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PFAPA:
The Reoccurring Fever
Who It Effects, How It Effects And How Is It Treated
Research by: Keshia Flinn
What Is PFAPA
Periodic fever Aphthous-stomatitis, Pharyngitis,
Adenitis Syndrome or PFAPA, is a relatively rare autoinflammatory disorder causing re-occurring fevers, with
no sign of an infection. These debilitating fevers
usually last 3-7 days.
The child grows normally, is very healthy and shows no
sign of sickness in-between episodes.
"Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome
(PFAPA) (Juvenile)." N.p., n.d. Web. 03 Dec. 2013.
What Is The Cause Of PFAPA
The answer to this question is not yet known. There is
no gene defect found in PFAPA, although sometimes
more than one family member has the disease. It is
clear that the inflammatory process is active during
episodes, but it is not clear why this happens.
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2013.
Who Is Affected By PFAPA
PFAPA can be diagnosed in young children as early as
age two and can continue until age eleven. This
syndrome is non-contagious, it only affects the child
with the syndrome.
Some children have been found to have rare cases
starting at an earlier age, but this can depend on ruling
all other illnesses out and the parents’, or caretakers’
knowledge of such an illness.
PFAPA is mostly male predominant.
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2013.
What Are Symptoms Of
PFAPA
The symptoms vary for every child, but there is one thing in
common: high, reoccurring fevers ranging from 102-104*F
Swollen lymph nodes
Fever chills
Aching or swelling of joints
Headaches
Mouth ulcers
Loss of appetite
Sore throat
Fatigue
*Note: These are just some of the more common symptoms for PFAPA, for every child
deals with different symptoms and side effects to the syndrome.
"Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)
(Juvenile)." N.p., n.d. Web. 03 Dec. 2013.
What Are Short Term Effects Of
PFAPA
PFAPA can make it very difficult to attend school regularly,
socialize properly, and live an active normal lifestyle
because of its re-occurring episodes. The fever episode can
range 3-7 days, one week out of the month, sometimes
more, with low grade fevers (100-101*F) in between
episodes. These fevers are very punctual in their
reoccurrence.
"Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)
(Juvenile)." N.p., n.d. Web. 03 Dec. 2013.
What Are The Long Term
Effects
Although continuing to sometimes age eleven or
fifteen, there are no long term health affects on the
child.
According to D., Theofanidis, MSc, PGDipEd, Clinical
Collaborator ATEI, the psychological effects coming
from childhood illnesses and syndromes can cause:
“Mental shock, stress, sentiments of anger, sorrow, and
increased intensity in their interpersonal
relationships.”
These psychological effects are based on a child that was previously healthy.
How Does PFAPA Effect the
Family
Although the child is mostly well, diagnosing PFAPA is
a very long process. This process creates a lot of missed
work or school days, stress on the parents or care taker,
frequent doctor and/or hospital visits, stress on the
other siblings and tension in the house hold.
Support Groups For Families
A live feed for parents’ to come together and receive information
and support from other parents’:
exchanges.webmd.com/pfapa
A live feed for parents’ to express concerns, gain insight and
recieve respectful advice from other parents’:
www.inspire.com
A Facebook page dedicated to the awareness of PFAPA from a
parent’s perspective:
www.facebook.com/pages/Pfapa-Awareness
How Is PFAPA Diagnosed
There are no laboratory tests specific for diagnosing
PFAPA. The disease is diagnosed based on symptoms
and physical examination. White blood cell counts,
sedimentation rate and the C-reactive protein, all of
which can be measured with a blood test, increased
during attacks.
It is important to exclude all other diseases that may be
present with similar symptoms (especially a
Streptococcus infection) before confirming the
diagnosis.
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2013.
Other Rare Auto
Inflammatory Diseases Like
PFAPA
There are many other periodic fever syndromes like
PFAPA, all are diagnosed differently and all have
different short and long term affects.
Familial Mediterranean Fever
Tumor Necrosis Factor Receptor Associated Periodic
Syndrome (TRAPS)
Hyperimmunoglobulin D syndrome (HIDS)
*Note: These are the most common and closely related syndromes to PFAPA.
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Is There A Cure For PFAPA
PFAPA is not curable, but it is manageable. There are a few
ways of managing the symptoms and re-occurring fevers
once diagnosed:
Tonsillectomy: removing the tonsils’
Prednisone: most common treatment, is a type of steroid
Cimetidine : a medicine that is used to treat stomach ulcers
"Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)
(Juvenile)." N.p., n.d. Web. 03 Dec. 2013.
Cases Of PFAPA In Children
Ekaih Flinn: Age five, diagnosed with PFAPA at age three,
with misdiagnosis for one and half years. Ekaih had
reoccurring fevers at the beginning of every month, with a
temperature of 102-105*F. Ekaih’s fevers lasted 5-8 days,
with minimal breaks in between.
Treatment: Prednisone
Outcome: Prednisone becomes very effective, with less
frequent fevers and no symptoms.
*Note: Ekaih Flinn is my daughter, all information is based on my experience.
Case Number Two
Zachary Cutler: Age seven, diagnosed with PFAPA at age
five. Major symptoms include: 103* F fevers, mouth sores,
headaches and sore throat.
Treatment: Began with Prednisone, but due to the
frequency of the fevers, prednisone is no longer
recommended and Zachary’s mother and pediatrician
decide to do a tonsillectomy surgery.
Outcome: The tonsillectomy is effective and Zachary no
longer lives with PFAPA.
*Note: These results were given to me in a direct interview with Zachary’s mother, Theresa Cutler.
October 15, 2013.
Is PFAPA Deadly
PFAPA is not a terminal illness and usually subsides by
age 11, up to age 15. After being relieved of the
syndrome, children usually live to lead healthy, normal
lifestyles.
"Sign up and Be Well." Cleveland Clinic. Cleveland Clinic, 2013. Web. 02 Dec.
2013.
Review
There is no cure for PFAPA
There is no known cause for the syndrome
PFAPA is not contagious
PFAPA occurs only in children, usually ages 2-11 years
Fevers normally last 3-7 days and are punctual and regular
The child is completely healthy between episodes
Fevers are very high ranging from: 102*F- 105* F or higher
There are only three methods to manage PFAPA:
Prednisone, Tonsillectomy and Cimetidine
Video Describing PFAPA
http://www.inspire.com/groups/the-childrensinn/topics/pfapa/
Dr. Lachmann, medical expert in rare auto
inflammatory diseases, discusses the symptoms of PFSs
and their impact on a child's quality of life as well as on
the child's caregiver.
*Note:This video is done by Novartis
(http://www.novartis.com/corporate-responsibility/access-to-healthcare/r-d.shtml)
Citation
Hashkes, Philip, MD. "Periodic Fever, Aphthous
Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)
(Juvenile)." American College of Rheumatology. American
College of Rheumatology, Mar. 2013. Web. 16 Oct.
2013.
Martinon F, Burns K, Tschopp J. The inflammasome:
a molecular platform triggering activation of
inflammatory capiases and processing of proIL-beta.
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mutations in a new member of the RoRet gene family are
likely to cause familial Mediterranean fever. Cell 1997;
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Piaget, Jean, and Bärbel Inhelder. The Psychology of the Child.
New York: Basic, 1969. Print.
Montessori, Maria. The Absorbant Mind. New York: Dell
Pub., 1967. Print
"Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis
Syndrome (PFAPA) (Juvenile)." N.p., n.d. Web. 03 Dec.
2013.
"Sign up and Be Well." Cleveland Clinic. Cleveland Clinic,
2013. Web. 02 Dec. 2013.