As someone who has blogged regularly for over four years (my other blog is writing to survive), I am not used to both leaving a blog hanging without posting as well as not answering comments or following up. Since my last post, I've taken two short and very intense psychology classes (both requirements for the counseling master's degree program I am entering in the fall) and ran out of time and concentration to do much else but study and do (most of) the other things life requires. So I apologize if you've been looking for information or if I've left you hanging. I'm thinking of you in particular, Laura. But I am back! And I promise to update this more frequently, especially when the boy develops fevers.
Because the fevers have not gone away. We were heartened when he went for an unprecedented 36 days without a fever, and also when that fever was mild and lasted only three days. Was this the slow work of the cimetidine? Was this the beginning of a trend? We still don't know, because a week ago -- 28 days after his last bout -- he became sick again, and the fever lasted for four long days.
His symptoms are definitely milder. Generally, his fever stays in the 101 - 102 degrees Fahrenheit range. His head doesn't ache as much and his throat is dry, but he does not complain of it hurting. He still vomits at least two or three times during the bout. We saw a new rheumatologist recently who doesn't believe this is PFAPA, but given she was wrong about the expected effects of cimetidine (she confused it with prednisone, at least that's how it appeared), I am not putting too much stock into her opinion. She was pushing for familial Mediterranean fever. Doesn't seem like it to me. Still, I can't be positive that his periodic fevers can be called PFAPA, which makes me think we should try the prednisone once to see if it has an effect. I don't want to treat the fevers on a long-term basis with steroids, but I would like to see if the fever immediately goes away with one treatment, which would identify it more firmly as PFAPA.
As far as the cimetidine goes, he's been taking it since March and the fevers are still with us. I'm beginning to think his fevers are part of a chronic, perhaps not curable illness, one that is hopefully temporary.
Hi Jennifer,
ReplyDeleteMy name is Emily and I am a mother to 3 1/2 year old Ella who has PFAPA. I came across your blog today and feel I can completely relate. Ella started developing fevers in January and had about six fever episodes before she was diagnosed. We see a pediatric rheumatologist here in Salt Lake City who was trained at UCSF (near you, right?) and we think she is amazing. I'm sure she could recommend someone in the bay area if you are interested. At our first visit with her she immediately recommended we try prednisone to clinch the diagnosis of PFAPA. When Ella developed her next fever we gave her a single dose and within a few hours she was absolutely fever free, eating, playing, etc. It was pretty miraculous. We even went to the park that day. I'm sure you can appreciate how amazing that is. Her next episode seemed to come a little sooner (3 weeks) and the prednisone was a little less effective. We gave her two doses (24 hrs apart) and the 2nd dose completely stopped the fever. We are on day 26 now, and thankful for every healthy day we have.
I am actually a physician (radiologist) and have looked in to prednisone side effects. The concern with prednisone use really only pertains to daily chronic use. I am completely comfortable giving Ella prednisone one or even two times a month since I feel the benefit far outweighs any negligible risk. Ella's doctor also feels this way. Also, the prednisone dose is a low dose.
We started Ella on cimetidine about two weeks ago so we will see how that goes.
I hope we can be a source of support for one another. I am happy to help in any way that I can and would love to hear more from you.
Hi my duaghter is also going through this pfapa. We are giving her cimetidine, since 2 month now , but episodes of fever are getting closer now . Rather than every 21 days, it is now every 15 days and then after prednisone, it comes back after 5 days. Is cimetidine working for your kid. My daughter is just 2 and half.
DeleteMona
Emily, I know it's been a while, but I want to ask you if you are stills giving your daughter Cimetidine??? How did it go? My 5 year old started 6 weeks ago and she's having an episode now. I don't know if we need to keep waiting for the medicine results. Thank you!
DeleteHello,
ReplyDeleteThanks so much for your comment. UCSF is near us and we have been seeing rheumatologists there, though I get the sense that they aren't that concerned about our son, in part because he doesn't have a long history of periodic fevers and he doesn't fit the total profile for PFAPA (since he started at six, which is on the old side for PFAPA). At the moment, it's been about five weeks since his last bout, though he had something going on a few weeks ago -- I don't think it was PFAPA, at any rate.
At this point, I think maybe the cimetidine *is* working. Or maybe the fact that the long term strep infection on his skin -- not his throat -- finally got antibiotic treatment is having an effect on his overall immune system. It's one of those things that is all-encompassing when it happens every three weeks and a little more iffy when it becomes unpredictable and not as intense.
