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 :)

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...

It's been awhile, yes?

It would take me hours upon hours of typing to fill you in on the last few months... quite frankly, I just don't have the time - I am racing against the clock as it is with nap time about to end. Here is the Clif's Notes version:

• Brice had a Nissen Fundoplication on December 27. The surgery took less time than expected. His recovery was AMAZING! We were initially told to prepare for 4-7 days in the hospital post-op. Guess who was released at a whopping 48 hours post-op --- I was shocked with how well he did. But I do have to admit that my baby broke my heart when we were initially in recovery. He was crying saying he wanted his mama and I kept reassuring him that I was holding him and I was his mommy, but it didn't matter. He was very out of it. The first 24 hours were rough because he was NPO and had an NG tube suctioning his stomach out. A small dose of Ativ@n, some IV M0rphine and Tor@dol took us through those first 24 hours beautifully. Once the boy was able to get some food in his belly, life was good again. He and I played in the play room and took a few walks. The LSU Golden Girls visited us and even gave Brice a gift! He actually received a few gifts while we were admitted from various groups visiting the hospital. On Day 2, once he proved he could tolerate breakfast and lunch, we were discharged! He wasn't overly concerned about his incision sites except during bath time. I received very strict orders, from Brice, to not touch his "boo-boos" - which I had no intentions of! Tomorrow is his post-op visit. You know what the best part is - I haven't hear "mommy my tummy hurts" in almost 2 weeks! After hearing it multiple times a day for 1 entire year, it is amazing what this surgery did for him!! Dr. Hansbrough was an amazing surgeon, who completed his surgery in an hour less than we expected and her bedside manner is superb. Again, Our Lady of the Lake, completely exceeded my expectations, treated my sweet boy like a prince and overall, did a fantastic job!
• Christmas was SO SO SO much fun - but I will do another post on that :) Being able to experience the magic of the Christmas season through our children is so heart-warming. I LOVED it this year! 
• While I was in the hospital with Brice, we received some not so good news from their immunologist... Pierce's pneumococcal titers are non-existent again. He went from having 2 out of 14 (May 2011) to 11 out of 14 (August 2011) - this was after receiving the Pneumovax23 vaccine.... right back down to 2 out of 14 (December 2011). Brice's titers decreased as well but not as drastically - Brice had 3 out of 14 (June 2011) and went up to 13 out of 14 (August 2011) - post Pneumovax23 and went down to 8 out of 14 (December 2011). Brice has also had frequent infections since the Pneumovax vaccine - croup, bronchitis, right ear infection, croup and a sinus infection. Pierce has (thankfully) stayed relatively healthy - he's only had bronchitis. Oh, well, they both had Hand Foot & Mouth in early November but that doesn't really count towards the infections that matter. so................. where do we go from here....

They've both been labeled as immunodeficient. Pierce is called IgG3 Deficiency with Specific Antibody Deficiency and Brice is called IgG3 Deficiency with probable poorly functioning antibodies. They've both displayed really poor Memory B cell function, too.

Brice will be starting IVIG infusion therapy at Children's sometime in February (early-mid). We have to wait on insurance approval and then a spot to open up (or eventually, he'll just get squeezed into the schedule). He will be admitted to Children's once a month for about a half day where he will receive donor antibodies (via blood product) through an IV. This treatment will definitely last for 12 months. If his immune system seems to be responding, they will continue for another 12 months. If he isn't responding, I am unsure of what the course of action is. Either way, he will come off of the IVIG treatment and have bloodwork at 6 months after the final infusion. By then, his immune system will have cleansed itself of all donor antibodies and should be producing antibodies on it's own. If his levels are down again, they will resume IVIG therapy with no true end date in mind. Since Pierce has stayed relatively healthy on his own (yay!), they will not start IVIG on him just yet. If he starts getting sick more frequently, they will begin treatment on him as well. We will not know if this is transient (something they will outgrow) until they are 6 years old. They will be followed every 4-6 months, while not on IVIG, until then to see how their immune systems are/are not responding.

That's kind of it for now - just in a wait and see mode until we can begin IVIG... More details to come as I get them

Appointments Galore!

