Tuesday scare

Pierce has been known to test my heart's function several times - thank goodness my heart has been able to withstand it all ;) We've dealt with things from him turning blue/stopping breathing/needing Heimlich (from aspirating) to scary breath holding episodes (from injury not out of anger/discontent). Tuesday was the day he decided to add unresponsiveness to the list.

Tuesday morning did not start like any other for Pierce. If you know that sweet little blonde haired boy of mine then you know that he goes a mile a minute from the moment he wakes up. He defines energy fairly well. You would also know that he is very persistent in getting what he wants, when he wants - he gets it from his mama. He also has the SAME routine every morning... as soon as we open his door, bright big smile followed by a hello then a "Milk, mama?" --- I give him a cup of milk, he crawls into "his spot" (also known as George's spot) on the couch, I put on the toddler equivalence to taking LSD (also known as Yo Gabba Gabba) and he becomes enthralled while drinking milk, curled up with his blankie........ So, Tuesday morning..... he came to us asking for milk - George told him to wait a minute. He asked me, I said to wait a minute, too. He puts his head down on my stomach and falls asleep for a solid 30 minutes. I needed to get up to get things ready for Brice so I placed him on the floor next to where I was. He woke up and asked for milk. I fulfilled his request and he laid on the floor drinking his milk... and continued to lay there... 7:30 rolls around and he is still there... George gave me a look in passing like something's not right because Pierce had his back to the tv... he was just so listless and so very lethargic. I took his temperature - a nice 98.5*. George left for work. 8:10am... Pierce is STILL in the same spot on the floor. I had to sit him up to get him dressed and I laid him back down. 8:35am... time to leave to take Brice to school... Pierce is still in the same spot/position. I pick him up and take him to the car... we head off to school.

At school, one of Brice's teachers asked if Pierce was okay. She commented on how quiet he was and how pale he was. I gave her a brief run down of our morning and how odd it was but that he was basically asymptomatic (aside from being lethargic and listless). It's now 9:30... I stop to get him a smoothie... he actually perks up for a solid 45 minutes! We leave one store to head to another one that is less than a minute drive from the first... he and I are talking and mid-word, I hear his voice drift off. I park, turn around and he is completely out. I take his vitals - resp rate in the mid to high 40s and heart rate is mid 130s. I notice that his arms, hands, legs, feet and torso were twitching - not convulsing - but twitching.... nothing alarming because he does this ,occasionally, when he is falling asleep - usually lasts about 5 minutes. 10 minutes pass and he is still twitching... I call our immunologist to get her opinion - she's in clinic so I leave a message.... another 5ish minutes pass and he is still twitching with the same vitals. I call our pediatrician - she doesn't have any sick visits but they suggest I take him into the ER. It hits me - my maternal instinct is telling me something is just not right but what? What is it? So I call my mom trying to hold back tears because I am legitimately worried. She says to go straight to Chnola. I call George, he suggests attempting to wake Pierce up... and then my phone starts to die.

I go home to grab my cell charger. While there I try to wake him up... except, that I can't. Several minutes pass by - I am shaking his leg and practically shouting his name... I start slapping his other foot, while shaking the opposite leg and saying his name very loudly... his head pops up, his eyes open for about 2 seconds, close and his head falls back to his chest. I decide it's time to head to the ER. I made another attempt, at a red light, to wake him up = unsuccessful... another attempt and he opened his eyes for a second... another attempt = unsuccessful. We get to the ER, I get him out of the car, walk towards the hospital, talking to him... he begins to become more alert and responsive. I wait outside for my mom to meet me - question whether or not I should bring him in because he seems better.... he pees all over my leg. My mom comments on how pale, clammy and cold that he is - she suggests that maybe it was a low blood glucose episode. She is diabetic so gold star for her on that brainstorm. We go to the car to change him and decide to head home for now to continue monitoring him (<-- any time I can bypass an ER trip, I 110% will). I gave him 5oz of milk on the way home - oh and Nana shared some gummy candy with him, too!

