Consider this a statement....and a story.

Whew. Just when we think we have a handle on life; BAM. More lemons get tossed our way....good thing I can drink a gallon of lemonade daily otherwise I'd be up to my premature wrinkles and graying hair in citrus fruit. I don't mind challenges; I welcome them so really I can't complain too much. It's just another walk in the park for this momma :)

Bruce is a trooper. He is a day shy of 15 months and I am wondering when in the world I became mother of a 15 month old....time may not have flown so much for me but I am still simply amazed I have been blessed with him. God is good. Trying, but good. 

The last thirty days have brought three stays in Children's Mercy for Bruce. When he was born, he had some major heart issues but with treatment for his Pompe disease, those heart issues were eradicated. Or so we thought. His heart has an extra pathway for the electric currents to jump and start beating super fast. Like 270+ beats per minute fast. Anything can start a SVT episode for those who have the ability but for Bruce, it's vomiting. Fortunately, it's not something he does daily but he is a baby in the middle of teething and has a primarily liquid diet through a g-tube. Unfortunately, we cannot stop them with the tricks most people can and it generally takes high doses of medication only bigger hospitals keep in stock. 

This last month when Bruce has had a SVT episode it is always at night and generally a weekend day...because nothing exciting happens on a Monday morning; it's Finagle's Law of Dynamic Negatives. 

SVT #1 :: Started on Friday the 13th and ended up with life flight ride #2 out of our local hospital. We were lucky to catch it but when I had my hand under Bruce's arm to hold him on my lap to clean him up, I felt the rapid beats and instantly knew what we were dealing with. The trouble was, we were 30 minutes from a county hospital and 90 minutes from Children's Mercy. We stopped at our local hospital, they did everything they could but they did not have the next level of meds available so a chopper was called and little man flew away in the night. Because of the lay of the land and direction we traveled in, I was able to watch the flickering lights of the chopper for 30 miles....green flicker red, green flicker red. Nerve-racking, humbling, exhausting all wrapped up in one long experience. 

As soon as they landed and when his favorite PICU doc put the stethoscope on him, he converted and spent the rest of the time flirting before we arrived. His medicine was adjusted and they sent us home that Sunday. 

SVT #2 :: Not nearly as exciting except we bypassed our local hospital and drove straight to CMH. The only time ever I nearly passed out from John's driving but he did good to get us there quickly and safely and again, meds were adjusted and they sent us home after a couple days. 

SVT #3 :: This time, not so quickly. Thursday night when Bruce started vomiting, I started panicking. I knew what was coming and I think I was getting more pissed off than anything. Certainly not with Bruce but with the situation, his medicine should be covering this. I quickly checked myself before I wrecked myself and away we drove after he started his episode. However, not after we tried all the vagal maneuvers they taught us : ice pack to the face, inverting and a quick cold shock shower. All to no prevail so we were southbound and down. 

When we got to the ER they took us right back and John and I started in on our routine - we told them everything they needed to know about Bruce, what meds worked for him, what didn't, a quick history and after they checked it out in the computer records, he was able to convert back to normal heart rate in less than 15 minutes after we arrived in the ER. That's a record and John and I felt like super advocates for Bruce. Rather than have to go through the normal routine they do for SVTs, they were able to get down to business. Momma likes. We got to the floor early the next morning, slept a bit and started in on a new plan with a new medicine. Momma really likes. 

But, this medicine takes time and close observation and an extended stay. Discharge is nearing and I'll get some time at home to do a load of laundry or six and get packed up to come back to Children's for this weeks round of as scheduled appointments and infusion. 

And  I do it all with a smile because who can resist this face.....

"ROOOAR"...means 'I love you' in dinosaur....

Boy's first haircut......that grin. Oh my.

And now for the statement. Living in the country is amazing. It's home for me. I am at peace. I wake up and every day I feel like I am vacation when I look out and I see nothing but rolling hills and prairie and trees and my neighbors are cows. And the way the sunlight hits the hills in the morning and EVERY sunset at night? I can't even put in words. It's glorious. 

It's also 118 miles one way to Children's Mercy and when we average over 10 appointments a month and have three unexpected stays in thirty days, it's exhausting. And frightening. And just plain silliness nonsense. So, Bruce and I are moving FROM a four bedroom, two living room, giant kitchen house with original hardwood floors and wood trim with a full basement, new deck and did I mention giant kitchen TO a one bedroom apartment. But, it does have a garage. And it's only 17 miles from CMH. Smart, indeed. 

I may sound a little resistant but really I am excited for the new adventure. The new place is actually really nice, great amenities and probably just as big as my house with exception the lay out is much different. I'm already planning quirky little DIY designs and have a new sleeper couch picked out for guests, mainly aunt Kacie when she comes to town :) And of course, margarita nights because I am only a few miles from a few ladies whom I know will join me in my quest to properly utilize all the citrus fruit in my life.

John and I are the best advocates for Bruce, with exception of a couple key players at CMH, and absolutely determined to make the decisions for our son. We are partners when it comes to Bruce and friends when it comes to us. Stress and pressure has taken its toll on our relationship but there is no one else in this world I would want to make parental decisions for Bruce with. Some days, I think John feels the same.

My brother is home from deployment #2 in Afghanistan. It's a chest-swelling feeling of relief and fear and pride and bittersweet sadness because he still has 2 years, 1 month before he is out of the Marines. I have never wished time to go by so quickly in my life. I love you, brother. You are MY hero....you and your handsome nephew.

Until next time, my friends.

x

One Year Down.....Many To Go!!!

Every time the thought passed through my mind the last couple months that we were about to celebrate Bruce's 1st Birthday and what a blessed milestone it is for that happy and handsome little boy, I couldn't help but slightly and silently freak out that it's been a full year for so many events.

 May 20, 2012 :: Bruce Jackson turned One Year Old. Wow. I put a lot of energy and time into making it a special day for him because he truly deserves it (even though he won't remember it) and a HUGE THANK YOU to everyone who helped celebrate at one of his three parties....he is loved :)

Bruce was born on a Friday afternoon - he was one of a few or four scheduled c-sections that day. I was 38 weeks and had been having some issues with my placenta which resulted in a lot of non-stress tests at CRMC. Which resulted in hanging out with all the awesome nurses there and getting to know them. Finally, the doctor gave in and we were on the books. At 1:38 pm, we welcomed our son into the world. At 6 lbs and 6 oz, I was pretty sure he wasn't our kid - or done baking yet but he checked out as healthy as can be. A couple hours later when I got to hold him for the first time, it was like my life finally had purpose. I gave thanks over and over in my mind to be able to enjoy that moment and wanted it to last forever. 

Much to the CRMC staff's dismay, we took up residence over the weekend. With the storms coming through and my spinal headache, I wasn't ready to leave yet whether it be new momma's nerves or just a gut feeling. I think higher powers in the end had a play. After a couple epidural shots and a lot of caffeine later, I was ready to take our boy home Monday morning. I hung out in the nursery for a while late Sunday night still on edge from the Joplin tornado and bright-eyed and bushy-tailed from a 2 liter of Coke. The nurse and I took note of Bruce's legs swelling around 1 am or so and she put me back to bed, assuring me she would wake us up if something happened but it was probably fine. A few hours later, she did just that. Things weren't fine.