So I 110% dropped the ball this morning... I forgot about Pierce's neurology appointment... and it was written in my planner :( I am a bit frazzled at the moment and I am sick on top of it all. I think I need to invest in something like this:

source

 We had a nice break from running the doctor circuit but it's about to start back up --- here is a glimpse:

Fri Sept 30 --- 9:30 --- Pulmonology (Pierce)
Mon Oct 3 --- 9:00 --- Pediatrician (Brice - 3 year well-visit {super late!})
Wed Oct 12 --- 9:15 --- GI (Brice)
Wed Oct 12 --- 11:00 --- Neurology (Pierce)
Tues Oct 18 --- 9:15 --- Orthopedics (Pierce)
Wed Oct 19 --- Tosillectomy, Adenoidectomy, Bronchoscopy, Laryngoscopy (Brice)
Mon Oct 31 --- 9:45 --- ENT (Pierce)
Wed Nov 16 --- 1:45 --- ENT (Brice - post.op)
Thur Nov 17 --- 10:30 --- Swallow Study (Pierce)
Tues Dec 13 --- 10:00 --- Immunology (Brice & Pierce)
Mon Dec 19 --- 10:30 --- GI (Pierce)
Tues Dec 20 --- 8:30 --- Genetics (Pierce & Brice)
Tues Dec 20 --- 3:30 --- Opthamology (Pierce)
(And Pierce will have his 2 year well-visit at some point after December 18)

And in between all of those doctor appointments/procedures/surgeries, we have several birthday parties, 1 wedding, a cute little red-head's Pumpkin Palooza at school, a USMC Boot Camp graduation, an engagement party, some Black Friday shopping, a much-needed trip to Baltimore to kiss and love on a sweet new(-ish) baby, a wild little blonde's 2nd birthday and the holidays! Fingers crossed that I make it to January 1, 2012 all in one piece ;)

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.

Answers on Thursday {hopefully}

We followed up with immunology today... Before I dive into the specifics, I want to explain a few things. I have had some friends and family tell me that they are so lost in the medical lingo but they try to keep up with the boys. I am going to try and break it down for you :)

• Immunodeficiency - as with many other things, can be of varying degrees, can be short-term (transient) or long-term (chronic, life-long)... without getting too specific, basically on some level, their immune system appears to not be working like it should be. 
• Their immune systems did not respond to one of the normal childhood vaccines, PCV-7 specifically. This vaccination offers protection against ear infections, sinusitis, pneumonia, meningitis and blood infections. It is given in 4 doses - at 2 months, 4 months, 6 months and 12-15 months. Once you've completed the 4 dose series, you can receive PCV-13 (protection against an additional 6 strains of pneumococcal bacteria). According to immunology, after 3 doses, a child should have adequate protection. Pierce and Brice both received 4 doses of PCV-7 and Brice received 1 dose of PCV-13.
  They each had bloodwork to look at their titers (antibodies in the blood reflective of past exposure to an antigen - or something that the body did not recognize as being a part of itself). The test looked at 14 different pneumococcal strains (the most common strains). Pierce showed protection against 2/14 and Brice was 1/14. Even without the respective 4 and 5 vaccines they each received, their levels should have been higher given their past history with infections.
• IVIG - this is a type of therapy that can be used for many different health conditions, one of which is immunodeficiency. IVIG = Intravenous Immunoglobulin is basically IV infusion of antibodies. I explained the process a little more HERE

Okay, I hope that clarified things a little more :) Now onto updates ---

• Brice --- presently on a prophylactic dose of 250mg Amoxicillan once a day (for headaches, tender sinuses upon palpation , purple under eyes). His Memory B Cell function came back low (labs are in the car but I will post when I can). On a scale of 8-19, he was a 4 and his IgM memory was a 9 (scale of 10-19). If his titers come back low, he will start IVIG.
• Pierce --- presently on a prophylactic dose of 480mg Amoxicillan once a day (poor Pierce starts with fever no more than 2 days after he completes a course of antibiotics - previous courses include 10 days of Omnicef, 3 weeks of Augmentin). His Memory B Cell function came back low. On a scale of 8-19, he was a 2 and his IgM memory was a 4 (scale of 10-19). If his titers come back low, he will start IVIG.

