jojo + PFAPA

glitter clip // tank // pants // shoes

just in case you missed the (extra long) explanation on the 'gram stories, I wanted to share here about Jolie and what's been going on AND that we finally have a diagnosis.

you have no idea what a relief it is to have some answers. 

I honestly didn't see the effect it was having on me, my emotions, and my heart until I was basically walking on a cloud yesterday after our appointment with the rheumatologist.

taking you back a couple weeks ago, we got a call from the clinic because someone had cancelled their appointment and there was a time slot available. it happened to be a Tuesday, which is my usual day off during the week AND Jordan was off to be able to go with me.

perfect.

truth is, I was a little hesitant about going and I sort of walked in with very low expectations of the appointment. our pediatrician had mentioned about a week prior to getting the call about the open appointment that we shouldn't worry about going if we had to wait until OCTOBER to get in.. and that she really feels like infectious diseases should be involved instead of rheumatology.

within just a handful of minutes of visiting with Dr. Stewart at the Children's Hospital of Dallas she had a diagnosis for us. 

PFAPA

"..excuse me, but do you have a handout on this?"

Periodic Fever, Aphthous Stomatitis, Pharyngitis, Cervical Adenitis (PFAPA) is a syndrome that consists of recurrent episodes of fever, sore throat, mouth sores and swelling of the glands in the neck.

Jolie has all of these things. 

she has fallen into a period of 4-6 weeks between episodes and her episodes of 104+ fevers last about 7-12 days.  these fevers completely knock her on her rear.  she's lethargic, doesn't hardly interact or play, and definitely doesn't eat.  occasionally this is coupled with nausea and the lymph nodes in her neck swell and become painful. 

in regards to the mouth sores.. I've noticed they come about a week before the episode.  and as a matter of fact she complained about her mouth "burning" a few days ago.. I didn't think anything of it then but now I know what I know, and she's been 6 weeks fever free, so I'm anticipating a fever any day now. 

ugh.

we do have a plan, though.  for now, we are going to watch and treat her for the symptoms as they come. maintenance medication is the last option for both us and the doctor mainly because the side affects of these drugs can be hard on her little body (stomach upset, damage on her liver, ect.). we could also treat with steroids with the onset of fever.. but steroids have a track record of causing the dormant time of these episodes to become shorter (i.e. 2 weeks between fevers instead of 4 weeks) which we experienced when we were treating her for what we thought was an infection in her lymph node when all this mess started.

we go back to the rheumatology clinic the first week of January and most likely we will plan the surgery to have her tonsils and adenoids removed. 100% of the comments I received talked about their success with the tonsillectomy- and Dr. Stewart agrees that this would be the best option for Jolie to find ultimate relief in these symptoms. apparently tonsils house a ton of bacteria and can be the root of all these issues Jolie is having. 

AND most importantly we don't want to be walking into kindergarten with these fevers, you know? 

I hate this for her, BUT I am thankful for answers. I shared the news on IG stories yesterday and the amount of people that came out of the woodwork to offer encouragement because they have directly gone through this with their child or know someone that has, was incredible. something so foreign to me is actually much more common than I could have imagined! 

the tips and tricks and trial and error so many mamas offered was phenomenal. 

I cannot thank you all enough!

update | p + j

finally, right? 

I'm writing this to give a much warranted update (even though there isn't much an update to offer on Jolie), but also because we have been going through some emotional things with both Parker and Jolie that I hope to never have to deal with again, but if I do, I'll be able to walk through this experience with knowledge and someplace to look back on if I write all this out on paper.. err, the internet.  I started this blog as a documentation of our life, but also as a place for ME to look back on of memories as well as for times when I'm thinking to myself (in regards to the other two babies in the house) "what the heck did I do (in this situation) with the twins?"  I read the monthly updates of all the kids more often than you might think, ha!

many of you have been praying, emailing, messaging about Jolie and I am SO thankful.  we have been covered in prayer and those prayers have been felt!  I don't want you to think that I'm just dangling you along and carrying on our merry way when our daughter has been going through some very scary things for a 4 and a half year old.  because thats not the case. 

not.at.all. 