John and I packed the room up like gypsies when she said there was a chance he would be transferred to Children's Mercy. I finally felt better to move and didn't feel like my head was going to explode but I remember the night nurses keeping a steady dose of painkillers in me being just a few days after surgery. Angels. The pediatrician came in her pjs and I knew it wasn't good. We met her earlier that weekend and she was very professional - I was excited to have met her. Then to see her in her flannel pants and hair in a ponytail at 5 am, I couldn't help to think, Shit. 

The flight crew from Children's was on call because of the tornado and made good time getting to Cameron. It didn't take much looking of the chest x-ray to know something wasn't right, even though they assured us it was probably just the computer screen. Right. After the crew in the jumpsuits started a routine iv, little man was ready for his debut helicopter ride. Some kids get to ride in a car for the first time but mine got a steel bird. John and I watched from the parking lot as they took off and we were right behind. I called parents from the road, yelled at John for getting lost, and managed to stay relatively calm. Even though I yelled. 

We took the last bed space in the NICU and it was clear to see the staff wasn't thrilled. No one really knew why he was here and what tests to do first. For a while that day, a midst shuffling family in and out to see him and semi-abiding by the sign-in book Nazi's ridiculous rules, we were at a stand-still. I was nursing Bruce that afternoon when the first clue came and it was a doozy - his heart rate jumped over 300 bpm and I witnessed the nurse actually tapping the monitor like in movies, then realizing what was happening. A famous CMH/KU doctor happened to be rounding and was the one who stopped it. A lifetime of gratitude to that man. 

Immediately an EKG and an Echo was ordered and I will never forget the cardiologist who took all of 6 seconds to see the monitor over the tech's shoulder before he walked away. Later that night, we sat in a conference room across a table from him and about 10 other doctors, nurses, social workers, and I'm pretty sure the chaplain was there, while he drew on a piece of paper what was wrong with our son's heart. And it wasn't good. After they left the room with more than enough apologies and promises they would do their best to figure it out what was causing it, I had a breakdown where I'm pretty sure I cried tears out of my nose. 

This is the point a year later where I am having a breakdown. To go from the greatest day of one's life to the absolute worst news you have ever heard just a matter of hours later, was more than overwhelming. Up to that point, I was able to find some humor in Bruce flying in a chopper and his village of people taking turns pissing the desk lady off  - as long as he was ok. And healthy. And up until that point, I was living in my own happy little oblivion.

The story goes, if you have been reading at all any this last year, that we did find the culprit and treatment was started immediately, at day 13. The longest 10 days of my life to be patient while they poked and prodded and asked us questions about everyone in our families, minus the first settlers. His heart corrected itself within two months of life and is now NORMAL. Take that Dr. Bad News. I actually saw him at CMH a couple weeks ago and kept my eyes to the floor - it wasn't his fault he had to deliver the news but I will forever associate him with that horrible day. Just a part of it. I still nearly hyperventilated when I turned the corner. 

It has been tough to watch the news the last few days because everywhere you turn, it's about the Joplin tornado. Later that night after our conference and we had shared the news with family, the hospital gave us a room at the Ronald McDonald house within the hospital. I shared the twin bed with John and we smuggled my sister in, who occupied the floor. The rooms aren't meant for long term stays or for anyone over 4'6" but handy to have when you can't leave/aren't willing to exit the same building until forced out of the hospital yet. I was being a pain in the ass and not keeping up on pain meds and refusing wheel chairs when anyone looked at me so obviously, sleep was terrible. But I did manage to get a little bit and when we woke up that next morning, through the paper thin walls, I could hear a dad talking on the cell phone with a Southern Missouri accent. I cursed him a bit for waking me up out of my horrible sleep to my horrible realization I was a parent of a child in Children's Mercy but immediately started praying for him and his family when I heard what he was doing - he was making funeral arrangements for one child back home in Joplin while he and his wife were up in Kansas City with another. I never saw him or heard what had happened in the end and maybe it was just God telling me that it was all going to be ok because there were worse things happening that day, I don't know. But that was the point when something clicked in my mind that my whole life was about to change. 

You think having a baby himself would have been enough to change my viewpoint on the world but that day was the beginning for me. Now I have a little man who has showed me a whole new world and brought me closer to God....and good Lord willing and the creeks don't rise, I will do everything in my power to keep him happy and healthy. 

May :: We're about to come full circle....

It's May! AGH! Where in the world has time gone....it's been a journey and without getting too sappy on you, here's the latest Bruce update ::

Infusions are going great - we're about to start the new drug trial with the adult version of his enzyme replacement therapy next month and Children's has been busy-busy getting paperwork finalized behind the scenes....better them than me, I say.

We met with neurology a couple weeks ago and she was PLEASED to see Bruce as he is now...seizure/spasm-free since November 2011, happy, smiling and growing well. I have NEVER seen that woman pleased so it made my day. Week. Year. Lifetime. She does want a MRI soon just to see what the abscess looks like and  that's just a drop in the bucket for this pro.

PT/OT is making some amazing strides. Since starting in December, Bruce has mastered rolling over, sitting up, weight bearing on the legs and is close to pushing himself to sitting up, staying up on all fours and taking steps while holding hands. When he started after all of his hospital stays, it was way too fuzzy to say when he'd be doing any of this so to have these goals in the bag so soon is amazing. He's a strong little guy and practice makes perfect!! He's pretty much given up on the bottle so his calories are primarily baby food and bolus feeds down the g-tube. We're slowly working off the night time continuous feeds because sleep is nice for parents.....

During April, Bruce had a couple poor hearing exams. Yesterday he had an ABR where I kept the poor guy awake by rolling him in a wagon through the hospital after his morning PT appointment so he could people watch all the interesting people you see at Children's Mercy and then he slept through the hearing test of all the pitches. Low and medium pitches were normal but associated with meningitis as he had are damaged cloacaes...(I always thought that was chicken anatomy but apparently different spelling) and high pitches are affected. He is borderline failing and quite possibly going to get worse as the cloacae grows incorrectly as he grows. So, we get to add an ear doctor to the large team of Bruce's plethora and maybe I get to meet the entire staff at CMH! So, that doctor that asked me if I worked there last time could quite possibly be our new doctor.....Anyways, we'll do our best and take whatever treatment necessary to ensure that Bruce hears his momma say, 'Get out of that mud puddle' or 'Leave that poor dog alone' or 'Stop chasing the calves' or whatever this ornery little boy will be up to....

Coming up we have the Muscle and Nerve Rehab Clinic, Neurosurgery follow up and Cardiology follow up. We are not expecting any major changes and are looking forward to the rehab clinic for their initial assessment and if Bruce is a candidate for any specialized mobility tools. I don't think he'd use them too long as much progress as he's making but if it helps in the meantime, we'll take it!

On the family side - Congratulations to (my sister) Aunt Kacie and her new husband, Uncle Jake!! He's pretty much always been Uncle Jake but it was nice of her to finally make it official :) The wedding was beautiful and so much flippin fun and they had a great time living the honeymoon life in Mexico...now they're back in Iowa and ready to start the rest of their lives together!! I'm not even going to say it but Bruce would like some cousins.....ONE DAY.

Bruce's Uncle Seth (my brother) is now on his second deployment to the Helmand Region of Afghanistan and hopefully the last if politics and government ever get their heads out of their asses and ya, ask me how I really feel. We pray every day for Uncle Seth and all of the Marines and troops on deployment and will write him all the time!! WE LOVE YOU, UNCLE SETH!!!!!!!!!!!

