Lasting Effects

So I guess I was wrong about our sweet boy being "totally back to normal." We have seen some "lasting" effects of this Prednisone, and are praying that they are not permeant. We haven't been able to find anything that says they would be, but my husband found some information that stated that the medicine could possibly stay in your system for a couple of months! Wow!
I  know that Rowan has a lot to deal with already, with being 2 and all, and this probably just intensifies everything. Poor baby.
We received the blood work results, and according to what the internet says and our new ped, it looks like everything is pointing to a complete diagnosis of PFAPA. His ESR and CRP results were pretty high while he was in the middle of this last episode, but were normal this past week when we had blood taken again. He also seems to always have a low LYM in his WBC results (even back when he was really little). Not sure if this has anything to do with it, but you can bet we will be asking about that.
We are waiting for his PIDS doctor to return from being out of the country so that we can set up an appointment and decide on what the next step should be. I guess we also have to get the "official" diagnosis from him.
Rowan starts a wonderful MDO program next week. He has been so excited about "going to school" and "making new friends." This program/school is such a blessing, and I am thrilled that we were able to get him in! We had open house yesterday so that we could meet his teachers and see his classroom. Rowan also has a peanut allergy (which I thought would be "his thing" until PFAPA came along), and thank goodness this is a peanut/nut free school. One less thing to worry about with him. When we met with everyone yesterday, they were SO wonderful in providing us with all the necessary information about the school and how they make every effort to prevent a peanut reaction. Rowan is the only child in his class with a peanut allergy, but I know there are many in the program who also have Epi-pens for different allergies.
So here I am, talking to the teachers, and they are so prepared for his peanut allergy, and all I can think about is this "bomb" I am about to throw on them about PFAPA. When I started to tell them about it, they were so sweet, but really had no idea what I was talking about. I brought them a hand out about PFAPA so that they could do a little reading if they felt like it. I was so thankful that they were so willing to learn more about it. They asked me what it was that I needed them to do. I wasn't really prepared for that question. So here are the things I told them (incase another mommy gets in this position)...
1. I would try my best to not send him to school if I believed we were about to start an episode. (No attendance policy- thank goodness!)
2. I would love open communication between myself and them about his behavior and how he is acting, as well as any questions/concerns about PFAPA.
3. If he looks like he is dragging, has red cheeks, or is not acting himself, to take his temp and call me. One of my fears is that he won't have a fever before he leaves for school, but develops one while at school, and them not catch it. I told them that his temp can increase pretty quickly, and that this could possibly cause seizures.
4. I asked them if they would be willing to administer medicine to help control a fever if it happened while at school. Typically they don't administer fever meds, but they said they would make an exception for Rowan as long as I had a pharmacy put a Rx/label on the bottles. (So to do this, you have to call you ped and ask them to call your Pharmacy with the exact dosage for administration. They pharmacy will then put a label on the bottles, and you can drop them off at the school. Thank you so much to my wonderful friends A and M (pharmacist) for giving me this info! Don't know what I would do without them!
5. I let them know that he is not contagious while going through an episode, and that they should not be worried about him "infecting" other children. (This kind of broke my heart to think that others may  fear being around him thinking that they could "catch" what he has.)
6. I really wanted to ask them to LOVE my baby as much as I do. =) He's very easy to love.  (except for maybe when he is on Prednisone....just kidding)
You never think that you will have the "sickly" child of the class when your little ones start school. Having been a teacher, I know the anxiety and stress it can put on a teacher. I am so grateful that his teachers and school seem to be easy going and eager to learn all about him. When we left yesterday, he had a very unusual meltdown. I sat in the back seat of the car to keep an eye on him on our way home, and saw such a difference in color between him and my daughter. He looks so pale and worn out with circles under his eyes. I pray that we find a "cure" soon, and he can get back to being a healthy little boy!

Pics of Rowan playing outside today!

 Love the squat!!

 Loves to run and play! All boy!

My happy boy!!


**Addition**
My daughter has been running a low grade fever the last couple of days. Can't seem to find anything wrong with her (just went in for a well check up last week). Praying that she doesn't have PFAPA too. This is about the time Rowan's fevers started, and they started low.  Anyone hear of siblings both dealing with PFAPA???