About Memory B Cell functioning, as I previously mentioned,  it's experimental. It is something that Children's Immunology is doing research on. They have a general threshold set (8-19) but it is not age-specific (that is what they're still researching). Since memory b cell functioning is reliant upon previous exposure to a microbe, the theory is that it is going to be lower for younger individuals. But it's a catch 22 because both the boys have a rather extensive history of infection. Those results are not for diagnostic purposes but they will be used, with all of their other labs, infection history and clinical findings, as support for insurance authorization for IVIG.

Brice also had bloodwork to check for some of the more common food allergens. He showed a slight issue with wheat but not as a "true" allergy. Given his GI issues, the gameplan is to treat this empirically. We will remove wheat/gluten for a 3 week trial - if we see improvement with his stool and belly pains, we know it's real. If we don't, then we re-introduce wheat/gluten. His level could be the beginning of an issue or it could be nothing.

And because my memory stinks lately and I use my blog for referencing at doctor appointments...
Brice - 92.5cm ( 23rd% ) and 13.6kg ( 34th% )
Pierce - 80.6cm ( 19th% ) and 10.6kg ( 12th% )

Check back on Thursday for our update - have a Happy Tuesday!

I am in for it...

... why do you ask? Oh my boys are such a mess (figuratively and literally)... here are a few examples of words I've recently heard and actions I've recently witnessed:

(1) Brice wanted to go play in his room... of course, little brother wanted to do the same... Brice wanted nothing to do with that ---
Brice: You can't play in my room. Get out.
Pierce: starts crying
Mommy: Brice that isn't very nice.
Brice: attempting to close his door NO mom! Don't talk to me!
Mommy: Are you almost 3 or 16 and wait, don't you have a penis? I thought only girls were mouthy like this Brice if you can't speak nicely to mommy or share, you're going to sir in time-out until you're ready to do both
Brice: NO mom! I don't want to share! I don't want Pierce in my room! Stop talking to me.
Pierce: continues crying
Mommy: Brice, it's time to go sit in time-out...
Brice: Noooooo mom, I'm going to share and be nice. I don't want to sit in time-out.
Mommy: I am glad you want to share and be nice but you still have to go to time-out
Brice: crying and reluctantly walking to time-out
Pierce: tears stop, walks into Brice's room and happily plays

(2) I put Pierce down for his nap shortly before George came home for lunch. We could hear Pierce talking (loudly) and playing. I asked George to put Brice down for his nap while I checked on Pierce. Upon opening his door, I found him standing on the edge of his bed, diaper-less, watching himself pee onto the floor. Seriously. [side-note: he's really moving towards wanting to potty-train; he has a horrific yeast-related diaper rash that had him in so much pain Thursday afternoon, he could barely walk. I put him in the tub for some relief but before I put him in, he peed in the toilet! He said pee-pee, put the toilet seat on the toilet, climbed on the step-stool, I sat him down and he PEED! I was SO excited!]

(3) Yesterday (Friday), I went into get him up from his nap and oh poop... there was poop EVERYwhere. He took his diaper off (duh) and pooped - either on the floor and transferred it to the bed or vice versa. I was so grossed out. He had it all over his legs, the sheet, his bed, blanket and pillow. I took his shirt off and put him in the tub... cleaned his room... and snuggled on the couch with him afterward. [side-note: I may or may not have taken a picture of said disaster... and I may or may not have sent it to my husband saying "why me?"]

I think I have provided enough evidence as to why I am in for it...

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...

{fear}

[this is the post in which so many of you will see that I am really not as strong as you think I am]

This weekend, I read a post on Band Back Together and it was like a light bulb went off in my head... definitely a defining moment for me.

I spoke with my psychologist today and she confirmed what I already knew... I am suffering from PTSD, anxiety and panic attacks.

Here is where I attempt to become transparent... Any time Pierce sleeps longer than he should or later than he should, I internally freak out. Recently, Pierce slept until just after 7am (not normal for him). While most parents would gladly welcome an additional hour of sleep, I laid awake from 6am on.  By the time 7am rolled around, I was in a full blown panic attack - heart racing and pounding, feeling like my sternum would give way at any moment and my chest would rip open. I laid there thinking those thoughts... you see, after you've watched your child turn blue and stop breathing, after you've watched your child turn blue and go limp countless times, after you've watched your child choke on things he likely shouldn't... your thought process changes - at least for me it has. I lay there thinking "what if I open his door and he is blue"... "what if I open his door and he is dead" - (as I type these words, my heart is pounding). And while I've been reassured that because of what I've been through, those thoughts are normal (for the circumstance) - what I say next, isn't normal. It takes me minutes - at times a couple, sometimes almost 10 minutes - to gather enough strength and courage to open his door... because I have this image of my blue baby stuck in my head... I am afraid that when I open his door, that's what I will find. And that fear paralyzes me.

