BABY NEEDS NEW WHEELS

The time has come. No use putting it off any longer. Not sure how it's going to happen, but it will happen. So here's the poop: we need a new van. Not a “new” van, a different van. Something bigger with less mileage than the minivan we currently have. Our van has served us well, but Colby has outgrown it. Plus it's starting to rack up some miles - 112,000 miles currently. Time to kick in the fundraising again! Hey, if we can raise the money to help us get a house, we can raise the money to help us get a van. That's my thought anyways.

So you may be asking, how much money is needed? I don't know. I have no idea actually. The ideal situation would be we get a bigger and better van for Colby and a little, cheap car for me to drive to work, run errands, etc when I don't have Colby with me. Even if I don't get a car and we get another van only, I can't imagine we'll find something decent for under $30,000. Ouch, I know. Trust me, I know. Nice wheelchair accessible vans ain't cheap!

The next question you might have is, when are we getting this van? I don't know. Again, I have no idea. I'm predicting it will take a long time to find the kind of van we need. I also don't know exactly what I'm looking for in a van just yet. Better storage, room for more passengers, less mileage – that's as far as I've gotten. There are advantage and disadvantages to rear vs side entry, lowered floor vs raised roof, etc. Gives me a headache just thinking about researching to find what we need, then actually finding it.

All I know is our current van is aging and is too small to meet our needs. I also know there is no room in my budget for a car payment, so the money will have to be upfront to purchase. And how do we come about getting this money? Work our booties off for it, that's how. Fundraiser after fundraiser after fundraiser. Been there, done that, happy to do it again to get what Colby needs. I think after this venture is over, I should hire myself out as a professional fundraiser. I'll also Google/research any grants or not-for-profits that might be out there to assist in financing. Or win the lottery, which would require actually playing the lottery. Or, if anyone out there is looking for a good place to drop $30-$40,000 – I know a kid. Stay tuned, more fundraiser fun and details coming soon!

CRA-CRA WEEK LAST WEEK, NOT SO MUCH THIS WEEK

Last week is a total blur. Monday a friend of mine came over and we loaded up the table and chairs I had borrowed from another friend for Colby's birthday party. She drove 25 miles to return them for me. See what about I mean about having wonderful, caring friends? Then Colby had an appointment here in town with his pediatrician. Poor bubba had to have 2 immunizations, one in each arm. Boo. Then I wanted to go see my dad. Got home around 8pm and started packing us up for the next couple of days. Colby had a PT evaluation at Cincinnati Childrens last Tuesday. It was his only appointment for the day. I got the brilliant idea that we (Colby, nurse and I) would stay up there overnight, and we would take Colby to the Newport Aquarium on Wednesday. So I printed out my “Colby out of town list” and started packing, organizing, repacking, reorganizing. Took the front seat out of the van so we could take the portable Hoyer lift with us. Bungeed down that sucker. We left home in time but somehow managed to be 11 minutes late for Colby's appointment. I HATE being late for appointments, especially Cincy ones. I was not a happy camper. Once we got to the hotel things didn't get much better. Unloading, moving furniture around in the hotel room, trying to find everything I packed, which trust me, is sooooo much stuff. The nurse and I were exhausted by the end of the night. Then back up at 6:30 to get Colby ready and everything packed and reloaded to head to the aquarium. And Colby didn't even like it! He had a high heart rate most of the time and kept shutting his eyes, especially when I'd say oooh Colby look at that. Maybe he was tired from his shots. Maybe he's just not that into aquariums. I feel like all that extra work, and money, was really for nothing. But now I know, take the aquarium off the fun things for Colby to do list. The experience was quite un-fun actually.

Wednesday we got home at 3:10 and I had to be at work at 4:00. We had to unpack the equipment and supplies Colby needed for the rest of the night. I was slinging cords, CoughAssists, vents, IPV machine, trying to get out the door to work on time. Needless to say, that didn't happen. Got there a little late, but I stayed late and finished out my time. I was one tired tater by the time I got home.

First thing we did Thursday morning was give Colby his bath! He usually gets a bath on Tuesdays and Fridays. Yes, I only bathe my kid twice a week. Don't judge. He does, however, get washed up every day. You know, all the important parts. Well, that didn't happen since we were out of town, so the first order of business, after giving meds and respiratory treatment, was to wash that stinky boy. He had PT/OT at the Kids Center later that day. We had to cut his therapy a few minutes early. I needed to drop off Colby and the nurse so I could make my dentist appointment at 2:45. I had already rescheduled the appt twice when my dad was so sick. So I was determined to go and get it done. I loathe going to the dentist. Anyone who knows me knows I freak out even having my teeth x-rayed and cleaned. I have a tooth that has resorption issues. I thought you just wait. This will be the day they tell me I can't wait any longer and the tooth has to come out. But, I caught a break there, thank goodness. They told me as long as the tooth wasn't bothering me, we'll let it go until it does. Fine with me. Last thing I have time or money for right now is dental work.

Friday Colby had the last of his summer camps at the Kids Center. There were 4 camps, and he was able to attend 3 of them. I didn't know how it would go with our schedules, the heat, etc so 3 out of 4 isn't bad. The last one's theme was carnival, and there were all kinds of fun activities the kids were doing. Colby got to smash a shaving cream pie in someone's face and also dunk someone in the dunking booth. He had lots of assistance to achieve those tasks, of course, but still, that's fun stuff! We got home and let Colby take it easy the rest of the day. Then that night we took him to his first concert! I local band, which I happen to love, was playing a free outdoor concert. The weather was beautiful so I thought hey, we'll give it a try. If Colby hates it, we pack him up and come home. He loved it! Absolutely loved it! He never got fussy once. Heart rate stayed nice and low. He watched the band, the people dancing. Who knew?! Note to self - no to aquariums, yes to concerts for Colby.

I was never so glad to see a lazy weekend in my life after that week. Colby had pet therapy this past Saturday at noon. But other than that, nothing else scheduled that we had to do. This week is already better. Today the only thing we had scheduled was Hosparus came out to check on Colby, as they do every few weeks. I was able to run some errands and get the rest of the luau decorations down. That's right, Colby's birthday party was 9 days ago, and I just now got all the decorations put away. Again, don't judge. I took care of the important stuff right after the party. Put the leftover pulled pork and cake in the freezer, and put the extra beer in the fridge. Priorities, you know. Another friend was nice enough to help me get everything packed up today. She returned the decorations I borrowed from the Moose Lodge, so that's saves me an errand to run. Ah, such wonderful friends. Don't know what I did to deserve such caring people in my life!

Ok, I've rattled on long enough. Time to clean up the mess where I fried zucchini and start getting ready for bed. Looking forward to zero scheduled appointments tomorrow!

I'M SUPER TIRED, BUT SUPER THANKFUL

I wanted to thank my family and friends for always being there for Colby and me. And I mean ALWAYS. This has been an odd year for me. I've had the lowest of lows and the highest of highs. And one constant has been the unwavering love, support and care from those closest to us. Please know I see it and feel it. Please know I appreciate it so much. Please know I don't take it for granted. Seems like I'm always needing help in various ways. I swear there are days I don't know whether to scratch my watch or wind my butt. Lately my dad has been ill, and I've learned the hard way you can't be two places at once. When I'm with Dad, I feel like I should be with Colby. Then vice versa, of course. There have been times my Dad has been really sick, sort of sick, not too sick, doing great, doing not so great, sick again. Those of you dealing with aging parents know exactly what I'm talking about. I always thought when one of my parents starting showing signs of illness and aging, I'd be able to handle it. After all, look at all I've been through with Colby, right? Plus having a parent get sick is the more natural “circle of life” occurrence in life, right? Wrong! It has been just as hard watching my dad be sick than anything I've experienced with Colby. I've really had to depend on Colby's nurses to help with Colby. They've been great. I've had to ask friends to help out with Dad when there are times I just can't be there. Everyone has stepped up and been a huge help. Thank you, THANK YOU so much.