Once we get home, our immunologist calls. I brief her on our morning. She thinks the low glucose idea is a good thought. She still wants us to go in to the ER. She doesn't think that it is an infection but she wants someone to physically lay eyes on him and run some labwork. We (reluctantly) head back to the ER. Everything was okay until the triage nurse made my blood boil. Seriously, why ask a question if you aren't going to wait for an answer? "Why do you think it could be a low glucose episode" - "Well my mom is diabetic, she mentioned it and I ran it by our immunologist who thoug..." - "Does he have any health issues" - "yes, he has a mitochondrial disorder and is immunodeficient - his specific diagnosis is IgG3..." - "Why do they think he has a mitochondrial issue" - "well he had a muscle biopsy in November..." - "yeah, but what are his symptoms" - "what do you mean - then, now... I need..." - "what medications is he taking" - "leucovorin, levocarnitine, and OTC supplements" - "oh my god, he is just the cutest little thing - hey sweetie" - "so I know that he looks good but Dr. Pratt wanted us to come in to get him checked out, she wanted..." - "have a seat in the waiting room" - UGHHHHHHHHHHHHHHHHHHHHH, I was pretty pissed. I notice these two teenaged girls sitting across from us (likely 14-16 years old) - they were mouthing off to someone... I realize it's an NOPD. He tells them "I will not take those handcuffs off of you because you're not going to hit me and while you're in my custody, I am in charge. If a doctor needs to treat you, he will do it with you handcuffed." @#$#^$#^WHAT? Sure enough, they were both in handcuffs. I whisper to my mom that we need to find a new section in the room to sit in. As we pass by one of the girls GROWLS at Pierce (because he isn't terrified enough of Chnola, right?!). I stopped dead in my tracks with my head turning back to look at her with whiplash type intensity. I very bluntly said "Really?" to which she flippantly replied "yeahhh, really" (like whatcha gonna do about). I mustered up every bit of of self control I could find within my body and told her to grow up while walking away to them laughing at us. Sigh...

We waited about 30 minutes before we were called into the back. I asked our nurse if she wanted to actually listen to our events and his medical history and she did :) A doctor came in 10 minutes later and I told everything to her as well. She wasn't concerned about his glucose levels because we've never had an issue with those in the past. She gave him a physical, everything looked good. She ordered labs only because he was a mito kid. Everything looked good except for his WBC count being high. She said things would probably get worse before they got better and that he was probably contagious and that she expected it to result in a stomach virus. I emailed our immunologist asking her to take a look once she got in to the clinic in the morning. She emailed me back yesterday with the following:

I agree his labs support some sort of infection, likely viral but no way to know by the basic labs. His cultures are still pending so I will send the labs once they are all back....just remind me! How is he doing today? Has genetics ever said that he might decompensate quickly because of his mitochondrial disorder? wondering if that has anything to do with it. Glad you went to the ER so we have some support of a cause. Oh and his glucose was fine.

Talk soon

Yesterday, he wasn't himself energy wise but he also didn't have any new symptoms. And today, he is pretty much back to normal, no new symptoms to report either. We are still waiting back the results on his blood cultures although I anticipate those to be negative since he seems okay.


But I have to admit that I am REALLY confused about Tuesday morning. I am also a bit concerned/wanting to know more about the idea of him quickly decompensating due to his mitochondrial disorder. I placed a phone call on Tuesday to our geneticist... sent him an email yesterday and as of now, still no response. 


I wish I had answers. I wish I was smart enough to be able to answer my own questions. Tuesday morning scared me to my core - and for me it's a tad more frustrating because I have no answer as to WHY his body reacted that way. So really, I guess that's it... have a Happy Thursday.

A tiny glimpse...

First, you can tell it's been awhile since I blogged... I had no clue that Blogger made a bajillion changes to it's format... oh well, bare with me if I screw anything up.