Tonight I told George that I needed to tell him something but I was afraid that he would think I was absolutely crazy (because really, how normal is that thought process?) I was so hesitant in sharing with him, but I reluctantly did. I started talking and explaining the fear that paralyzes me... that puts me into a panic attack... I began to say "I don't want to open his door because what-if... what-if" as I was biting my lower lip because I didn't want to cry... I just flat couldn't get the words out... he looked at me and said "you don't need to say anything else, I feel the same way. I have the same fear." And as good (I know, maybe not the right choice of wording) as it felt to be validated, it felt that bad, knowing that he feels the same way - because it's a horrible way to feel. He explained that's why he checks on Pierce 3, 5, 10 times a night (we both check on the boys several times a night). Now with that said, his fear isn't as extensive as mine. He doesn't become so scared that he won't open the door... but he still fears the worst. He also said that it's becoming less of a big concern and more of a smaller one... his biggest worry with Pierce as of late, is him choking - we've been dealing with it much more than we'd like - let's just say, performing the Heimlich 3 times in 7 days aren't the greatest of odds.

I hugged George. I thanked him for not thinking I was crazy. My heart was hurting that he carries the same fear as I do. I thanked God for truly giving me my perfect soul mate.

I hate this fear. I hate the unknown - I am such a control freak and I feel as though know I have such little control over all of this... and I don't like it... not one tiny bit. But I do as I have to... I put my brave face on. During our crisis moments, I am grace under fire. I run on adrenaline. I do what I have to take care of my baby... to advocate on his behalf. And it's tough because to look at him, Pierce looks like your normal 17 month old - his insides just don't match his outsides. But I have my moments, where it all breaks down... and when fear creeps in like a thief in the night... I try not to let it steal anything, but clearly, sometimes it does.

bumper cars - missed memo?

I apparently missed the memo that I would be involved in a game of bumpers cars yesterday...

Pierce had an appointment with pulmonology at Children's. He had a clear (!) chest x-ray with NO sign of lung disease. He still has a residual cough (mainly at night) from his upper respiratory infection. Dr. L thinks it's one of two things: (1) he's had multiple lung infections and he wheezes from time to time (especially when he's sick) - so the night-time cough could be an asthma-like cough or (2) with viral (respiratory) infections you can experience asthma like symptoms for 4-5 weeks after the infection subsides... so for now, he will receive a breathing treatment at night, before bed. Dr. L wants to see us back in September when "lung season" begins. I am imagining that we will be on a 2-4 times a year checkup pattern with him. He stated that if he ever sees a problem cropping up that he will let us know how he thinks it will play out ahead of time so that we can make a decision in a non-stressful environment. He reminded me (in a kind, not fear-mongering way) of the down sides of mitochondrial disease (ie G-tubes, serious breathing difficulties, etc). He seems to be very intelligent (you can tell he knows that he is but he reels himself in avoiding arrogance) - he was the Director of ICU at Children's for 25+ years. Long story short - I REALLY like him and I am glad to have him on Pierce's team.

On our way home, literally about 1 mile from our house, we were rear-ended --- seriously, being in a car accident with my child had always ranked in my top 5 mommy fears. With this accident, I knew it was going to happen when I looked in my rear view mirror. Poor Pierce was sound asleep - the scream my baby let out on impact was horrifying. When I knew the impact was inevitable, I kept thinking "I am going to get out of this car and give that person a piece of my mind... how could he hit me? And with my baby in the car? What part of stopping couldn't he understand?!" --- and after Pierce screamed all I thought was "How quickly can I get out of my seatbelt and get him out of his carseat?" It took a good 10 minutes to console him and it broke this mama's heart. A state trooper came out and wrote a report.

Pierce seems to be having some anxiety with getting into his carseat. It has happened four times now - he freaks out and doesn't want to get into his seat. I am a little sore. I must have clenched my teeth because my jaw hurts in addition to my neck, shoulders and lower back.

