Halloween Fun at Sesame Place

This past weekend, we visited Sesame Place to check out their Spooktacular event....dressing up was encouraged so we thought it would be fun to stick with the Sesame Street theme and put together our own little Cookie Monster costume for our Cookie Monster loving guy.

He even had cookie wheels. ;-)

Isaiah loved his first trip there almost a year ago, when we got to experience the park, full of Christmas spirit.  Given how much he loved Disney World this past June, we were confident he'd get a kick out checking it out again.

Our first stop was meeting the Count.

Just Cookie and The Count hanging out.

After we met with the Count, we visited Murray across the street!

Isaiah was a little put off by him....inching away...lol, but he still smiled and wanted to touch Murray's fur.

After that, we checked out one of the shows, "Who Said Boo?", where Elmo had everyone yell "Boo" at the beginning and we had a fur-real meltdown...(get it? fur real? lame.)....Isaiah ended up calming down once I had him in my lap and he enjoyed the rest of the show...especially when Zoe danced right in front of us!

Throughout our day, we were even able to Trick or Treat throughout the park, which was great practice for Halloween. :) I wish I had gotten one of those experiences on video, because it was super cute how Isaiah would try to roll up holding his bag out for goodies.

He loved meeting Abby Cadabby (above), mostly for her wand ;-) and seeing Cookie Monster...it was like looking into a mirror.  If the mirror had a clown image on it. :-P

We're just learning the ins and outs of wheelchair decorating...but those cookie wheels didn't make it past Cookie....we left them with him after we met him because they kept falling off.  (Next time we'll try zip ties.)

The big excitement of the day was that Isaiah sat in a restaurant standard high chair!

Twice! :) and both times after I wiped them down with clorox wipes. He really rocked that high chair.  Isaiah had no issues sitting up...which is so amazing.  Kiddo has gotten SO strong!

Isaiah's favorite show of the day was the The Not Too Spooky Howl-o-Ween Radio Show.  I took a few videos which I've uploaded to youtube...one that shows how a few characters came right up to Isaiah during a show.

It was a busy day at Sesame Place and we had a blast....we even skipped nap time and waited around for the parade!

Which was totally worth it :) Big Bird, Prairie Dawn, and even Grover came up to say hi to Isaiah.  I was quick enough to catch his interaction with Prairie Dawn.

It was an awesome day.....we loved all the Sesame Street excitement and we were grateful that it all put us in the mood for Halloween.

Hernia Surgery (Last Month)

It was just over a month ago that Isaiah had his hernia surgery.  He had a hernia pop up in late spring and we had it diagnosed at his next pamidronate treatment.  (That's when we go to the hospital for his bone treatment.  It won't cure his Osteogenesis Imperfecta, but it basically helps strengthen the bones and promotes growth.)

Before he could get cleared for surgery, we had to stop in to see his orthopedic surgeon to verify a fracture in his right leg since it was on the same side as the hernia.

The fracture was healing but teeny tiny.

With ortho's blessing we prepared ourselves for Isaiah's third surgery.  (First surgery was to place his port and ear tubes and second was to place a shunt to alleviate his hydrocephalus.)  We stayed at the Ronald McDonald House across the street from the hospital because we had to be there bright and early the next morning.  

He handled pre-op rather well.  We hung out watching tv while I signed papers and met with his doctor, nurses, and anesthesia team.  They accessed his port and gave Isaiah a mild sedative.  Amazingly, they agreed to have me help position him on the operating table.  I suited up in what they lovingly call "the bunny suit" and I helped wheel Isaiah to the operating room.  

It was cold and white and full of many people scrubbed in and wearing face masks.  So many times now I've been able to face things along side Isaiah and this one really caused me to hold my breath.  Being in the room where they'd be working on him.  Intubating him.  Fixing what needed fixed.  This was such a minor surgery but minor surgeries still scare me because of Isaiah's respiratory history....and surgery.

The operating table had something like a bed of warm air for Isaiah to lay on, on top of a special gel pad....all to keep him warm and to protect his bones.  There was a fan that was blowing warm air into it constantly.  With the nurse's direction, I laid Isaiah right onto the pad and I helped position his head and shoulders to their best position for him to be intubated and to breathe the clearest.  They allowed me in there until the tears welled in my eyes....(guys, sh*t was getting real in my brain.), watching him go under. I was there while he went into a deep sleep on a better sedative, gave him a kiss, sung him his Honeybunch song, and walked out to wait for it to all be over.