Q and A

Ok, so this is more of a Q than and A, but still. I have so many questions going through my head, and have read so many conflicting stories about PFAPA. If you have experience, or know someone who has, please answer any or all of the questions. Any kind of help will be beneficial!

First off, I had a nurse friend of mine make a comment about the blue fingers and toes. I thought I would share her response incase anyone else maybe dealing with it.
"The blue of the fingers is pretty simple to explain. Simply put, it's a correlation between high heart rate and low volume (some level of dehydration) as you mentioned. The body does a shift where it pulls blood into the "core" until it stabilizes again."
Thank you so much Paige! Now I am armed with information incase it happens again. I will push more fluids. We are getting pretty good at "bribing" him to drink. This past episode, my husband started to offer him gifts (books, stickers, toys) and money (coins) to put in his piggy bank, if he took a sip of water. Not sure if it's a good thing that he already knows money has value, but kind of thankful that he is not quite aware of the value that each coin has. We were pulling out every penny we could find!

1. Recommendation for a really good and accurate thermometer??? I have tried the quick digital one (under his arm) and I have to do it three times before I can get it to go as high as his temp. I have another digital one that takes about 60 seconds to read. I feel like this is the most accurate, however, try holding a 26 month old down for 60 seconds without moving the thermometer 100 times a day! ;) We also have the ear thermometer. This one gives me different readings every time I take his temp. I know it says to point it toward his nose, and I really try to do it the right way, but it doesn't seem to work. I figure we need to invest in one that works pretty well, regardless of the price.

2. I worry about giving him too much medicine. It says on the box that it can cause all sorts of liver and internal damage to people. Should I be concerned about the fact that I am alternating Motrin and Tylenol, every four hours, for days, every month??? I don't know what our other option would be. We have been told not to let the fever spike because it can cause febrile seizures, especially when it spikes quickly (and his do- 100 to 104 in 30 minutes!). Febrile seizure are more of a concern than a fever, from what we have been told.

3. Has anyone noticed that the fevers get higher the longer they deal with the syndrome? When he was younger, they wouldn't get over 103 before meds. Now they are going to 104.5 with meds. I have read that some deal with fevers as high as 107! What do you do at that point? ER? I have been told to put him in a luke-warm bath, but also told not to. They said that if his body gets cold in the water, then he starts to shiver and his body tries to warm itself up- increasing his body temp. We have experienced that with just a normal warm bath while he was sick. =(

4. I have read mixed reviews about surgery being a cure. I pray that it is. To have their adenoids and tonsils taken out, could stop the syndrome. However, I have also read that for some, the fevers come back after, and others say that the fevers stop, but all of the other symptoms are still present. Any light shed on this would be helpful. Also, what age should the child be to have the surgery? We have been told that Rowan is too young, and should be closer to 3 before considering the surgery. Something about not being able to get younger kids to drink enough to stay hydrated???

5. Alternative medicine??? Anti inflammatory diets? Tart cherry juice? Extra vitamin D? Have these things worked for anyone?

6. Just from research and asking questions, my husband and I think that the groin pain is from swollen lymph nodes in that area. Also, maybe some of the joint pain is from swollen nodes around them (behind the knees, in the feet)? Anyone out there dealing with the same thing, or have knowledge of where lymph nodes are located? What about the back of your neck/head?

7. I have also read that after giving Prednisone, the episodes become closer together. This terrifies me. If someone could clarify- did it happen after just one dose of Prednisone? How much closer (I have read half of the normal time)? If you don't give it again, will they go back to 4-5 weeks? Are they worse?

8. Has anyone else experienced skipping a month in the summer (or anytime) and had the next episode be a really bad one? Ideas about kids skipping in the summer? Heat related???

9. Finally, what does it feel like? I know there are adults living with this syndrome. If you could shed some light on what my baby is feeling, I would be so grateful. Anything that I can do to make it easier on him during the episodes would be wonderful.

If anyone is dealing with the "roid-rage" and wonders when it will be over. Rowan is 100% back to himself today!! I am so very thankful. So the Prednisone was given Friday morning around 9:30am. It is now Wednesday. That's how long it took! Blah!
Grateful for the healthy weeks in-between. It's amazing how you can be around him now and never know what he just went through last week.  Just a few pictures of Rowan and his sister playing this morning. Made my heart so happy:

                                         (Rowan showing her the "rainbows" on the wall.)

