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.

HAPPY BIRTHDAY SWEET BOY! (one day belated I guess)

Colby turned 15 yesterday. Can you believe that? How amazing is that? I mean really, how incredibly awesome is that boy? First he and 5 of his friends went bowling at King Pin. That was quite a sight to see. There was a 5-year-old who had never bowled before, a second grader, 2 kiddos in wheelchairs and Colby's St X buddies who are now juniors in college. Actually I think everyone had a great time. I know Colby did. We only had time to bowl one game and he started crying after it was over. Silly boy. Then we all headed back to the house for cake and ice cream with more family and friends. Colby was showered with attention and got tons of nice cards, gifts and birthday cash. Yeah, it was a great day. For all those who helped me prep for the big day, bowled, came to the party or wished Colby a happy birthday, thank you so much. Colby turning 15 is a really, REALLY big deal, and I appreciate you caring and helping to make it special. Did I mention it was Colby's birthday? Did I mention it was a really big deal?

Today I was determined to rest and relax. Not exactly my forte but I tried and pretty much succeeded. Colby got some cool items for his fish tank so we got those in place. The weather wasn't disgustingly hot so the nurse and I took Colby for a walk around the block. I tried to encourage Colby to use his eye gaze. He's found a new set of words and is saying different things, but still not forming sentences or communicating his needs. The battle continues.

Tomorrow it's back to reality. Colby has FINALLY been approved for the Home and Community Based Waiver. I've been working on it since freaking November. It was a giant pain in the fanny. But anyways, the main reason I wanted this waiver was so Colby could get briefs and chux pads. We've had to beg, steal and borrow briefs for months. Colby got his first shipment this month. Fingers crossed we continue to get shipments monthly. One of the benefits from this waiver is also some respite hours. We use a different agency for briefs and respite than we do for Colby's full time nursing. It's confusing I know. The nursing agency hasn't found a temporary replacement for the nurse who broke her wrist. She'll be out another 4-6 weeks. Bleh. So the waiver agency is sending out a nurse tomorrow to work one day. This should be interesting. Usually I wouldn't do it, but I'm up for giving it a shot. This nurse better not be a stupid douche bag, that's all I've got to say. I'll also call our regular nursing agency, Maxim, to see if they have someone to cover for the nurse who is out. The never ending nursing drama.

But for now, right now, I'm still enjoying the weekend. Colby and I need to discuss how he's going to spend his birthday money. I'm thinking some new school clothes, maybe some supplies for the rabbit. I'm also thinking of getting Colby an Echo. We could program statements on his eye gaze so he could communicate with Alexa. Not sure if it would be a bust or not, but we have to keep trying on eye gaze. I refuse to give up on Colby using it effectively to communicate. Lord, grant me patience.

Okay, that's the update for now. Again, thanks to everyone for making Colby's birthday the special day he deserved to have. I sure do love that kid. And I know many of you all do, too. We are so blessed to have you in our lives.

YES, I REMEMBER HOW TO BLOG - TYVM

I could make a promise to blog more often. But let's face it, I'd be lying. I would like to share some good news, though, so here's an update on things Amy and Colby.

Colby's new eye gaze machine was delivered yesterday! Hip-hip-freakin-hooray! Colby's speech therapist submitted the paperwork back in January. Yes, 5 months ago, and it's been caught up in insurance and Medicaid hell ever since. Thank goodness we lit a fire under the eye gaze company's butt. Colby's health insurance is getting ready to change June 1, meaning we'd have to start all over with the new company if not by June 1. And the pre authorization for the device was about to expire May 24. So luckily we got the thing shipped and here before then. It's a beauty! Thinner than his old device but the screen in bigger. Colby's speech therapist, who happens to be the most wonderful therapist in the universe, made a special trip here today to move all the info off his old device onto the new one and move the mounting piece off the old loner device onto the new eye gaze. Oh, much happiness to get this marked off the to-do list! If we had to start this process all over June 1, pretty sure I would've had a small meltdown of some sort.