Bruce has his first birthday this month!!! There is much to do yet for the big celebration but that'll all come in time...so I keep saying :) This coming weekend we will be walking with some friends and family in the KC March of Dimes Walk and I cannot wait to be a part of that!! Hopefully we'll get to see some familiar faces from the beginning days in the NICU and to be a part of such an amazing organization will be neat....Go TEAM BRUCE THE POMPE MOOSE!!

Little man wanted to take momma's chair and watch cartoons during his last infusion....of course I gave up my seat!

I think that's it...so much for keeping this blog updated with short and sweet posts :) Thanks for reading anyhow....God Bless!!!!!!!!!!!!!!!!!!

"No, I don't work here."

EEEEEIIIIIIIIIIIIIAAAAAAAAAAAAAAAAAAAAKKKKKKKKKKKKKKKKKKKKKKKK SSSSSSSSSSSSSSSHHHHHHHHHHHHHRRRRRRRRRRRRRRIIIIIIIIEEEEEEEEEEEEKKKKKK.

Bruce's new words. He doesn't cry or throw fits but boy, does this kid shriek. A lot.

The last several days have been chaotic, crazy and wonderful. Last Friday was Bruce's first day at the Early Childhood Center in town and I don't think he could have had more fun! He loved seeing all the kids and even ate the best for me he had all week so I think he's going to do just fine...and I'm sure learn new tricks and share his own tactics....sorry, other moms. 

Saturday, Bruce and I walked in our first 5K! It was a lot of fun and a huge thanks to the Driver family for inviting us to the Eagle Egg 5K at Summit Christian Academy in Lee's Summit! We weren't exactly at the top of the group but we weren't at the very end, rounding up the cattle drive with our small herd of children and strollers. I am looking forward to doing more this year - Bruce really enjoyed the mass of people and scenic ride!

Jake and Kacie and papa bear John met us in Kansas City Saturday evening and the four of us big kids headed to the Sprint Center for some rank bulls and tight fitting blue jeans! We all had a lot of fun at PBR and I can't wait to take Bruce with us - next year he will be making the trip with us! 

<----- Me and my beautiful sister, Kacie!! 

 Her wedding is coming up SOON.....

...17 days soon. 

My family and I made it back home to the ranch on Sunday but long enough to mow the lawn, unpack from the weekend trip to KC and get ready for the next two days of appointments at Children's for Bruce.

Monday was Infusion Day. All went well minus a late start - sitting on 1-35 at a complete standstill with no sight of a bathroom for miles is scary...I need to rethink my morning caffeine intake on our long commutes to KC should the event of another car fire occur and I am left nearly desperate for a port-a-potty. 

Tuesday had an early start in radiology with his upper GI consult from his random week of vomiting early February but fortunately, there is no obstruction nor reflux. Hard to say what that was all about but I imagine he had some of the flu bug that plagued his momma and daddy and left us nearly lifeless and just did a better job handling it than his sorry parents. Nonetheless, a consult was ordered, we complied and now we know little man fine....it never hurts to check with this one :) 

Later Tuesday morning we met with PT and Bruce showed off his new moves. She was really pleased with his progress and we discussed our next steps with little man. As she was saying she wanted to see more floor mobility before we started with standers and walkers, Bruce was rolling all over the place and she said, "Like that." We meet with rehab clinic in a couple weeks and I think we'll have a better idea when we can start with some mobility tools and get this kid upright and moving!

That afternoon was another hearing exam. Same results as last time and we aren't surprised that there is some hearing loss after his bout of meningitis last summer. Our next step is an ABR exam where they will test his ears while he sleeps to find out just how much loss there is and then decide what kind of devices he will need. Now's the time to be getting on this as he develops his speech and mobility...maybe if he hears himself scream and screech, he won't do it as much?? 

A momma can only hope....

And to the nice doctor who asked me if I worked at Children's Mercy, I hope it was because I look doctorly. 

It is amazing the amount of dirty dishes John can accumulate in just a couple days of 'baching' it but I don't have time for those today. Nor do I have time for laundry and clothes so I will be working from home today in my robe...

A parting view of our neighbors to the south ::

Even with all the chaos, screeching,and mountains of work and tasks awaiting me, I AM BLESSED!!

And that, gentlemen, is Bermuda Hybrid #5.

I loooooooove that movie. Who doesn't love some Dennis Quaid in baseball pants?!

Bruce Update :: Infusion went great. Too early for final hearing exam results but round 1 shows mild to moderate hearing loss from the meningitis. Judy at PT was impressed with rolling over but still pushing for more results - I like that about her. 

Momma Update :: New camera. It's Spring. I'm already worn out from the rain and appointments this week but I'm home and can work the online marketing galaxy in my pjs.....life is good. 

Papa John Update :: Got his new boots and they don't make his feet look big - his words, not mine. But his feet ARE big - my words, not his.

Daddy loves baby.

Baby loves bananas.

A lot.

Momma loves a happy baby.

 And chunky baby legs.

And Papa John's new boots (:

Top o' the mornin to ya!

Happy St. Patrick's Day! I've gone from celebrating in a bar with green beer to waking up to Bruce's own special pot of gold! Who doesn't love giving a cute baby a bath after a morning diaper blowout?!

It's been a great week so far! A couple nights ago, I was working on supper and John laid Bruce down on his back in the living room. When he walked back in after a few moments, little man was on his belly! After a few months of PT, we have a roller! I immediately gave up on creating a culinary masterpiece and hid behind the door to catch him on video...within a few minutes, I had plenty of proof!

The next scheduled infusion is Monday and I can't wait to see the looks on their faces after Bruce shows them his new trick!

The Feeding Saga :: Parents 1 pt , Bruce 0

Each day, feeding time is a like a box of chocolates. You just never know what you're gonna get with Bruce. 

So when he refused his bottle for the third day in a row, we added a little chocolate. 

That's right. Breastmilk + formula + thickener + chocolate malt Ovaltine.

I ended up spoon feeding it to him so he would have a taste of it and practice the spoon some more. 

This is the result! 

I don't think we solved the bottle drama but at least we know he's a chocolate malt kind of kid :) 

Sometimes you just need a good cry...

When I was supposed to be researching material for a blog that was due yesterday for work, I came across a story of a young family in Kansas who lost their little girl when she was 11 months old to cancer.

You can imagine the state of my eyes right now. And my work blog is not getting anywhere near done.

We are coming up on a year of all that we went through with Bruce. His first birthday is right around the corner in May and I should be so excited to start planning a great party for him. And I am. That little boy deserves nothing but the best and as his momma, I intend to do my best.

But I have so many emotions I thought I checked at the door when we checked out of Children's a few months ago. There is something to be said about anniversaries and I think looking back, I'm even more scared of what we went through as a family than when I was when we were living it daily.

There have been some issues with the enzyme treatment that Bruce receives for his Pompe Disease due to a shortage within the manufacturing company. Our AMAZING doctor called me yesterday when we were at the salebarn watching the second round of calves this year sell to fill me in about her trip to Boston last week to meet with the drug company suits. And any other doctor would have been mortified to their patient's mother answering the phone with an auctioneer screeching numbers in the background but not her - she knows how we roll and has seen a cow or two in her lifetime.

But her call was to inform that she has procured enough of the drug to last til his first birthday. Then he will start a new drug - the same exact drug as the first but because it is made in a bigger/different bioreactor, the FDA has not approved it and Children's has start a clinical trial and Bruce is the star of the show. The mountain of paperwork is underway and by time May comes, it will be smooth sailing.