(She adores him so much!)

                                     (My sweet happy babies- what a great morning it was!)

"Roid-rage is real!!"

NEVER again will we use the Prednisone! (Unless there is a serious issue and it is the ONLY option we have left). The fever episode was terrible, but at least my sweet boy was still with us. It is now Tuesday. The steroid was given to him on Friday, and I am just starting to see my sweet baby again! He is still having some behavior issues, but nothing like what he had for the first 3 days. Now I have my masters in special education, with a specialty in Autism and Behavior Disorders. I have dealt with behavior of all kinds before. However, dealing with your own child is so different. I continue to have the utmost respect for all families dealing with Autism and/or behavior disorders. You all are a pillar of strength. I believe I only got a small piece of what you go through on a daily basis.
We have been walking on eggshells since he woke up from his nap on Friday. He woke up SCREAMING  at the top of his lungs. I had no idea what was wrong with him. No one warned us or said anything about this causing unusual behavior. Typically I can comfort him by picking him up and hugging him, or loving on him. That was not the case with this. Any kind of touch set him off even more. Any noise, including our voices made him crazy. It was almost like all of his senses were in overdrive.
He screamed for 3 hours straight! NO BREAK! My heart was breaking for him. His heart was racing, and I'm sure his blood pressure was through the roof. He was also in attack mode. He would attack anyone near him. If there was no one close, he would come after us- mostly me. Thank goodness he only knows how to hit. I can't imagine what it would have been like if we had already started preschool and he knew about all of the other aggressive behaviors that you can have!
It seemed like it was much worse after he woke up- in the mornings and from naps. He wouldn't drink anything, so after all the screaming, his little lips would be almost cracked from being so dry. The look in his eyes is what scared me the most. It was like he was looking through you, with all of this rage, but also like he was begging for help. He hardly used any words at all. It's like he forgot how to communicate. He didn't want to eat, and when he was calm enough to eat, it was a battle to get him to eat anything remotely healthy. (Rowan is a pretty good eater. He likes all kinds of fruits and veggies. Wouldn't touch them during this time). I called the ped that afternoon, and they said, "If you can't deal with the behaviors, give him some Benadryl." Are you kidding me???? The last thing I am going to do is put MORE medicine in my child! And just to vent, I wasn't calling because I couldn't deal with the behavior, I was calling because I wanted to know if it was normal or if I should be concerned. I don't think they understood what the extent of his rage was.
Saturday morning we thought we might have to take him to the ER. Again, he was screaming and crying for hours. I couldn't get him to drink or eat. His heart was beating through his chest. There was NOTHING we could do to calm him down. All we could do was wait for him to wear himself out to the point of exhaustion. And he did. That was our life until Sunday night.
I am so blessed to have a daughter that is so chill and relaxed about everything. She is amazing at entertaining herself. However, after a week and a half of being so focused on Rowan, I felt like I was failing at being there for her too. She has a new tooth coming in, and I didn't even know it until it broke through! She's a great sleeper, and has slept through the night since I stopped waking her to eat, so I should have known something was up when she woke up fussy a couple of nights in a row. However, my only thought was, oh no! I have to get her back down before it's time for Rowan's meds again, or before he starts crying again. I pray that she doesn't have PFAPA too. I don't know how families, with more than one child going through this, deal with it.
Sunday night, after we put the baby down, Rowan finally sat on the couch with us. Oh the joy in my heart can not be put into words. He even put his hand on my leg and snuggled up to me. With tears streaming down my face, I thanked God. We watched a movie- I don't even know what it was because I was looking at him the whole time.
Monday morning- again the rage. Much less this time, but still there. It only lasted about half of the morning, and then again after his nap and a couple more melt downs throughout the day. Warning- TMI: After Rowan finally had a BM (it had been almost a week), his behavior started changing. I don't know if the meds were being absorbed over and over because he wasn't drinking anything (only 3 wet diapers in 24 hours- and that's pushing it), but I was thrilled. He sat in my lap! That night, again he snuggled up in my lap and we watched part of a movie. Monday he slept A LOT. He slept until almost 10:00, and then took a nap from 2-5:45, then went to bed at 8:00. If he hadn't seemed so exhausted at 8:00, I probably would have kept him in my lap for the rest of the night.
This morning he did well. No screaming or crying, and easily distracted when he would start. He wanted to play with his sister again, which was so heartwarming. She missed him so much. My sweet, polite boy is coming back. I am so thankful. The Dr. called yesterday (Monday) and said that his throat culture came back negative, so to stop giving him the antibiotics. I know that the blood work showed that he had a bacterial infection, so I decided to continue to give it to him to be safe. So when I gave it to him today, this is what happened!