We're making progress on getting Colby the new (and only) drug to treat SMA. Again, insurance problems. Of course they denied Colby getting this drug initially. United Healthcare denies everything initially. Then they said he needed a PT evaluation before approving Spinraza. Took Colby to Cincy for that several weeks ago. Now insurance wants further genetic testing on Colby to see how many copies of SMN2 he has. We've drawn the blood for that and it has been shipping to Massachusetts for testing. None of this surprises me. I know lots of other SMA families who have had to do exactly the same thing to get approval. We're inching our way closer. That's how I have to look at it. Not sure how the new insurance change will affect getting the drug approved for Colby. Guess I need to look into that this week. Putting it on my to-do list now.

Tomorrow is the last day of Colby's freshman year! Did I just type that? Colby is a freshman, soon to be sophomore? Be still my heart. Last day is supposed to be Wednesday so he would have 3 days left. But he doesn't go to school on Tuesdays so that leaves 2 days. The nurse that goes to school with him needs the day off on Wednesday. There is no other nurse through the school corporation's agency who is trained to take Colby to school. Since I can't go to school with Colby because I'm a “liability,” which is such total bullshit, I guess he'll be staying home Wednesday. Leaving tomorrow as the last day. One more day. I mean, you know, not that we've been counting down or anything.

I've been keeping this news under my hat for quite a while, and now it's time to share. We're packing up and heading on a vaca this summer. We haven't been on a vacation in a long time, so we're overdue. Where we're going is the most exciting part. Taking Colby to a special needs camp in upstate New York. Yep, this crazy mama is packing up her sweet boy and traveling 800+ miles one way. How will Colby do during the trip traveling that long? I have no idea. I do know he's the absolute healthiest he's ever been, so if we're going to attempt a trip like this, I feel NOW is the optimal time. The best part is we'll be meeting another SMA family there! They're driving down from Vermont to go to the camp. They've been many, many times. I'm nervous and thrilled and excited and anxious all at the same time. So much to get done still! The last week before we leave will be spent organizing, packing, packing, repacking, reorganizing, rethinking, packing, repacking, reorganizing, thinking again, packing again, etc. You catch my drift.

It's so great to share good news. Things around here are going well. As I type that I hold my breath, cross my fingers, rub on the rabbit's foot and pray it can stay this way a while. We have good, dependable nurses. Okay, when's the last time you heard me say that? The house is still as beautiful and wonderful as ever. I do kind of wait for the other shoe to fall. We're an SMA family, no way things can be going this well. That just doesn't happen, right? Trying not to take the good days for granted. Thankful for every single moment Colby stays healthy, or “Colby healthy” anyways. Thanks for reading and checking in on us. We're blessed beyond belief for sure. If I could only get this goofy rabbit to mind, I would have a perfectly charmed life indeed.

STUBBORN BOY, CUTE BUNNY, TIRED MAMA

Good grief! I haven't blogged in over a month? Shame on me. You know the reason. Been busy. Actually I've also been tired and overwhelmed along with being busy. Not really sure why. Maybe it's because it's winter and we haven't seen the sunshine in, oh I don't know, weeks now! Maybe it's all in my head and I need to just get over it. Nah! We'll blame my current blah mental status on gloomy weather and being busy. Yeah, let's do that.

