Recurrent unexplained fevers

[BT2016]

Hi, my daughter is 1 year and 1 month old. Ever since she was 5 months old, she has had unexplained fevers of up to 41 degrees every 3 weeks to the day. We can predict when she is going to be ill and has now been ill 13 times in 7 months. When ill she is lethargic, skin is all mottled and she gets purple lips. She vomits too. Have taken her to the GP and out of hours countless times to be told it is a virus. Eventhough she gets checked over and they can't find any symptoms such as sore throat or ears etc-no other symptoms whatsoever. Had an appointment with the paediatrician who told me she is normal eventhough she has dropped from 91-95th centile to 70th centile, he thinks her weight and length are 'correcting' themselves (she is long eventhough my husband and I are short) I suspect she has periodic fever syndrome but paediatrician said she doesn't fit the criteria because he doesn't believe she is failing to thrive. She doesn't eat for those days she is ill, takes her almost a week to fully recover and get back to normal. Last time once her temp returned to normal it dropped to 35 degrees. She felt ice cold and NHS website says below 35 and it's hypothermia? Paediatrician also said that was normal....She seems to be getting ill again now, bang on schedule. I am fed up for her, she needs a break, we only get 2 weeks of a healthy happy child at a time (that's if she doesn't have a cold in between these episodes). Hate seeing her so unwell all the time. Should I be pushing more or is she normal? It doesn't feel like it-yet the GPs and paediatrician are making me feel like some silly clueless mum. This is my second child by the way-both stay at home, no nursery etc and eldest is never ill/picks up anything from my other daughter who (if I were to believe the drs) keeps picking up viruses every 3 weeks exactly....Please help with advice, should I be doing more for her?

Thank you so much tedsterteddington for the response- it really helps hearing the steps you took, in order to understand what it is I should be doing next. DD's temperature is rising tonight, am expecting to be confronted by a feverish baby either middle of the night or in the morning, am dreading it. Always hope that this is the week where it stops. I think the important thing I need to do is keep taking her to the GP like you said to back up the notes. We have a follow up with the paedatrician in a couple of months too so will argue my case with him too if nothing happens with the GP before that.

Also tedsterteddington-what does the treatment consist of?

Hi . My son had exactly the same symptoms as your daughter and was eventually diagnosed with cyclic neutropenia. A very rare disorder where the neutrophils fall at 3 week intervals virtually like clock work. He is now treated with medication and is doing well . Hope you get answers soon

I have encountered so many doctors over the last few years, we have ended up in various A&E departments and GP surgeries up and down the country. I think there is only one who had actually heard of periodic fever syndromes/autoinflammatory conditions! We met a new GP last week who said that when he hears the word syndrome it usually suggests rare! The autoinflammatory alliance have so much information, I'm also part of a Facebook Group called autoinflammatory diseases - rare but not alone which has been incredible. Parents facing similar all aver the world and highly knowledgeable admin, they may be able to recommend a doctor in Bristol. There is Professor Brogan at Great Ormond Street who has a periodic fever clinic too.
Sorry to hear about your daughter Popsy, Cyclic Neutropenia is one of the conditions they looked into and excluded with my son, glad to hear she's doing better.
There seem to be a number of treatments available, my son has only just started a medication recently and is currently 4 days overdue for an episode so I'm hopeful that it's maybe having an effect. I think it depends on what condition they diagnose. For PFAPA they seems to find that removing the tonsils fixes the problem, I think most others are lifelong and need to be controlled with medication.

Thank you so much for the information tedsterteddington- fingers crossed that your son is about to skip an episode, that would be amazing, I often wonder what that would feel like :-) hopefully the medication is working and he can start feeling a bit more normal. I feel a bit more hopeful because the paedatrician did mention periodic fever syndrome (so it's obv something he does believe in/know about, unlike so many others) especially after I spoke about it as something I thought could be wrong, and he said it could be a possibility but only if her weight continues to drop? Surely they shouldn't just be waiting for that-doesnt seem to make sense as it is dropping but she does manage to eat more at other times during the month-she has always been a big baby (91-95th centile for height and weight consistently until around 9 months when I stopped breastfeeding her-this was when her weight started dropping as when she was ill at least I could breastfeed her even just for comfort but she just refuses food now and won't eat during an episode) so her weight isnt dropping as quickly as other children. I expect if I stopped breastfeeding a lot sooner, her weight would have dropped that much quicker. Doesn't change the fact that she could be suffering from it- I will be discussing this further with him- at least it has been mentioned though-am hoping if I do a clean catch urine sample and rule out a uti they might then seriously consider a periodic fever syndrome.
Popsythe1st- I am so glad that your son is doing better now, must be a huge relief to find a diagnosis and treatment.