I hope the cimetidine works well for your daughter and I would love to share information and support. no matter what.
Thanks again!
Thanks for your reply. I hope I didn't sound like a drug rep for prednisone! I was just a little surprised your son's doctors haven't tried it yet. I have my fingers crossed that the cimetidine continues to make his episodes farther apart and less severe.
ReplyDeleteThis morning, 27 days since the last episode, Ella woke up with a fever. It's not as intense, though, so maybe the cimetidine is working for her too. Normally she isn't able to eat or get off the sofa and today she has had small snacks and is currently sitting up watching Sesame Street. So I'm hopeful.
First, I am sorry to hear that your daughter is having an episode. I know that feeling of hopefulness and then disappointment when another bout hits. I hope this one continues to be milder and also shorter.
DeleteAnd you didn't sound like a drug rep for prednisone. :-) I know a lot of people are nervous about steroids, for valid reasons, but the dosages are so small and (hopefully) infrequent in these cases that I don't think it would do any harm. It does seem like there are a variety of approaches with PFAPA and that doctors aren't on the same page.
So far, so good with our son. I hope it stays that way.
Ella was only sick for about 24 hours thank goodness. It seems like the cimetidine is helping because this episode was less severe. The fever didn't seem as high and she complained less of stomach pain than usual. We also gave her the prednisone which helped again.
ReplyDeleteI'm curious how you get your 6 year old to take cimetidine? Pills or liquid? Any tips? I'm having a hard time getting my daughter to take it since the liquid is horrific tasting and ground up pills aren't much better. I've tried stuffing pills in chocolates, grinding them up and adding to smoothies, etc. She can taste the pill no matter what we try and is starting to resist.
I am glad that the cimetidine appears to be working! Our son has stomach pain, too, or at least has what he calls a "throw-up" feeling that can last for hours before he actually throws up. That has happened every episode, even after months of cimetidine.
DeleteThe liquid really is foul. We started with that and switched to pills after less than a month. He's had very few problems. At this point, the twice daily pill is quite routine. I think it's worth trying, even though she is younger than he is (and you remind me that I have to change my info on him -- he turned 7 this summer).
hi - our daughter had PFAPA and outgrew it. (took some repeated pushing thru Inf Diseas consult etc but eventually we got to Stanford Lucile Packard Children's hopspital with the rheuma specialists there. they did the genetic testing to rule out the other periodic fever courses. the steroids provoked a more frequent response. cimetidine worked for us but our daughter seemed a little sluggish while on it. after a year and a half on ...we tapered her off and now she has been symptom free for several years seems ok. i found it helped to bring her in *Unmedicated* to document the fevers. which at 105-106 was frightening. we also saw subtle signs of joint inflammation during episodes. agreed re cimetidine being foul liquid - but she began to self administer like a shot (w/ supervision) and got dessert right after - and somehow being in control of taking the meds - seemed to help.
ReplyDeleteThank you for sharing your story (I've been replying to lots of old comments -- my apologies for the extremely long delay). I've been wondering if/when we should taper the boy off the cimetidine. I admit, I am pretty scared of what might happen. He had a brief vomiting episode in late fall that was probably just a tummy bug, but which also brought back the feverish times. No one wants those to return.
DeleteHi I read this after trying to find more information on pfapa. Of course, anything I could find was about young children. I am 15 years old and my episodes started when I was 3 months old. I took a test, to see if it was fmf or a different venison of pfapa since my episodes were quite different from pfapa but still didn't fit fmf. My fevers are extremely high, and I get every symptom of pfapa every 28 days, on the dot. During my visits to rheumetology, I've had as much as 5 doctors in the room investigating my case.
ReplyDeleteThey decided to put me on cimetinde, which I have been taking twice a day. According to this, it should make the fevers less intense, but for me, they dissapear. Only the fevers though. I get every other symptom there is. Every 28 days, the exact cycle as pervious. So for the person who said their child grew out of it, they might not. I still have it. And any ideas as to why cimetidine didn't fully work? My doctors don't even know what to call what I have now. Thanks
Hello,
DeleteI am sorry for letting your comments languish for months (am finally catching up). I am also so sorry to hear about the difficulties you've been going through. From the parent's perspective, the fevers are draining and it is also so hard to see one's child feeling terrible. You've got the deeper perspective as someone who is going through it. I hope since you've written this that the cimetidine is helping or that the fevers have subsided.
