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!

Finally....A Good Note!!

There has been nothing normal about the last two and a half months with Bruce. His health hasn't been the greatest. He's on old folks medication and gets all kinds of tests and labs drawn each week. John and I know half the medical staff here at CMH and have regular conversations about Bruce's diagnoses and what's next in the plan to get him better.

And if you've only read one of my blogs in the last two and a half months then you know well enough that this has become the norm for us as parents. Rarely do we get to have conversations with positive news and never have we had a conversation like this before. Until Thursday.

The nurse practioner for our cardiologist called our room Thursday evening:

Her: "Bruce's echocardiogram came back normal."
Me: "Ah. Well, you mean normal for Bruce."
Her: "No. Normal."
Me: "Normal, normal?"
Her: "Yes, normal."
Me: "What does 'normal' mean?"

She went on to explain his heart muscles in the left ventricle weren't thick like were when he was born. By this time I was so shocked I blocked out rest of the conversation and it wasn't until the next day we ran into her and our cardiologist in the hallway did it really sink in his heart was normal. Luckily John was there with me to be witness!

Our geneticist, the doctor who in the beginning was the one who suspected Pompe Disease was the culprit of his heart disease, came to our room the next morning and looked almost as excited as we were as parents for the news. The enzyme treatment has made a tremendous difference in Bruce's health and thanks to her and her team who have fought so hard to get the Pompe diagnosis and the treatment ordered, we have seen our first and most important improvement being his heart. Medicine and prayers are powerful.

Bruce can never go without the enzyme treatment every two weeks and as his momma, I will make damn sure that whether it rains 20 inches or snows 20 feet, John will rowing us in a boat or blading roads with the tractor to get where we need to get. I don't think he will mind one bit! I have had days of doubt and in the beginning, I was a hot mess. John has always been a rock. He's never doubted and always kept me strong if I was even remotely worried too much. He's one proud daddy of his son.

Yesterday Bruce had another procedure to help drain his brain abscess. There wasn't as much gunk which can be a good sign but the witty neuro and his team of blondes are keeping close watch. The part of his brain that is being affected is what determines personality and moods and some motor skills. Yesterday morning, Bruce was full of smiles and was making great eye contact and melting his momma's heart. If any brain damage, I don't see it. Time shall tell but this is a strong kid. He's already whipped hypertrophic cardiomyopathy.

That's the latest and greatest health wise. On a sidenote, not all nurses are created equally and I've been getting into gangster movies thanks to Mob Week on AMC. But don't worry, I'm not getting any ideas.....yet. I also have been watching Shark Week on Discovery and I really want to swimming! I might just be getting a little stir crazy in these walls but I'm not about to stuff needle-toting nimwits down the elevator shaft or jump into a feeding frenzy of tiger sharks off the African coast.

PLEASE keep your prayers and good thoughts coming!! They never go unappreciated! Medicine may be working in Bruce's body but it takes more than just that to keep him getting healthy and his momma from tossing malfunctioning oxygen sensors that beep every five minutes out the 5th story window!

Really. We're still here.

If Children's Mercy had an A-List, John and I would be on it. Let me rephrase that. Bruce would be on it. John and I are just the ones who carry his luggage and change his messiest diapers when the nurses sneak out of the room when they smell that smell.

But really, Bruce has caused quite a commotion among the doctors and nurses in this place and just about every department has heard his name and a brief synopsis of his case. And as Bruce's parents, we spend a fair amount of time in hallways and in the cafeteria and just passing by updating all those who are curiously wondering about the latest this mystery child has produced.

And latest would be the brain abscess. We came in for pneumonia, which led to meningitis, which led to seizures, which led to the brain abscess. It has taken three procedures to drain it, so far -- the most recent first thing this morning, and many tests and cultures to determine what caused it. We're making progress, even though it seems like one step forward and a half mile back. The highly intelligent brainac docs tell us that it's common to have to drain these multiple times and eventually it'll subside. On a side note, our current neurosurgeon has one of the greatest & driest sense of humor I've known...it's been refreshing.

