IVIG

My lone post for the month of March touched lightly on our IVIG journey. I have to say that so far it's going very well. Brice has completed 4 infusions and Pierce has completed 1. Brice presented with an ear infection at this 1st infusion --- He also got sick about a week after infusion #3 (double ear infection and red, swollen throat - Rx'd a z-pak) --- IVIG #1 = migraine 24 hrs post; IVIG #2-4 = headache 48hrs post. Pierce's first infusion went a bit more smoothly than Brice's but it lasted much longer. And poor Pierce had a migraine for an entire day, starting a little less than 24hrs after. He was miserable. We will see how they continue to do - they may need a small dose of steroids at the 24hr-post window or they may need to switch to a different brand. They are presently on G@mmagard.

Infusion day has gotten REALLY tough with Brice. He knows what's going to happen... he knows he's getting an IV... and he knows he doesn't like it. His first IV went okay - the second was a disaster. By the third, they knew what to expect so they brought in the anesthesia team to do his IV. They were prepped to take him down to a procedure room to give him some nitrous, do his IV and bring him back to the floor. Thankfully, they didn't have to... but the kid has a reputation now. For infusion #4, in walked an anesthesiologist to do his IV. He isn't allowed to eat for at least an hour before in case they have to give him any nitrous.

Our immunologist called in an Rx of Syner@ for us. Our first time using them will be this coming Wednesday. It's basically a lidocaine/tetracaine patch that we will put on as we are leaving the house. It needs about 45 minutes to take full effect. Fingers crossed this helps reduce some, if not all, of the pain they experience during the IV process.

Last month, I spoke to the immunologist [that we see each month for IVIG] about switching to sub-q. I knew that we would have to wait because it was Pierce's first infusion but I wanted to know more about and what his thoughts were regarding it. He thinks it's a very reasonable option for both of the boys. He prefers to do in-hospital infusions at first just to see their reaction to the immunoglobulin and to have that one-on-one time with the parents. He also thinks that we will be able to find a suitable area for the sub-q infusions somewhere in the boys' flank area. As much as I don't want to me the "mean guy" having to stick my kids, I will gladly welcome it because it's a teeny needle and we get to do it in the comfort of our own home. Basically, we insert a thin needle, under the skin/into fatty tissue (sub-cutaneous), tape it down and begin the infusion process. It will take about 1.5 hours and we will do it once a week. The pump is similar to an insulin pump but a little bit larger. Home health would come in and teach us what to do, etc. The side effects are much less with sub-q because they are receiving a smaller amount of it each week. The purpose of any immunoglobulin infusion is to administer enough into the body to last for "x" amount of time. In the hospital, they are given a 4 week dose... at home, they would be given a 1 week dose.... so we may be able to completely eliminate the migraines/headaches! I plan on bringing it up again after our infusion this coming Wednesday. I would love to be able to start this route in June. The other good thing is that if sub-q doesn't work out, we can switch back to in-hospital infusions without it messing anything up! I would say expect an update next week but I make no promises because my life has the ability to go from very calm to insane in a matter of seconds... but for now, here are some pictures chronicling our infusions.

IVIG #1

IVIG #2

IVIG #3

IVIG #4 / IVIG #1

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 :)