GPs seem very sceptical about removing tonsils etc don't they- I know that I have had mine removed and my dad also. I think it may be hereditary, myself and family are Mediterranean and my mum is Chinese so I know these ethnic backgrounds are more prone to these type of disorders? Maybe it would be worth me mentioning this- I should have already but the last 2 visits they haven't let me get a word in edgeways and i have left demoralised and upset.

BT you have a right to request a second opinion. Your GP needs to take your concerns seriously and you need to be polite but firm about the request if the GP initially trys to decline. Is there another GP in the practice that you can see perhaps?

I had the same battles with the original
Paediatrician as my son was 91st centile, he has dropped to 75th more recently. Don't let them fob you off with this, I have a very poorly little boy who happens to eat well and catch up inbetween episodes and genetically is from a family of tallish and solidly built people! Just because he's not underweight it doesn't take away from how ill he is and the huge impact this has had on his life
I think that if they diagnose PFAPA then tonsil removal is the preferred treatment, my son doesn't have PFAPA so this isn't an option for him, he's closer to FMF/TRAPS. Obviously a rheumatologist would be able to get you on the right path. Don't give up

Take somebody to your next appointment with you for moral support, partner, friend or family member! Also have all your notes together with any questions written down. I forget everything if I don't go in organized

Getting a clean catch urine sample is simple- ask the GP for stick on urine collection bags- wait till she's ill, bath her, dry her with a clean towel, stick the bag on- let her run around with no nappy, just the bag, then whip it off as soon as she pees. Then transfer to a sample bottle.

Or when my son was in hospital they put sterile gauze in his nappy, then squeezed the urine sample out from the gauze using sterile equipment! Probably not ideal but he was very uncooperative so left them with little option!

Thank you for the comments-I think I will try another GP this week. Thank you for the advice about a clean catch sample- I had no idea but will ask when I go in. All of the comments from everybody really have helped me- it's hard to talk to the same people about it- having your views and support make it a little less daunting especially as we approach another episode. So thank you, will try and keep people updated

Stay strong and please let us know how you get on. I really recommend joining one of the groups I've mentioned as the support we got from them has made all the difference 👍🏼

Thank you :-) this is going to sound V strange but DD was showing all the signs leading up, temp was rising, eating lots and then no appetite, funny noises, ice cold hands etc etc, had a temperature on the night she was due an episode, woke up with a temp and then has been fine since?! Just wondering whether it is to do with her having a cold? (DH was speculating whether her immune system could be attacking the cold rather than her own body for a change?) This is the first time a cold has ever coincided with her episodes, she seems happy and has no fever. A first for us, I feel happy. I know it prob isn't the end but for now I feel happy. I will join one of the groups as we all def need a bit of outside support when it happens again- by the way I got some of those stick on bags to collect her urine sample for next time-shame you have to ask for one and they don't just provide one straightaway!

Ah that's good news! It could just have been a very mini flare, my son's flares have usually come every 2 weeks and lasted between 3-6 days but we once went 6 weeks between them and have had a couple of flares that were much shorter and milder. I have never know why they were like that. Just keep it all written down as it will all help the doctors with identifying any pattern! My son has had some flares recently that started with a high temp just for a couple of evenings before it properly started. It's all very strange and I don't have any explanations.
Wishing us all a happy and temperature free Christmas

Well the urine collection will be easier with the bags- well done. God knows why you have to push just to get them! Good luck

Thanks- this is the first time she has ever had a mini episode-it has been strange but v nice indeed, especially with Xmas coming up and eldest DD playing up a bit. She has been super happy, eventhough she is a little poorly with a cold. Colds I can deal with though! Yep am still recording everything. Tedsterteddington-how is your son? Did he manage to skip an episode in the end? Hope you all have a lovely Xmas x

Hi everyone, I am so glad that I've found this thread. My DS who is almost 18 has been having periodic fevers since September 2015 and we just get fobbed off by the GP who seems to think he is idle and trying to get days off school (he hasn't missed a single day and he was ill when we were on holiday and on Boxing Day plus you can't fake a fever!). As he is 6ft tall and healthy in between his fevers, he doesn't seem like a priority for them but he is doing A-levels and I won't feel happy if he goes off to uni without a diagnosis. I have had to end up going to a private GP for tests but bloods are all normal so I am going to ask for a referral to a rheumatologist.