A sample of the abscess was sent to the University of Washington last week to see what was causing it. Today we learned the name of it and I can actually pronounce it. John, however, cannot. He said it right the first time and every time since has been a complete different version...I bet he failed as a child playing the telephone game. Bacillus pseudomycoides. Commonly found in soil, but then again so is everything else. We live on a cattle ranch. I make a living selling agronomy products to dirt farmers. I ate a healthy dose of dirt as a kid and I am willing to bet John did too. Bruce, on the other hand, is having an adverse reaction to what's in soil and he's never even seen it before. Let alone swim in a mud puddle or lick the popsicle he dropped on the ground.

The infectious disease doctors have no idea how he got the bacteria into his system. There are several theories and lots of possibilities but there is no way to tell now. We all just hunch, for lack of better medical term. And it sucks. Another medical statement we hear from the docs often. It could have come from anywhere and is actually a common skin contaminant. I probably have some under my fingernails now. But how he got it is in the past and now we need to focus on zapping it out of his system so his head can heal.

Bacillus pseudomycoides is not known to cause brain abscesses and herein lies where the highly intelligent team of docs are confused...why now. Why this time. Why Bruce. He's been on some hefty antibitiotics and now he will be on some different ones. There really is no antibiotic specific for Bruce's case but the team has confidence in this different antibiotic and hope it will do what needs to be done. Again, Bruce is a medical wonder. At 10 weeks of age.

So, that's the latest. We lost track of how many different rooms we have been in when we hit eleven last week. We have learned that when Bruce is feeling good he loooooooves the night life and the ladies. He has spent many an hour out at the nurses' station "just cuddling" while either John and I are away for the night or we're sleeping because that's what diurnal mammals, or parents who are awake during the day, do. That habit will have to change when we get home or John gets to hire a cute nurse to tend to the baby in the wee hours....which ain't happening, let me tell ya.

Moral has been tested but it hasn't been lost. John and I are doing as great as can be expected, maybe even a little better, and we still have not killed each other yet. My mammary glands no longer hate my guts and I am sad to have seen them go. Maybe one day I will get those implants...I plan to unpack the bags underneath my eyes soon with a little rest and relaxation but I'm not too worried about how frumpy I look as long as John looks at me the way he still does. Oooh la la....

Bruce has proven to be a perplexity to raise and I feel a little robbed of some of the mommy time I'm missing out on when he's so cute and cuddly. Soon, he will be a raging ball of testosterone with the capability to walk and drive and form his own opinions that probably will differ from mine...but nonetheless, he is and always will be my handsome and truly amazing little boy whom I undoubtedly love more than anything in the world.
No matter what he's got going on inside...

And.....We're back.

Bruce is an amazing little creature. Today marks two months he's been in this world and every day I'm proud to be his momma. With that said, it's been a long two months. Parenthood is life changing, no doubt, but he's brought with him a whole new meaning to late night feedings and diaper changes. A typical day for me as a parent includes a talk with some sort of extremely highly intelligent doctor with more degrees hanging on their wall than I have wallpaper in my house. It's become the norm for us as parents to discuss the insides of our little boy with health care professionals like we're talking about the newest calf crop this spring. It's just natural. Which is unnatural...

I'm on a very humbling path right now in life. As many days as we've spent in Children's Mercy, one has to be. My world, my life, my very existence has spent more days than not swaddled up in a generic hospital blanket hooked up to monitors keeping record of his every breath. He takes it all in stride because it's what he knows right now. It's mine and John's responsibility to make sure that he knows life outside the joint as what it is: full of family, love and cows...and one very confused basset hound who wants to play with the little creature in the car seat rather than just sniffing him!

