Understanding PFAPA: The Basics
PFAPA, or Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis, is the most common cause of non-infectious periodic fever in children. It is an autoinflammatory disorder, meaning it's caused by an abnormal immune system response, not a virus or bacteria. The episodes are characterized by a sudden onset of high fever, typically lasting three to seven days, alongside other key symptoms.
Symptoms often include:
- High fever, often over 102°F (38.9°C)
- Aphthous stomatitis (mouth sores)
- Pharyngitis (sore throat), sometimes with white patches on the tonsils
- Cervical adenitis (swollen lymph nodes in the neck)
Between episodes, children are typically completely healthy and show normal growth and development. The exact cause of PFAPA is unknown, and the condition is not contagious.
Medical Management of PFAPA Episodes
Aborting a PFAPA Flare with Steroids
For many patients, oral corticosteroids, such as prednisone or prednisolone, given at the onset of symptoms, can dramatically shorten or end the fever episode within hours. However, a key consideration with this approach is that while it provides quick relief, it may also shorten the time between episodes for some individuals. Repeated use can also carry side effects, so this is a treatment option to be carefully discussed with a healthcare provider. Standard fever reducers like acetaminophen or ibuprofen often have limited effectiveness against PFAPA fevers.
Prophylactic Treatment to Prevent Flares
For individuals with very frequent episodes, some doctors may recommend daily medication to help reduce the number of flares. Two medications are sometimes used for prophylaxis:
- Colchicine: Originally used for Familial Mediterranean Fever (FMF), colchicine is an anti-inflammatory medication that can reduce the frequency of PFAPA episodes in some children. It is a long-term treatment option, but its effectiveness varies among patients.
- Cimetidine: This is an older medication with immune-modulating properties. It has been used with some success in the past, but studies on its effectiveness are limited, and it is less commonly prescribed for PFAPA today.
Surgical Intervention: Tonsillectomy
For patients with severe or frequent episodes that don't respond well to medication, tonsillectomy (surgical removal of the tonsils) can be a highly effective treatment option. Studies have shown that tonsillectomy can cure PFAPA in a high percentage of children. While surgery carries its own set of risks, it is a definitive option for many and should be considered after consultation with a specialist, such as a pediatric rheumatologist or an ENT doctor. It is important to note that PFAPA can occasionally persist into adulthood, sometimes requiring more complex surgical approaches like lingual tonsillectomy, particularly if the initial tonsillectomy didn't fully resolve symptoms. For more information on tonsillectomy, consult resources like the American College of Rheumatology.
Managing Triggers and Lifestyle Factors
While PFAPA is not caused by infections, certain factors can sometimes precede or trigger flares. Stress, in particular, has been identified as a potential trigger in some individuals. Focusing on overall wellness can help manage the condition:
- Stress Management: Identify and minimize emotional or physiological stress where possible. Encourage a balanced lifestyle with plenty of rest.
- Maintain a Healthy Diet: While no specific diet cures PFAPA, ensuring a well-balanced diet rich in vitamins and nutrients supports overall immune health. Some research has explored the role of Vitamin D supplementation, though further studies are needed.
- Adequate Rest: Ensuring the patient gets enough sleep can help manage the immune system and general well-being.
PFAPA Management Comparison
Treatment Approach | Primary Goal | Effectiveness | Potential Drawbacks | Application |
---|---|---|---|---|
Corticosteroids | Abort episodes quickly | High, for ending an acute flare | May shorten the interval between flares; side effects with repeated use | Short-term, acute flare management |
Colchicine | Prevent future episodes | Moderate, works for some individuals | Gastrointestinal side effects possible | Long-term prophylactic treatment |
Tonsillectomy | Provide a definitive cure | High success rate, especially in children | Surgical risks; not effective for everyone | Option for frequent, severe cases refractory to medication |
Conclusion
For those asking how to get rid of PFAPA, it is crucial to understand that while there is no universal cure, effective management strategies exist. For most, the condition resolves spontaneously by late childhood or adolescence, and in the meantime, treatment options can significantly improve quality of life. Whether through abortive steroid treatment for flares, daily prophylactic medication like colchicine, or surgical tonsillectomy, a personalized treatment plan can be developed with a healthcare professional. Consulting with a pediatric rheumatologist is the best step for diagnosis and tailoring a management plan that works for you or your child.