This past weekend Colby turned 16. I can hardly believe it. We had a huge party for Colby, complete with 52 guests, a beautiful 3-tier cake, a great luau theme, excellent food, and a fire spinner. That's right, there was a young lady in our driveways spinning fire batons and hoops. Colby's party was one of the highlights of 2018 for me. I wanted to do something really special to celebrate his 16^th. Again, I needed help, and my family and friends were more than willing to help. People helped decorate, bring food, address invitations, run errands, clean up afterward. Whatever needed to be done for the party got done. I loved every second of it. I loved celebrating Colby's big birthday! Having a house full of love and laughter is when I'm at my happiest, for sure.

I just wanted to take a moment to thank everyone who has helped us over the last few months. Thank you for checking on my dad when I can't. Thank you for helping with Colby's party. Thank you for understanding if I didn't return a call or text, or if when you talk to me it seems like I wasn't listening. Honestly, I probably wasn't half the time. I've just had a lot of worries and to-do lists running around in my brain like squirrels on crack. I'd like to say my schedule will calm down soon, but I'm afraid that's not going to happen. A lot of decisions are currently hinging on my dad's health. Colby is due for his next Spinraza injection in August, along with getting back to school. I keep telling myself everything is going to be okay. Take one day, one hour if necessary, at a time. Prioritize, do what has to be done not necessarily what I want to get done, accept that I can't be all things to all people all the time, and just keep chugging along. So, time to watch an episode of OITNB then go to bed. Tomorrow is Monday and I have the to-do lists ready to go.

CINCY VISIT SUMMARY

Our trip to Cincinnati Childrens on Thursday made for a long, exhausting day. But, Colby got all good reports and that's really what matters. It's almost scary how amazingly well he has been over the last couple of years. I know I shouldn't say that – I'll totally jinx it. And let me just say, I have the sweetest, most tolerant, adorable, bravest kid on the planet. Seriously, I do. Colby is laid back, goes with the flow, never seems to get undone about much of anything. Yesterday he was poked, talked to, talked about, driven 200 miles for no-fun dr appointments, and moved in and out of his wheelchair. He handled it all like a champ. Very proud of my sweet boy. Well, I guess I should say sweet young man, since he'll be turning 16 next month!

First order of business was to get labs for Colby. We're following him more closely now that he's getting the Spinraza injections. Since they need to test his coagulation, using his port for a blood draw is not an option. The heparin sitting in the port throws off the coagulation. So that means a peripheral stick for Colby. Sigh. The phlebotomists were fantastic. They accessed Colby on the first poke. Then started to draw the 10, yes 10, vials of blood they needed. Colby stopped bleeding after 7, so they poked him again and were able to get all the samples they needed. Next stop, on the complete other end of the hospital of course, was a DEXA scan. Colby gets Zometa infusions to help with his low bone density issues. So he gets a DEXA scan yearly to see if the Zometa is working. Getting this done was easy peasy. Check it off the list. After that was an EKG and Echo down in Cardiology. Also easy testing for Colby. Then we went up one floor to Pulmonary so they could test Colby's vent settings. They measure his vent pressures, his leak, and I don't even know what else. Basically they said we should be putting more water in Colby's trach cuff. They tell us this every---single---time---we're up there. And my answer, every single times, is that Colby is doing fine with the amount of water we currently have in the cuff. Furthermore, he doesn't like extra water in the cuff. You can tell by watching him he's uncomfortable with it. So, until Colby starts having ventilation issues, we ain't fixing what ain't broke. Then we went back down to the 4^th floor for clinic. After all this testing and running around, it was time for Colby to actually see a doctor!

In clinic, Colby saw the neurologist, pulmonologist, cardiologist, nutritionist and rehab doctor. The nice thing is we stay put in one room, and the docs come in to see us. As I said, all good reports. Not much to tell really. Colby is scheduled to get his next Spinraza, insurance pending, in August. We'll be returning to Cincinnati Childrens to get it done instead of Louisville. Don't ask. It all has to do with insurance negotiations, what the company who makes the drug will and won't approve as far as getting on their free drug program, etc. Basically a bunch of red tape BS. Pulmonary suggested maybe Colby needs a bigger size trach. Maybe. He's never been upsized in the 4 years he's had his trach. We'll address that next month at Colby's ENT visit. Colby has never had cardiac issues of any sort. The cardiologist said we can mark them off the list. Colby doesn't need to be followed by them unless specific problems arise. Yay! We're more than happy to do that. Colby's weight is fine. The nutritionist didn't make any changes to his formula recipe. And I swear in the almost 16 years we've been going to Cincinnati Childrens, I'm not sure what a rehab doctor does. She asked if we needed any new equipment, braces, etc. Nope, Colby is in good shape there. We're working with his PT/OT here in Louisville to get a new bath chair and neck brace. Arrived at the hospital at 8AM, and at 4PM they said we were free to go. We got in some Cincinnati traffic heading out of town, and also some traffic getting back into town on the Watterson. Colby's nurse, Colby and I were all soooooo tired when we got home. The nurse, bless her heart, worked a 14-hour day. And as I mentioned, Colby was so good all day. Never complained or acted grumpy one time. Unlike me haha. Colby's next appointment at Cincinnati is July 10^th for ENT and Endocrinology. This will be an “easy” day for us. Anyone who knows Colby knows getting him somewhere isn't exactly the easiest task ever. But having only 2 appointments that day will be a breeze compared to what we went through Thursday. It's all perspective, right?

Thank you to everyone who wished us safe travels and good luck on Thursday. We did it! Another Neurology clinic over, done and we survived. Time to start working on something much more fun, like Colby's upcoming birthday! He'll be 16 – how in the world did this happen? Slow down, time. Please just slow down a little.

IT'S ALL GOOD

I said I was going to do better at blogging. Obviously I lied. But I do have exciting news to report. The good news – the best news ever – is that Colby has received his 5^th Spinraza injection. He was due for it on March 15. There were issues with Colby's insurance. It changed on February 1^st to United Healthcare (UHC) - the suckiest insurance on the planet. Colby was denied Spinraza twice. Then the company that makes the drug offered to put Colby in their free drug program, meaning Biogen would give Colby the drug at no charge for a year. That's over $300,00 worth of medication being given to Cincinnati Children's! But apparently Cincy hasn't decided whether or not they will accept Biogen's free drug program. Ok, I totally don't understand that. It wasn't just Colby who was getting the screw job on this issue. There were 3-4 patients who were also affected by this UHC denial mess. I was very disappointed in Cincinnati's inflexibility, along with them seemingly making no hurried attempt to work on this issue for the patients who needed a quick resolution. So, there we were. A company was willing to give Colby the one and only drug that would help his SMA, yet Cincinnati Children's wouldn't accept it for whatever reason. Talk about frustrated.

Then enters the family liaison representative from Biogen. She is A-MA-ZING. She works with SMA families and is a crucial link between Biogen and the patients who need this drug actually getting it. She was able to help me switch gears and think outside the box. Long story short, Colby received his Spinraza injection here in Louisville at Norton Children's. I was amazed at how quickly we were all able to pull this together. Colby had an appointment with the Louisville neurologist. Then he needed a CT scan and x-rays. While we were there we met the radiologist who would be performing the injection. Then we got it scheduled and bing, bang, boom, Colby got his Sprinraza this past Thursday. Incredible. I'm so, so, so thankful Colby was able to get his Spinraza within a reasonable amount of time from when it was due. The next injection is due in August, so we can put this worry on the back burner for now.