His surgeon was wonderful, telling me something like "I've got this".  I still offered to scrub in, because we have that kind of goofy relationship (this is the second surgery he performed on Isaiah, the first being placing the port), and he told me to get the heck out of there...but something funnier, because he has a similar sense of humor as Dave and I. 

Once surgery was over, Isaiah stayed sedated and intubated and was wheeled to medical imaging for his very first DEXA scan.  A dexa is a bone density scan; it measures the bone mineral density. After that, the plan was to take him to the PICU (The Pediatric Intensive Care Unit) for him to be watched overnight...but things didn't go as planned....

Instead, Isaiah handled everything so well during and after the surgery that they brought him to the PACU instead....which is the place you'd typically go after a minor surgery.  (I was shocked...I had been prepared for a night in the PICU.)  I was brought in to see him as he was waking and watched him drift in and out for a bit.  Once he was more awake, I asked him if he wanted water and Isaiah downed an entire ounce.  We waited a few minutes and I was able to offer him more and he guzzled another ounce down.  No vomiting.  He was waking up more and more.

Then I asked if he wanted a cracker and he said "ok, cracker"....and then downed that.  More water.  "More cracker"....six crackers later, he was cleared to go home.  I was floored.  Go home?  He JUST had surgery!  but yes, he was cleared to go home by everyone so they took off all the wires, deaccessed him....and we headed for the exit.

I double checked to make sure they didn't mean for us to stay at the Ronald McDonald House....nope, they meant for us to go home.  home HOME.  So we did.

Isaiah inhaled his dinner when we got home and honestly, you'd never know he had surgery just hours before.  He had an incredible night's sleep and it was just...all....surreal.

His incision has healed beautifully and this surgery really showed us how strong Isaiah has become.

I should hopefully be posting again on Friday....we have had quite a few well visits and follow ups the last week and a half, so I'll share the details of those in the next post. :)

Physical Therapy Update

When we went to Isaiah's first OI clinic a few weeks ago, we met with a physical therapist (PT) at DuPont.  Isaiah has a PT that he sees weekly (actually twice a week because he also sees her for water therapy!), and that we adore; she was thrilled to hear that we were meeting with a PT with lots of OI experience.

Meeting with a PT who frequently sees other children with OI gave us the opportunity to get lots of ideas for what PT should look like...and a lot of that was already implemented...rolling over, tummy time, reaching passed midline....I could on, but she also gave us the go ahead to try some things that I have been hesitant to try at home with Isaiah's regular PT. (Basically, stop being such a chicken, momma.)

One suggestion she had for Isaiah was that he needs to try "short sitting"....coming up with something for Isaiah to sit on took some creativity but once we figured it out, it was amazing!  He's rocking putting some weight on his legs 

"Hello? Dr K? It's time to take this splint off now."
(This photo is from a few weeks ago, when he had a broken arm)

We're even working on getting him short sitting in his wheelchair like he should be. 

This picture was shot in the middle of changing things up. ;-)

 Originally he was short sitting in his chair, but then he couldn't reach the wheels because he was sitting too far forward.  I put off finding a solution until we talked rods with his orthopedic surgeon (rods will likely lengthen his legs, so I didn't want to change his cushion if it was only for a short period of time, especially since he's been successful propelling his chair sitting the way he's been).  I chatted with some fellow OI moms (once I knew surgery is being put off) and some suggested cutting his cushion....I couldn't do it though; that little sucker costs hundreds of dollars!  Instead, I found some thick foam at a local craft store and cut that to size.  Bonus is that it was a remnant; I got it for $5. I've got enough for a foot rest and an extra seat cushion!


Beyond short sitting, we're also trying to give Isaiah ample opportunity to scoot on his behind.  Now that he is rocking independent sitting, we're hopeful he'll start trying to get moving.  We want to give him a good, stable place to work on moving, and one suggestion we love is to give him baths in a laundry basket.