Last Friday at home at the first sign of a fever, John and I loaded Bruce up and headed for Cameron to see our primary doc. After a quick look, she sent us southbound to Children's Mercy. He was fussy and irritable for him and I didn't get the quick fix I was looking for. I know it doesn't work that way...We got to the ER and they covered the basics: blood cultures and xrays. Bruce was a trooper even though for not feeling good and they sent us home that night with amoxicillin and a diagnosis of a little bit of pneumonia in his upper lungs. I thought we were done there. Progressively over the weekend, little man got fussier and if he could've talked, I think he would have told me he had a headache equivalent to the hangover to the time I swam in the Chariton River fully dressed and lost my cell phone in the bathroom closet...he was miserable. After two long nights of no sleep and one momma at the brink, we made our way back to the ER Sunday morning. More blood tests and xrays showed the pneumonia was clearing up but the spinal tap indicated he had meningitis. No wonder the kid hurt.

They sent us up to the sixth floor and we were settling into our corner suite with a view, twice the size of our NICU room, when Bruce had his first seizure. Within minutes, the rapid response team was sounded and we were swept away to our new room in the PICU. He's never had seizures and within 15 minutes, he had two. All day long, he had three total. More tests were ordered and John and I got our game faces on for another day of consults. They were fairly certain it was bacterial meningitis but the source of where it came from was and is still sketchy because of the amoxicillin doses he had at home that did its job and killed some of the bacteria. Because of the seizures, an EEG and MRI were done with abnormal results. EEG showed constant seizure activity in his brainwaves and his MRI showed a mysterious spot in his front right lobal of his brain that is more than likely result of a mild stroke which is more than likely from the meningitis. The neuro surgeon really couldn't commit to a diagnosis today so that's where we're at now: 'more than likely'. Another spinal tap will show us how he is responding to the antibiotics (Infectious Diseases had a conference today where Bruce was a main topic -- let's hope all those minds agree he's on the right antibiotics) and we also should have an idea from more scans how the drugs are affecting, if at all, the mysterious brain spot.

None of this is related to the Pompe Disease. He can still receive his infusions as scheduled as long as he is feeling good, which is why we were so concerned in the very first place when he spiked a fever. Fevers are no good-a. Our goal is to make sure he's as healthy as can be and we were lucky as well as on the ball to get him where he needed to be so the docs can do their job!

So now, we wait for antibiotics to run their course and get little man back up to eating and being his handsome, old soul self. Each day is an improvement. We'll have more doctor visits to add to our already busy schedule once we get out of here again, but we'll take it all day by day. Like the overgrown white sasquatch, Winter, from Santa Claus is Coming to Town -- it's one foot in front of the other. My little family, the three of us, are all inhabitants of the island of misfit toys...On a side note, John and my blood test results came back. Bruce has both a deletion and a mutation of the chromosome that determines his Pompe Disease. The deletion came from John and the mutation came from momma...see? We're a family of misfits!

I share all of this info for the whole world to read because if I didn't have a way to get this off my chest, I would explode. Every day folks here ask me how I am holding up and if I'm OK. I truly am OK. Of course, I'm tired. That's to be expected I would reckon. But, writing/blogging/updating the world on Bruce's conditions is my way of dealing with Bruce's conditions. And, of course, my amazing family and village of support keep me up and running too. John and I are as best as we ever have been and I find strength everyday. In God, in Bruce, in this big ol' crazy world.

We're back on the sixth floor where we were Sunday, briefly. We have a corner penthouse complete with a bed for momma, a recliner for daddy, a bathroom with a shower, a tv, mini fridge and a window view of the chopper pad 20 feet away that brought Bruce here when he was 3 days old...it's like we've come full circle!

The Great CMH Escape...

For a while there, I didn't think they were going to let us out of the joint and keep our baby for ourselves....but they did! But they forgot to send home one of those nurses who kept watch on him 24/7. And fed him. And changed diapers. And gave meds.