So I need to clarify something. There was another local man with the same name as my dad. He and my dad lived maybe 6-10 miles apart. Apparently this other man had a wild side and ended up in jail a couple times. When my dad was still teaching, one Monday morning he had to explain to the principal of his building that he was NOT arrested that previous weekend. It was the other guy with the same name! Well, this gentleman passed away a couple of weeks ago. I assure you it was NOT my dad! My dad is very much alive. I've received a couple of condolence calls. Again, it's NOT my dad. Yes, my dad has had some health issues lately, along with a couple of hospital stays. And while I appreciate people reaching out to give their sympathies, again, it's NOT my dad. Nothing like some total confusion thrown into the mix of my already stressy life!

I'll end with this cute Colby story. As we know, most of the time he doesn't use his eyegaze to communicate. Watch YouTube, change the TV channels, sure, but proper eyegaze communication has never been Colby's strong suit. His eyegaze was set up Friday, the day after his Spinraza procedure. His nurse asked him how he was feeling. He went right to his feelings page and said, “brave.” Damn skippy you're brave, Colby Michael. He's the bravest, strongest, most stubborn, wonderful human I know! Sounds like he has some sense of how proud I am of him. Warmed my heart when he said that.

BRONCH AND BS

Took Colby to Cincinnati Childrens today for his annual broncoscopy. Grandpa Kenny was kind enough to volunteer to take us. I had to turn off Colby's feedings at 3:15am. Started him on Pedialyte and apple juice and turned that off at 7:15. My goal was to hit the road between 7:30-7:45, and we pulled out of the drive at 7:40. Nice. We didn't have to be there until 10:15, but I didn't know how traffic would be, so I felt the need to leave a little early. Everything went easy peasy. Colby was very anxious (loud and jabbering) when we first got there. He eventually calmed down. Actually he was awesome the whole time. He's such a trooper! His procedure was done right on time. The pulmonologist said Colby's trach, stoma and lungs all looked “pristine.” That's what we like to hear! Colby had to spend hardly any time in the PACU. By the time we got back to see him, he was already awake and alert. The ride home sucked! The roads were horrible. Colby's head flew out of his head piece several times because we hit so many huge bumps, pot holes, whatever it was. So yeah, I'll be following up on that new head piece for Colby's wheelchair tomorrow. Glad this appointment is done and off the to-do list.

Colby's next Cincy appointment is on March 15^th for his 5^th dose of Spinraza. Ok, let's talk. As of right now, it's tentative that he will actually get the procedure. And I am one pissed, stressed, worried mama at this point. Colby's health insurance changed February 1 from Humana to United Healthcare. Humana had approved all of Colby's loading Spinraza doses without too much trouble. United Healthcare, however, not so much. They are saying that Cincinnati Childrens must order Spinraza from their vendor, Accredo. The Cincinnati Childrens higher-ups are saying no, you can't tell us where to buy our drugs. So there you have it. When I first heard this news last week, I flipped my lid. Actually it's more like I shut down. Shoved and tucked all that worry and emotion waaaaaay down deep. But now I'm wearing my big girl panties and am ready to take on this next challenge. I talked to the drug rep for Biogen, the company that makes Spinraza. She helps families navigate insurance, Medicaid and Biogen to get the drug approved for SMA patients. Now that she is aware of what the United Healthcare issue is, she's on it. Just knowing that someone out there can help me get this drug and the procedure approved for Colby is a huge relief. She absolutely talked me down off the ledge yesterday. She didn't promise me it would all be approved by March 15^th. But she said Cincy and United HC are negotiating to get this issue resolved. She heard maybe by April 1. She told me this is well within the amount of time Colby can receive his next Sprinraza dose. So I'm trying to stay calm. I'll follow up with her later in the week to see how things are going.

Yesterday I worked and Colby went to school, and that's been our week so far. I'm keeping Colby home from school tomorrow. Give him time to get all the anesthesia out of his system. Currently I'm on the couch watching “This Is Us” and the nurse is getting Colby ready for bed. The rabbit and cat are hilarious this time of night. The cat will jump up on the couch with me. The rabbit will jump up on the other side. This freaks the cat out, so she'll jump down. Which freaks out the rabbit, who then jumps down also. I'm just glad they're getting along. Charlotte is still leery of Colby. I think she needs some time to get used to his machines and tubing. But I pick her up and take her to Colby every day so they can get better acquainted. Hoping one day I'll go in Colby's room and see the two of them all snuggly in his bed.

Time to wrap this up, tuck in Colby, feed the fur babies and go to bed. More blogging soon.

SERIOUSLY, THANKS FOR CARING

Looks like it's been a week since I've blogged. Honestly, I thought it was 3 or 4 days. Shows you how well I keep track of time. So what is best? Give a quick update to what we've been doing every day for a week? Just write about the last couple of days? Or forget updating everyone on what we're doing and blog about whatever pops up in this brain of mine. I guess there isn't a right or wrong answer. I do enjoy doing this blog. And I think there are some people out there who actually enjoy reading it – go figure! Mostly my mom and best friends, haha, but that's ok. I know there are people out there who like to know what Colby and I are up to, making sure we're surviving this circus that is our lives. Yes, we are both doing just fine currently. And believe me, I know that could change at a moment's notice. I try very hard to not take the good, ordinary, uneventful days for granted. It's easy to get hung up in the BS of daily life. Especially when Colby's care is so involved and I'm doing much of it solo. Yes, we have nursing help. But trust me, that can be a whole other set of circumstances and problems. And yes, I have tremendous support from my parents and friends. Without their constant support, love and confidence in me that I can actually do this single mom to a medically fragile kid gig, I would've dissolved into a huge puddle of goo long ago. It takes a village has never been more appropriate when it comes to sweet boy and me!

By the time this past Sunday rolled around, you could stick a fork in me, I was done. Tired, just darn tired. Colby's feeding pump went off around 6:30am. I got up and turned it off. Unlocked the front door. Texted the day nurse to come on in and that I was going back to bed. I don't do it often. I always think I can get this and that done while the nurse gets Colby up for the day. Not that day. Slept in until 9:30am! It was a thing of beauty. Then I did 4 loads of laundry and watched the Patriots LOSE the Super Bowl. Woot woot! And of course stayed up to watch This Is Us. Ohmygosh, if you're not watching this show, what is wrong with you? It's soooooo good. I didn't cry at all during the episode, so I guess that's confirmation that my heart has turned completely to stone. I also spent a great amount of my time following after Cupcake, picking and wiping up her little pees and poops. I don't know what is going on with this damn rabbit! She used to be so good at using her little box or chux pads. Now she's just letting it land wherever she happens to be. Driving me nuts! I bought “Rabbits for Dummies at Christmas for Colby and me to read so we would be better bunny owners. I've read about 80 pages so far, but haven't gotten to the part about litter box training. I thought we had this under control but obviously not. Time to skip ahead to that chapter. Good thing she's cute!

Yesterday Colby went to school until 12:30. Then I picked him up and we met his PT at the pool for swimming. Colby hasn't been swimming yet this year, so I know he enjoyed it. Last night he fell asleep within minutes of us tucking him in. Today after school he gets his port flushed, and Tuesdays are also bath days. Colby's regular nurse for Tuesday PM called off tonight. Luckily they were able to get one of Colby's other nurses to cover the shift tonight. Her regular Tues client is out of town, so she happens to be free tonight. I can certainly give Colby a bath by myself. I've done it many, many times. But I cannot tell a lie, it's much easier with some help. I said I was going to dedicate February to working on Colby's eye gaze. Getting some pictures on it, learning more about the technology of it and what it can and can't do, etc. Yep, it's on my to-do list. There's still plenty of February left so I'm not panicking yet.

