pfapa: the reoccurring fever who it effects, how it effects and how is it treated research by:...

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 auto-inflammatory 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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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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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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Other Rare Auto Inflammatory Diseases

Like PFAPAThere 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.

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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-childrens-inn/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. Mol Cell 2002; 10:417–426. CrossRefMedlineWeb of Science

Citation

International FMF Consortium. Ancient missense mutations in a new member of the RoRet gene family are likely to cause familial Mediterranean fever. Cell 1997; 90:797–807

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.