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.