I'm going to hop off here and get my shower before Colby gets home from school. This is the 1^st Tuesday in a long time I haven't worked that 2^nd job. I'm enjoying doing chores around the house and not being so rushed. I'll miss the extra money for sure, but like I said, I need sanity too. More blogging soon, peeps. Thanks for reading this and for taking an interest in our lives. We live “differently” from most, but that certainly doesn't mean we're not happy. Colby, Cupcake and I are snug as bugs in rugs around here. Looking forward to a more laid back week and some down time with my kiddos.

FULL DAY FOR US

Colby has had his IPV treatment and is getting tucked into bed. Cupcake is hanging out in front of the fireplace, gnawing away at a chew toy. Hey, at least it's not the wood trim. I'm poking around Facebook and not watching the president's speech. Just not feeling it. I'll catch the highlights on tomorrow morning's news. 

Colby had school today. Yes, he's still in school even though it's winter and cold/flu season. I'm sure many people think I'm completely cra-cra for sending him to school right now. They may be right. Supposedly the flu peaks in February. I may up and one day decide to put him on home/hospital for a few weeks. Just depends. Once he was on the bus, I headed to my second job. Yep, more proof I'm completely nuts. I've been working at a law office since before Thanksgiving. I don't know what I was thinking taking on another part time job. Oh wait, now I remember, I need the money! But it got to be too much. Too much driving back and forth. Too much time away from home not getting stuff done. So today was my last day there. It's bittersweet. I liked the people I worked with and the job. I certainly like the extra money. But Colby comes first, and there are times I just need to be at home to make phone calls, schedule appointments, get Colby to PT/OT, clean, etc. I'm hoping the law office calls me to work there occasionally. Or I really hope I win the lottery.

I got home from work about 7 minutes before the bus got here. The nurse and I got Colby out of his wheelchair and into his bed. I stretched him out and put on his hand splints. Then I headed to the gym. Yes, don't laugh, I go work out sometimes. Then as soon as I got back, the evening nurse and I took Colby up to Great Clips for a hair cut. He needed it – his hair was looking funky! Then we gave him his Tuesday night shower. He was doing his eyegaze after that. His thing now is YouTube. He'll bring up the internet on eyegaze and go straight to YouTube. Then the craziness begins. He'll open YouTube over and over again, so there are multiple Spongebob, Sesame Street and Veggie Tales running at once. It's enough to make me want to jam rusty ice picks in my ears. So today was a very full day for Colby. We just got him tucked in and he's already about to fall asleep. Most nights he stays up until around 11. I'm not far behind. I need some QT time with the bunny, a shower, then I'm heading to bed. Tomorrow Colby has school and I have my first job. Yep, really need to win the lottery. Good night.

WHEELCHAIR WOES FIXED? Hopefully

Waking up at 4:15 is not how I like to roll. Getting up at 5am is definitely not how Colby likes to roll, either. But we did it. Did an up and back trip to Cincinnati Childrens today. Fingers crossed, I think we have solved the wheelchair head piece dilemma. Colby tried a Savant head piece, and we were all pleased with the fit and support it offers Colby. So NuMotion will order the Savant and return the Stealth. Same old blah-blah. Papers have to be signed by a doctor. Paperwork has to be submitted to insurance for approval. I'm not sure how long it will be before we get the new head piece. The guy from NuMotion said well, you can pay for it out of pocket and you'll get it much faster. I gave him the look of death and said yeah, right. Price is roughly $550. Think we'll just wait for insurance or Medicaid approval, thank you very much.

Then we went aaaaaaaaall the way to the opposite end of the hospital so Colby could get labs drawn. I told that bimbo to call for the Vascular Access Team, but noooooo, she said she could access Colby, no problem. And do you think she accessed him? No. She got one chance. After she couldn't do it, I was furious. But I had on my big girl panties and calmly asked her to page VAT to do the blood work. VAT brings an ultrasound machine so it's much easier to get access on someone like Colby. Long story longer, the blood work is done. The neurologist also ordered tests that require a urine sample. We tried to collect it while at the hospital today, but that was a no go. So I'll have to get an order from the neurologist and find somewhere to take a sample for testing this week. I get to have all the fun, huh?

I have big news. Really big, super big news. I'm getting another cell phone. That's right, the time has come for me to put my iPhone 4 out of commission. A friend of mine gave me that phone over 3 years ago. It's been real and it's been fun, but that phone has to go. I don't know whether to laugh or cry at how very slow it runs. I can't download apps on it. Often it tells me I have no more storage. I could go on and on about the issues of having a phone that old. Well, we'll see. I'm very technology stupid. I'll probably get intimidated and chicken out in the next couple days. But as of right now, I'm doing it!

Time for The Good Doctor. Must go. More updating soon.

WHEELCHAIR WOES

The good news is Colby has his new power wheelchair. We went to Cincinnati Childrens in August and got it all lined out. There are many, many things to consider when ordering a wheelchair for someone as medically complex as Colby. First he was going to get the newer model wheelchair. Then I was told the tray that slides onto this wheelchair would be permanent. Nope, won't work. I have to be able to take off the tray to get Colby in the van. So we were able to get the same style of wheelchair Colby had before with new seating, arms, head piece, etc. Colby's chair was delivered December 28^th, which is amazing! We've never gotten a wheelchair that quickly before. It has to go through insurance, which always denies it. Then it goes to Medicaid for approval. Once it's approved, then it has to be ordered and built. So getting the wheelchair within 4 months really is incredible. The bad news is we've had some problems with it, especially the head piece. Of all the parts and pieces Colby needs to work properly on that chair, it's the head piece that's THE most important. We (two wheelchair dudes, PT, and whoever else wanted to take a crack at it) have worked hours on that damn thing. I'm ready to roll that chair in the Ohio and watch it sink. My opinion is the head piece was fault from the start, which is hard to believe because both his old and new head pieces are made by Stealth. Not sure how we had such good luck with the one on his old chair and such bad luck on the one on his current chair. Actually we've been using the old head piece on his new chair because the new head piece is that crappy. Lovely.

So that will be appointment #1 tomorrow. We'll leave here around 7:30 tomorrow and go to the Perlman Center to get this wheelchair looked at and hopefully fixed. After that, Colby's appointment #2 will be getting labs drawn. Unfortunately they need to be drawn peripherally, meaning they will have to stick him to get the blood. They aren't able to draw the blood from his port because they need to do a PT and PTT to look at his clotting factors. You can't do this test with blood from a port because there is heparin sitting in his port, and that will mess up the clotting factor values. I hope this peripheral draw goes as easily as the one he had done at Cincy in December. I had the lab technician call the Vascular Access Team, who brought an ultrasound machine to find the best spot to access Colby. One stick, which is fantastic. Hoping for the same tomorrow. Then we can head home. Really hoping to be back on the road and home before afternoon traffic starts getting crazy.

I don't know if it's proper or not to ask for prayers for a wheelchair, but I am. Really hoping the Savant or the HeadPod that Colby tries tomorrow is the solution to his head piece issues. I'm not giving up until we have this resolved. Can't have sweet boy's head not properly supported. We'll see.

CINCY VISIT HIGHLIGHTS

I was told over Thanksgiving that I needed to do a blog update. Well, here I am 4 days before Christmas, doing just that. Actually I'm just going to update on Colby's recent Cincinnati Children's visits.

My dad, a nurse and I took Colby to Cincy on Tuesday. He had one appointment that day to meet his new endocrinologist. Well that didn't happen because she had her baby the day before. We met with some other endocrinologist and discussed Colby's recent DEXA scan. One of the 3 measurements they take was improved – the other 2 not. Colby is going to continue his Zometa infused for another year or so, then we might discuss him taking a break for a while since the medication isn't really helping to improve his bone density. However, it is helping to prevent bone fractures, and that's our main goal.