I decided to take Colby out of school for Jan and Feb. Too many nasty germs and viruses going around. I usually send him to school during the winter, but something this year said don't do it. It took the school system quite a while to find a home/hospital teacher for Colby. He (Mr. Randy) comes twice a week, each time for one hour to work with Colby. Poor Mr. Randy! He's finding out just how incredibly stubborn Colby Michael can be. Colby doesn't like doing schoolwork at school, so he sure as hell doesn't like doing it in the comfort of his own home. Oh sweet boy, where do you get such stubbornness?! We had Colby's eye gaze going with Mr. Randy was here yesterday. The only choices Colby had were Yes, No, I Don't Know, Maybe, Seriously, All Done, All Gone. Mr. Randy was trying to get him to answer yes/no questions. Colby started answering “seriously” for every question he was asked. Really, Colby? It was kind of funny, but let's face it, Colby was being a total smartass. So then I hid that button, so it was no longer available for Colby to say. Then his answer to questions was “I don't know.” To every---single---question--- Mr. Randy asked him. I about lost my flipping mind. I finally hid all the buttons except Yes and No. Wow, can't wait for home/hospital instruction again tomorrow. Want to take bets if Mr. Randy calls out? Haha.

And speaking of calling out, we're having nursing issues again. Oh boy, dealing with nurses, my absolute favorite. We had a full time second shift nurse. He and I got into a disagreement last Tuesday. He looked and me and said, “I'm going home!” Got his shit and walked out half way through his shift, and he hasn't been back. So then the nursing agency found a replacement. This nurse was going to pick up some shifts while her regular patient was in the hospital. She worked one full shift, then called the nursing agency and said she didn't want to work with Colby again. She complained the drive was too far and that Colby was too big and heavy. Due to her back problems, she would be unable to properly care for Colby. That's two nurses in one week. But wait! There's more! We have a nurse who only works one shift a week for us, Friday evenings. After next week, guess what? Yep, she found a full time case closer to her house, so she's taking that patient and dumping us. That's three nurses gone in one week. She is going to pick up extra shifts next week, then start her new job the week after that. So we're good until next Thursday, then we will have no evening nursing. ZERO. Zip. Hopefully the nursing agency can find a replacement soon. If not, it'll be okay. We still have a full time day nurse and a part time nurse who helps on the weekend. If we go a week or two without night nursing, maybe it's the universe's way of saying I need to spend more time with Colby. And I'm fine with that! 

Tuesday it was pouring rain so we didn't take Colby swimming. Instead, I gave him a haircut myself. I probably shouldn't add barbering to my resume anytime soon. But I think I did a decent job? Right? 

And what about me? I'm keeping busy, for sure. Upon careful examination of my checkbook, I've come to the realization that I'm broke. I was geared up to start looking for another part time job when the nursing crap started. So as soon as we have more nursing, and dependable nursing, I'll be on the hunt for a j-o-b. Not sure where I'll end up or what I'll be doing, but hey, I'll figure out something. Not sure how in the world I'm going to still care for Colby, take care of the house and work more, but I'll figure something out. After all, I am Super Mama.

We have a new member in our family. This is Cupcake. I wanted to get Colby a cat or kitten for Christmas, but we had a nurse who said he was highly allergic and that he would have to quit if we got a cat. Well, this is the same nurse who up and quit so obviously we should've just gotten the cat and let his sorry ass leave! But I don't regret getting a bunny. She's sweet, curious, super soft and we love her to pieces. Things I've learned about rabbits: they like to eat. They poop, and poop, and then poop some more. Our rabbit is very nosy, but she's been pretty good about chewing. She hasn't gnawed on any of the woodwork like people said she might. She has chewed through one of Colby's pulse/ox probes and also a feeding extension. But once I caught her and bopped her on the nose for it, she has pretty much stopped. Now I'm debating on whether we should get a cat or not. Yes, apparently I'm a glutton for punishment. If we're going to have a nice, big house, might as well fill it up, even if it's with furry critters. Or maybe a parakeet...?

 

And lookie here! We finally got our driveway repaved. Hooray and zip-id-eee-doo-dah. I think they did a great job. Don't know if you can tell or not, but there are huge ruts on each side. Well, if anyone is going to step off the driveway, into these ruts and gets hurt, it'll be be. No doubt. I caught the foreman walking by our house (who happens to be a total hottie BTW, too bad I'm probably old enough to be his mama.) I asked him if they were planning on filling the ruts soon, and he says yes. I probably should've gotten his idea of “soon” but I didn't. Hoping very soon. 