I have just bumped onto this blog. We have a 5 1/2 year old son who was diagnosed with PFAPA 4 years ago by a infectious desease doctor. His fevers have been text book and we treat with piggybacking tylenol and motrin (when on the shelves) or generics which I swear don't work as well. About a month ago we came across some research regarding Tagament...called our IF dr and she said go for it. We were driving to Florida for Spring Break when our son woke up with his fever, started the tagament, 20mgs am and pm. His fever was the easiest yet! Only lasted 3 days and only hit 103!!! That was March 22 2013. Today is April 25 and no fever, we started giving tagament on the 20th because he had the "glossy eyed" look that he tends to get a few days before his fevers. We are crossing our fingers, hoping!!! My one question that I can't seem to find an answer for is, Are people treating with Tagament every day, during episodes, or trying to prevent? Would love to hear a reponse.
ReplyDeleteCrossing my fingers!!
and for the one who asked how to admnister... we split 200mg pills down 8 small pills. put one pill into a small spoon of applesauce! works great!
Hi,
DeleteMy daughter has papa. How is your son doing? This is the first I have come across the Tagamet treatment. Do you still do it? So at 5 1/2 you gave him 200mg daily? Thanks! Mary
We use tagamet daily to decrease the frequency and severity of our daughter's episodes. When this all began a few years ago we had our daughter see a pediatric rheumatologist who diagnosed her with PFAPA essentially based on the history we gave her, the office exam (normal) and a blood panel to screen for underlying rheumatologic disease (also normal). After the office visit she had us administer a dose of prednisone during our daughter's next fever to "clinch" the diagnosis. The prednisone completely aborted the fever and she was normal within hours. Then she started our daughter on a daily dose of Tagamet to help prevent the fevers. It definitely works, prolonging periods between episodes as long as 3 to 4 months. We had to adjust the dose a little after about 7 months because she suddenly had two fevers only two weeks apart. The doctor increased the dose a little and split it to twice a day. That has seemed to work great as she has only had one mild episode since December.
ReplyDeleteIn the beginning our daughter's fevers lasted 3-4 days and reached 104-105. Now with daily tagamet they last 1-2 days and rarely go above 100. Before she couldn't get out of bed and would often throw up during episodes but now she still eats and plays a little. She just rests more and watches TV when she's sick. It's made a huge difference!
Hi,
DeleteMy daughter was diagnosed with papa a few weeks ago. We are thinking about the tonsillectomy. We gave her the first round of steroids last week and she was fine in an hour. How much Tagamet did you give your daughter? And how is she doing now? I would love an update. Thanks! Mary
Hi Emily, may I know the dosage of prednisone that you gave to your daughter when she had high fever?
DeleteHi, I had to go dig up the old prescription (it was 2012!). It says this: prednisolone 15mg/5mL solution. I gave Ella 6 mL which is equivalent to 18 mg of prednisone. Good luck to you!
DeleteThe literature seems to say having tonsils taken out cures PFAPA. Just wondering why no one trying tonsillectomy ? Why wouldn't docs recommend it ? It is surgery, but pretty mild surgery...
ReplyDeleteMy daughter is being treated at Vanderbilt Univeristy Hospital in the Infectious Disease clinic for PFAPA. This is where Dr. Katherine Edwards is on staff. She is the one who formally diagnosed PFAPA and gave it the name. Within the last three weeks, my 22 month old daughter has had four episodes of fever which has been treated with Orapred. Unfortunately, they have decided that she is one of the patients who has more frequent fever episodes due to the Orapred. As of yesterday, she has been changed to the Cimetidine. Dr. Hasalan told me yesterday that if Hannah is going to respond well to the Cimetidine, we will see no fever within the first two months. If she does well, then we will leave her on the twice daily medication for six months. Then she will be taken off of it and given time to see if the fevers reoccur. If so, she'll have to either go back on the medication or have a Tonsillectomy.
ReplyDeleteI'm anxious to see how well this medication works for Hannah. Quite honestly, I was wanting them to recommend the TNA at yesterday's appointment. The studies show that the surgery has a higher success rate of stopping the fever than the daily doses of Cimetidine. However, the doctor recommended giving the medication a try since we are coming upon the holidays and our schedules will be putting us out of town for three weeks next month. So we will give this a try and hope for the best.
Please let me correct myself it's T&A for tonsils and adenoids.
DeleteI've just come across several groups on Facebook that are dedicated to discussions just as you're bringing up. I'm finding them very helpful! There are three that I can see: pfapachild , PFAPA.Community and Periodic Fever Syndrome (PFAPA)
DeleteHope that helps!