The medicine got on his face because he turned his head, and cause what looked like burns! If it's doing this to the outside, what is it doing to the inside? SO, no more antibiotic. This poor little guy had had a rough week. Looking forward to a few weeks without him being sick. He starts MDO at the end of the month. Thank goodness they don't have an attendance policy. I don't know what we will do if he is still dealing with this when he starts school. =(
For now, my sweet guy is back- play and loving on all of us. (With a few typical two year old testings here and there!)

The worst one so far!

The actual fever didn't start until August 2nd. I knew by the way he was acting, and by the fact that his fever was already at 103.5 during the first 24 hours, it was going to be a bad one. Usually his fever peeks around the third day, and then starts to decrease. Most of the time it doesn't go over 104, so it worried me that it was so high already, especially with us giving him medication every 4 hours.
I also knew that we would have to get blood work done this time around, and since it started on Saturday, I knew that day 3 would be on Monday- not real good timing.

(My sweet baby sleeping with a 104.5 fever)

On Monday I took him to the Dr. (we couldn't see his normal one because she doesn't work on Mondays). I called before heading there and told them that we needed to come in for blood work that was prescribed by our Dr. at Tulane. They told me I HAD to have an appointment with a Dr. to get it done. I made sure they looked it up and had the orders, and then asked (to make sure) that they were able to take blood for these three specific things...CBC, ESR and CRP. They said yes, absolutely.

Between the time I called and made the appointment and the actual appointment, Rowan woke up (he tends to sleep later some days when he has been up and down all night). He was clingy and uncomfortable. I held him on the couch while he got some fluids into him. I noticed that his toes looked blue and felt a little cool to the touch. I asked him if he was cold. He said yes, even though his fever was pretty high. I covered him with a blanket and continued to watch him. About 2 minutes later, his fingers started turning blue too. Of course this really freaked me out! My husband took him outside and tried to get his blood flowing, thinking that maybe the way I was holding him cut off the blood flow. We have been told that Rowan has a slight heart murmur, so I was concerned that something was wrong with his heart. I tried to call his ped., but they kept saying, "we don't know if it's part of the syndrome or not. You need to call his other doctor and ask him." Finally I asked them what they would to say to any other mother that called and said their child had a high fever and was lethargic with blue toes and fingers. She said- "I would tell them to go to the ER." It angered me that she wouldn't have said that initially.

(Bad pictures, but you can kind of see the blue tint)

After about 10 minutes, the color returned to both his hands and feet. I emailed our Dr. from Tulane to ask if this is a possible symptom of PFAPA. I also called and left a message with his nurse. She called back and said to take him to a Dr, to get him checked out anyway. I was pulling into the peds office when she called back.
So here we are at the Dr., with a very high fever and a miserable, very sick child, waiting for blood work. Typically he is great at the Dr. However, this time he was so upset. He cried the whole time and wanted me to hold him. I felt so bad. When the Dr. came in the room, he asked what was going on. Due to Rowans crying, it was difficult to keep a conversation going, but I let him know that we needed blood work done and told him about the blue toes and fingers. He said that he's seen "thousands of PFAPA cases, and that kids just out grow it." He acted like it was no big deal, and that the symptoms were not serious. This is the FIRST time anyone in that office has mentioned PFAPA. Going in there the million times before, you would think someone would have mentioned something before our PIDS sent the possible diagnosis over! Right?? Hmmmm.....
He checked Rowan out and said he was unable to do the blood work in the office, but that he would send me to a lab that could. I was SOOO mad. I called and specifically asked if it could be done there, and they said YES! While I was trying to process this information (with a screaming baby), the Dr said, I want to do a strep test on him because I have seen a couple of kids with it recently. Before I could even object, he was swabbing his throat! I still feel bad about not responding fast enough to tell him NO! I felt like he was just doing whatever he could to get $$! Of course it came back negative, and after all that we still had to go somewhere else to get this blood work done.  He did say that sometimes with a high fever your limbs can turn blue because all the blood is going to cool down/save your vital organs. I don't know how much I believe that. His fingers and toes turned blue at least 3 more times throughout this episode.
We ended up getting the blood work done at a lab down the road. They were wonderful! I was so hard to see him go through that, knowing that he was already so sick. His fever was at 104.3 when we got the blood work done. The only thing that helped was knowing that they should defiantly see what they need to with his fever being so high.
When we got home that afternoon, I noticed his neck looked swollen. Now he has had swollen lymph nodes before, but this was terrible! They were like golf balls, and were red like they had fever in them.