Jolie Grace

there is little update to give, and honestly I wish I had more to type here about her. 

a couple things.. NO FEVERS.  its been three weeks of a completely fever free Jolie. YES.  AND she has been acting more of herself as far as my normal joyful JoJo versus the BEAR of a child we witnessed during the last several months. we finally made an appointment with a rheumatologist for OCTOBER.. and I'm sure the poor lady setting our appointment could tell my frustration with that time frame and quickly asked if I would like to be on the cancellation list.

"um, yes.  thank you."

after running through some details and events over the last several weeks with our pediatrician she thinks that autoimmune could be a stretch.  OK, so more unknown and misinformation and a complete reach for a diagnosis.  don't get me wrong- I am positive they are seeking the greatest possible care for our girl and finding a diagnosis is at the top of their list, but they just don't know what to do because her symptoms are very inconsistent.  the west nile virus has now been thrown on the table.  its a very violent disease and apparently there are two types- one is viral and presents like what Jolie has suffered with and the other attacks your nervous system and is completely debilitating- much like meningitis. I've watched a friend deal with the latter and it is the scariest thing I've ever witnessed in my life. so, in this situation, I am thankful Jolie doesn't have that.  while, health wise, things are looking up for our girl, emotionally she's wrecked.  I was walking through all this with Jordan (we talk about it daily it seems..) and have placed blame on ourselves for not explaining to sweet Jolie about what she was going through.  dozens of doctors and procedures and blood draws.. putting her little body in tube after tube for tests and never once did we really sit her down to walk through what she was about to do or why we were doing these tests on her.  WHY?  WHY didn't I/we explain it???  I think part of it was that I was trying to figure out myself how to process what was happening with her and I completely forgot to include JOLIE in this.

 parenting 101 and I got a BIG FAT 'F'. 

she's been traumatized through her experiences and has now developed severe anxiety thats keeping her connected to my hip 24/7 and up all night long in fear of who knows what.

I can't even sit them down for dinner and walk back to the bedroom to pee without her following me back there in tears because I "was taking too long". if I am putting a baby to bed she will sit outside the room against the wall waiting for me to come out.  bedtime takes about an hour once we get them in bed- the up and down and more reassurance and kisses and hugs.. she's starting in her room but wakes almost nightly with night terrors and screaming, so we basically have a permanent palette on the floor next to the bed.

Parker Jane

she's always been the more emotional one of the two girls.  she's also the sweetest most gentle soul I've ever met.  this girl has SO MUCH love to give.  she also has developed this emotional anger that I have no idea how to control.  this started after the first weekend we separated them to take Jolie to the hospital over several days for tests and exams.  they had never been apart for more than 24 hours and Parker was with her BB and Papa for 3.5 days.  we FaceTimed and sent pictures, of course. the person Jolie cried for after coming out of her first CT scan wasn't mom or dad or BB, it was Parker.

"I need my Parker."

we FaceTimed Parker and they were both in tears before the call was over.  

the twin bond is very real.

after Baker was born Parks had some anxiety just to the extent that she needed a little extra love before bedtime, but this time its overwhelming the amount of stress this girl has. my going back to work has put her over the edge, coupled with Jolie getting extra attention, PLUS a new baby.. its a lot for her little heart to handle right now. I've gone on multiple dates, just Parker and me. Jordan has done the same and her BB has picked her up a time or two just to spend some quality one on one time.  all those things have helped!

goodbyes and bedtime are the most stressful time of the day. leading up to me leaving for work or saying good bye to the nanny (she's with them three days a week) can last up to 20 minutes and still end in a tantrum because I didn't wave from my car goodbye or say I love you eleven hundred times.. even though I DID.
the tantrums.  so bad that I've googled "psychiatric disorders in children related to tantrums".

truth. 