Sometimes our doctor tells me too much info. I didn't even know she was flying to Boston to meet with the company. And I damn sure didn't know there was a shortage. But when I brag on her and say what a great doctor she is, I don't mean it half-heartedly. She's a bulldog. She will fight for Bruce until her last day because she is passionate about medicine and helping children. And I am grateful to God that he sent us to her. He knows, and she does too, that we can handle everything that has been put before us as parents to this little boy who has to live his life navigating through uncharted waters with a brave and happy heart.

He'll have a bad day now and then but because his momma is getting them out of her system now, she'll be there for him.

Happy RARE DISEASE Day 2012!!!

Today Bruce is 9 months, 9 days old and we are 9 months, 6 days into the journey and maze of doctors and diagnoses and appointments and medications and all the excitement that comes with! I never thought I would participate in celebrating Rare Disease Day because I never thought a rare disease would be such a  part of our lives. But, it is and to know our son, Bruce, you would never know he has Pompe Disease, a rare inherited neuromuscular disorder that causes progressive muscle weakness in people of all ages. He is blessed with an early diagnosis and available enzyme replacement treatment (ERT) that he will receive for the rest of his life. Not all people celebrating Rare Disease Day are able to say that they have treatment, let alone a name to the disorders that affect them or their families.

Bruce met with his cardiologist yesterday and keeps getting great news....NORMAL. To think that when he was only 3 days old with the diagnosis of hypertrophic cardiomyopathy (HCM) and we were desperately trying to find out what caused it and if he was a candidate for a heart transplant that today we can say he has a normal heart! That's a miracle if I have ever seen one! He will continue to stay on his heart meds for now but only because he has a history of fast heart rate episodes (SVT) -- not because he needs it for anything else. 

To have answers to all the questions we were faced with in the beginning of our journey is simply amazing. We have learned that Pompe disease caused the HCM and regular ERT treatment would help correct it. The genetics team never said that it would actually correct it altogether and we would see results so quickly...but when Bruce was two months old we had the first NORMAL heart results and ever since, he hasn't looked back but kept on keeping on. The cardiologists were reluctant at first to say it was the treatment but I think after the last 7 months, they are slowly becoming believers :) We sure are!! 

Bruce's second medical dilemma was the CNS brain abscess from bacterial meningitis when he was 2 months old...just as we were getting used to the idea of Pompe disease and all the treatment! The abscess was hard on the little guy but he was a trooper and has healed great. Our last appointment with neurosurgery was before Christmas and had great news that the abscess was nearly disintegrated. His VP shunt for the hydrocephalus is working great and we were clear for 6 months with out another checkup. We go back in May and hope it's just a routine visit!

Last month we met with neurology for the Infantile Seizures that the abscess caused and she kept him on his seizure meds. We have not seen an episode since Thanksgiving and go back for yet another hopeful routine visit in April. It's too early to tell what damage has been caused in the right front lobe when the abscess was but time will tell and if I have heard it once, I have heard it a thousand times -- babies have so much plasticity and he should be just fine!

Things we watch out for for signs of damage are his controls and functions. Eating has still been a challenge but thanks to the gastronomy tube and momma's good (mixing formula) cooking, he's putting on weight just fine. During the day when we aren't on the road for appointments, he get free choice at bottles (all that leftover breastmilk I worked so hard on pumping when he was in the NICU!) and during the night he gets hooked up to his pump. Otherwise if you see us rolling in the stroller at Children's Mercy we look like a food cart -- Bruce on his pump and momma's cup holders full of diet coke and coffee! 

Physically wise, he looks like a 2 year old he's getting so big! He's nearly 30 inches and just shy of 20 pounds. Long and lean like his daddy! He wears anywhere from 18-24 mos clothes even though he just turned 9 mos old. They vary so much anyways. Developmentally, he's in the 6-7 months range. Rolling over is SOOO CLOSE and he can sit briefly without support. He loves to mimic tongue movements which I am sure will come in handy one day when he learns to pick on siblings (no, not even close to that yet :). And he is a great little helper when it comes to medicine time and feeding time -- he thinks he can grab all the syringes and tubes himself......yesterday he was insistent on helping with the EKG and echocardiogram and the little old nurses thought it was too cute...Geesh, he's already winning them over with his charms. 

And that's where we stand on our first ever Rare Disease Day! I am blessed to be momma of this little boy and we will continue to be the best advocates we can be as his parents! So many people are affected by rare diseases and do not have the treatments they need and yet they are even more positive than we are....those are my true heroes in all of this. I have changed so much personally in the last 9 months; rather than seeing a sick child and saying a quiet prayer and walking on the far side of the hallway, I want to stop and talk to them and their parents and get to know their stories. I am not intimidated anymore of the 'taboo' Children's Mercy Hospital filled with illness and sadness yet cheerful and hopeful for all of the patients and their doctors who work so hard. God has a plan for all of us, that I know. He shows me mine everyday! 

Thank you to everyone who has supported us in our journey and continued keeping us in your prayers! Bruce hasn't been fortunate enough to meet most of you but I hope that will change soon!

Oooooooh, my.......

Has it really been nearly THREE MONTHS since I have posted last? Yikes! I forgot how much I missed this little box to type in...it's a great escape for me to ramble on and on!

So much has been going on!! Which is half of the reason for the lack of blogging lately but also because I am so honest in this blog, I kinda put it on the back burner so all of my family readers wouldn't check me into the loony bin for opening up my stressed-out mind :) And, man, was it ever stressed...the last stay in October for Bruce in Children's Mercy nearly done me in. We were not expecting such complications and we certainly weren't expecting the team of doctors to fail so poorly at communication with each other and cause a ruckus.
But, they did. And we survived. Because that's how we roll!

Hanging with momma! 

The last couple months, Bruce has been making tremendous strides with his health! His last MRI in November showed great results that the abscess was nearly gone. His infantile spasms were still occurring at the time but since Thanksgiving, they have been controlled with medications and the neurologist was optimistic then they would disappear altogether as the abscess dissolved. And we're in the middle of January with no issues to speak of!! Tomorrow, bright and early, Bruce has an EEG and a neurologist follow up so we are very excited to see what that has to say. And a little apprehensive.

Other great results have been in physical therapy. We tried a couple different places but settled with a lady at Children's Mercy (imagine that!) who has seen another child with Pompe before. In our search, I was explaining a lot what the disease was and what other results have been but Judy at CMH knew all about it and honestly, was very worried when she saw Bruce's case. The first appointment she shared with me that when she watched Bruce and I in the waiting room that day, she knew already Bruce was doing MUCH better than most infants with Pompe and she was happy to see that! We are very blessed!

Bruce has P/T every week and that will continue for some time. Eventually the sessions will taper off and we'll move over to the rehab center but for now, it's weekly P/T with Judy. When we first started only last month, Bruce was very 'iffy' about his feet because he pretty much didn't know he had them! When you spend so much time lying in a hospital bed, I guess your feet are the last things you really think about. Since working with him, he now knows he has two rather large feet and loves to pull his socks off...he certainly gets that from his barefoot momma!!

We also work on weight bearing and yesterday she introduced leg splints much sooner than she imagined! They are pretty cool looking - red with rocket ships. When daddy saw them he said they look like the ones he uses for baby calves when they have wobbly legs, minus the rocket ships. I'll never forget, many moons ago in the beginning of our journey, when we saw a baby calf running out in the pasture all cute and awkward-like he said, 'That is going to be what our kids look like!' I reckon he was spot-on!!

