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!