(Swollen under his ear)

I kept thinking that maybe that Dr that did the strep test stirred something up in there, or made something worse. Mommy guilt- again.
Monday night was a rough one. Most nights are bad. Neither of us gets a lot of sleep . I have to set my alarm every 4 hours to wake up and give him meds. He is up and down a lot in-between those times crying and hurting. His fever got up to 104.5  that night. It broke my heart.

(His paleness makes his sister look tan- they love to play together in her bed!)

Tuesday (day 4) his fever started going down (103). Wednesday, down another degree (102). Thursday his fever started to go back up (104.2)! What is going on???? This was so NOT our normal pattern. Thursday afternoon he developed a rash on his face, arms and legs. Nothing on his trunk. It almost looked like a bunch of mosquito bites. Of course, I called the ped. again and said that I wanted to bring him in. I described the rash and told them his fever was starting to spike again. Because it was 30 minutes from closing time, they told me to just "keep and eye on it" and if things were still bad in the AM to make an appointment. Again- another mommy fail. I should have listened to my instincts and demand to come in. I couldn't sleep Thursday night. I knew something else was going on. He was complaining of all the normal pains, but also that the back of his head was hurting. The rash was worse when his fever spiked.

(Rash on his arms, legs and face)

8:00 AM Friday morning, I called and made an 8:45 apt. He was so sick. He was very pale and lethargic. He had dark circles under his eyes and was crying all morning. When the Dr (our regular one) saw him, she realized just how sick he was. They took blood again and did a throat culture. The blood work showed that he had a bacterial infection on top of the PFAPA. I knew something wasn't right. He was so sick that he fell asleep on me (the second time to ever fall asleep in public- even when he was an infant he wouldn't do that). His fever started spiking while we were there, so they gave him Motrin, and then a dose of Prednisone (which we were supposed to do at the next episode) 2mg/kg, and a big dose/shot of antibiotics- Rocephin 750mg. They also gave us a Rx for Suprax 200 mg/5. (I called later to get the names and doses of each medication for my records.)
His fever broke in about 2 hours. Lymp nodes went down and rash started clearing up. BUT the behavior/rage from the Prednisone was terrible!!!!! (Roid-rage in next post)
By the way- We are getting a new pediatrician!
**If you are reading this and have questions because I am not clear enough, please ask. There is so much going through my head, and with the lack of sleep this last week- sometimes I don't make sense.

June Fever

We were ready for Rowan's fever to come at the end of June/early July, but it never did! I was hesitant to "breathe out" for fear that it would show up late or something. Plus we read somewhere that some kids skip episodes in the summer months- no idea why.  He did run a 100.9 temp on July 3rd, but it disappeared the next day. I kept thinking- "Maybe I'm wrong! Maybe it really was just a lot of viruses!" We continued on with life, and started to look at a fever free future. Then the fussy behavior and clinginess started around the 28th of July. I knew that we were working toward another episode. Rowan tends to "let you know" through his behavior and actions, that he is starting to feel bad. He has a lot of language for a 26 month old, but he doesn't know how to explain what he is feeling internally all the time. SO I look for the signs.

Just some pics of my sweet boy! Love him so!

 He loves to be outside playing!

 He is the sweetest big brother, and loves his sister so much.

 A gentle soul.

 Reading a book to his sister...

 Keeping cool in this heat!

 Always wants to be next to mommy or daddy.

 The love they share is so special. Love that he wants to hold her hand all the time.

My snuggle bug!