Bruce's first Christmas was fantastic! It took me nearly four days to organize and put all of his toys away but he made out like a bandit and is a very loved little boy! I'm excited as he grows to teach him what Christmas is all about and that while over-the-top gingerbread mansions and over-indulgence on grandma's baked goodies are great and all but really the day is for Jesus and to celebrate him in our lives! I will never forget how blessed I am and I want to share that with him!

2011 was a year I am sure I'll never be able to forget. Even if I were to try. It came in peacefully, roared like a freight train throughout and left us in the end stronger than ever before. It all did happen. John's dad beat prostate cancer. Bruce entered this world the same weekend as the Joplin tornado and left an aftermath that seemed at times to rival mother nature's fury. My brother returned home safely from his first Afghani-hell deployment and spent Christmas in Missouri - the first time since becoming a Marine. I left my first after college job as a big girl and said 'see you later' to the great guys I worked with. My sister got engaged to a perfect man for her I am proud to call Uncle Jake. Relationships were put through the ringer.

 BUT....!!

I became Momma to a child who has taught me more about life, myself and faith more than anyone before. I feel stronger, wiser, a little more tired than usual :) and I think if I had listened to up above sooner, I would have questioned less and gone with the flow better. I wouldn't trade a dang thing for the experiences I had in 2011 and I am forever thankful to God for bringing Bruce into our lives!

Here's to 2012 and the memories I can't wait to have with my family!

Keep a weather eye on the horizon....

We're coming up on two weeks back in Children's Mercy and a lot has changed in the last couple weeks. In Bruce, in myself, in future plans. But that's life; you can't change it but you can adapt. And I am like a karma-karma-karma-karma-karma chameleon.

Health update :: If you recall back to last month, we thought Bruce, er, we were told by neurologists and gastroenterlogists, he was having a type of reflux that triggered 'seizure-like' episodes. So antacids were prescribed and Bruce got a purple pill a few times a day...and just when I mastered how to give a 4 month-old a pill, plans change. Big time. Last week a new neurologist came on board and met Bruce herself for the first time. She had heard about these episodes and wanted to see for herself. Since Bruce had been admitted I happened to have one captured on video on my new fancy phone and showed her. I'm a face reader and I didn't like what I saw on her face. Her funny resident with a bow tie saw the look too and knew what she was thinking. Her diagnosis, Infantile Spasms. And they are as scary as they sound.

Most babies are born with electricity in their brains that shows up on EEGs when they have Infantile Spasms. Bruce was not born with them so his EEGs have never shown the hypsarrhythmia that detects spasms but they can be triggered by issues in the brain and in Bruce's unique case, his brain abscess is the culprit. This is not something they have seen from brain abscesses but more typically from brain lesions or tumors.  So, there was much arguement between all of his doctors that this was an incorrect diagnosis and we shouldn't call it Infantile Spasms but really after much debate, it is what it is.

He's started on a new medication that seems to be helping so far. He is not yet worked up to the dose where they will hopefully go away all together but that takes time. We have some options, medication-wise, to treat them because the goal is to eliminate them all together. And it is a possibility that he will grow out of them as his brain abscess heals. Infantile Spasms are kinda like super seizures...seizures don't hurt or do damage, unless you have one and fall and hurt yourself, but they don't cause brain development issues like spasms do. Bruce is behind physically developmentally but we knew that from all of the time he's spent in a hospital crib. During the second brief time home, we started working with Missouri First Steps and that will definitely continue but as a parent, I need more. And time will tell what, if any, damge has been done from the spasms.

When I was pregnant, I made some life-changing decisions with my unborn child in mind. It was a year ago exactly that my whole world changed and I am so thankful. But pregnancy does that for every woman. It is beautiful. I was a bubbling ball of hormotional momma hormones and willing to change my life plans and make every decision for the heartbeat growing inside of me before I ever made a decision for myself. And now that Bruce is here and I can touch him and hold him and shower him with momma kisses, nothing has changed. I am still making every decision for him as my number one priority.

So much changes for people when they become parents. For those of you who know John's and my history together, it's been trying at best. But we have overcome so much together and now we are raising a baby with needs we never dreamt about. Hell, we are raising a baby with needs no doctor ever dreamt about. An infant diagnosed at 13 days old with Pompe Disease who overcame hypertrophic cardiomyophathy within two months of a new treatment who then caught a common bacteria but then turned into bacterial meningitis and resulted in a brain abscess that is now causing Infantile Spasms? That's like a twisted medical version of the M.A.S.H. game I played as a kid to figure out which sports car I would drive and early 90's heartthrob I would marry. It's certainly not text book. And I drive a Malibu.

Plain and simple, Bruce is a medical miracle. We have a wonderfully unique little boy who is so danged cute. And we're going to make decisions that are out of the box because we have a very out of the box situation and want the best for Bruce, no matter what.

Last week ended with a conference with five of Bruce's many specialist doctors to come up with a plan for little man. I think they've got a good one in place and tomorrow is a new day. This week he will be getting a permanent feeding tube in his belly that will help administer all of his meds and always work on feedings. Also this week I have the numbers of some development programs to enroll him in. Where ever and whatever, it'll be for his best interest!!

It's me again, Margaret....tehehehehee!!

Do I sound like a broken record?? One would think they would go craaaaaazy for as much as we have been through but I don't think I'm too bad.....YET!

Bruce is back in Children's Mercy. His infusion Monday went fantastic but I brought to attention his newest medical mystery....a black tongue. From what I heard, there was some slight panicking on behalf some of the staff but quickly a cause was found and it's from one of the strong antibiotics. Hopefully next week when his 4 months of antibiotic therapy is over, his black tongue will go away. (On a side note, I did make Halloween cupcakes the day I noticed it and they did have black marshmallow fondant but I can assure you, my 5 month old did NOT eat the cupcakes.) I thought it was ironic though.

But the tongue is not why he's back. On Tuesday, he had Special Care Clinic and Infectious Disease appointments. For the last week now little man has been incredibly fussy, which babies do, but as parents you know your child and if something is wrong. With all that Bruce has been through this summer, the docs agreed a ct scan would be helpful to make sure everything with the shunt was alright and nothing was out of place. Fortunately, everything looks ok shunt wise but on the ct scan, they noticed the ventricle is slightly enlarged. Which is concerning. So they admitted him and a bunch of tests have started again.

Right now, we really don't have any answers. The neurologist stopped by today and upped his seizure meds because come to find out, he's chunky. I guess he's been putting on too much weight but as a momma, that's music to my ears. There is nothing wrong with a healthy, well-conditioned baby. We're waiting to hear from neurosurgery if they have any suggestions but last we knew, they weren't too concerned. So, it's literally a waiting game. Bruce is still fussy but the nurses on 5 Sutherland are glad to have him back. Secretly, I think he's happy too to see his ladies.

Back to the 'too many chefs'. Every doctor has their own agenda when it comes to Bruce but the end goal really is to get him healthy. The communication has been lacking some but hopefully our geneticist is going to step up and herd up the free-ranging cats and get everyone on one page. Her theory to Bruce's poor eating habits is from the damage done to his brain from the abscess and suggests maybe a more permanent tube in his stomach for feeds. We were hoping as parents that the antibiotics were the culprit and once he was off them, he'd feel better. It will be interesting to see how next week plays out and how Bruce responds. A g-tube may be necessary but one thing is for sure, we will work with Bruce each and every day to make sure he is learning and growing and doing as best as he possibly can. Soon we meet with a Behavior and Development specialist at CMH and we'll get the ball rolling with as many programs and work as he needs!

