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 :)

Updates

I clearly suck at blogging - I always want to write something. And I write these elaborate posts in my head but I never have time to type them out...

Brice is doing very well with his IVIG. His first infusion was January 25, 2012. Of course, he presented with a left ear infection that morning but overall, the infusion went very smoothly the day of. The following day, wasn't so great. He went to school and I was called around 10:45am because he was crying and had a headache... a migraine. My poor baby screamed the entire 10 minute drive home... then for the next 25 minutes... in addition to wretching (because of his Nissen he can't vomit). I gave him a dose of Motrin, turned all the lights out in the living room, closed the blinds and he passed out. He woke up feeling much better after the Motrin and his nap.

His second infusion was on February 22, 2012. Attempting to do his IV was a VERY traumatic event for him, myself and the nurses involved. I laid on the bed next to him with my legs wrapped around his in a death grip form (because the kid is strong) and each of my arms held one of his arms. From time-to-time I had to pin his chest down with my own. Nurse #1 held his head... Nurse #2 held/stabilized his arm... Nurse #3 did his IV. All the while Brice is screaming at the top of his little lungs - he would also insert the following phrases between long screams: "Get me out of here," "I want to leave this place," "I want to go home. I don't want an IB (because he can't say his V's)" etc. It was TRAUMATIC to say the least. I think it took around 20ish minutes and he and I were both drenched in sweat. His infusion went smoothly and no migraine the following day!

Pierce is fully potty trained! I don't remember the exact date (but I will reference FB for the date for his baby book) - but some time in early-mid January! We used the same method we used with Brice - which really wasn't a method - just child-led but once we said goodbye to diapers, that was it - no diapers or pull-ups while sleeping, etc. And it worked! And while it feels liberating to be freed from giving a ton of money over to Pampers each month, I also miss the days of just changing a diaper. Seriously, y'all... having to get up 5 times during dinner because the kid needs to potty and can't wait is it a bit tiring ;) and I am a germophobic, so the whole having to be in a public restroom so frequently gives me the heebie jeebies. And it's bittersweet - my baby isn't really a baby any more...

Brice had a very difficult time transitioning back to school post-Nissen. I think it took about 6-8 times (remember, he only goes on Tues & Thur) before he wasn't freaking out every time I left his classroom door. But he appears to have finally re-adjusted.

Brice no longer sees GI!! His Nissen was one of the BEST decisions we've made medically for him. He is no longer in pain. He is HAPPY! He no longer takes Nexium. He is eating foods that he had previously given up. Thank goodness for that surgery!!

Pierce had a genetics follow-up yesterday with our main genetics doc. He is VERY pleased with Pierce's progress developmentally. Pierce is still a peanut - weighing in at 11.5kg and 85.4cm tall. I asked if it was possible for Brice to have a very mild mito disorder as well given his health history (minus the gross motor developmental delay) and since they both are immunodeficient. We spoke more in depth about some of the ways to acquire genetically linked disorders/diseases. 1) Recessive - both George and I are carriers of the gene but we don't have the disorder but we passed it along to our children - he went into more depth mosaic (not effecting every cell) etc. 2) X-linked - maternally linked -- women are XX so they are not affected and men are XY so they are affected or 3) abnormal mitochondrial DNA - in what's considered normal, biologically-speaking, a person has 46 chromosomes (23 from mom and 23 from dad). But we all have "extra DNA," so to speak, inherited directly from the mother - called mitochondrial DNA. Both sperm and ovum have mitochondrial DNA, but the ova destroys the mito DNA in the sperm and only the maternal mito DNA is passed along to offspring.