Yesterday we had to be back at the hospital at 7:30, yuck. Colby's first appointment was with ophthalmology. Colby's prescription has only changed slightly. Basically it has gone from 20/20 to 20/25 if we keep his current glasses, which is what we're going to do. That's what the eye dr suggested. I asked her about Colby getting contacts. I said I would be the only one to handle them. I certainly don't expect the nurses to go sticking their fingers in Colby's eyeballs. But the ophthalmologist said because Colby has astigmatism, she doesn't recommend contacts. Something about he doesn't blink enough, and contacts that you wear with astigmatism you need to blink a lot. Something, something, blah, blah. Next we headed to the lab where Colby had to get a peripheral blood draw. I requested they go ahead and call the Vascular Access Team since Colby is a horrible stick. She used an ultrasound machine and was able to access Colby on the first time. Yay! They drew 8 vials of blood, plus Colby had to give a urine sample. What I thought would be a horrible experience ended up being easy-peasy. Then we went on to pulmonary lab to test Colby's vent settings. That was painless, and I was even able to get some vent connectors from them. Score! His last appointment yesterday was PT. They evaluated Colby to see if he has made any improvements since being on Spinraza. Well duh, we all know Colby has done amazing things while receiving this drug. Unfortunately, the increased movements we're seeing in Colby don't add up to points on their scales. I'm calling bullshit! But they made notes about Colby having a better heart rate, vocalizations, stamina, etc, along with their PT measurements. I don't care what they say, in my books he has done wonderfully on Spinraza, and I won't let any test tell me different. We were all over that dang hospital. Started in Section E, then A, then C, then back to A. Very busy, very exhausting day by the time it was finished.

Today we went to Neuro Clinic. Meaning we show up, get Colby comfy on the exam table, then all the doctors come to us. Ah, much easier. Colby saw the nutritionist, rehab doctor, pulmonologist, and neurologist. We're going to tweak Colby's formula recipe a bit, both calorie and fluid intake adjustments. No vent settings changes at this time. Then we hightailed it down to Ortho Clinic. They took x-rays of Colby's spine. Every time I see those, I'm amazed at the amount of hardware running down my kid's back! X-rays were fine, no worries. Thank goodness.

Got home about 4pm. Got Colby settled and most of the unpacking done. I'll work on that some more tomorrow. The rest of the night involves not much. Colby is in his room doing YouTube on his eye gaze. I'm lounging on the couch in front of the fireplace, yelling at Cupcake when she tries to reach up and eat my houseplant. I'll blog again soon and fill in the last couple of months. Yep, I'll get right on that.

Ok, I'll try.

OCTOBER OCCURRENCES

Hey, guess what! You're going to be shocked to read this, but we're short on nursing. Yeah, can you even believe it?! Colby has a nurse who works the weekend evenings, Sat and Sun from 4p-12a. She needs surgery and will be out for the next 4-6 weeks. I'm not going to even attempt to get her shifts filled. We're darn lucky to have a weekend nurse, and I'm pretty sure the nursing agency will have trouble filling the shifts. Screw it. I have plans to go to a Halloween party on the 28th. One of Colby's other nurses said she would work that night so I can go out. Other than that, Colby and I will just spend some QT together. As one of my friends said upon finding out we'll have another nurse out, it's one step forward and five steps back with the nursing situation. When nurses call off it doesn't even phase me anymore. I'm actually more shocked when they all manage to show up for their shifts in a week. I have no control over it, so I better learn how to deal with it.

Colby started attending school on October 2^nd. That means we're both getting up earlier, and the nurse has to be here earlier in the mornings. We're slowly adapting to the new schedule. And I've decided to send Colby to school all 5 days a week instead of 4. I really like his teacher this year. She has already learned how to navigate his eyegaze. I think she sees potential in Colby and is also willing to push him when it comes to his extreme stubbornness. There's no way I'm taking Colby's swimming away from him, so we'll be doing that one evening after school. Hoping it doesn't wear Colby, and me, out too much. We'll see how it goes.

Spinraza injection #3 is done! Colby did as well this time as he did with the first two. One more loading dose on Nov 15^th, then he won't have another dose until 120 days, which is March 2018. Our appointment was at 6AM. Damn, that's early. I gave it some thought and decided doing an up and back trip would probably be easier than packing, loading, unpacking, unloading, repacking and reloading all of Colby's stuff. So we left at 4-freaking-AM last Thursday. Everything went smoothly. Colby and I both seemed much more at ease this time around. I don't take chances with him developing a lumbar puncture migraine, so I make him lie flat for 48 hours. He was not digging it! I could tell he was totally bored. Sorry kid, Mama's rules. It's a small price to pay to avoid complications from getting the injection.

We're getting excited for Halloween. I've been doing some decorating here and there as I've had time. I've decided my decorations are all too cutsie. Time to step it up and get some bigger, scarier stuff. Also need to decorate outside more. Okay, I don't know if I NEED to decorate more outside – I just really want to do it. If I had the time and money I'd turn this darling little bungalow into a creepy, scary, awesomely decorated house. Wonder if I could find a good price on a coffin somewhere...

So that's a little update. I'm working some extra hours this week so I figured I'd better blog while I had a little time to do so. You know, gotta make hay while the sun shines. More soon, as time and energy level allows.

SPINRAZA #2 - DONE!!

This week has been crazy busy for us. Some for the good; some for the bad. Let's start with the bad, then end on a good note.

The transmission went out on the van. Not cool. I get frustrated with my dad sometimes because it seems like he's always finding something wrong with the van. I'm sure there are many of you who have dads like that, too. Well, he mentioned a couple of times there was a “whining” with the van. I ignored him and also started ignoring the whine. You know, just turn the radio up and the problem will go away. Well it didn't. He took the van to get it looked at and sure enough, the transmission was going bad. Not something you can mess around with for sure. Dad took the van to get fixed and brought me the old, big, brown van to drive in the meantime. The Terdmobile. Good old Terdy, still getting us from Point A to Point B when needed. I couldn't take Colby anywhere in it because the lift is broke, but I was able to run errands, go to work, etc. There were some issues in getting the van back, but we finally did on Friday morning. Just in time because we had a trip to Cincinnati Childrens this week for Colby's Spinraza injection. More about that later in this blog.

The water chamber on Colby's vent tipped over last Wednesday, causing water to travel through the vent tubing, into his trach and yep, into his lungs. Colby's nurse somehow got a pillow caught in the tubing and caused the water chamber to spill. I know it was an accident. I know she didn't mean to. I know she felt horrible about it. However, that doesn't change the fact that her carelessness put my kid in great danger! Thank God I was home when it happened. We immediately began doing CoughAssist treatments and trach suctioning with Colby. I was online with other SMA moms to get advice on what to do, how aggressive to be with treating the issue, etc. We monitored Colby very closely, gave him extra CoughAssists throughout the day, and re-positioned Colby frequently for postural drainage. I have to idea how much water got in his lungs. Scared me to death. My mind started racing. He'll get sick. He'll get pneumonia. We'll have to cancel the Spinraza injection. You might think I'm overreacting, but really I'm not. All that very well could've happened. But it didn't, and I'm thankful we were able to “fix” Colby without having to go get x-rays, get on antibiotics, him getting sick, etc. Living in fear isn't very fun sometimes.