When we were riding up the elevator Tuesday to Bruce's first appointment, we ran into his old NICU buddy's grandma. His buddy was born in March, two months premature (his due date was the same as Bruce's) and had a lot of respiratory issues. Finally after 7 months, he was getting to go home!! We were so excited for them and wish them the best of luck on the outside!!! Hopefully one day his momma and I can get the two ornery fellers together for a play date and we can tell them both how many headaches and sleepless nights they gave us....and how proud we are of them for growing big and strong!

Surprisingly, but then really not, we are in our same room we had six and a half weeks ago. It's like they saved it for him. It feels a little too much like home.....but I do enjoy the view of Union Station twinkling in the night lights as I sit here and type!

I started working, YAY!! It's a fantastic opportunity to do some things I really love....organize, data entry, emails and blog! I know, I'm a dork. It's for a Kansas City-based online marketing company, Turn The Page Online Marketing, and I get to do pretty much everything from my computer. And my computer goes with me everywhere - home and hospital - so I have something to focus on in between doctors and nurses popping in and out and while little man snoozes away at night. It's a great company and has some really great people working and I'm soooo excited! While I miss all the guys and farmers and office folks at the coop in Iowa, I'm happy I can do something that allows me to learn a new industry and be with Bruce as much as I am. I'll still spend some time this winter sitting around drinking coffee with all my favorite farmers though....

So that's the latest. I haven't made arrangements to stay at Ronald McDonald house yet because who knows how long this stay will last but if it looks like we'll be here throughout next week, I'll get a room. For now I'll be bunking in Liberty at mamacita's...home cooking beats cafeteria food!

Bruce sends his love and his parents say THANK YOU for the continued support and prayers!!

It's so quiet in the middle of the night but I should be sleeping....

Woohoo! It's Fall, a close second favorite to the non-'burn the hair on your toes, it's that hot' days of summer! Momma is happy...and a happy momma means less grumbly comments under her breath and more smiles, less "find something in the fridge yourself to eat because I had a bowl of cereal and you're a big boy" and more crock pot cooking, less laundry on the kitchen table and more festive decorations, and (probably John's favorite) less "knock off your damn snoring or I'll shove your face in a pillow" and more, well, ok that hasn't change too much!

September was here...I think?! It's still been great to be home! Bruce is really growing and his body is healing from this oh-so-crazy summer. Yesterday, he had a MRI and it showed the brain abscess is regressing (great news!) but there is some concerning swelling the neurosurgeon is going to keep an eye on (eh) but the vp shunt is working great! Infectious Diseases has ordered another month of his super strong antibiotics (eh) so the feeding tube is staying put til those are finished but he has been eating fairly well lately! We started some cereal last night and that has been a hit so far. GI gave the go-ahead to start solids but he's to remain on the Nutramigen AA and off breastmilk.

Bruce's seizures started acting up again a couple weeks ago and the neurologist placed him on some stronger anti-seizure meds. When the kid started tapping his left leg and left arm and keeping time, I knew something was up because his father and I have no rhythmm, whatsoever. None. Nadda. But, the meds have helped and we haven't seen any new activity since, except for what we thought were seizures the other night. All of the medications Bruce is on are wicked strong and keep his belly upset, hence the feeding tube. Which really is the handiest tool for giving meds when he's sleeping......no fights. Yet, a catch-22.

But these latest 'seizures' are in fact, not. They are episodes consistent with Sandifer's Syndrome which is when the baby is rolling his head and moving his arms repeatedly in attempt to open his esophagus to let the acid out. I called back down the hospital earlier this week and the neurologist said this was reflux activity and to call GI. Luckily, we had an appointment already after the MRI. I was describing the episodes to the GI doc and he started to go off on an intelligent spill about an Englishman named Sandifer and I kinda burst his bubble when I said I found it on the internet the night before....Momma's head got a little inflated for playing doctor but quickly deflated when he said I had to give Bruce a pill twice a day. Yep, to a 4 month old. Chuckle, chuckle, funny man. But, I will say your joke about Bruce's 'Howitzer' antibiotics compared to normal 'pea-shooter' antibiotics was funny...

There are other ways of giving Prevacid but this is the best way to get the medication through the stomach and into the intestines where it needs to be. Bruce doesn't think so and every time I place the little dissolving chunks on his tongue, I go through the CPR steps in my head. I think I get a new gray hair each time! He's been on the suspended version from the pharmacy for a month or so now and it's not working. Babies typically grow out of reflux and it's something we will have to watch, not that we weren't already. When I found the info online on Sandifer's Syndrome the night before the GI appointment, the article mentioned how rare it was for babies to have this. Less than 1% of reflux babies ever show the symptoms but when the article said 'rare', I knew he had it. This kid does rare to a perfection!

Bruce is over 14 lbs and topped 25 inches a couple days ago! Long and skinny, not surprised. His daddy used to be that way....sorry, love, just checking to see if you're reading or not :) Each day I go to get him out of his crib and I see a change in his face. It's amazing how quickly they grow! We have reached the stage of blowouts, though. It's like a light bulb has gone off in his cute little baby head that says, if I lay like this and I grunt like this, I will make it go up my back like this! And to see momma's face is fun! Yay! And I'll wait til when daddy's not home! And I'll do it only when I'm sitting on momma's lap!
Ok, it probably doesn't play out like that in his head but it sure seems like it!

We've been able to get out and enjoy the nice weather some lately. We started what hopefully becomes a new fall tradition of apple picking with great grandma and great grandpa!

I think here is grandpa telling Bruce momma better be careful putting the full bag of apples on the stroller like that....

Because not 10 minutes later I nearly toppled the whole rig, baby and all...

Bruce telling daddy the tale of momma's scary stroller driving later that day...

Still acceptable now, but if this is the case in 30 years, we have to talk, son.

Letting it all hang out during an infusion...notice 'I don't care if you take this picture now but it better not be on the internet later' look on his face....

Learning to smack his tongue like silly momma...you're getting there! Kind of...

Bruce's first visit to the neighbor's dairy when they were chopping silage that day! (More ranch pictures to come in the following posts for those of you who care....we don't just talk baby poop and breastmilk here!

A relaxing day at home with mom sticking her camera in my face...again...

And finally, 'ok, I will smile once more if you would kindly remove yourself so I can watch Monday Night Football....'

And there you have it so far, folks! Later today (because it is after 2 am and OH. SO. PEACEFUL), Bruce and I are heading to the Amish shops at Jamesport, MO with some of the lovely ladies in my family for our annual kickoff to the fall season! Daddy has requested that we bring back some baked goodies and a box of gate handles from the farm store down the road...he seriously knows where to find the good deals when it comes to farm supplies...I know this because I have driven all across the countryside for him...and I love it! Still checking to see if you're reading :)

Happy Fall, y'all!!

GOD BLESS!!

And now a word from Waylon Jennings...

"So wherever we're going, Lord, it's good to be at home..."