Pierce had more bloodwork yesterday to look at his mitochondrial DNA. There are still SO many unknowns with mitochondrial disorders (just like the immunology field, both are rapidly expanding every day - plus they are soo much more under-funded than other fields like cardiology, etc). The mito DNA bloodwork will look at 1200 different things along with 12 (or is it 15?) transverse RNA sequences.... it could come back with various abnormalities or low levels of this, that or the other... but they don't know what it all means. They only thing that they are sure of are 37 different pathogenic mutations. If he has one of those 37, we will get a confirmed mito diagnosis (if you remember, he has been clinically diagnosed because of his healthy history, symptoms, abnormal organic acids/ketosis/acidosis and abnormal yet inconclusive muscle biopsy). If he was one of those 37, I will be tested next. If I am found to have any low levels, then they likely won't even have to test Brice because he will have it too - as a matter of fact, any of my offspring would be. The difference in asymptomatic vs mildly to highly symptomatic happens while the zygote is forming. It all depends on how many abnormal mitochondrion are transferred during that process. I know deep stuff - and I may have messed a bit up in translation but that's what I took in yesterday :)

Pierce had an immunology follow-up this morning. Because Pierce and Brice are following the same exact patterns immunologically, we are just putting off the inevitable with waiting on IVIG. So the decision was made today to start him on IVIG. They have to begin his paperwork and get insurance approval, which as of stated before can be a lengthy process. The game plan is for Pierce's 1st cycle to take place with Brice's 4th cycle (in April). Thankfully (maybe/maybe not) I will be able to take the boys on the same day for their infusions. The only thing that makes a bit nervous is trying to manage both while getting their IVs. I know they won't be done simultaneously but feeling like I can comfort both of them is a concern to me. We also briefly discussed starting Brice on home infusions this coming summer. He needs to do at least 2 more infusions in the hospital. If we switched to home infusions, it would be done sub-q. Home health would come in and teach us how to administer everything. We would do his infusions once a week and they would take about 2 hours to infuse. George is a bit nervous with this option because we won't be in the hospital if something were to go wrong. If it doesn't work out, then we will just go back to monthly admits for infusions.

Pierce had a sleep study in January. He has mild sleep apnea. They've recommended airway assessment (to check for an anatomical obstruction but our primary ENT wants to hold off on a sleep endoscopy until after our next sleep study (in June). He also stated that because of the underlying mitochondrial disorder, he likely won't do a T&A. He thinks that a T&A would be an unnecessary surgery because his apnea could be related to a muscle tone issue not an obstruction. If Pierce's apnea index score gets worse, the ENT wants to a CPAP trial. I mentioned all of this to our geneticist yesterday and he's not really on board with the idea of CPAP without having done a T&A first. He thinks a T&A would he helpful so that Pierce has more room, so to speak, for air to travel. He would like for us to get a second opinion - we are working on scheduling one.

Whew... I think that's it... one of these day, maybe, I will try to blog about more exciting, non-medical stuff...

Inside of...

... less than two weeks...

Mr. Brice has endured the following:

four IV's
two OR visits
two intubations/extubations for anesthesia

two ER visits

two bouts with dehydration
several bags of fluid

one colonoscopy

one upper endoscopy

one tonsillectomy

one adenoidectomy

one nasal endoscopy

one bronchoscopy

one laryngoscopy

lots of Zofran, Motrin and pain meds

and even more love, hugs, kisses and snuggles

On Thursday, October 13, Brice had the colonscopy & upper endoscopy (with a duodenal aspirate). His GI scheduled these procedures because of some belly/poop issues involving blood. Thankfully, upon visual (endoscopic) evaluation, everything looks as it should. We will have the biopsy and aspirate results back on Monday. Everything seemed okay. We took him to Chick Fil A for lunch and Target for a new toy. He played with Pierce and even went to his cousin's 7th birthday party. And then 8:30pm hit and HOLY PUKEFEST FOLKS... always a joy having lots of vomit, the consistency of pudding, on your shoulder, down your shirt, into your bra... lovely, just lovely (and yes, you can thank me later for that stomach-turning visual... and thank me even more for not giving you a far worse visual). And he continued to vomit until the poor kid was vomiting bile... 8 times inside of 2 hours. I took him into the ER. They ran bloodwork, started an IV, took an abdominal x-ray and administered Zofran (I swear, this drug is like a miracle worker - maybe I should write their manufacturer a letter - it's saved my life more times than I can think of... well maybe not my life, because that seems a tad extreme, but my sanity). His bloodwork showed dehydration and the x-ray was consistent with a person having a colonoscopy (not sure what that means). The doctors concurred (ER and GI on call) that the vomiting was likely due to the procedures.... or so we thought...