Okay, time to get kid tucked in to bed. Will blog again when I have the time and energy.

HAPPY 2017 IS GETTING HAPPIER

Well, the start to 2017 was totally sucky. But we are doing much better now. Thanks to everyone who called, texted or asked on Facebook how Colby was doing. There's no doubt in my mind we are surrounded by so many people who love and care about us! Took Colby to the pediatrician here in town Tuesday. I was leery because his regular doc was out of the office, so we had to see just some other doc in the practice. I really liked him! Turns out Colby had an ear infection. Not really sure how. We didn't go swimming last week because I was sick. Didn't go swimming this week because Colby was sick. But anyways, he's on Omnicef for 10 days. I think we caught it just in time before it started moving down into his trach and lungs. So I've been getting up through the night to do a respiratory treatment for Colby. Nebulizer, IPV, CoughAssist, the whole shpeel. For 2 nights he was up with a high heart rate and low oxygen. For the last 3 nights we've been doing extra treatments. How much sleep are we not getting this week?! But if it helps Colby get better, that's fine. I'll do the same tonight, then reevaluate tomorrow based on how his numbers hold up, the color and amount of his secretions, etc.

I've made the decision to put Colby on home hospital for school during the winter. I'm one of the few SMA parents I know of who sends their kid to school during the cold, germ-infested winter months. Colby has never been sick and in the hospital once during the winter. So weird. He's had major illnesses in September, July and March, but not during the winter months. And there's this little voice telling me, hey, don't push his luck. We could use the rest, that's for sure. There are lots of things we can do with him at home that are educational, if we choose. Plus he'll have a teacher come out for a couple hours each week to bring him his school papers and work on them with him. Pretty sure I'll never regret the decision to spend more time with my kiddo. I've already told him swimming will be decided on a week-to-week basis, based on weather, how he's feeling, etc. He probably didn't like that. We've also always taken him to the pool in the winter. Yes, I know, I'm one cra-cra mama. Never claimed not to be!

We are still without a bunny around here, but it's not my fault, I'm trying! I e-mailed the lady at the rabbit house. She e-mailed back the next day saying I needed to fill out the online application, which I did immediately. Now I haven't heard from her in almost 2 weeks. I called her once, left a voice mail, and yesterday sent her another e-mail. Uh, hello, we're in need of a bunny to put in this ginormous cage set up in the living room! I think it's supposed to be warmer next week. I guess if she doesn't call back, we'll run Colby up to the local pet store to pick out a rabbit.

I have some happy news to share. Last year my endocrinologist found a cyst in the bed where my thyroid used to be. Okay, that's obviously not the good news lol. She sent me to a neuroradiologist. He thought the cyst was nothing to worry about, but wanted to do follow-up a year later. Went to the neuroradiologist yesterday and now he's almost totally convinced the cyst is only a cyst and not a recurrence of cancer. He's calling it a “post surgical cyst thingy,” very scientific term isn't it. The mass/cyst doesn't look suspicious and has shrunk in size instead of grown. So zip-id-eee-do-dah, I'm still cancer free. I could have reached up and kissed him right on the mouth, but managed to hold my composure instead. He wants to look at it again in a year just to be on the safe side.

But the really, really, big, huge exciting news is that with any luck, and work and coordination with Cincinnati Childrens, the drug company, Colby's insurance company, etc, and with the pharmacy gods smiling down on us, Colby will be able to receive the recently approved drug to treat SMA. This is such a huge deal people! After 14 years, there is FINALLY some hope that the progression of his SMA will stop worsening, and maybe, just maybe, he will gain back some strength and mobility that he has lost over the years. The wheels are in motion. Best case scenario, 2-3 months. Regardless, Colby will be receiving a drug to fight his SMA in the near future. I'm flabbergasted this has come to fruition. I honestly never thought a cure or treatment would be available during Colby's lifetime. Never been so glad to be wrong about something in my whole life! I'll keep everyone updated, of course. Right now I've done what I can do. Signed a consent form and signed a start form. Next Colby's neurologist fills out the rest of the start form, submits it to the drug company. Then Cincinnati has logistics to figure out like which department is administering the drug, do they need to hire more people, stuff like that. Amazing. Simply freaking amazing.