So I owe a few dozen updates to those who actually still come here to read... but today I am exhausted. I did want to share this with you. I contacted the Beads of Courage program to see if the boys were eligible. CHNOLA only offers BOC to oncology patients but we are eligible for the Beads From A Distance program. I am really excited because I feel as though this will give the boys something more tangible to express all that they've been through... and it's quite a bit for their 2 and 3.5 years of existence. I had a tally form with various categories and sub-categories to complete. They will each receive a certain bead for each thing they've been through... here is what I was able to tally for each of them:

Pierce

• 1 inpatient admit
• 8 nights in the hospital
• 1 NG tube
• 21 x-rays
• 4 times of being NPO
• 2 IV antibiotics
• 1 time on isolation
• 8 IVs
• 24 pokes for bloodwork
• 2 echocardiograms
• 2 EKGs
• 5 swallow studies
• 2 upper GI series
• 4 ER visits
• 1 ambulance ride
• 1 muscle biopsy
• 2 brain MRIs
• 1 ABR
• 2 brain CTs
• 1 upper EGD
• 1 lower EGD
• 1 sleep study
• 1 EEG
• 1 upper airway fluoroscopy
• 2 cardiology visits
• 10 GI visits
• 4 opthamology visits
• 9 genetics visits
• 7 ENT visits
• 4 neurology visits
• 6 orthopedic visits
• 5 immunology visits
• 4 pulmonology visits
• 2 IVIG infusions (well 1 is this coming Wednesday)
• 1 surgery
• 1 trip to AL for ortho consult
• 17 trips to Baton Rouge (1 hour away from NOLA)
• approximately 94 therapy sessions (speech and physical)

Brice

•  5 IVIG infusions (1 this coming Wednesday)
• 12 IVs
• 13 ENT visits
• 6 immunology visits
• 10 GI visits
• 3 pediatric surgeon visits
• 1 pulmonology visit
• 1 genetics visit
• 7 x-rays
• 3 hearing tests
• 15 pokes for bloodwork
• 3 surgeries
• 7 ER trips
• 2 inpatient admits
• 5 nights in the hospital
• 2 upper EGD
• 2 lower EGD
• 1 bronchoscopy
• 1 laryngoscopy
• 1 nasal endoscopy
• 1 NG tube
• 1 brain MRI
• 1 ophthalmology visit
• 2 urology visits
• 3 ultrasounds (1 abdominal, 2 renal)
• 2 foley catheters
• 2 upper GI series
• 1 swallow study
• 1 nuclear gastric study
• 21 trips to BR
• approximately 88 Speech Therapy sessions

After seeing all of this, it made me realize why the last 3.5 years have flown by... the sad thing is that this doesn't even encompass what seems like the hundreds of times we've been in the pediatrician's office for sick visits, well visits and childhood immunizations.

That's it for today --- if you have the ability, I really encourage (and would more than appreciate) you to make a donation the Beads of Courage program. You can do so here!

A post coming tomorrow about our scary day with Pierce yesterday... off to go release some stress while working out :)

titers and vials and more

Yesterday, my sweet little man had his quarterly follow-up with genetics. Since I don't feel like getting all fired up again, here's the annotated version:

After almost 45 minutes in waiting room with screaming toddler, our brazillant nurse crossed my path (not her best move) and I wanted to drop-kick her for her craptastic attitude... fast-forward another 45 minutes, doctor finally joins my terroristic toddler... he immediately apologizes for the "unacceptable wait time" (his words, my thoughts --- someone double booked his 9am slot - mind you, our appointment was scheduled for 3 months... sigh)... I express my dissatisfaction with his nurse... he acknowledges that "changing the system is hard, but he's diligently working on it."... we move on to the real reason for our visit - sweet Pierce. I tell him about our ST's concern with his decrease in words - he says we are probably seeing some regression but the positive thing is that he will progress back up. Mitochondrial disease has it's ups and downs - like a roller-coaster. I told him that we saw allergy/immunology and that Pierce's IgG levels came back low. We discussed Pierce's history of pneumonia, (several cases of) bronchiolitis, wheezing, neb'ing, etc... he thinks we need to add Pulmonology to our (ever-growing) list of specialists. He also said that Pierce is anemic - his levels aren't critically low. We are adding Poly-Vi-Sol to his daily regimen of medications/supplements. If that doesn't help, he may need elemental iron. We waited forever and a day for his labwork (due to miscommunication... again)... his left arm pooped out after {barely} two vials (I knew it would as soon as they stuck him because of the way his blood was flowing...)... his right arm was such a champ... 8 vials of blood later, one teal bandaid, one red bandaid one orange and one grape lollipop, two glittery Elmo stickers and we were FINALLY out of there... a whole 3 hours later.