On Saturday, we took the boys to the pumpkin patch at Brice's school. Instead of carving their pumpkins, we decided they could paint them. So imagine this... we're en route to Target... to purchase said paint... Pierce starts coughing... I ask if my little buddy is okay... he looks confused... and then it happens... PROJECTILE VOMIT... undigested hotdog chunks and all (oh yes, people, you're getting another visual because I had the pleasure of cleaning it up... you're welcome). We went to a Halloween party at our neighbor's home... and Pierce puked again... the next morning, he puked again... all over the couch. Seriously, I don't know if I've ever experienced that much vomit in my life - and I've experienced a lot from other people (hey, it was our younger 20s - no kids, lots of time to experience all that NOLA has to offer). But I digress... obviously, a virus thought out home was a lovely place to visit.

And then Monday happened... that evening the virus decided to slam both George & I straight into the floor. I thought there was some unwritten rule somewhere that both parents could never, should never, ever be sick simultaneously... our ship was slowly sinking... it was a rough 24 hours. [total side note: George puked in the kitchen sink - no worries it's stainless and I scrubbed it - but the noise that it made scared the bejeezum out of Pierce - there I sat, on the couch, with one screaming kid, the other one crying, trying my hardest not to puke myself... oh the memories]

Fast forward a couple days... Wednesday, October 19, Brice had the T&A and other scopes. The doctor took a nasal biopsy to look at the cilia (for a few reasons - will discuss once we get results). His adenoids were VERY bumpy/rocky looking - consistent with being chronically infected/inflammed. He has bilateral nodules on his larynx and a broken blood vessel. The nodules are a tad confusing because those usually are a result of chronic vocal abuse. ENT told us to mention it to ST but even she was confused by it - especially since he doesn't have a raspy/hoarse voice (common with nodules).

Of course, in true Brice fashion, he wouldn't take his pain medicine by mouth. The (less than brilliant) recovery nurse suggested that he take a 30-45 minute nap and then maybe he would be willing... ummm no. I should have asked where I could send her a thank you note. Because of that and us having to wait 45ish minutes for an IV dose of morphine, the poor kid's pain was out of control. The morphine dose had NO effect on him. We had to hold him down and try to force his other pain med down orally. He slept on and off for hours... refused to eat or drink. At 3:30pm, our nurse said that if he didn't turn the corner within the hour, he would be moved to the floor because the short stay unit closed at 6pm. George and I took Brice on a walk and made a game out of drinking (you have to take a sip when we reach the window/door/hallway)... and it worked! He consumed 4oz of apple juice and 2 oz of ice cream - we were discharged exactly one hour later!! Brice did very well Wednesday evening - he tried to eat a mini powdered donut and a piece of pizza. Thursday was a decent day until 3pm. Friday morning, we thought we were going to have to head back to the hospital. My older sister saved the day with a smoothie - I was able to sneak his meds into the smoothie and life seemed okay. Saturday was MISERABLE - we reluctantly landed in the ER around 2:30pm.

When the nurse was doing his IV, she mentioned that with the way he presented and the fact that he was post-op, he was going to be admitted. The doctor was FABULOUS and it didn't hurt that she was the momma of a redhead - she could totally commiserate with the strong will they have! He was given a bolus, steroids, morphine and then they started a dextrose drip. He had a small bout with vomiting (well it came up and he forced it back down) so they give him some Zofran. Around 6pm, she and I discussed our options... 1) she could admit him or 2) we could stay another hour for a little more in fluids and another morphine dose. We decided to go with option 2 because it's always much nicer to sleep in your own bed on your own couch.

Today was another rough day... no medicine and nothing by mouth. We told him that we would go to Chuck E Cheese if he would drink something (you know we were desperate!). We bought him a special cup while there and he took a few sips. When we got home, he drank a small bit more - maybe a total of 2 ounces. Around 5pm, we were preparing to head back into the ER and this time, knowingly, to be admitted. But somehow, I was FINALLY able to convince him to drink something... and he took in 6 ounces!! He still isn't eating and as I type this, he no longer wants to drink, but hey, we're seeing minor progress. Hopefully it continues!