Time to tuck in Colby and go to bed so we can get up at 2AM for a respiratory treatment. Oh goody. G'nite all.

HAPPY NEW YEAR PEEPS

My oh my! I haven't blogged since November 16? I've had a couple people ask me if anything was wrong because I haven't updated lately. I guess it's a combo of being busy, preoccupied with any number of other things or I just flat out haven't bothered to do it.

Hard to believe another year has rolled around huh? Just doesn't seem possible. This was our 2^nd Thanksgiving and Christmas in our new house. Unbelievable! We had 12 here for Turkey Day. I was on top my game. Had all the dishes listed so we didn't forget to set something out (who does that?!) Even had times written down so that everything was done at once. Didn't want the hot stuff getting cold and the cold stuff getting warm! Just one tiny little problem. The turkey was under cooked! (I wasn't responsible for the turkey, just sayin') I go in to get Colby up in his wheelchair only to return to a 16-pound turkey rotating/cooking in the microwave. It was kind of funny I guess. Better to have a microwaved turkey than food poisoning, that's my theory.

Christmas was fantastic. Actually, I was sad to see it come and go. Seems like we work, and plan, and decorate, and bake, and shop, and wrap and then boom, the family rolls in and in 4-5 hours it's all over. Took Colby to the Hosparus Christmas party again this year. It's such a great time and the people who organize the party do a fabulous job. It seems to get better every year! Colby got some awesome presents this year including a bowling shirt with his name on it, glow light and items for his fish tank, a couple of board games, movies, clothes, gift cards. Mama Santa is getting Colby a bunny rabbit. Oh my Lord what have I done? On Christmas he unwrapped all the stuff for the cage – hay, feed, toys, etc. Next step was to assemble the cage, which we've already done. Next step is to pick out a bunny! Turns out they have shelter houses for rabbits just like they do for dogs and cats. Did you know this? I sure didn't. So I've filled out the application, and I'll call them this week to see if we can set up an appt to look for our future house pet.

Colby had 3 days of appointments at Cincinnati Childrens Hospital the week before Christmas. By the end of Day 3 we were all pretty much exhausted. But overall the trips went well. Up and back on Tuesday. Then up on Wednesday, stayed overnight and back to the hospital Thursday morning for appointments all freakin' day. Overall the reports were good. No major surprises. I need to call and schedule an appointment for Colby to have a broncoscopy sometime in the near future. Need to talk to the nutritionist to cut about 50 calories daily out of Colby's diet. My sweet little boy is no longer little! He weighs 101 lbs and is about 4' 10” now. Holy crap.

I would say I'll make a New Year's resolution to be more consistent with blogging, but let's get real, we all know better than that. I didn't really make a “resolution” to do or not do something this year except for being wasteful. I want to be conscience of not wasting anything. Don't waste water or electricity, don't waste time and energy on people who don't deserve it, don't waste my time with the never ending what-if's that penetrate my brain, etc. Anyone know how to just turn your brain off sometimes? I need to know that technique. Geez.

And of course my main focus will be to keep Colby as healthy and happy as possible. He's such a good boy! He deserves so much better than the shit cards he got dealt. I'm debating on whether or not to send him to school during the winter months or put him on home hospital schooling. Of course the weather has been so warm it hasn't really been an issue yet. I don't know. We'll see.

Okay, that's all I've got. I'm looking forward to 2017. I think some really wonderful things could happen this year. May take some hard work and stepping out of my comfort zone, but hey, what else is new?!