one time she was throwing a fit so out of control that it took me and her Papa holding her down in the carseat to buckle her in and then she puked all over my car in exhaustion from screaming so much.   she freaked out on me at 2am one night (of course when Jordan was at the fire station) when I told her to go back to her bed and that tantrum consisted of nearly kicking a hole in the wall, woke everyone in the house and lasted over 60 minutes.  she's lashed out at her sisters. kicked the nanny.  told me she hated me numerous times..
thrown food all over the floor and destroyed a handful of toys.
life has been TOUGH. as tough as its been for me and the rest of the house, I know that she's having an even harder time trying to process her feelings.  we recently met with a social worker in hopes she could offer some help and insight as to how we should be dealing with her- because nothing is working!  thankfully, after she assessed her history and chatted with her a bit she didn't think this was a permanent emotional disorder.  girlfriend is just really anxious. 
in turn, we have changed the way we do bedtime (reading stories, no television, sleeping in our own bed) and have started practicing saying goodbye and good night so they don't last forever and end in distress.  I've also begun reading a recommended book 'anxious kids, anxious parents' and have come up with tools to help Parker work through her emotions while also keeping my cool.. it is SO EASY to lose my mind on this girl when she is misbehaving.
theres a FINE line between discipline and managing Parker's fits.
good news is that the tantrums have subsided from everyday multiple times a day to less often.  I think/hope that we've been able to make her feel comfortable enough to work through whatever emotions she's dealing with at the moment as well as diverting her attention to something else, which is actually working really well. 

obviously, you all basically see the 2x2 happy highlights of our life- and why shouldn't you??

assumptions can easily be made by what it looks like from those posts, and yes while most of the time we are living a happy life, you will not see the time when Parker has torn her room to shreds and taken a bite out of my leather couch in pure uncontrollable anger and frustration..

I'll also spare you the terrifying moments when Jolie is running through the house screaming "MOMMY!!!" in fear searching for me at 1:37am in the morning because she's had yet another night terror.

tears are rolling down my cheeks as I type and relive some of these events of the last month or so- partly because I have never been this stressed out and exhausted in my life and also because I feel vulnerable to share all this after I get many complements for how well I handle 4 children.. well here it is:: I feel completely hopeless as a parent.  not in my wildest dreams would I think that I would be dealing with this kind of thing EVER, but here we are.  I can see the light at the end of the tunnel. I can.  I've got some happy and healthy kids- life is chugging right along and I'm loving being a mom to Parker, Jolie, Baker, and Brady.  BUT- do not assume that I have my crap together because I most definitely DO NOT. tomorrow I'll follow up this post with something light and cheery, lets talk about makeup or food.. or what we did over the weekend! but not because I'm sweeping over the hard stuff in my life, but because its helpful to remain positive during the hard times Jordan and I have been dealing with. we have a very strong faith and leaning on each other as well as pressing hard into the Lord allows us to keep a smile on our face. it is EASY to fall into the doom and gloom "poor pitiful Amber".. but we won't.  life moves on with or without us and dwelling on something like this is easy, trust me.  

the good, which is about 90% of the time is SO GOOD.  we are so blessed and life is SO good.. just going through some tough stuff right now.

we will get through this stage of life and move forward.. looking back to forget the really hard stuff and focus on how we grew as parents, in our marriage, and as a family from this experience.

Jo

FIRST of all.  a GIANT thank you to everyone that has called, texted, emailed, messaged, and commented on social media since we briefly shared whats been going on with Jolie. its been the most humbling experience walking through this process of trying to figure out what in the world is going on with my child, and I've gone from crying out in hysterics to fuming angry about all this.  trying to process it all and then having to explain to those that are concerned is overwhelming because honestly I'm at a loss for words to adequately express what the plan is at this point.

I guess I'll start from the beginning?  

this started probably about 4 months ago.  Jolie had been having random fevers, virus like symptoms off and on.. and sleeping NON-stop.  she would take two or three naps/day along with sleeping about 10-12 hours at night.  since she was in preschool I chalked it up to being a stomach bug and she would get over it.. and she would.  symptoms were always short lived.  about a month ago we were visiting my parents and sweet Jolie was, once again, with diarrhea and her fever was getting up pretty high.  we were treating it with Tylenol and Motrin, rotating it every three hours or so and it would come down, but sure enough, within 3 hours she was spiking 102 once again.  we were getting ready to go to dinner and Jordan was brushing her hair and rubbed his hand across her neck and she winced in pain.. thats when we found it.. the GIANT lump on her neck. it was huge and her neck was red and it clearly hurt to the touch.  we sort of panicked and called our pediatrician to ask about taking her to the ER or seeking guidance for what we should do.. and they told us that it was not a big deal and to come in on Monday.  So, Monday, May 22nd to be exact, we went into the pedi's office and saw the nurse practitioner (that we love!). this was day 5 of persistent fever (running 102-104) and I was done. 