Welcome Lung Season

So Brice had croup on Monday. He was fever free on Wednesday and Thursday. We sent him to school yesterday, but an hour after I dropped him off, his teacher called and said that he was crying a lot. When I picked him up she stated that he was not acting like himself. Last night, his cough (which sounded less barky) was much more frequent. He would have moments in between coughs where he was gasping for breath. He couldn't sleep and was very miserable. We gave him a breathing treatment and eventually he was able to fall asleep.

First thing this morning, he said "mom, I need to go to the doctor." Our ST came at 8am and I scheduled a pedi appointment for 1:30pm. He started feeling warm so I took his temp --- 101.3* --- after taking his temp, ST stated that Pierce felt a little warm --- his temp was 100.7* --- yay ;(

The pedi examined Brice and spent a good bit of time listening to his chest... his bout with croup moved into this chest. He now has bronchitis. She could hear crackling sounds throughout his lungs but no focal spot. She gave him a shot in the hopes that if this is a pneumonia trying to occur, maybe it will won't happen. He also has an ear infection (right ear) and is on azithromycin for 5 days. While there, I mentioned that Pierce had a fever this morning and I could hear him coughing during his nap. She listened to his chest and examined him --- he also has bronchitis and is on azithromycin for 5 days. If Brice gets any worse over the weekend, she said to take him into the ER and if he isn't any better by Monday, she is ordering a chest x-ray.

I spoke with our immunologist about our appointment. We are just to keep track of everything. For now, we are still scheduled to see them in December. If infections start happening more frequently, we will be seen sooner. IVIG talks happened (very briefly mentioned but still) and possible prophylactic antibiotics again. Thankfully, if we do wind up on the ER, she is on call this weekend!

Here is to hoping my boys feel better quickly - especially Brice because he is FLAT MISERABLE. It breaks my heart that I can't do anything more to make him feel any better. I am really hoping that he makes some progress over night! I am also hoping that lung season is a little less eventful!

Have a great weekend!

all over the place...

Blah... I suckity-suck at blogging again... I mean, I've pulled out the laptop several times to blog but then something takes away my attention... oh well...

Both boys look like they've responded to the Pneumovax23 vaccine:
BRICE


Pierce


We follow-up in December for more labwork - fingers crossed their levels remain stable! If not, IVIG talks will resume. But for now, we are loving the fact that things are looking positive!

A very cute little redhead that made me a mommy had a birthday on August 28 --- we had a blast! Brice's party will have a post of it's own :)

Speaking of Brice, he saw ENT today and is scheduled to have a tonsillectomy, adenoidectomy, bronchoscopy and laryngoscopy in mid-October. Our ENT said that Brice's history has way too much substantial evidence in support of the above surgeries/procedures, He is fairly confident that Brice stands to benefit quite a bit from having his tonsils removed. Given B's history with pneumonias, bronchiolitis, wheezing, croup, etc, he wants to scope his airway/lungs to make sure everything's ok. He doesn't anticipate seeing anything off per say but since Brice will be under general anesthesia, he said he may as well do it now.

We were in the ER last night with Brice... he has a pretty nasty case of croup :( Poor kiddo is in quite a bit of pain and his cough makes my lungs hurt. He started with a low fever (100*) on Sunday evening. I gave him a dose of Motrin to prep for a nighttime spike... 12:15am = 103*... 6:45am = 102.3*... 11:45am = 102*... 5:45pm = 102.3* --- between his Motrin doses, he would stay in the 101s --- in the ER, around 11pm, he was 103.8*. They did a chest x-ray (and included his neck in the field) --- no focal pneumonia (!) but it did show some viral patchiness in his lungs and steeples sign (consistent with croup). They administered a dose or OraPred (oral steroids) and gave an Rx for two more doses. Today was rough on him... any time he coughs, he cries. He sounds like he is wheezing but apparently it is stridor since croup is upper respiratory. He is hoarse but fever free as we speak :)

Pierce had a GI appt today - some other doctors on his case were marginally concerned about his lack of weight gain but his GI isn't. Pierce had a swallow study on August 23 --- it showed pooling in his valleculae - the good news is that he clears it with a swallow... the not so good news is that it puts him at a much higher risk for aspirating. The speech pathologist noted that he has inadequate tongue base retraction. Dr. A said this is unfortunately something he can't fix and that will never fix itself. Pierce will eventually learn to accommodate this issue on his own - he will probably eat slower, etc. We have a follow-up swallow study in November and Dr. A plans on repeating swallow studies every 3-4 months for awhile. For now, we are thickening and fortifying his feeds (5oz coconut milk + 1 scoop of Neocate, Jr + 1tbsp oatmeal). He is not allowed to drink out of straws as it presents liquid at a greater force of speed and really ups his chance of aspirating.

