The Kawasaki Disease Forum

I have noticed simularities between some children currently experiencing unusual post KD symptoms and PFAPA.

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

What is PFAPA? Recurrent episodes of fever that affects children in early childhood. Episodes decrease in frequency and resolve after the age of 10.

What causes PFAPA? It is clear that the inflammatory process is activated during episodes but it is not clear why it is triggered. It is unknown whether the cause is genetic or due to an initial infection. (It is important to note that infection, trauma, stress, and vaccination are all suspects in triggering PFAPA).

What are the main symptoms? High episodic fevers (104-105F), sore throat, mouth ulcers (mostly occuring on lateral side of tongue), enlarged cervical lymph nodes (glands in the neck), fatigue, chills, abdominal pain, headache, joint pain, rash. The episodes last for three to seven days and recur every few weeks. The child looks very ill but, not all children have all symptoms (especially mouth sores).

How is PFAPA Diagnosed? Inflammatory blood tests like white blood cell count, ESR, and C-reactive protien are increased during attacks. There are no labatory tests specific for diagnosing PFAPA.

How is PFAPA treated? There is no cure. The aim is to control symptoms. The fever does not respond well to Tylenol or Advil. Steroids (usually Prednisone) have been shown to shorten episodes.


PFAPA is frequently discussed with other Periodic Fever syndromes. Although rare they are worth a mention.

Familial Mediterranean Fever (FMF)
Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS)
Hyperimmunoglobulin D Syndrome (HIDS)

I hope this information will be of help to the children struggling with health issues after KD.

KCLP
I appreciate the information. Very interesting and a lot of big names! I looked it up. Lots to read. Tonsils were not a problem for my family but sinuses were and still are???
If you get a minute maybe you could give more information and details how this was finally dignosed.
Marshall's disease, am I right? Discovered as far back as 1948. The ulcers of the mouth though - my husband has them every now and again and the same for his father, and my two kids. They are older, not children. Their ulcers are concave, side of tongue, inside of cheeks, and lips, but not roof of mouth.

liquidambar,

PFAPA (Periodic Fever Syndrome) was first recognized in 1987. The Syndrome was initially called Marshall's.

The Dx of PFAPA is becomming more common.

KCLP-
Thank you so much for this information. That is really interesting, especially that the fever doesn't respond to Tylenol or Advil. In all the years we've dealt with Paige and the long term fevers she had, I never heard anyone mention this.....and they were struggling with her case. When I get some time I am going to read up on this.
It surely sounds similar to KD. I think some of these vasculitides have to be related. It also seems like a lot of these kids have asthma---
Thanks again,
Sandy

Most of those vasculities diseases, my family comes so close to fitting them and yet doesn't quite fit in any of them either - except for my daughter's Kawasaki's, and I have no doubt my son had an atypical form of it.
One of those symptoms would be Recurrent fevers for sure though, and tylenol being a useless, cabinet space wasting drug would be another!
I read they discovered it 48 but 87 they upgraded or improved their knowledge, or maybe it said- had more kids than every before, so they could study it more - What ever it is - sounds like it is new, unheard of just like Kawasaki's.

Tylenol works great with KD and brings the fever down for about 4 hours and then it will spike back up---it is definitely not a waste and that is the part I find interesting about PFAPA--that it DOESN"T respond to it. Tylenol tends to mask the fever of KD, our daughter took a lot of it and it really helped with the gallbladder pain during the KD. It worked almost immediately to keep her from screaming and it lasted exactly as long as it said it would--she was on the max dose for her age and it worked miracles for the pain.

hmmm!
When my daugther had Kawasaki's Tylenol did nothing as far as I could tell. Her fever seemed on some natural cycle. It went down some in the early afternoon, only to begin to rise again in the evening. It was not her gall bladder, but her spleen that swelled up her upper belly . Not till seven days in the hospital did they finally start the asprin. I don't know maybe she was getting over the worse of it on her own, but the mega doses of aprin seemed to really make the difference.

I do know that asprin was what I learn to reach for inspite of Reyes fear when either of my kids were sick with the fever.

I've never heard of a spleen problem during KD-although I think it was reported in adult KD once, nor have I heard of swollen stomach either. The mega doses of aspirin during acute KD are for the high platelets and not for the fever.....however I am sure it brings the temps down also. I have also never heard of KD having initial fevers that start low in the day and then increase later in the day unless from drugs suppressing the fever. I suppose it could happen that way though. Prolonged fever of 4 to 5 days in the 101 and above range should start doctors thinking about KD. I have heard of one case of KD without fever, but that is exceptional.