Let's move on to better, nicer topics. This past Monday was my birthday. My mommy offered to take me to lunch, anywhere I wanted to go. I said hey, there's a Frisch's Big Boy down the street. Lived around here 20 years and have never been. So that's where we went! Patty melt and hot fudge cake made for an excellent birthday lunch. I got lots of cards, calls, texts, visits and gifts. It was a great birthday. Thanks to everyone for taking the time to make me feel loved on the celebration of the day of my birth!

To say van transmissions ain't cheap is an understatement. We had some very considerate family and friends help us pay for the van. I cannot thank you enough. When I found out it was the transmission, my head started spinning. I had no idea how I was going to work enough extra hours to pay for it. I feel with these Spinraza injections, Colby needs me now more than ever. I'm working on getting him extra PT sessions and also OT. We need to really work to make the most of this medication he's receiving. Thank you to everyone who gave me “birthday money” which was really funds to help pay for the van. It has taken a huge worry off my mind, and it's appreciated so much.

And last but certainly not least, Colby's 2^nd dose of Spinraza is a done deal! We went up on Sunday night and stayed again at the Ronald McDonald House. Had to be at the hospital at 7:30am. Colby's lumbar puncture went as smoothly this time as it did last time. He did, however, wake up at 2:30am and didn't go back to sleep. So obviously he's having some anxiety about all this. Understandable! I think for next injection, October 12^th, I'll see if they can prescribe him a little something-something to help with that. Everything went very smoothly, just like last time, and we were home at 2:45 yesterday. I checked on him hourly through the night. He had no issues with sleeping. He's doing great. Keeping him flat for 48 hours. I've been giving him Tylenol since he had the procedure. We're going to stop the Tylenol and see how he does. He certainly doesn't seem to be in any pain or discomfort. Such a tough kid! Looking forward to getting these loading doses done. Halfway there!

Okay, I really, really need to stop typing and get something done today. Is it bad that I want to curl up on the couch and watch movies all day? But the mound of mail on the table and loads on laundry waiting on my tells me that's probably not an option. But then again, would it really matter if I took a couple hours to chill out?! Maybe throw in a load of clothes, then some couch time? We'll see how my energy level lasts.

POST SPINRAZA UPDATE

I'm going to make this update short and sweet.

Colby's first Spinraza injection went well. Very well. His procedure was scheduled for 8:57AM and they wheeled him into the OR at 9:03. The radiologist came out in no time and said no problems and Colby's injection was done. He was able to find an opening in Colby's spine easily, injected the Sprinraza and bing, bang, boom, done. Colby stayed in the PACU (recovery) for 2 hours, and we headed home. Got back to Louisville around 3:15. I can't express how damn glad I am to have this first Spinraza over with and done!

I made sure Colby took it easy for a couple of days. He stayed flat for 48 hours. I'm sure he was bored, but that's just too darn bad. Mama's orders. I bought him “Kong: Skull Island” so he watched that a couple of times. Overall he has done very well. His heart rate was really low Tuesday. I assume that was because of the anesthesia. We sat him up in his wheelchair Wednesday, two times, one hour each. Thursday we increased it to 1 hour 15 minutes twice a day. Today he sat up 1.5 hours twice. It's been 4.5 days since the procedure, so I'm assuming he's out of the woods for any complications.

THANK YOU to everyone who stayed in touched. The calls, texts are Facebook communications are much appreciated. We are so loved. I realize that more and more as time goes by. Fingers crossed for a successful injection #2.

SPINRAZA Q & A

What's the schedule? My goal is to pull out of the drive at 12:30 tomorrow. Colby has been invited to a birthday party, so we'll go there first. Stay about an hour. Party on the way to the hospital, that's how we roll. Then do his IPV and CoughAssist treatments and feed him while there. Load back up in the van and head to Cincy. Staying at the Ronald McDonald House tomorrow night, which is excellent. It's right across the street from the hospital and of course much cheaper than staying at a hotel. So again, I request that when you go through a McDonald's drive-thru, please consider dropping your change into the Ronald McDonald House donation box. It's a wonderful organization and I'm really glad we got a short-term stay room there for not only Colby's first injection, but also his 2^nd injection on Sept. 25^th. His 3^rd injection is in October and his 4^th is in November. The rest of Sunday night will be spent getting unpacked and organized. Hoping to get to bed early. We report to Same Day Surgery at 7:30am Monday morning. Colby's procedure is scheduled for 8:57am.

How long will the injection take? Not sure. Colby needs to have anesthesia, so of course his procedure will take longer than someone who just needs lidocaine and no anesthesia. I'm thinking 45 minutes to an hour, start to finish. Then he'll stay in recovery for at least 2 hours. Maybe more since he's getting anesthesia. Once we're discharged, we're winging it. It's recommended that after receiving the Spinraza injection to lie flat as much as possible for several hours after the injection. We've taken out the front seat in the van so we can lean Colby back in his wheelchair to be flat vs riding sitting up like he usually does. I'll give him some Tylenol and we'll start heading home. If Colby starts to have any issues at all, we'll either turn around and go right back to the hospital, or stop where we are, get a hotel, lie him out flat and stay out of town another night. I don't have any idea how the procedure will go or how Colby will react to the drug and the anesthesia. Uncharted territories.

What results will Spinraza have for Colby? Don't know. Only time will tell. At the least it will hopefully stop the progression of SMA. Yeah, not only does Colby have this hideous disorder, it's progressive, meaning he gets worse and weaker as time goes by. Hopefully this drug will stop SMA from making Colby lose anymore lung function and/or muscle strength and mobility. I've heard wonderful testimonies from many SMA parents who say Spinraza is making their SMA child louder, stronger with better head control and improved muscle movement. Yeah, we'd take any and all of the above. Who knows?!

Heck, the Interventional Radiologist could come out and say sorry, we couldn't find an opening in Colby's spine and weren't able to administer the Spinraza. It could happen. Colby could have zero side effects or migraines, spinal fluid leakage, respiratory issues, and who knows what else. I'm opening my head and heart to any and all possibilities with this drug. I'm praying for the best and preparing for the worst. We didn't have a nurse until 4:00 today, so I spent the morning having a long discussion with Colby. I've tried my best to prepare him, but that's kind of hard since there are so many unknowns at this point.

To everyone who continues to follow, love and pray for us during this SMA journey, thank you so much. Actually I can't thank you enough. I'm not going to lie, I'm scared shitless for Colby. There have been times I'm like wait a minute. I'm agreeing to give my kid a drug that may or may not work, and he may or may not have some pretty severe side effects. Sounds a bit cra-cra really. But overall I truly feel trying Spinraza is the best decision for Colby. We can always stop the injections if necessary I suppose. So away we go. We're Spinraza bound!! No turning back now.

BUSTING AT THE SEAMS TO SHARE THIS

It's time to share our big, BIG news with you all. Colby will receive his first Sprinraza injection on September 11^th, just a couple of weeks from now! How about them apples?! I've known about this for a couple of weeks. However, Cincinnati Childrens called once to reschedule. Plus I had an over-the-phone consult with Anesthesia yesterday, and wanted to make sure there were no surprises with that. So I've been sitting on it, making sure it is a total go, and it seems to be. Oh my, the thoughts and emotions running through my little brain right now!

Here's how this will all go down. Colby will get 4 “loading doses” of Spinraza, the first being Sept. 11. Then 2 weeks later on Sept 25. Then a couple weeks later on Oct 12 and his 4^th loading dose is scheduled for Nov 15^th. Yep, we're going to be very, very busy in the near future. After the loading doses, Colby will receive the drug every 120 days forever. Ok, here's the part that freaks me out: the drug is administered via lumbar puncture (LP.) Couldn't just crush up a pill and flush it through Colby's g-tube, could we? Nope, of course not, that would be too easy. The procedure will be done down in the Interventional Radiology (IR) department. I've heard wonderful things about the IR doctor who has given other kiddos this drug from other SMA moms. They've been so busy down in IR giving Spinraza that he has also been training his fellow to do some of the LPs. But I know for a fact the doctor, not the fellow, will be administering Colby's Spinraza.