Overall, Pierce is doing VERY well. He climbs non-stop and has an amazing amount of energy for someone that is barely 24lbs and 32" tall! We are amazed at his ever-expanding vocabulary. Lately, he gives the BEST squeeze hugs and big open-mouthed wet, sloppy kisses!!

Brice started school --- another post of it's own --- he goes two days a week from 8:45-2:45 and L-O-V-E-S it!! I have days where I just stare at him and think "you're not a toddler anymore - you really are a little kid" - how has time gone by so quickly?! It's very bittersweet - I love the kid he is becoming but I dearly miss the baby/toddler he once was.

As crazy and chaotic as things are lately, I am really enjoying life. I am happy and learning how to fully embrace being perfectly imperfect. It's tough but I have a great group of guys in my life - from the daddy down to the littlest man... life is good.

stickers, tummies & blog changes

The boys (unexpectedly) had their blood work done today. They both were having their pneumococcal titers checked from their June 28th vaccination with Pneumovax23 and their memory b cell function tested. Brice was also having a food allergy panel done. They both did great... well Brice had a massive meltdown over having to wear a band-aid after the fact... and it only got worse when he was given the option of choosing stickers. OH.MY.GOODNESS. I was sweating by the time we walked out... he was so upset that he didn't realize he was saying no to the Toy Story stickers he ultimately wanted.... but then, he wanted the WHOLE roll. I explained to him that he could have a few and the rest had to stay for other kids having blood work too... while he is normally a great share, today, he had no desire to do so. He l.o.s.t. it... the woman that drew his blood said he could have the entire roll... to which I 110% refused. He was not being rewarded with an entire roll of stickers for not wanting to share and for having a meltdown over not wanting to share. Finally, he took one of each type on the roll and we left.

Speaking of food allergies... the boys have a new tummy doctor (aka GI specialist). As much as loved our GI doctor in Baton Rouge (and will forever be thankful to him for stabilizing Pierce as a teeny babe), I just couldn't make the 1+ hour trip any longer. So back to the new tummy doctor... while I can't say that I was overly impressed, I can say that I think I am going like having him on our team.

Dr. K had lots of things to say about Brice and I honestly cannot remember each one because holy gees did my kiddos bring out the crazy for the first half of our appointment. These are the things that I do remember: (1) We're reducing Brice's Nexium intake from 20mg twice daily to 20mg once daily; (2) He's curious if some of Brice's belly issues could be related to food allergies or sensitivities; (3) He reviewed Brice's EGD biopdy report from March --- Brice did not have any evidence of esophagitis but that could have been masked by him being on Prevacid --- Brice had duodenitis; (4) If Brice's belly pain gets worse (especially to the point we were at a few months ago) he wants to do another EGD and a 24 hour pH probe study; (5) the duodenitis could be the culprit for his belly pain... actually visceral hypersensitivity could be. Dr. K said that once the inflammation from the duodenitis subsided, the nerve endings may not have properly healed and could be hyper-sensitive now... so anything moving through the duodenum (part of your intestines), in essence, could cause pain. If this were the case and if his pain were to get worse and if it were to start interfering with his day-to-day activity, they would treat the visceral hypersensitivity. Dr. K did give me a fair warning that the treatment for it is "old-school" anti-depression medications. He wants to see Brice again in two months.

As far as Pierce goes, he is in a pretty stable place GI-wise. I explained to Dr. K that pulmonology wanted us to get another swallow study and I really didn't think Pierce needed to undergo another swallow study (aka pharyngogram or MBSS - modified Barium Swallow Study or VFSS - Video Fluoroscopic Swallow Study) My two reasons are: (1) I am just not convinced that his recent choking episodes were related to anything mechanical - I am of the opinion it's more of an ENT issue (while he was sick) and (2) my poor kiddo has been exposed to SO much radiation in his short 19 months of life that if we can avoid it we need to. While he respected my desire to avoid the MBSS he thinks it's necessary. Due to Pierce's mito diagnosis, he wants a baseline study, if in the event we were to see issues arise in the future. The hospital will be calling me at some point this week to schedule the MBSS and we follow-up with Dr. K in two months.