When they first even mentioned the word 'discharge' I sent John a text about it and made him swear to keep it on the down low. I didn't want to jinx it. I had this horrible vision in my mind that we would jump through all the discharge hoops and get all excited and then they would find something else to do to the wee one....and you know what? THEY DID! They day we were all set to leave, they decided last minute to do another blood transfusion. Kind of like one for the road. The very nice and polite doctor smartly kept his distance from me when he told us they wanted Bruce to stay one. more. night. for observation. The man was small enough I could have taken him in one tackle...and he knew it. But, everything worked out just fine and the extra day was nice to have to get everything in order. I was a little overwhelmed, but in a good way. One week ago last Thursday, when the nurse FINALLY walked us to our car and made sure Bruce was strapped in tightly in his seat, we closed the doors and I hollered to John, "PUNCH IT, Margaret!!" Of course, he did not punch it and drove us home in a non-racecar/blue hair/road-rage driver manner while I sat in the backseat with my eyes forward in case a random nurse or doctor came running from the elevators looking to stop us. Bruce slept.

The first few days out of the joint we stayed in Liberty at my mom's house just in case we needed to be close. Within 10 minutes of being there, the doctors called. Twice. Luckily, just for some minor medication changes..whew! It was going to take a search team to track me and my baby down...I can pack a diaper bag well enough to hide out in the woods for as long as it takes to throw off the hounds. The first few days it was fun being at home with the kids to help out...wish I could have packed one of them in my diaper bag to bring to the ranch!

We've had a couple doctor appointments since and with exception of a few minor issues, Bruce has checked out just fine. The cardiologist was impressed with his tone and function given his heart condition and we return in one month. Our next infusion is Monday and we look forward to being outpatients then! Within 24 hours of being back in Hatfield, I purchased a giant dry erase calendar for the wall and it's filled up pretty quickly with upcoming appointments...which is just fine by me! As long at the end of the day he's in his crib in our home, I'll drive daily to Timbukto for doc visits! Which is pretty much the same distance from Hatfield to KC :) The wee one has a great team of doctors watching out for him and we're very lucky to have them on our side.

It's fantastic to be home and getting the house back in order...being gone 6 weeks, you knew there had to be a few issues but the ac is blowing cold air again, the Dish guy comes back next week to finish fixing the satellite and a new refrigerator has been ordered and will be delivered the end of the week...compared to what we've been through lately, un-thawed meat in the freezer dripping down into the wires of the fridge was nothing!

Most importantly, Bruce is doing as great as he can be. Fortunately, he loves really stinky formula and has been eating like a champ...which is beyond great! I can't complain about the middle of the night feedings because I love it when he eats like a horse and he's starting to get those cute little wrist fat rolls! Sadly though, his hairline is starting to fill in. I am really going to miss the old man receding hairdo he has...

John is getting caught up at the ranch and VERY happy to have us back in the neighborhood to cut down on his trips up and down 1-35. Out of the whole deal though, he's a much better city driver and I feel comfortable riding with him downtown KC. For the first time ever. All I need to work on now is getting him to quit talking to the other drivers in their cars...they cannot hear you.

It took John four and a half coolers to get all of my milk out of the fridge...he was a trooper hauling those through the hospital to the car because you know people weren't thinking he had beer in there. Or maybe they were. Glad no one stopped him to look inside!! ( I know, I know...again too much information...)

We'll miss our friends and staff at CMH but it's not like we'll never see them again! We will. Often. Before we were discharged, Bruce sent his buddy next door, Lucas - who has been in much longer than us, a giant cow balloon and one that said, "Milk it for all it's worth." If anyone knows how to milk hospitals, it would be Bruce and Lucas! They were the heart breakers of E-Pod.

We have a long road ahead of us and a lot to figure out on how to adjust to this really different lifestyle, but John and I have great support and through the whole ordeal, we didn't kill each other. Er, he didn't kill me...So we still have each other! John is amazing and I'm very blessed to have him! And, of course, a very handsome little man who is changing and growing daily!

No doubt life will be interesting in the coming months and years and I'll keep you all posted!!! Thanks again for thoughts and prayers and hope life is well with all of you!!

John, Sarah & Bruce