Introduction

We are a family of four (plus two furry puppies) who recently moved to Louisiana from Alabama. Our son was born May of 2012 and in April of 2013 we found out we were pregnant with our daughter! She was born a month early, November 2013, and we moved in December 2013 so my husband could start his new job with his family near. We have been very busy this last year, but are so grateful for all of our blessings.

This blog is really for me to document what we go through on a monthly basis with our son, Rowan, as well as get some of the feelings/questions out that I have running through my mind. I am hoping that it helps other families through their journey with PFAPA, and brings all of us together to help find a way to make life easier for our sweet babies.

PFAPA= Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome.
From what I have read, they named it based on the symptoms because no one knows what causes it or where it comes from. It is a rare syndrome that includes fever episodes, every 4 weeks or so, which last from 3-7 days, and in our case include: mouth sores, sore throat with redness and enlarged lymph nodes. Rowan also experiences severe stomach pain, joint pain (which causes clumsiness = lots of scrapes and bruises), groin pain (which keeps him from being able to sit on hard surfaces for too long), loss of appetite (which causes weight loss/very slow weight gain) among other symptoms. We are one of the "classic cases" being that Rowan has all of the main symptoms that make up the syndrome.

Our journey began when he was probably 10 months or so. We didn't really see a pattern until after we moved and he was around 18 months. I remember taking him to the doctor in Alabama for fevers and mouth sores. They would always run a strep test (because his throat was red), and it ALWAYS came back negative. "Another virus" or "Hand, Foot, Mouth." I was a first time mother, but because I had been a special education teacher, I knew that kids could get sick a lot the first couple of years, so I tried not to stress out too much over it (yeah, right!). The week we moved, Rowan had one of his highest fevers- 104.5. We were in this new house, with no furniture, sleeping in pack n plays and on blow up mattresses, with a brand new baby (a preemie), and one very sick little boy. I didn't even know where the hospitals were yet! After giving him Motrin and stripping him down, the fever started to go down a bit. I already set up an appointment for my daughter at our new Pediatricians office, so I was able to call them in the morning to see if I could bring him in. (Of course I called our old peds office that night to see what to do. I didn't get a call back until 3 hours later- some mess up in the after hours clinic or something). Our new ped. told us that it was just a virus (after a negative strep test and taking blood), and that some kids just tend to run higher temps when sick. SO we took him home to wait it out. A month later, another fever. Again, took him to the dr. They did another strep test and took more blood. Another virus. Each month we would go through the same drill. Things just didn't seem to be adding up. We never went anywhere, being that we were in a new city and didn't know where anything was, or have any friends yet. I guess I could see a toddler getting a virus each month if in MDO or a daycare, but Rowan stayed home with me. AND no one else ever got sick.
Of course I turned to the internet and started searching recurrent fevers. I found PFAPA and it sounded just like our sweet Rowan. Expressing my concerns to his doctor and getting nothing in return except the "crazy first time mother look," I decided to call Rowan's previous pediatrician to see if I could get a referral to a specialist. I needed to see if this might be what he had. I was told to call and schedule an appointment with a Pediatric Infectious Disease Specialist- cause that doesn't sound scary at all- at Tulane. After making many many many phone calls, I finally was able to get an appointment (without a physicians referral) for June 17th.
I found that one of the most important parts to getting a diagnosis was to record each episode, along with the symptoms, so that the doctor would be able to see the pattern. I called and requested medical records from both pediatricians offices, along with a copy of all the blood work they had taken over the course of Rowan's life. I went through and highlighted each visit that involved the symptoms of PFAPA so that the PIDS would be able to see all of the info we had easily. I went back and recorded every date I could remember and could find based on his records, and was armed with all of this information when it was time for our appointment.
We met with Dr. Schieffelin on the 17th, and for the first time I didn't feel like I was going crazy. He was so nice and knowledgeable. After listening to us and EVERYTHING we had to say, he said it sounded like a classic case of PFAPA. Since we researched this on the internet, it was familiar to us, and was more of a confirmation of what we already knew. He said that he wanted some blood work done during Rowans next episode and then some done while he was in-between episodes so that he could compare them. He also wanted to give Rowan a does of Prednisone during one episode, and if it ended it, then that would be the real confirmation that it was PFAPA.
We went home feeling like we were armed with new information and had a direction to go in. We were ready for his June fever (which should occur around the end of the month/beginning of July).