way, has anyone ever told you what an absolutely, adorable, handsome lovebug you have.

Lisa Johnson said...

I am so glad to find information like this and blogs like yours. I have been searching for months for what is wrong with my son. He also has monthly fevers and after discovering PFAPA last night on the web, I am convinced this is what he has!! Have you considered taking his tonsils out? That was something that I notices was recently discovered to show practically cures it.

Carli Westcott said...

Thank you for sharing your story, I have been researching PFAPA for over a year now and it took that long to convince my pediatrician that my 5 year old son had it. We were eventually sent to Childrens Hosp in Boston, where it was confirmed. Anyone that suspects their child may have PFAPA the best thing is to keep detailed records!!!

Natalie said...

Hello from Atlanta Georgia. My story is a little different than the average. I have two children with PFAPA. Not only is that odd but they are ages 7 and 15. The seven year old was diagnosed at Scottish Rite Chilrens Hopspital over a year ago. The fifteen year old was just diagnosed this year. Normally this disease is prodominatly seen in young children. They both have the classic symptoms that reoccur about every six to eight weeks. Prelone is their saving grace. Tried Tagamet with no success. So we have our doctors pondering the possibilities of it being a hereditary disease.

marcie said...

My daughter is now 16 and she has PFAPA. It all started when she was 18 months old. She gets the mouth ulcers, and high fevers. She also suffers from joint pain and pain in her back and legs ankles and feet. I don't know if the pain is because of the fevers, but would like to know if any other parents are going through this with their children. One good thing is that Erin did have a wonderful 4 years of no fevers.. 8-12...yeah..

Anonymous said...

my daughter has had PFAPA for 2 years (she is 2 and 4 months)...that is a fever every 16 days, fever for 5 days and then 11 good days, like clock work ranging from 105-107 degrees. we planned our lives around it, i think they thought we were crazy at first, but eventually we got a diagnosis. she had tonsils and addenoids out in Dec 2010, not one fever since.....

Jaime said...

My 4 year old was diagnosed with PFAPA in January and we went to see Dr. Licamelli in Children's Hospital in Boston, who has been doing research on this syndrome. My daughter had her tonsils and adenoids removed last month and our fingers are crossed, no fevers thus far!

Anonymous said...

Hi everyone, my id twin boys keep getting reccurent fevers since aug 2011..about once a month. they were in preschool at the time so doctors kept telling me that it's just a virus/flu. They have been both out of preschool since Dec 2011 and the reccurent fevers have continued, about every 3-5weeks..sometime every 2weeks they get a fever, sore throat, very tiny sores in their mouth (sometimes) chills, and sometime foot pain. What I don't understand is that they also get red flat spots on their palms of hands and soles of feet..which fade a bit but don't completely go away until the fever is gone aobut 3days..5 different doctors have to me it's a strain of hand & foot and mouth desease...grrr..very frusturating. Three weeks ago I did my own research and read about PFAPA and it fits my boys to the T..beside the rash. Has anyone's children gotten the red flat spots on the palms and sole's of feet? I've also requested a complete blood count in which they found that my boys are a little anemic..but are going to be getting more blood work done the near future to rule out other genetic disorders.Sorry so long...reading about other families that are or have gone through this helps alot. oh yes my boys are almost 4. Thank U!

cori Phillips said...

I have a 15 year old son that was diagnosed with PFAPA syndrome when he was 2 1/2. He started with symptoms at 4 months old and we were so happy to finally get answers when we did. I must say it was a long haul!!! We treated him with prednisone every episode (monthly and sometimes bimonthly) for about 6 years when he finally out grew it at age 8. We haven't seen anything since, until now. He has been a very healthy athletic boy. But... he just had his wisdom teeth removed and some other oral surgery done and went under anesthesia for the first time and he is running a fever of 104 and the doctor says it is not related to the surgery. I'm wondering if the trauma of oral surgery has brought the PFAPA back on. I sure hope not. Wondering if anyone else out there has older children that were diagnosed with PFAPA when they were younger and outgrew it only to have it return again when they were older. Would love any info. Thanks.

Anonymous said...

Hi Cori Phillips, I noticed your post. I have a 3 year old with PFAPA and was curious to know what if any side effects your son experienced from taking the prednisone for such a long period? This is one question I can't seem to get answered. And can't find many people with children at older ages. Thanks.