And how do I know this? Let's just say oftentimes nothing is easy with Colby. Most patients receive this drug with no anesthesia. They lie on their sides or back, are given only a local numbing cream to prepare to get the LP. Well, if you remember, Colby had spinal fusion surgery a few years ago, meaning of course his spine is FUSED. So the trick is to find an opening big enough in that fusion to administer the drug. The IR doctor has looked at Colby's back/spine x-rays and consulted with the ortho doctor who did Colby's fusion. He has said that for Colby, this will be a “very difficult procedure” and that he will be handling it, not the fellow. And Colby will need to have anesthesia. That brings up another can of worms of worries because SMA patients aren't supposed to fast, and of course when you're having anesthesia you have to do that. I've been in debate with the neurologist on whether or not Colby needs to be admitted to the hospital the night before to get PPN for the procedure. She insists he doesn't need it. I insist he does. So it appears we've reached an impasse. She called me yesterday and said now don't worry, Amy, you'll see everything will be fine. I cannot stand when people talk to me like that! Of course SHE'S not worried, it's not HER child undergoing anesthesia and having a lumbar puncture procedure done. I'll be so freaking glad to have this first Spinraza done!

I keep talking to Colby about all this. Trying to reassure him that many other kiddos have had this done and that I would never agree to something for him unless I thought it would help him. People keep asking me how he feels about it. Honestly, it's hard to tell. I've told him several times if he's scared or has questions to please use his eye gaze and let me know. That hasn't happened, so I guess I'm to assume he's ok with it.

What a way to end SMA Awareness Month, huh? To share the news that Colby will soon be receiving a drug to treat his SMA is still mind blowing to me. We've waited 15 years for this. Honestly I thought there would never be a treatment for SMA during Colby's lifetime. And here we are, just a couple weeks away from Colby receiving the first and only FDA approved drug to treat SMA. Yes, I'm scared to death about the whole anesthesia/LP stuff. Yes, I'm scared because we don't really know what to expect in improvements for Colby. We're rolling the dice and taking a huge chance. But I can't let me fear take over the decision to do something positive for Colby. And I certainly can't let Colby sense that fear. So, we're moving forward with Spinraza, and I'm praying I'm making the right decision for Colby. We'll soon find out, won't we?

NO NURSES, NO SCHOOL, BUT REALLY CLEAN TEETH

Greeting from our casa! Colby is getting his IPV and CoughAssist treatments done. I'm watching Ninja Warrior and eating a Lean Cuisine pizza. Aren't you jealous you're not here right now to party down with us? Here are some updates.

We had a good weekend. My brother, sister-in-law and niece were in town for my niece's cross country meet. So they came over afterward and we took our mom out for a belated birthday lunch. I suggested we go to Orange Clover, a cute, yummy, locally owned restaurant. Would've worked out perfect except they are CLOSED on the weekends. What the hell?! What restaurant is closed on Saturdays. Our second choice was Buckheads. Can't go wrong there. Good times were had by all. Then when they left to head back home, I went into work. Packing pills on a Saturday night, oh what fun. Yesterday we took it easy for the most part. I took Colby for a nice long walk. Let him practice driving his power chair. Unfortunately he was in more of a turn in circles mood. His choice I suppose.

The nursing situation stinks even more than it did last week. Broken Wrist is still out and now Nurse Baby Daddy is on paternity leave until Sept 12. We had one fill-in nurse who was working with Colby 2 shifts per week. Found out today she's cutting back to 1 shift. So there's another opening. And the school system's agency still hasn't found a nurse to go to school with Colby until Broken Wrist returns. They asked a nurse who had done home health nursing with Colby for years to do it, but she said she didn't want the job. Not sure what happened there, but I'm not worrying about it. If she doesn't want to do it, we don't want her. So we started the process for Colby to go on home hospital until he is able to start school. This means a teacher will come here to the house twice a week to work with Colby. With any luck, that will get started this week. Actually I think not having nurses here all the time for a couple of weeks is a blessing. Colby and I enjoy our one-on-one time. I'm a little more relaxed without any employee in the house for sure. And on the weekends I can sleep in until 7:50AM! Yes! As long as I can get Colby where he needs to be and work my part time job, we're good.

Took Colby to the dentist last Thursday. I was a complete freakin' wreck. Actually I was a nervous wreck since I made him the appointment months ago. I'm one of those people who hate going to the dentist with a passion. I despise it with every fiber of my being. Probably explains why I hadn't taken Colby in 6 years! Hey, don't judge, we've been busy haha. Last time he went to the dentist it was a disaster. He cried and cried. His heart rate shot up to around 160 and we had to bleed 2 liters of oxygen through his bi-pap the whole time. This time was the complete opposite. It was a breeze for him. And trust me, that hygienist was scraping the you-know-what out of Colby's teeth. I was amazed at how well he did. Seriously, why do I ever doubt this child?! He took it like a champ. A stud. Owned that teeth cleaning like a boss. Kicked ass and took names. The looks he gave the hygienist were priceless. The dentist recommended we take him back in 3 months for another good cleaning, then hopefully we can move to every 6 months.

We went to Colby's school today so he could meet his teacher. Yep, another new teacher for Colby. His teacher from last year has moved to the post-high program. Colby's teacher is especially new. This is her first year teaching. Took Colby's eye gaze so she could see Colby working with it. The first words he said were, “bedroom, bedroom, television.” I said that's Colby's nice way of saying he doesn't want to be here and wishes he was home, in his bed, chilling and watching TV. That kid! I think she might also be the teacher who comes here to do home/hospital with Colby, and that would be awesome. That would give her a great opportunity to get to know Colby. So maybe this whole can't go to school right now might turn out to be a blessing.

Time to tuck in sweet boy. Swimming and bath time tomorrow.

BUSY MONTH - here's a short update

Spinraza: We've made baby steps forward with getting Colby the only FDA approved drug to treat SMA. We did the extra blood work needed, involving a peripheral draw for Colby. In other words, they had to “stick” him and get his blood through a vein and not his port. Not a simple task, as Colby is a horrible stick. The staff at our local LabCorp were fabulous and patient. They were able to get his vein accessed on the 3^rd attempt. Colby's PT/PTT/INR levels were all within normal limits. So, Neurology said they would send the referral to Interventional Radiology so that Colby could get on the schedule to start receiving injections. The Neuro nurse did indeed send the referral last Wednesday; however, the neurologist hadn't signed off on it. This is what I found out today when I called to follow up. Hopefully the neurologist signed the referral today. I'll be checking with IR in the next day or two.

Power chair: Colby is due for a new, shiny, fancy power chair! Insurance will pay for a new chair once every 5 years. Doesn't seem that long since he got his current chair. But hey, if it's been 5 years then heck yeah, let's do this. There are 2 companies we could use in Louisville for the chair. They are bad and worse. I refuse, and I mean refuse, to use National Seating and Mobility here in Louisville. Their customer service sucks at best. Yeah, I said it. They're horrible! We've never used Numotion before, and unfortunately, I haven't heard good things about this company either. I asked several SMA families and PT's their experiences with them. None were favorable. But that's who Cincinnati Children's suggested we work with, so I'll give them a try. Fingers crossed I'm not blogging about how much they suck in the the near future. Wednesday a Numotion rep will be here to access Colby's needs. I've been making a list of everything I like and dislike about his current chair. I'm kind of stressed. I want to be sure we get the best possible fit for Colby because like I said, he'll have this chair for the next 5 years. Then on Thursday we'll go to Cincinnati Children's Perlman Center to meet with their PT and this Numotion guy to firm up the wheelchair specs. Then we wait. And wait. And wait for paperwork to get submitted, insurance to approve or deny, etc. It'll be months before he actually gets this new chair. Oh how we hate the waiting.

