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.