The information provided here with PFAPA is interesting and basically I was looking at what documented evidence separates it from KD. Pain killers such as Tylenol are not normally given other than for gallbladder pain during KD as far as I know.

My daughter's temperature ranged from 104 to 105 and when I said it went down in the early afternoon I meant it went down to 102-103.
When you say the tylenol took down the temperature do you mean it took it down to normal or do you mean that it took it down a point or two?

I suppose tylenol took down my daughter's fever a point or so, but at that temperatuer why would it matter?
By the time they gave her asprin she was already showing some improvement on her own. I mean she stopped sleeping (coma like) and got up and emptied that swollen belly into the potty????
Asprin did not take the temperature down to normal either by the way - but lower than the tylenol did.

It was Kawasaki's that my daughter had: red rash espcially were the skin had been exposed to the sun; lips and tongue swollen and red (not off red but red!), fingers swollen and peeling, eyes very pink, and yes swollen belly. When she started to get better she still had a molten, spiderweb look about her skin, like were all her blood vessels had been inflamed and left a bruise. Oh, yes the SED rates were high.

She continued to have a temperature of 100-102 for almost four months, even with asprin. She had a temperature of 99 - 101 for close to nine months, asprin would take it down, but again not to normal.

You might be splitting hairs here with Marshalls. I admit I have not read extensively much more about it, but I will. What little I have read there is once again like all the rest of the vasculaities diseases (except Kawasaki's) almost fits, but not really.

Children who have PFAPA can have T's as high as 106F sometimes, resulting in fever induced seizures. If alternating Tylenol and Advil helps reduce the T by one or two Degrees, it is helpful.

I found that when researching current information on PFAPA much was "left out" about the realities of living with this syndrome. Some of us have found the same with KD. Part of the reason for this is due to the newness of these syndromes.

There are many simularities between KD and PFAPA. I am starting to find information recognizing confusion between diagnosing incomplete KD or PFAPA. This is of concern to me because PFAPA can be life altering but KD can be life threatening (as well as life altering). The correct diagnosis is very important.

Liquidambar,
PFAPA (Periodic Fever Syndrome) or Marshall's was recognized for the first time in 1987.
Marshall-Smith Syndrome was recognized in the 1940's and discovered as genetic in 1971. The symptoms include unusual skeletal mutation, including respiratory difficulties and MR.
These are not the same Syndromes. If you are looking up only "Marshall's" you could confuse information.
I appreciate your interest.

Momcat,
If you do want to rule out Paige having a form of Periodic Fever Syndrome, I would suggest keeping a journal of the frequency of her fevers. Juvenile Infectious Disease Specialists or Juvenile Rheumatoid Specialists seem to have the most knowledge of PFAPA.
Thanks for taking an interest in the simularities between KD and PFAPA. I also, find it interesting. If you do have time to research the two and find anything new, I would appreciate it if you would share.

KCLP

Here is a link. Is there any thing new it this???

I don't know, it is like they come so close and yet can't connect the dots.

But I have only thought on this a little and it does need more study. http://padeh.net/new_files/PFAPA.pdf

Jason,

Headache can be an indicator of Periodic Fever Syndrome. PFAPA resolves itself in early adolescence, yet there are other types of Periodic Fever Syndrome that are genetic and continue into adulthood. Think about your family history. Did a relative suffer from the same or similar health issues you do?

It is important to remember that Periodic Fever Syndromes are not associated with KD.

I hope you find the answers you are looking for. Your sharing of your situation and ideas is a great contribution.

liquidambar,

I do not think KD and PFAPA are the same syndrome. Classic KD and PFAPA are distinguishable.

Incomplete KD and PFAPA could be more difficult to diagnose. That is the reason education and awareness of the two is so important.

Do I suspect that KD can initiate PFAPA? Honestly, yes.

But, I make it clear that this is a theory from a mother searching for answers. There is nothing suggesting an association between the two and no trials have ever been conducted that I am aware of.

You believe your children developed PFAPA as a consequence of KD? Did they eventually "outgrow" it?

I understand your reasoning.

Thanks for your response and question.

The fevers for my daughter ended about her second year in college, that is a long time. The fevers for my son stopped about 17 years old. These fevers for my children did seem to decrease in severity about 9 or 10 years old.

I think it is just common sense to think that PFADA and Kawasaki's is probably the same thing or related, I am like you on that.

Just my 2 cents...Bobby's spleen and liver were significantly enlarged during the acute phase of KD. It got so bad that his "inney" belly button popped to an "outey"...that's when I demanded that his illness (which had been poo pooed as a virus, by his Doctors) be taken seriously and an appointment scheduled with the lead physician in the family practice. Bobby was hospitalized within the hour of being seen!