I filed a claim this morning. At first the (at-fault) driver's insurance company did not want to replace both carseats but one simple statement changed their mind: "... that's fine. I would like a written statement from your company accepting full liability and responsibility in the even that Brice's carseat were to fail us in the future." Carseat safety is a huge thing to me - I refuse to take any risks with my children's lives.

At the end of the day, I am so thankful that our car was significantly damaged and that my baby and I were not seriously injured.

More Labs

[Before I go into the details of Pierce's labs, I want to welcome my new readers from Kelly's Korner --- and a special thank you to the moms who've reached out because they've been in my shoes before.]

I received a large portion of Pierce's most recent labs - we're still waiting on his CoEnzyme Q10 and Carnitine levels and Organic Acids (those are sent out of state).

Pierce had an upper respiratory infection that started 2.5 weeks ago but he has vastly improved since then. When the URI started he was hoarse, wheezing, sounded very junky, had tennis-ball green nasal discharge, a not-so-lovely cough and mid-grade fever that would come and go. On Memorial Day, I took him into the ER because he started sounding like a bullfrog while breathing - the ER doc heard lots of upper airway congestion but couldn't hear anything in his lower lungs. He said something could have been going on but because we were already doing 3-4 albuterol breathing treatments daily, he couldn't be certain.

He still has an occasional cough - nothing like what it once was or near as frequent - and we can still hear a bit of congestion when he's breathing. I was surprised when I saw his WBC (white blood cell) count...

(1st column = normal results --- 2nd column = abnormal/out of range results --- 3rd column = range)

His platelet count has been high on his last two sets of labs - in addition, his lymphocytes (lymphs) have been high and his neutrophils (segs) have been low  ---

6/7/2011

(1st column = normal results --- 2nd column = abnormal/out of range results --- 3rd column = range)

3/7/2011

(1st column = normal results --- 2nd column = abnormal/out of range results --- 3rd column = range)

Yesterday, I spoke with Dr. P (allergy/immunology). She informed me that platelets are an acute phase reactant - so a high level can indicate a reaction to infection or inflammation. Neutrophils guard against bacterial infections, and are found in the blood stream, which allows them to respond quickly to trauma and move to the site of infection or inflammation. A low neutrophil count can mean depleted or compromised immunity. Lymphocytes are T cells and B cells - they form part of the adaptive immune system, B cells create antibodies in blood which are used to kill foreign bodies, while T cells help in the activation of cells that will neutralize threats. Low neutrophils and high lymphocytes generally signal a viral infection - the elevated count is a result of the body's attempt to fight the infection. So something is definitely going on inside of him at the moment. The good thing is that his body is semi-responding the way it should - he just doesn't have the antibodies he should to fight off infections.

His neutrophils were low and lymphocytes were high in both March and June. His WBC count was normal in March and high in June. Dr. P plans on doing another CBC when she checks his titers after the Pneumovax23 vaccine. If his lymphocytes are still high, they will look deeper into it - whether it's his T-cells or B-cells, etc.

I also expressed my concern over the Pneumovax vaccine and his age (according to Merck, it's safety and effectiveness has not been established in the under 2 population). Dr. P reassured me. Early childhood vaccines are protein conjugated because that's what the immune system recognizes when it's still developing. Pneumovax is a polysaccharide vaccine - typically young children respond poorly to this. The hope is that since Pierce's immune system responded poorly to protein-conjugated vaccines that his body will mount a different (positive) response. I feel better knowing that he isn't going to have some sort of negative reaction to Pneumovax - the only negative reaction would be his immune system not responding to it and us having to move on to a different path.

For now, we are keeping things a bit more low-key until he receives the Pneumovax vaccine (even though it's not a guarantee then) - we are staying away from large crowds - especially lots of kids, any one sick or recently sick, etc. I am just so thankful that he hasn't been more sick than he has.

That's all for now - a mountain of laundry and two little crazies are calling for me :)

Have a wonderful weekend!

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.

{just breathe}

When it rains, it pours {at times}...

I am feeling very overwhelmed...

I know it's said to "count your blessings, not your problems" - but on some days, just thinking about that idea is tough...

As far as George, myself and the boys go, we're fine. But outside of that, my family (specifically my immediate family) could use all of the prayers, thoughts, wishes you have to offer. My heart is broken and heavy. For now, please respect our wishes that this stays a private matter.

Now onto other stuff...

