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 :)

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!

{monday mumbles}

{monday mumbles}

• Potty Training is going VERY WELL! Brice has been in underwear 24/7 since Thursday morning. He's had a few accidents - mostly at naptime. It's weird --- he stays completely dry overnight (so 10ish hours) but the last two days he's woken up wet from his nap. He's napping now so we'll see... Overall, he's doing fantastic - no pullups, only underwear... all the time... and 5 accidents TOTAL. {I am proud mama if you can't tell}. But here's my concern... pooping. I think he's a bit backed up because of all the barium he had last week. He's only had one BM since last Sunday (yikes!) I keep asking if he needs to poop and he tells me no... I've heard that this can be the tricky part of PT'ing... fingers crossed.
• So far, all of Brice's testing has come back normal, within normal limits or negative - bloodwork, stool studies, nuclear gastric emptying study, VFSS and Upper GI Series --- his upper endoscopy found a hiatal hernia and his duodenum aspirate showed a staph aureus infection.
• 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 and probably a few more that I cannot think of! His laugh and smile are still just as infectious as ever! Some words that I forgot: PaPa, Elmo, woof-woof, ahh-ahh (monkey sound), boat, choo-choo, cat, bubbles, roar, yum-yum, ball, two, cat, blue, hello, hi --- almost 40 words!
• We went to French Quarter Fest on Sunday and had so much fun! George's brother, David, came with us and Brice had so much fun with Uncle Dabe (Dave). You couldn't have asked for a more gorgeous day but it was hot... H-O-T! Brice sucked down 20oz of fruit punch (definitely not in my top 10 of liquids to give to him but we were in downtown NOLA and it was free!)... and he had two accidents around 45 minutes later... both happened within minutes of each other. And it one of my not finer moments... I had a dress on because it was H-O-T... it was also windy... {do you see where I am going with this...?} --- my dress flew up in the back (while I was pushing the stroller) --- I screamed (which probably only brought more attention to me) and made George push the stroller so I could hold my dress down --- sigh...
• Brice's appetite sucks lately... I know it's due to his stomach problems... I don't know if he know has aversions because of the pain - he's made negative associations with food? We give him {almost} whatever he asks for. He typically takes 1-3 bites (if that) and that's it. The one thing he will eat is white bread - toasted or not - it's a guarantee. He drinks lots and lots of water. He no longer wants to take his Nexium - I think he knows medicine is mixed into whatever I have in the bowl in my hand (I typically mix it into Greek yogurt or applesauce), especially since he did not request whatever is in the bowl. 
• We have a few appointments this week for the kids - Pierce is getting his tooth fixed on Thursday and Brice has a follow-up GI appointment on Friday {I can't wait for that appointment}... I just want an answer and a solution to Brice's GI problems and I would love a special way to get medicine into him!
• The boys have been coming into our room around 5:45am (it's cute to see them both walking in with their blankets but I also enjoy seeing the inside of my eyelids at 5:45am too!) My husband is wonderful... he let me stay in bed for an extra 45 minutes this morning - I really needed it.
• I am keeping my fingers crossed that another little path in my life continues to open up - if and when it does, I will share about it :)
• On Thursday, Jenni and I are taking our {four} boys to either the Zoo or Aquarium! I haven't seen her in months... I am excited about spending time with them :)

a story about {eggs, a tooth and more}

This may be a bit back and forth, so hang in there while reading it...

Tuesday: Brice had his nuclear gastric emptying study. We left at 6am, arrived at 7:30am and upon checking in, we were told that his test had been canceled... ummmm, excuse me? I very politely informed the lady that we were originally scheduled for Thursday but after speaking with Erin (the tech) she rescheduled us for Tuesday. We went back and forth for a 2ish minutes at which point I asked to speak with Erin. Instead, the lady called Erin, we worked things out, got checked in, paid the rest of our insurance deductible (blech!) and patiently waited... waited... waited. {read: we were told to be there at 8:15 and on Monday someone called and said we needed to be there for 7:30 --- well, they were wrong --- we were not taken back until 8:35am}

They made Brice a scrambled egg with a radioactive tracer... in a styrofoam cup... in the microwave (I should probably hop off my soapbox before I even get started about styrofoam and the fact it is not biodegradable and what it emits once heated... sigh). He went to town eating it... until it burned his mouth... after a few tears {from him} and kisses {from me}, life was good and he finished his radioactive egg. Then it was time for fun {read: NOT}...

Brice had to lay on a table...

{source}

while papoosed {his head was not strapped down and he had free range of his arms}...

{source}

for NINETY {90} MINUTES... pure torture for a toddler...