Stats --- 23lbs 4oz (16th% weight) and 78.9cm (20th% height)

I received a phone call today from his allergist/immunologist team. they received his titers back... not so great news.

Pierce received his Prevnar (PCV7 - now it's PCV13) vaccine at 2 months, 4 months and 6 months of age; at 15 months, a child receives a Prevnar booster. A child's immune system is typically where it should be against the 14 most invasive strains after the first 3 Prevnar vaccines... Pierce's is not. 

They want to see at least 7 of these serotypes greater than 1.29 --- as you can see with Pierce, only 2 of his are... and just barely. Because of his low titers and low IgG levels, they are going to give Pierce a vaccine called Pneumovax 23. They said his body will (or should) mount a different response to this vaccine. Approximately 6 weeks after, his titers will be re-checked. If his levels are still low, we will discuss our next step (possibly close monitoring of his infection levels or straight on to IVIG). [side-note: I am slightly nervous about this vaccine seeing as how it has not been established in the under 2 population... Pneumovax23]

It's so hard seeing this sweet, smiley-faced boy that looks a-okay on the outside but really isn't on the inside... that's all for now... I feel depleted... but in 30 or so minutes, that sweet smiley-faced, blonde curly haired boy will be calling my name... and showering me with wet open-mouthed kisses... and that my friends replenishes my spirit.

bloodwork news

I just spoke with Pierce's geneticist to get some clarification on a few questions his neurologist had. The respiratory chain enzyme assay was completed. He plans on doing the mitochondrial DNA bloodwork in the future. Pierce does not have any Inborn Errors of Metabolism.

And the great news is that his medications are working! His ketones and amino acids are back within normal limits! Pierce's CoQ10 levels were lower than Dr. N wanted to see. His level was 271 and he wants to see it in the 300-400 range. He is fairly confident that it is most likely in that range as he upped Pierce's ubiquinol dose from 0.5cc to 1cc twice daily (back on March 7).

Yay for some great news :) I will take it however it comes in whatever amount - small or large!

As for Brice, his nuclear gastric imaging study has been moved to Tuesday morning. I was attempting to research what to expect with this particular test. While reading CHOP's website, I noted that they stated you cannot have an upper GI series 48 hours prior to nuclear gastric imaging. I had already placed a call to the imaging center to speak with a tech on what to expect. When they called me back, I explained to her what I read and she didn't feel it would interfere but said she would check with the radiologist just to be sure.... well, sure enough, it could cause inaccurate counts on certain images - (one point for me!) - they were able to squeeze him on Tuesday morning.

He has to be NPO for 6 hours prior to the study (including pain meds)... Here's the laughable part of all of this: Brice will eat a radioactive scrambled egg (made in a microwave)... he will have 10 minutes to consume it (not sure how that will go, especially due to his lack of appetite)... and here's the kicker, the ideal way to perform this test is for him to LAY STILL for 90 minutes! If {more like because} he cannot stay still, they will take images every 15 minutes. We will have a 2-2.5 hour break (while still NPO) at which point we will return to have another image taking at the 4 hour mark.

We will have his swallow study and upper GI series the following day.

His GI is out of the office all next week. I am hoping his partner will interpret the results so I can get some answers next week instead of having to wait. We will see :)