Yesterday, I spoke with one of Pierce's doctors (new to his team of doctors) - I was concerned that some cold-like symptoms were a result of his allergy skin testing (performed on Wednesday). She said that they were not. She was also happy to hear from me because she was going to call me. They did some bloodwork on Pierce on Wednesday - to look at his immunoglobulin levels (basically his immune function) - IgG, IgA, IgM, IgE, IgD (if you've read my blog for any length of time, then this will sound slightly familiar as we've {kinda, sorta} traveled this path with Brice). For Pierce, his:

• IgE levels were low - this is a good thing. IgE is related to allergens. If your body meets a particular offender (that you're allergic to), it will build IgE antibodies in response to it. Even with this finding, Pierce is still labeled as "Milk Allergy."
• IgA levels were normal
• IgM levels were normal
• IgG levels were low (not a good thing) - about 20 points below normal. "IgG antibodies are found in all body fluids. They are the smallest but most common antibody (75% to 80%) of all the antibodies in the body. IgG antibodies are very important in fighting bacterial and viral infections. IgG antibodies are the only type of antibody that can cross the placenta in a pregnant woman to help protect her baby (fetus)." source

We may be dealing with an immunodeficiency, specifically an IgG Deficiency (with the subclass undefined at this moment). We're in a wait-and-see boat at the moment... we're waiting to see what his blood titers look like. She anticipates having those results around the week of June 6th. Depending on those results, we may begin treatment soon. If those results buy us some time and he were to get pneumonia, treatment would begin immediately. If those results buy us some time and he doesn't get pneumonia, we will be watching him for any signs of infection over the next 2 months (ear infection, sinus, etc).

Treatment (IVIG) for this is with blood product (purified plasma) that has Ig antibodies in it. We are looking at once a month treatments for 1-2 years. From what I've gathered, it is done through IV infusion.

I spoke to his pediatrician yesterday about the results and she said the IVIG would probably be beneficial to him.

So we sit and wait...

But in the meantime, Brice will be seeing the same doctor. While reviewing Pierce's medical history and going over his family history, I mentioned Brice's bloodwork (if you didn't read the above link, Brice had similar testing done in August 2009 - his IgM levels were low, IgG and IgA levels were borderline low). She asked if his levels were ever re-tested and encouraged having his levels redrawn. Further into our conversation, I mentioned that Brice is presently being treated for pan-sinusitis (infection of all sinus cavities - it was incidentally noted during his May 6 brain MRI). She looked at me and said "he really needs to be seen again." The next available appointment was June 28.

Between now and June 28, Pierce has an eye specialist appointment, a genetics appointment (w/bloodwork), his 18 month well-visit (w/immunizations), an orthopedics appointment (w/x-rays) and Brice has a GI appointment.

{monday mumbles}

• Brice had a GI follow-up on Friday --- it was rather boring (which is NICE!) --- until Brice takes his Nexium, twice daily, for 2-3 weeks, we cannot assume that his pain isn't GERD related. We has taken it as prescribed since Friday :) {in addition to his $45 ear drops - 4 drops in his left ear (the one with the tube), twice daily - I about died when I had to pay for those... we follow-up with his ENT next week}... yay for not having to fight him! He has also been saying he's hungry, asking for food and actually consuming it too! I feel like we are beginning to make some progress and I am thrilled :) 
• I took the boys to the Audubon Zoo for their FIRST TIME ever - I know, crazy, right? We live in NOLA and I've never taken them (whoopsie)! We went with Jenni & her sweet boys - all 4 boys had so much fun! We went to every exhibit and spent a total of 5.5 hours at the Zoo. I enjoyed spending time with Jenni and not being rushed. It was awesome catching up with her! {cell phone pics to come}
• Our weekend was pretty relaxed... and I really enjoyed it! Saturday, we had breakfast with the EASTER BUNNY at Bravo's. I have to say for what we really got our money's worth. It only cost $10.95/adult and $5.95/child. They served a buffet of french toast, stuffed french toast, scrambled eggs, bacon, potatoes and fresh fruit (strawberries, raspberries, blueberries and pineapple). The kids were given a bag filled with candy and a plastic egg. The egg contained a free ride on the Easter Train at Lakeside Mall and one free appetizer ($12.99 value) and one free kids meal ($5.99 value) at Bravo's. They set up a little area to take pictures with the Easter Bunny and while he wasn't busy doing that, he was going from table-to-table taking pictures and visiting. 
• As of Friday, Mr. Pierce's tooth, that was fixed on Thursday, was back to it's jagged, chipped-self. We go back to the dentist tomorrow...
• We're hosting EASTER at our house this year - I am excited! I love to decorate - it's going to be fun!
• We have two new pets... TURTLES! They are named Nemo & Nemo 2 (I give you one, and only one, guess as to which of us named the turtles). Brice helps us feed them and he loves watching them... 
• In closing, I am going to leave you with two invitations I've recently designed for friends of mine:

finding words

It amazes me to have a child that has almost 40 words at {almost} 16 months old. Pierce is spontaneously acquiring more and more words! He says Bice (Brice), no (and he knows how to shake his head no), up, down, mommy, daddy, potty, poo-poo, Daisy, Duke, stop (top), Neo, kitty, duck, quack, moo, dog, uh-oh, night-night, go, out, bye, hop, PaPa, Elmo, woof-woof, ahh-ahh (monkey sound), boat, choo-choo, cat, bubbles, roar, yum-yum, ball, two, cat, blue, hello and hi. Pierce is able to communicate with us, with his brother, with his world. We don't have meltdowns, tantrums, confused looks because his parents have no idea what he is trying to communicate... once upon a time, with Brice, that's exactly what we had...

I remember it all clearly... I googled Early Intervention in hopes of finding out whether or not my babe was truly delayed with his speech. I was tired of people telling me "he's fine, don't worry," "boys talk later than girls," and "give him until he is 2 years old." My heart was telling me differently. I knew that he wasn't fine. And my brain fought my heart... hard. How could my baby not have words? We never used baby talk. We read books, we used descriptive adjectives, etc... why wasn't he talking? His peers were - some in two to three word sentences. I've prided myself on my intelligence. I maintained a 3.914 GPA while in college. I married a smart man. It hurt my brain in ways that I cannot even begin to describe. I felt like I failed him.

We had our initial meeting with Early Steps. He was approved to have an evaluation. Can he do this, that and the other... no, no and maybe. I remember feeling even worse after our evaluation. My irrational brain was in full overdrive... How have I failed my baby like this... when in reality, if I were to have listened to my rational brain, I would have known that they were attempting to establish a base and a ceiling. But I couldn't think straight... I really felt as though I failed him.

Brice had his third ear infection and we were referred to our ENT. Brice had a hearing test and he failed. After our ENT appointment I felt as though we had an answer for my baby not having any words. That certainly didn't make it sting any less when Early Steps called and confirmed that Brice was significantly delayed in communication and cognition. We had an answer and a plan of action. Brice received a PE tube in his left ear and had his maxillary and sub-lingual frenulums cut on April 13... one year ago today... my baby's body was given the physical opportunity to find his words. I can't thank Dr. Peltier enough.

In the early days of speech therapy, we were learning to use sign language to communicate. He learned signs rather quickly and we were grateful for having an opportunity to communicate his basic needs (milk, more, eat, etc). And then by his second birthday, he had a handful or words... not many, but enough to make me beam with pride. And slowly but surely, he really started to find his words. As days turned into months, he was able to piece two and three words together for sentences. He began to spontaneously acquire words. And then it hit us one day... Brice NEVER stopped talking! Some days, I find myself lost in thought while listening to Brice talk. I sit there and smile and think about how far he has come in one short year.

Brice had his one year reevaluation with Early Steps a little over a month ago... he no longer qualifies! She was beyond impressed with "his intelligence." I knew he wouldn't re-qualify and I was so happy because I know where he was one year ago... wordless. He still has a small articulation problem. We have three sessions left with our speech therapist. She believes that he will work through those in our last few sessions and will no longer need her help (note: we had considered continuing in private therapy with her). Our speech therapist is truly an amazing woman. She helped my baby find his words (in addition to helping my other baby as well) and for that, thank you doesn't seem to be enough.

My children have taught me so much and not just through this, but through every hurdle we've overcome with them. They've taught me that nothing is insurmountable. They've taught me how to hope when I thought hope was gone. They've taught me how to be more compassionate and less concerned about what others think. And most importantly, they've taught me how to love deeper than I ever thought was possible. And for that, I thank them.