So sorry to hear that ono40
Start keeping a written log of dates, temperatures and symptoms. Take photos of your son with the thermometer next to him showing his temp, then you can take all of this "evidence" to show any doctors you may see. I would recommend trying a different GP until you find a supportive one. We were fobbed off by many doctors but finding a supportive GP and seeing a rheumatologist were crucial. My son's bloods are fairly normal, aside from anaemia, when he is inbetween episodes. But his bloods are wildly abnormal during an episode! Stay strong and don't give up!

Thanks so much for this information. @Tedsterteddington
My son had his tonsils removed in March for suspected sleep apnea and three very bad bouts of tonsillitis.

Since having his tonsils out he has had two episodes of high fever with no apparent cause and the consultant has suggested that we watch and wait as he may refer him to someone who will diagnose PFAPA. However the fact that he has had his tonsils out and is still getting fevers has made me concerned that it is something more serious.
I have asked to join the Facebook group you recommend and would like to see a really good consultant about this. We are in surrey so great ormond street would be an option

From my understanding PFAPA would normally improve after tonsillectomy. I would request a referral to GOSH through your GP and see the rheumatologists there, or the Royal Free if your son is 18 now! These conditions are so rare that it requires some fight and determination to get anybody to listen, write everything down (temps/dates/symptoms) and try and see the same GP with each episode to get their support and for continuity. Keep me posted. There's a new Facebook group for U.K. Members RACC-UK!

Hi guys, quick update on my daughter. She still has recurrent fevers, in the middle of one now. At the beginning of the year, she had fevers but there were reasons for them, jabs or a cold and a cough. But she has started having unexplained fevers again. We spoke to the specialist who was pretty dismissive and is insisting that there is no serious underlying problem and that periodic fevers could be an explanation but he really doesn't think she fits the criteria as she isn't failing to thrive. I reminded him that she eats a lot in between episodes to make up for it, but it didn't seem to make any difference to his opinion. It's getting very frustrating. Before our follow up with him, he said he wanted urine samples every time she had a fever and these have shown no urine infection. At our follow up appointment, he just reiterated that he thinks she is normal and that the fevers show her immune system is working. I really do not know where to go from here, my little girl is V miserable currently- she goes purple and mottled from the fevers and it went up to 41.3 degrees the other night before plunging below normal but again the specialist said this is normal. Does anyone think this could have anything to do with her teeth coming through? Maybe not the high fevers but the fact that it really runs her down? Always seems to coincide when her mouth is in a right state. I don't know whether I am deluding myself because I don't want to put her through a lot of stress if it is something that might go away when her teeth are through.

My nearly 5yo has been having fevers for nearly 2yrs.
It seems to be ear infections. VBut the drums frequently burst and it akways responds to AB so seems very similar to PP with the dc with it dec-mar, tonsillitis.
However during this time she also has ended up with permanently swollen neck glands and a heart murmur.
I think when their temps get 40+ that can cause vomitting. Dd temp is impossible to control and like a pp said with the JIA it does rise evening and drop by morning, but i think possibly normal body temps do cycle anyway.

We are also in same part of uk to OP which is interesting.
I have worried that dd has one of
JIA (family arthritis history)
Glandular fever
Problem with adenoids
Lupus
She also had a tick bite at 2yo so lyme disease.

Op did the fevers start after stopping BF?
Could it be slight dehydration/constipation?

Hi Naty1, am sorry to hear about your little one, nothing worse than seeing them suffer. Her fevers started at 5 months which seem to coincide with when she started teething but also when she started weaning. She was breastfed until 10 months so not anything to do with that. Dr cannot find anything else wrong with her, nothing wrong with throat, ears and no urine infections either. Def not dehydrated or constipated, when ill she won't eat but she will drink lots and lots. The vomitting Def seems down to having such a high temp. Apart from these fevers, she has not really been ill from normal things like colds etc.