Home. Last Thursday Bruce was discharged at 12:05 pm. Within 10 minutes of signing on the line, we were loaded down like the Clampetts, complete with baby and six weeks of living supplies from our stay at the Ronald McDonald house, and headin' north with the hammer down, breaker-breaker rubber ducky, 10-4 good buddy, we got us a convoy, mashin' down through Wolf Creek Pass -- all of it. We made a quick stop at my mamacitas long enough for her to pack us moreso with her home cooking and tell her grandson to get his butt home, and to stay there. 20 minutes later, I made the realization that we completely bypassed the CMH inpatient pharmacy and forgot to pick up Bruce's EIGHT prescriptions.

And so, at the Holt/Lathrop exit on 1-35, we made a u-turn and headed back for the drugs.

In honor of his round the clock drug regiment, I would now like to inflict upon you the song that has been stuck in my head since Thursday night...(lyrics have been changed):: One, two, three o'clock, four o'clock, MEDS! Five, six, seven o'clock, eight o'clock, MEDS! Nine, ten, eleven o'clock, twelve o'clock, MEDS! We're gonna medicate Bruce around the clock tonight!                           You are welcome!! :)

Sleep isn't in my repertoire anymore and it's fine by me! Sooner (before later) it will catch up and I probably ought to be lying peacefully in my bed now but sitting here and watching Bruce make the funniest facial expressions while he dreams and listening to his daddy saw logs with a team of professional wood grinding beavers, is my slumber. Home. I can't say it enough!

So far in the last few days I have spent some amazing time with friends and family, eaten the best t-bone ever smoked and mamacita's best-ever banana bread, napped a ridiculous amount of times with my baby next to me, re-inserted the feeding tube in his nose because it wasn't in the right place, added 'maternal apothacarist' to my resume, cleaned and organized for hours without ever making a dent in the mess and mastered the moby wrap while wearing a robe. Yes, it can be done.

This weekend has been the best of my life, ever. I fall more and more in love with Bruce everyday and I enjoy learning and seeing who he is becoming, outside of a hospital crib. He's been doing fantastic. The feedings are going better but we do still have the ng tube in and will for a while and most of the medications should end in a few weeks.

We leave for KC in the morning for his next enzyme replacement therapy and a slew of appointments on Wednesday: Cardiology, Neurosurgery follow-up, Infectious Diseases and the Special Care Clinic. I feel like an anxious ex-con getting ready for a parole hearing, afraid of getting locked up again. Which is probably just the exhaustion setting in because I know Bruce is going to do JUST FINE with his appointments and we'll be home before I know it, back to my robe and snuggling with my son while he sleeps and I watch all the trashy Lifetime and TLC shows...He really is making progress and growing a belly like his daddy's! (But it's a cute belly, John! :)

It's great to see everything so green in northern Missouri! You can laugh all you want that this town has more digits in it's zip code than actual residents but I wouldn't trade it for anywhere. Someday, the beach but that's years down the road...we've got a family and cows to raise first!!

GOD BLESS!!!!

Prepared for battle...

The next time I write this blog, I plan to be naked, sitting in a bean bag chair eating cheetos! Ok, not really but we will be home and I more than likely will be in my robe...because who needs clothes when you're home?!

WE'RE GOING HOME. HOME!!! Like, tomorrow!

Six weeks, four days. Whew. I woke up this morning tired but prepared for battle. Bruce hasn't been eating well this past week and a half and it is really the only thing holding him up for discharge. It's not easy to make a three month old drink a bottle he would rather pitch it out the fifth story window and it's not like I can threaten him...'if you don't drink this bottle, no sweet-ease for you, son!' (Sweet-ease :: sugar water for babies you can dip their pacifiers in...aka, 'baby crack'. And as John found out the hard way, it cannot be recreated with Splenda). Besides, those threats wouldn't work in a children's hospital anyways....as soon as moms leave, the nurses let them have whatever they want! For Bruce, it's being held and hanging out at the nurses' station. But back to the battle.

There are several theories why he's not eating. Momma's is that his belly gets pumped full of a variety of meds everyday and quite honestly, his formula is gross. I can't blame the kid and he's been through a lot the last several weeks. Last week they placed a ng tube for his feedings. Bruce gets first chance to take what he can manage out of the bottle before his belly gets upset and he gets cranky, then the rest goes down the hatch in a small tube in his nose. Bruce hates them and that's why there's more tape on his face to hold it in place than one of John's attempts to wrap a Christmas present.

Come to find out, it is very common to send kids home with ng tubes who do have issues eating and getting back on their feet. But our docs weren't having it. We were staying put. This time yesterday, I thought our current attending was a puffed up peacock with too many ruffles in his feathers so I kept my mouth shut and waited patiently for John to leave for the ranch for a couple days so I could do some investigating. With a little help on the inside from one of the patient advocates who was assigned to Bruce's case in the very beginning, back in the nicu days, and I have had the wonderful pleasure of many a deep conversations on life and it's craziness, communication was restored and the docs were finally on the same page as us: We really don't want to live here forever. And compared to taking a baby home with a picc line, a feeding tube is small fish.
But I say that now....just wait til he pulls it out himself and we have to place it back in and he's screaming baby obscenities at me!

So, my battle preparation this morning, complete with warpaint (really just my makeup because I could use all the help I can get), was for nothing now that doctors know we feel confident we can manage with the equipment. And that we are capable of doing it as parents. And if they knew that we are capable of doing it because we both grew up on dairy farms where it was not uncommon to tube feed calves, well then they'd probably take our baby away for comparing him to a calf.

And that's that! We get to be home this weekend for the holiday, the unofficial celebration of the end of summer that Bruce has spent in a hospital! I'm going to use this to signify the end of his crazy fluke illnesses and a fresh start for our future...we will always be familiar with procedures and doctors and hospitals for his lifelong diagnosis but it's nothing our family can't handle....from home!!!

So now for the real battle.....getting Bruce adjusted to no cute night nurses to cuddle with..............

a BIG difference!

WOW! What a week! So from the last post it's been a whirlwind but I think things may finally be settling down for us here at the hospital...today marks 5 weeks 3 days that Bruce has been admitted for the meningitis and the complications that came with it. Which is exactly how long he was in the NICU! The kid just turned 3 months old on Saturday and he's already spent 11 weeks so far of his life as a patient of Children'$ Mercy...

Here are things he has no clue about in the big wide world: fresh air, clothes, his Gus dog, days without doctors poking and prodding, cows, and quiet nights without nurses holding him. The list goes on. It's going to be a reality check for him when he hit northern Harrison county and it's quiet...John's going to leave for the ranch and Bruce will take one look at me and decide this party doesn't have enough people and I should call some friends over. He prefers the blondes but has settled for brunettes.

The night before Bruce was due to get the brain shunt, he spiked a mysterious fever so it was postponed. He was pretty miserable from the pressure in his head and something had to be done so his neurosurgeon took out 26 ml from his left ventricle and rescheduled the shunt for this week...the next day, the pressure increased again and more fluid had to come out. This was Saturday and the on-call neuro came in....he walked through our door in his street clothes and looked like he just came from his kids soccer matches. Momma didn't panic too much. About 15 minutes later, our regular neuro called in and asked to speak with us. He said he was booking the operating room for the very next morning for Bruce's shunt because it couldn't wait any longer. As miserable as the little man was, John and I were glad to be getting this surgery so he would just feel better. I was missing my baby.