And... I've made a few more changes to my blog! I moved my blog roll from the sidebar:

to a page at the top (We like to read):

and I also added a few buttons as well :)

I am off to stuff and address 80 birthday invitations for my ALMOST 3 year old redhead... have a great night!

one more kiss!

I need to start blogging on a more consistent basis... not for you (sorry, just being honest) but for me. I initially wanted this blog to be a place for me to remember those moments... the ones that I want to remember for forever. It's turned into more of a place for me to document my children's health issues (which has come in handy more times than not) and from time-to-time, I get to document about those moments. My hope is to start blogging about anything and everything - whether it's about Brice's first day in Pre-K3 (coming SO soon!! September 6th to be exact!), Pierce CONSTANTLY wanting to "pee-pee", a picture of my boy's artistic renderings... on my walls, their medical issues or just my thoughts - I need to challenge myself to my blogging at least three times a week - hold me to it, people, hold me to it!

Today is my mom's birthday! We are celebrating with dinner and a cake here at our home this evening. Both the boys called her this morning to wish her a "Happy Birthday" but she didn't answer! So what did this momma do... pulled out the (cell-phone) camera of course and posted videos to Facebook (seriously, THANK YOU technology!).

Aren't they cute?

And my latest update on the boys... it looks like we're heading in the direction of IVIG therapy for both of them. Pierce has been living on antibiotics for weeks now (he did 12 days of Omnicef and is taking 3 weeks of Augmentin). Brice is about to start antibiotics as well for a sinus infection.

This is what our daily meds list looks like (minus whatever antibiotic Brice is about to start...)

They are both having blood work on Monday, August 2, to check their titer levels (post Pneumovax23) and to check their memory B cell functioning. The latter test is experimental but it can indicate if they have difficulty making cells necessary to recognize and fight off a microbe on repeat exposure. If Pierce's titers come back low, that will be enough to justify (to insurance - they need certain labs to approve IVIG since it is extremely costly) starting his IVIG course for 18-24 months. If Brice's titers come back low OR within an acceptable range but his memory B cell function is low, he will begin IVIG as well.

At our hospital, IVIG therapy is administered once a month, on Wednesdays only. It is a short stay admission to the hospital (typically about a half day so long as the infusion is uneventful). An IV will be placed and the infusion typically takes a couple of hours. Every 6 months, they will have blood work to look at their levels - IgG but specifically IgM and IgA. If at any point during treatment, their IgM or IgA level starts to decline they will be given the definitive diagnosis of CVID (Common Variable ImmunoDeficiency) or hypogammaglobulinemia. If that doesn't happen, once the course of IVIG is completed, they will have blood work 6 months after to check their levels. If everything looks good, they will still be followed because they are technically not out of the woods. At any point, their levels could drop off which would mean true CVID (as opposed to transient of childhood). If they do have CVID, they will get IVIG for the balance of their lives... that statement is a bit daunting.

Deep down inside, I strongly feel as though they will both be candidates for IVIG Therapy. I am thankful to know that we will most likely have an answer in about 3 weeks (versus in September!). I remain hopeful that if they do go in this direction, that it's just transient and not true CVID. Only time will tell... and for some reason, I just can't buy into the quote of "this too shall pass" because, what if it doesn't.

It's not fair for them. I feel like my body failed them. I know in reality that I haven't failed them. But if they have this... at what point during their development did something go wrong? I've felt like this for quite some time with Pierce's mitochondrial diagnosis. And while he is on the mild end of the spectrum, we have no assurity that it will always be like this. It can change at any moment... and that scares me. I feel like I am constantly questioning every off thing he does - Why is his appetite suppressed? Is he falling more than usual for a toddler? For himself? Why is he sleeping more than usual? Are any of these symptomatic of regression? And  now, the thought of him having a compromised immune system on top of it... that just sucks. It's bad enough that he will be on a list of medicines for the rest of his life, but to think he may need IVIG forever too... I despise that too. But then I look at them... and I see their happy, smiling faces. I see them playing with one another. I see Pierce becoming more of a toddler daily and Brice becoming less of one. I see so much love and happiness. And that makes every single moment I worry and stress so completely worth it. I would be lying if I said I wished things, medically speaking, weren't different. It's a lot to take in but I still get to fulfill requests of "one more kiss " (actually 8 more kisses) and "one BIG kiss"... I still get to kiss them good night, every night And those moments are some of the moments I want to remember forever.