"check for everything, and I mean everything."

so they did. and everything came back normal.

no mono.  no strep.  no infection.. no leukemia. 

yes, I made them check THAT.  I felt like something wasn't right and I didn't want to be back there in months with terrible news being delivered when I should have listened to my gut instinct.

her labs looked relatively normal.  of course a little elevated on her white cell count because she has been constantly fighting fever.  but otherwise, nothing concerning at all.  

"its a virus."

so, assuming this lump on her neck was infection related to the virus she had, she was put on a really heavy and strong antibiotic (clindamycin). she took 14 days of antibiotics that basically tore through her GI and caused even more GI upset than she was already having in the first place.  she wasn't eating too great, but she had stopped running fever, so we were on the right track.  she finished her medicine on a Sunday and on Wednesday she spiked 102 fever (again) and I couldn't get her fever to come down below 101 for 12 hours, even with cold bath and medicine. I called the pediatrician and came in the next day.. to see Dr. Butler.  she gave her a full exam after we walked her through the last month of concern and decided that, along with more lab work, we needed to get a sonogram of her neck since it hadn't changed at all in size, even with heavy antibiotics.  

she also checked her thyroid and about a billion other labs and tests to rule out a million things as to why this could be happening.  

we sent off a stool sample and did blood and urine cultures.. we did the works.  

that night we were up all night long with Jolie.  she had started throwing up again and couldn't hold down medicine to bring down the fever that had gotten up to 104. I was trying to dress her to load her up to go to the ER at 2:30am and she was like a potato sack. she wouldn't move or respond to me because her little body was just exhausted.  finally, after several attempts we got her to hold down meds for a half hour that brought her temp down to 101.  I'll take it.

we were able to get into the hospital to do the sonogram on Friday afternoon and we were expecting to just wait and hear on Monday from the doctor about the results.  we had plans to head out of town early on Friday morning for Jordan's grandmother's celebration of life that was on Saturday afternoon.  Butler really wanted us to get this sonogram done before going out of town, so we loaded up after the test and headed out.

our HOPE was that this sonogram would show that the lymph node was fluid filled or an infection causing the fevers and could be treated with medicine.

this sweet pic was taken right after everyone got up on Saturday morning! 

we headed downstairs to eat breakfast at the hotel.. I left my phone in the room and completely forgot  about everything that had happened the day before.. aside from Jolie waking up with a fever AGAIN.

just after I got everyone situated with their plate o' pancakes Jordan got a call.  I quickly realized who it was and I just sat there in silence trying to read his face to see if I could get some kind of idea as to what was being said on the other end.

he hangs up and doesn't even say anything.  just starts crying.  

"WHAT?!"

"the sonogram was abnormal.  its not fluid filled and its not an infection.  she has a large 5cm mass on her right side and the other side is abnormally small and appears that its not even functioning.."

the next several hours are a complete blur. 

we were told to leave as soon as we could and head back to Dallas for a CT scan.  we quickly packed everything back up, left Parker and Baker with BB and Papa and took sweet Jolie and Brady and booked it as fast as we could back to the hospital.  since it was Saturday we had to register through the ER and then finally made our way back to radiology.

we spent the next two hours waiting. Jolie had to drink the oral contrast, and getting a little person to drink THAT much was near to impossible.  but I attempted to make it into a game and we got enough down so she could go back for the CT. 

she had both oral and IV tests run and the preliminary results were CLEAR.  I was so thankful. 