Sunday morning came and the surgery went great. It was eerily quiet in the recovery room while we waited for the show to get on the road but nice for all the extra attention Bruce was getting from the nurses and doctors. The shunt was placed under the skin on the left topside of his head and the catheter was routed on the inside behind his ear down to his belly. Kinda crazy looking but it has done wonders for his mood and he's like a brand new baby without the pressure. It's something we'll always have to keep an eye on and be cautious of when he's playing and growing but it will give him some wicked awesome stories for the playground one day.



Recovery has been great and no signs of complications from the surgery. His feedings haven't been great so that is a concern and just about the only thing hindering our 'escaping KC' plans...Physical therapy has suggested acid reflux so we're going to try antacids and hope that's all it is. Other than having some spells when he's fussy and not eating, Bruce is smiling and laughing and melting momma's (and all the nurses and female doctors) heart. His eye movements are much better and he's still kicking like a ninja. His neck is getting stronger but it'll be a while before he's sitting on his own but it's ok; we love the HAPPY BABY!!!


P.S. I have a TON of pictures to share but I can't get them off my camera onto this computer so I'll put a slideshow one day of all the hospital craziness and his way too cuteness! (Thanks Millie for letting me steal these!)

Quick update...

This quick update turned into a longer one...it happens when there is a lot going on with Bruce! I may have temporarily lost my wittiness and my stress level within the hospital is at an all-time peak. I have my fair share of rope burns from hanging on to the end of my rope but the good news is that I'm still hanging on...So have no fear, you will not see me belly flopping off the construction crane next to CMH on the 10 o'clock news.

Ok, so the abscess from the bacterial infection in his brain is regressing. Great news! Bad news? His cerebrospinal fluid is increasing in his brain ventricles because some residual sediment from the infection that won't allow the fluid to reabsorb, as it should. Did you know that a baby can produce up to 700 ml of cerebrospinal fluid in one day? Our bodies make it and reabsorb it constantly but Bruce's body is having fits. No big surprise there. Pressure is building in his head and causing him to have a serious headache; yesterday, the docs did a lumbar puncture to help relieve some of that pressure. They took out 12 ml from his lower spinal column but so far today, little man is still pretty sore and fussy.

Tomorrow, neurosurgery is going to place a reservoir in his brain to be able to aspirate some of the fluid as needed when he gets too much in his system. Eventually, Bruce will "more than 98% sure" (words from the docs themselves) need a brain shunt to help him long term absorb the fluid that isn't absorbed. One major concern with that is infection and Bruce has had more than he needs of that so everyone is extra cautious as when to do the shunt placement. Infectious Diseases has given the go ahead but each neurosurgeon is different and ours would like to wait longer til he feels Bruce is ready...I'm on board with him. So, we do the Ommaya Reservoir in the meantime. Which is not a vacation spot in South Africa, aunt Kacie...

Bruce had another EEG this afternoon which he and momma slept through. Those rooms are too dark and too quiet, which again proves Musgroves can sleep anywhere...The reason for the EEG is that the docs suspect a little more than usual seizure activity going on in his head, which is more than likely from the increased pressure. They've already increased his medication but this will give insight as to what's going on in there for sure.

On the Pompe side, Bruce got his Port-a-cath on Monday and did just fine during the procedure. He is probably one of the littlest guys to ever to receive this appliance...we haven't heard the officials say that he is but there are hospital rumours that he just may be. The port is titanium and is just under his skin on his right chest and is what the enzyme replacement treatments will be administered through every other week. As he grows, Bruce will need different ports but they should last a couple years...hopefully. So as his growth spurts come, he'll get longer jeans and new ports!

We're hanging in there. I could mope and groan and complain but truly, it could be worse. I have to find the humour in all of it and thanks to my sister, I now also think of 'Ommaya Reservoir' as some distant, turbulent war-torn province that is always on CNN news. Bruce has gone through more than a lot of 80 year olds sitting in a nursing home and the kid complains far less than any of them. I'm proud of our little boy but I can't wait til all of this is a (MORE THAN A FEW) page in the baby book!

***Update to the update.....literally within the minute I posted this blog, our neurosurgeon came into the room to discuss tomorrow's plan. Bruce IS getting his shunt at 7:30 am and NOT the reservoir. Lots of risks to consider but he feels the benefits will be better for our little guy...and so once again, we signed another surgery consent form....

A Bob Marley and Diet Coke kind of day...

Two of my most memorable milestones from my pregnancy with Bruce: when the baby book said he could hear from inside the womb and when his testicles dropped...I was a proud momma. Of course, I had to celebrate the first with a couple of my favorite sounds in the world: Waylon Jennings and a Dodge diesel engine. To do so, I took the old Dodge work truck with my Waylon Jennings burnt cd permanently stuck in the cd player on a back road cruise to the home office one afternoon last fall. Later that night, I played ocean waves against my belly from my sound machine. The second milestone wasn't so much a celebrated event other than I told the whole world my boy had balls now...

I love all kinds of sounds but my new favorite since little man has been born is the 'Ah, dammit!' noise he makes when he can't get all the sneezes out. Because he's been sick the last several weeks, he is a little behind on developments...he's not quite able to hold his head on his own and rolling is pretty limited to side-to-back although he kicks like a ninja! He has found his ears and does move his hands to mouth but more importantly to momma's heart than all that, he's smiling like a champ and starting the cooing sounds! My eyes still tear up when he looks at me and gives me that toothless grin! This last week has been amazing to see the changes in him since it seems like the brain pressure is down from the abscess and the antibiotics are taking effect. Tomorrow marks 12 weeks of age and for all the boy has been through, I don't think he'll have much trouble hitting more milestones in the future!

Yesterday was a Bob Marley and diet coke kind of day...doctors were in and out, he had a hearing screen he refused to sleep for, an outpatient pulmonology appointment on the other side of the hospital we had to hike to and his NG (feeding) tube was removed. ((I did manage to score a pan of the Pioneer Woman's chocolate chip cookie cinnamon rolls from Aunt Kerrie and some great family visiting including some sisterly bonding I don't get enough of these days!)) I cracked a case of diet coke pretty early in the day and yesterday afternoon, after the medical masses left the room, I turned on some Bob Marley and little man snoozed on my lap. Momma loved that bonding time. Music is HUGE in my life and I love all kinds...I'm hoping Bruce will too! Today's kind of a wild card day and I'm playing Foster the People...see, I'm a cool and trendy mom! HAHA Yes! I did just say that.......

Infectious Disease holds the reins on the going home wagon. Bruce was yet again hot topic of another conference yesterday to discuss the best antibiotic treatments for him since he's such an odd case. From the meeting of the minds, it was decided to up vancomycin iv dose and an oral will begin today. All of his functions are looking great so far so they feel they are on the right path. We're currently awaiting MRI results from this morning to check the regression of the abscess, if any. Bruce may not be a prodigy pianist or a chess club champ (yet) but he has had five MRIs and has never been sedated. I wonder if that comes on a bumper sticker?

Pompe wise, the enzyme replacement therapy went great this week and the Genetics team is truly impressed with how he is responding. They can only recall a few cases of infantile Pompe in the last decade at CMH and Bruce has far exceeded any of their expectations. We're gonna keep on, keeping on with making progress!

John has been whipping out hay bales like no one's business back at the ranch thanks to help from neighbors and my awesome cousin, Dalton. I'm hoping if we build Dalton a house and find him a non-Amish lady friend, he'll make the move to the north and work full time. Or not, but it's a nice thought! John is heading back tonight and we're planning for a nice and relaxing weekend...I'm sure it'll last all of three minutes!