Updates

Sorry for not updating lately... to be honest, I just haven't felt like it. I feel like every time I post something it's like throwing a rock onto the not-so-good news side of my proverbial life scale... but I've come to accept that it is just the way it is right now. I also realized that I need to blog before I forget. My past posts have come in handy in terms of remembering 6+ months ago when I've needed to update a new doctor.

As much as the Type A in me wants to be all organized, this post will be somewhat all over the place...

June 13 --- Pierce's first appointment with Dr. Levine at Children's Hospital (New Orleans). I was very impressed and equally as happy to have him on our team of doctors. He seems extremely knowledgeable. I really liked when he said if he feels strongly that Pierce's health is going in X direction, he will let us know so that we can make an informed decision in a non-stressful environment (vs landing in the ER, being admitted and having to make a big decision for his health). Pierce's lungs looked good - no evidence of any lung disease. He gave us a prescription for Xopenex to use prn for wheezing episodes/night-time coughing. We follow-up with him in September - aka "lung season" - and if we ever feel as though a visit is warranted, his doors are open. He also wanted Pierce to undergo another modified barium swallow study due to choking and aspirating episodes. We scheduled a GI appointment with Dr. Keith at Children's for late July. {{After I typed this, I felt as though I've previously blogged about this but I can't remember}}


June 24 --- Pierce's 18 month well-visit; he was finally put on antibiotics - 12 days of Omnicef. His sinus infection started to clear up and magically his choking episodes started going away --- my mommy brain is telling me it's not truly a mechanical issue. I think it's either (a) his adenoids are/were swollen or (b) when he's sick he becomes weak (normal for anyone) but maybe it's affecting his suck/swallow/breathe function. I plan on discussing my theories with Dr. Keith.

June 28 --- Pierce received the Pneumovax23 Valent vaccine; Brice saw the same immunologist. We discussed his history and his previous immunology visit in September 2009. Brice had bloodwork to look at his immunoglobulin levels and titers. Brice also received the Pneumovax23 Valent vaccine.

July 5-8 --- Pierce started with fever the day after he completed a 12-day course of Omnicef

July 11 --- Pierce started with fever (again)

July 13 --- Pierce's fever climbed to over 104 with Motrin and Tylenol rotation getting it into the 103s. His heart rate was in the 140-150s and his respiratory rate was in the low 60s. We spoke with the pediatrician on call and she suggested that we take him into the ER given his medical history of mito diseaseand possible hypogammaglobulinemia. So off to Children's we went... 4 hours later + 1 chest x-ray + blood cultures = possibly something bacterial starting to occur but most likely viral

July 15 --- (today) Pierce STILL has fever. I spoke with his immunologist and she suggested that we go into the pedi to rule out strep and ear infections. Either way, she wants him on a 3 week course of antibiotics. She mentioned that it looks like he will ultimately end up needing IVIG therapy. We really won't know more until early September. Both the boys have repeat bloodwork to check their titers on August 23. It takes approximately 2 weeks to receive those results back. And speaking of titers, I received Brice's pneumococcal titers back today... {drum roll please}

They are non-existent --- seriously, what the heck?! His IgG3 level was low and his IGE level was high - allergy testing will most probably be tacked onto his August 23 follow-up.

So it appears as though both of my children do not respond to protein-conjugated vaccines. Fingers crossed that their immune systems will mount a lovely response to Pneumovax23. Come on August 23... and more importantly, come on 2 weeks post August 23.

I really just want answers. If one or both need IVIG therapy, I just want to get to that point. I just want something concrete... something that doesn't involve such lengthy wait times.

That's all for now because Pierce has a doctor appointment in about an hour...