Eye gaze: If you remember, Colby got a new eye gaze device a couple of months ago. I've been trying to do better at getting it in front of him and having him use it more. Yes, I could be doing even better. Baby steps, ok? We used some of his birthday money and got him an Amazon Echo. We love it! His speech therapist has programmed several commands on Colby's eye gaze so he can use the Echo. For example, he can choose one button and his eye gaze will say, “Alexa, what's the weather today?” And poof, the Echo turns on and Alexa tells us the weather. Pretty cool stuff. We also did the coolest thing with Colby's eye gaze a couple weeks ago. We made a phone call! How cool is that?! His eye gaze has Bluetooth so somehow (I'm not a technology person for sure) his speech therapist synced her phone to his Bluetooth on eye gaze. I went in my bedroom and closed the door, and Colby called me on my cell phone through his eye gaze! It was amazing! He could hear me through his eye gaze talking to him. He has programmed buttons for this application, too, so he was telling me stuff like, “LOL, how are you,” etc. Currently Colby is sitting up in his chair with his eye gaze in front of him. He has turned on the DVD player, played the Spongebob disc in there, and is watching it in fast forward. Don't know why he likes to do this, he just does. Old habits die hard I suppose.

School: Colby will not be starting school on Wednesday. As I mentioned, we're going to Cincinnati Children's on Thursday. The appt is very early, so we're going up Wednesday evening and getting the driving out of the way. So that really leaves Wed and Thurs out for school. Plus, the nurse that attends school with Colby is still out with a broken wrist. For another 4 weeks! So I've talked to the school nursing agency, and they are working to get another nurse to go to school with Colby for the next few weeks until Nurse Broken Wrist returns. Honestly the school thing is totally up in the air right now. Playing it as it comes along. What other choice do I have?

That's enough updating for now. I'll be so glad when the wheelchair is ordered, the Spinraza is scheduled, and they find a good nurse to attend school with Colby. Then it'll be on to the next group of items needed and/or phone calls to be done for this high maintenance sweet boy.

SPINRAZA

So what in the heck is Spinraza? Well, if you're associated with the SMA community in any way, you already know the answer. If not, allow me to educate you. Spinraza is a drug that treats SMA. Correction, it's the ONLY drug ever developed to help fight SMA. Hard to believe this drug is a reality. When Colby was diagnosed almost 15 years ago, there was no cure and no treatment. I honestly never thought we would see hope in a medication to help Colby in his lifetime. Well, guess that'll teach me not to dream big huh? Of course we've all prayed for something to help in any way, and there's a good possibility Spinraza just might help him.

The majority of the research and drug trials for this drug have been done in newborns and infants. Spinraza shows amazing results in reversing the symptoms of SMA. But what does that mean for Colby? SMA has ravaged his body for years, leaving him with very weak muscles that despite our best efforts are starting to contracture, along with being vent dependent. Colby can usually spend 2-3 minutes off his vent before his oxygen starts to drop. Will Spinraza “fix” all this? Will Colby be able to walk and talk soon? No. He won't. However, this drug shows some promise with the older SMA Type I kiddos who have been in trials. If Spinraza could stop SMA from progressing and making Colby any weaker, I'd be happy. If the drug could help Colby gain back some strength and movement that SMA has taken from him, I'd be over-the-moon thrilled. Spinraza is not a fix-all for Colby, but at this point, it's the ONLY hope we have for Colby in treating his SMA. Let's do this!

Not so fast. It's not that easy, of course. Never is with Colby and/or SMA. First was the process in getting the drug approved through insurance. The drug was approved by the FDA on December 23, 2016. Being a brand new drug and treating such a small patient population, the drug is outrageously expensive. The first thing Colby's insurance required was a pre-treatment PT evaluation, which we did back in Cincy on April 19^th. Next insurance required genetic testing that wasn't done back in 2002 when Colby was diagnosed. Check. Got that done as soon as the insurance company asked for it. Then on June 1, Colby's private insurance changed, meaning we had to submit this information to his new coverage and see what they had to say. They approved! Colby has been given the okie dokie to start receiving the one and only drug on the market to treat SMA. Exciting news, I know. But I'm also very apprehensive for several reasons.

You don't just pick up Spinraza at the Kroger pharmacy, crush up a pill and flush it down Colby's g-tube. Oh no. The drug is administered via lumbar puncture. Honestly, this scares me to death. A possible side effect could be a migraine if there is any fluid leakage during the procedure. I've lived with migraines for years, and that is a pain and discomfort I wouldn't wish on anybody, especially my sweet boy! So that's a concern. There's also a worry that since Colby's spine is now fused, finding an opening large enough to administer the drug might be an issue. We won't have any idea until we get to Cincy Childrens and the IR (interventional radiologist) looks to see the access availability for Colby to get Spinraza. And at the moment, Colby's blood clotting factors are abnormal, so there's no way he's getting this drug until that issue is fixed. Colby had a ton of nutrition labs done last month, ordered by his neurologist. She also ordered a PT/PTT/INR as pre-procedure labs. And of course they were off because the blood was drawn from his port and not from a peripheral. The next step was to get an order for a peripheral lab draw. Hoping that this clears up the clotting issue. Just got the order for that yesterday. Tried to find a company to come out and do the blood draw here at home. Unsuccessful. So tomorrow morning we'll be dragging Colby up to LabCorp to get his blood draw done. I'm also a nervous wreck about this, since Colby is a horrible stick! I mean absolutely horrible! I'm dreading it big time. Hoping I don't thunder punch anybody during the lab draw tomorrow.

Wow, this is a lot of info in a short blog. That's where things stand with Colby getting this drug. It's pending basically. Once everything is a go, we'll hit the road to Cincy. Colby will receive the drug on day 1, day 15, day 30 and day 120, then every 120 days forever and ever or until we decide to stop the injections. Honestly, I'm pretty damn nervous about Colby getting Spinraza. The drug is brand new, so who even knows any long term side effects. Plus I hate the idea of Colby getting this done by lumbar puncture. There are some SMA families who jumped as soon as the drug was approved. They wanted Spinraza for their child immediately. That's not me. There are some SMA families who aren't currently considering the drug for their child. They want more information. That's not me either. I'm in the middle I guess. Scared to death of what this drug will or won't do for Colby, but at the same time I feel I owe it to him to try. He's come so far and done so much beyond what doctors ever thought he would, why not push the envelope and see if now his SMA can maybe be reversed. Wish us luck! This is a very, very, very big deal for Colby. The process needs to go as smoothly as possible so I don't lose what little sanity I have left. Who would have ever thought I'd be blogging about a treatment for SMA?! How awesomely awesome is that?!

POST B-DAY PICS

Now that I've found the cord to my camera, I can post some pics of Colby's bday party this past Saturday. And maybe start the scrap booking project I should've started in February.

My friend Nancy made these towels for the bowlers. My mom helped me clean house and BFF Amy helped me get out tables, chairs and make icing. Yes, it takes a village.

Nancy and I made Colby's awesome bowling cake. Funfetti! We also had chocolate cupcakes and ice cream.

Colby's St X buddies still stay in touch and go bowling with him. How sweet!

What a crew! Hope everyone had fun bowling.

HAPPY 15TH BIRTHDAY SWEET BOY!

And the best part of having a birthday...presents! Colby opening the Artsplash gift he got from his friend Danielle.

It's hard to buy for Colby. Cash and gift cards always appreciated.