they had basically prepared us for the absolute worst: the lump on her neck is actually lymphoma and the nausea and vomiting and repeat fevers were all a result of the cancer spreading to the nodes around her GI track and in her tummy.. and that we would leave straight from there and transport Jolie to Children's Dallas where she would be admitted to the 6th floor oncology unit.

lets just go ahead and file that under: parental nightmares you never want to experience.

after the hour long CT scans it was finally time to leave.  since the scans came back clear (read by an adult radiologist) and we were free to go home. we still felt a bit unsure about the scans because the CT showed all clear or everything.. even the lump on her neck.  no one, not even our pediatrician relaying this information to us really understood that because we can see it and feel it.  something was missing. the pedi scheduled us for 12:30pm on Monday to come in for another exam and to produce a game plan.  she also had scheduled a conference call with the pediatric radiologist at the hospital to officially walk her through Jolie's CT scans.

either way we were happy to be on our way to spoil Jolie rotten for all that she has been through that day.  we took her to build a bear, claire's for some new jewelry, and then out to dinner for chips and queso at 9pm.  it had been a long day and everyone was so exhausted.  I cried myself to sleep with Jolie in my arms.  I couldn't let her go!

Monday morning came, along with my first day back at work (talk about terrible timing for all this), and I got a call from Jordan. 

"why is he calling me."

apparently when the pediatric radiologist read the scan that morning the mass WAS there.. the scans were just really poor quality because she was moving around.

um, yes. she's four.  she's terrified.  and she was bawling her eyes out.

 the adult radiologist that read the scans on Saturday missed it.  our 12:30pm pediatrician appointment was changed to a 2 o'clock appointment with an oncologist downtown.

 once again with the emotional roller coaster of confusion and fear..  

the appointment came and I felt like I could just hurl everywhere.  I hadn't quite closed the door on the possibility of it being "the C word", but the unknown was debilitating.  I hadn't slept in like a week which felt like a month. my body hurt with anxiety and I just wanted answers.  Jolie was scared and had yet to understand what was happening or why she was there. the moment we were admitted to the exam room she started crying.

"no more doctors, daddy"

all I could think was that this was just the beginning.. 

the hematologist/oncologist came in after what seemed like an eternity of waiting and she did a full exam on Jolie, just as our pediatrician did the week before.  she mentioned that the mass was elastic and not affixed.  she also mentioned that she could feel protruding lymph nodes all over Jo's body, which she reassured us that was normal for some people.

"I am confident to say that this is benign. that doesn't give you answers about the fevers and GI issues ..but I do not believe this is lymphoma simply by the way it is presenting itself. 

I could have kissed her! she then proceeded to schedule us for a follow up appointment in two weeks.. more blood work to see if anything has changed and assuming that if the lump doesn't reduce in size then we will be going the MRI/biopsy route.  it hasn't changed in a month, even after heavy treatment with antibiotics, so I foresee the next appointment ending with a scheduled MRI, but we shall see!  

Tuesday came and my phone rings again.. its Dr. Butler with more test results.  apparently the stool sample we sent out came back positive for EColi.  most likely picked up from a restaurant, Jolie's nausea and vomiting could be related to this.  

so, now were assuming the lymph node and throwing up and diarrhea are completely unrelated?

I guess.  can you get fever for a month from a food borne illness?  google couldn't give me a straight answer, so I dunno. to be honest I'm still trying to process everything and its all happening so very fast.  I swear everyday we are getting different explanations and plans of care.  Butler didn't want us to be sitting idle for 2 weeks, so in the meantime we are treating Jo for the EColi with more antibiotics and probiotics..AND eliminating dairy.. which totally sucks.

all I know is this girl is a freaking rockstar.  she's been such a trooper. never complained. and seems to keep her spirits even with all this mess, and while still feeling like total crap.

so, now we wait some more.  

hoping it all comes to the surface with facts and a solid treatment plan.  the lump still isn't fluid filled or infection, and I feel like no one really knows what is going on with our girl.  I admit to feeling a little silly and mislead for freaking out over something that wasn't there or is something completely different.. I wanted to scream for joy and anger when the oncologist settled our greatest fears.. but y'all I'm so relieved.