I brought Toy Story 3 with me and we watched it between tears, screaming and pleas for help (so heartbreaking...) - you would have thought it was Christmas when the lights turned back on in the room and he was able to move again. We had to go back at the 4 hour mark for one last {1 minute} image. We left and went to Chuck E Cheese for an hour because how else do you occupy a toddler when they cannot eat or drink... He won 140 tickets and got a snake and baby frog in return for said tickets. We went back and he started crying as soon as we walked in the room... begging me "no mommy no, please no" - why don't you just rip my heart out kid... but it was only for a minute... thankfully!

Wednesday: Oh Wednesday morning... the antics of Wednesday morning brought me to my knees. I think everything I've been through in regards to doctor appointments, tests, procedures, tears, cries of pain, worrying about test results, the unknown and so much more all finally hit me...

I was in the bathroom doing my makeup because we had to be at the hospital for 10:15am for Brice's Upper GI Series and MBSS. George was getting ready and the boys were playing at my feet... and then it happened... a loud thud that would make any mother's heart know that someone was hurt... then came the screams and my curious 2.5 year old saying "Uh-oh, what happened to Pierce?" --- exactly my thoughts sweet older brother, exactly.

Pierce somehow hit his mouth on the side of our tub. Once I pried his mouth open, wiped away as much blood and saliva as I could, I saw what I feared most... a fracture and an intrusion/luxated injury on tooth E... my poor baby's tooth was chipped (almost spanning the entire length of the base of his tooth) and partially pushed back into his gums. While I rocked him in the nursery, I sat there crying right along with him. As soon as I could compose myself, I called our pediatric dentist on his cell and left a message... then I called his hygienist (a friend of mine) and she said to head into the office for 8:15am. They took a few x-rays and thankfully the pulp of his tooth (nerve) was not damaged. We will go back next Thursday to have his tooth bonded so we can avoid decay (and his tooth will have a normal, non-chipped shape again). As for the intrusion, his tooth should spontaneously re-erupt. It could take quite some time but I am hopeful that because his tooth was not fully erupted to being with, it will continue with it's eruption pattern (maybe just a bit more slowly because of this injury)... 

We rushed back home to pick up Brice and George and flew to Baton Rouge. I called the hospital to let them know we were running late due to a minor emergency. We were suppose to arrive at 10:15am because his tests started at 10:30am... we walked ran in at 10:37am. Brice and I went into the back while my other favorite guys waited for us.

Brice started crying the moment we placed him on the table. I knew it was coming, especially after the day we experienced on Tuesday. I promised him that he would not be strapped down. My promises, at that point, meant nothing. He had to drink a liquid barium contrast while lying on his left side. He took one sip and after that, he spit the rest out. The PA really ruined it when she brought in a 60cc syringe. As soon as he saw it, he kept saying "no medicine mommy, no, please no." After that effort, the PA explained that we could either come back another day or they could call our doctor to get an order for an NG tube to fill his stomach. I told them to go ahead with the NG tube. While the PA was in the back, someone else (not sure of her title) came in and offered Brice a tropical punch flavored barium. He didn't like it. She offered strawberry and he drank it... one sip... then another. They started the test again and of course he no longer wanted to drink it. I promised him another trip to Chuck E Cheese and anything else he wanted (hey, I was desperate)... and it worked. He drank enough to fill his stomach. I thought that was fun... then they had to position him in all sorts of positions... he cried, I begged him to cooperate and soon enough, it was over.

After that, we completed his MBSS (modified swallow study). He was less than thrilled. He drank a few sips of the strawberry barium, wouldn't touch the pudding consistency barium and he consumed the graham cracker dipped in the pudding. They saw no aspiration.

After an hour long trip to Chuck  E Cheese, we went home... and it was lovely to just relax.

Today, the boys had speech therapy. While our ST was here, Brice started coughing hard while drinking some water. She said she thinks that he is fatiguing while swallowing because it happens around 5ish sips into drinking. We will let Dr. A know at our follow-up visit next Friday.

I had planned on sharing some exciting news in this post, but after some thought, I've decided it deserves it's on post! Come back to visit tomorrow to see our exciting news!!

Neuro {Pierce} and GI {Brice} Updates...

Yesterday, I took Pierce to his neurologist. I really love her. She is very down to earth, has amazing bedside manner and truly cares about your concerns/thoughts. To me, those qualities make for a great doctor!

These were the major highlights of our appointment:

• Pierce's episodes are Breath Holding Spells. Dr. S stated that they start around 12-18 months old. It's not a temper tantrum. It is involuntary (just as fainting spells are). She said when he is letting out his cry, something in him forgets to tell him to breathe back in --- that's why he stops breathing, turns blue, goes limp, etc. I told her about him going stiff and twitching, she said can be considered normal... he can actually go into full-blown convulsions for up to 15 minutes before he would be placed on any sort of medication.
• If he were to convulse for more than 15 minutes or if he were to start having breath holding spells that were not triggered by crying, then he would be put on medication.
• There is a correlation between breath holding spells and epilepsy (which doesn't mean that because he has BHS, he will have and/or get epilepsy).
• We spoke about Pierce's muscle biopsy pathology report. She and the pathologist (from Texas Children's) go "way back."
• Dr. S asked if Dr. N (our geneticist) had ordered the quantitative respiratory chain enzyme assay. I told her no and she thinks it's important to do it. It needs to be done on the muscle tissue so hopefully they still have some of his tissue left.
• She thinks Dr. N needs to do the bloodwork on the mitochondrial DNA
• Dr. S asked if Dr. N ever said anything about Pierce having a fatty acid oxidation disorder. I told her no; she was curious if he had ever been tested for it - again, to my knowledge, he has not.
• She thought it was really interesting to see the statement "This patient shows markedly reduced expression of Dysferlin." - she said this was the third patient she's seen with that. Dr. S said she has no idea what it means and that it could mean something or it could mean nothing. She planned on emailing the pathologist and letting her know that Pierce was her patient and that this is now the third mito patient they've seen with the Dysferlin issue. She said they may publish the information so that it's out there.

We will continue to follow-up with neurology every 6 months for awhile, unless something changes that warrants sooner follow-ups.

And for Brice... I called Dr. A yesterday and left a message regarding the great extremes we've gone to in getting him to take his medication. He called me back after 5pm. I told him that I couldn't get Brice to take his meds orally - in no form - at all. I also told him that Brice has been coughing/choking on things as simple as water, he's back to gagging himself again, and just seemingly very symptomatic for reflux. In addition, we're still hearing daily complaints about his belly pain. The following are the highlights of my conversation with Dr. A:

• We can stop the antibiotics (sulfamethoxazole). While Brice's staph aureus counts are definitely abnormal (350,000+), he is not insanely abnormal (1,000,000+). He's comfortable, at the moment, with us discontinuing the medication.
• He is concerned about Brice coughing/choking on water. He wants a pharyngogram (swallow study) to be completed this week.
• In addition to the pharyngogram, he wants an upper GI series as well. Those will both be done at the same time.
• He is also concerned about possible gastroparesis (delayed gastric emptying). Dr. A is ordering nuclear gastric imaging to be done this week too.
• There is the possibility he may order an esophageal manometry to look for esophageal spasms. Our doctor does not do manometries. He thinks there is one doctor in our area that does it and there is a doctor at Texas Children's that does it as well.
• And with all of the above on the table, he also stated that he is not taking the idea of Brice's gallbladder off the table either. I am thinking that is more at the end of the list, but it's still on there.
• Dr. A wants all of this testing done this week. He will be out of town next week but his partner will be in and we have strict instructions to call if things start falling apart and/or deteriorating. 
• *** Just got a phone call (10:30am) --- they are unable to schedule any of the testing for this week --- we will have his pharyngogram and upper GI series next Wednesday (4/6) and his nuclear gastric imaging on Thursday (4/7).

I think that's it! I will update again after we have the tests done.

seriously kid... quit testing my heart

When Pierce was 8 weeks old and hospitalized (for pneumonia), he had a few "breath holding spells" where he would silently cry and turn bluish in the lips (particularly when he was in pain - like getting a new IV). Since then he has had them on and off. Recently, they seem to have become worse.

Since Friday (3/18) he has had 3 of them that have resulted in his lips turning completely blue with the rest of his face taking on a bluish-purple hue, going limp in my arms with his arms and legs sporadically twitching. On Friday, when it first happened at this degree, I laid him on his back on the couch and continually blew in his face while tapping his feet. He began breathing again but was extremely pale after the fact. It happened again on Sunday while my mother was watching him. And today, about 2 hours ago --- this morning's episode was a bit worse. He was sitting on the floor in the kitchen when it started. I glanced down at him because I heard his head hit the floor. He was laying on his stomach, with his arms and legs twitching, his body was stiff - almost seizure like. I picked him up, he was blue in his lips and he went limp - I blew fairly forcefully into his face and he gasped. He remained very pale for about an hour after.

I am not sure if I should be concerned. I don't know if this is standard for "breath holding spells." Could this be interrelated to the odd breathing noise he makes upon inspiration (a deep, sometimes raspy, noise - not high-pitched - some have described it as though he is gasping)?

He's had a normal echocardiogram. They were unable to complete the EKG because he wouldn't cooperate. And he's also had a normal EEG.

It's frightening as all hell to witness... and I am tired - physically and mentally.

I was up from 2:30-3:00am with Brice because his stomach was hurting. If only he wasn't so difficult in taking his medication (Carafate) - but truthfully, my MIL told me it's absolutely disgusting (she took it before) - so I was up rocking him, comforting him, telling him everything would be okay... and finally, it worked - he fell back to sleep. I should probably mention that he is having a tough time going down at night. He used to go to bed around 7pm and fall asleep by 7:30pm. Nowadays, we put him to bed around 7:30pm and he is wide awake (escaping from his room and all) until typically 10pm. Is this him just being a toddler? I don't know how to fix it.... any suggestions?