The following article is copied from the Cleveland Clinic website. The doctor today said that there is nothing major that's wrong with Ethan and that it was most likely either viral infections that he's getting or PFAPA. He also said that the pediatrician did thorough enough testing and he didn't feel that we have to do more at this point. (YAY!)
So - it's pretty much what I thought and from here I'm going to see how he does the next few months and if he continues to have these fevers monthly the infectious disease doc we saw today can call out a steroid to try that can help with PFAPA.
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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.