Yup, a laundry basket!  The holes give and sides give him lots of areas to grab onto to support himself and pull. He was still getting baths in a baby tub, we're going to give him a few days outside the tub in the basket (to get used to being in it) and then we'll be moving on to baths in this basket, hoping that the water will give him the ability to move (hello at home water therapy).  I'm nervous that I'm going to have one slippery little boy, but he really can and should be sitting up in the tub to strengthen his muscles.  Isaiah hasn't been challenged at bath time, it's time to change that. :)


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Sorry for the lack of Wednesday posting, we had two appointments that day that took over my brain. ;-) More on that next week. :)

Fall Fun

Sorry for the late post, but we had some last minute fun today before I could get a post together for today. :-O  I have a few more OI-related posts but I wanted to take a break from that since I feel like everything I posted last week was all medical.

After a week of ugly weather, this morning we woke up to beautiful temperatures and sunshine...and we had no appointments on schedule!  I had gotten an email from Longwood Gardens this weekend and took notice of the "Pumpkin Playground"...we'd never been to a pumpkin playground...

So we went!

For this trip, I pulled Isaiah's wheelchair out for the first time since he broke his arm a month ago. We're scheduled to see Isaiah's ortho next week to check up on it and verify it's healed, but since he's been pushing up on his arms when on me and he was dying to push today....I'd say it's on the right track. :)

Can I take a minute to say how awesome, amazing, and fantastic it is that Isaiah can sit independently?!  

Isaiah was in a great mood the entire time we were there today...he was so interested in everything, it was just a fantastic day together.

I've noticed a few of our new followers on Isaiah's facebook page have commented about how it's nice we do normal things...I know that sometimes our life isn't typical, but we live beyond Isaiah's OI and hydrocephalus...if that makes sense...

 His condition(s) are a part of our lives, we accept them, but they aren't our lives.  We strive to raise Isaiah to live with his OI and his hydrocephalus, but not let them rule over his life.

Yes, we have a lot of appointments, we have surgeries, fractures, therapies...but we also have pumpkins and cuddles, story time and picnics on a bench...

This picture was post-picnic ;-)

Some of my goals when it comes to sharing Isaiah's life with you are that you learn about OI, that you learn about hydrocephalus...and that you see Isaiah...our amazing little boy.  He has those conditions, yes, but he is so much more than them.  

He's a smarty pants who has learned how to unhook his
wheelchair harness which means momma is in trouble. ;-)

Sleep Study Results

Sleep Studies.  If you're a follower on the blog, you know how we feel about sleep studies.

How anyone is expected to sleep with all of that attached to them, I don't understand.

But, this was Isaiah's fourth sleep study, so he's a seasoned pro.  ;-) For anyone new to the blog, Isaiah has had to do sleep studies to check his obstructive sleep apnea.  He used to live on a constant C-Pap 24/7 because he went into respiratory failure around one month of age.  His apnea was severe, with many "events" taking place throughout the night causing his oxygen to dip.  As he got older and stronger, he moved on to only needing the C-Pap at night and having much less dips in his oxygen.

When night nursing ended, I moved all of Isaiah's medical stuff into in to his nursery.  It's funny, I was trying to find pictures of it in his room but I don't seem to have any!  To be honest, I hated having it in there, his nursery was my one "baby" place...if that makes sense...it's completely silly, but I even got into the habit of storing his c-pap machine in the closet during the day so we didn't have to look at it.  It stressed me out.  Maybe because his going into respiratory failure was so awful and I just didn't see that coming?  I knew his bones would break, I knew we'd have challenges, but such severe breathing problems?   It was scary stuff.

Sleep studies bring it all to the forefront.  They are supposed to tell me how Isaiah's apnea is to let us know if he still needs the support.  To me, sleep studies are supposed to give me some good news and be the ending paragraph to the C-Pap chapter of Isaiah's life story...but gosh darn it, they never are!  The chapter is forever long.

Isaiah has had time off from the c-pap to test his body and see if he could handle sleeping without it.  He had a sleep study in April after months without it and the results were that his apnea was significantly improved from months before.  It was considered mild.  Isaiah's doctor said that if he didn't already have the c-pap in the house, he wouldn't prescribe it, that's how mild the apnea was.  At that point, Isaiah had really begun monkeying around his crib, so Dave and I made the decision to keep him off the night time C-Pap but continue to monitor his oxygen using the pulse ox....that was until this past August, where his monkey movements had him twisted up in the cord to the pulse ox, and/or chewing on it when we thought he was sleeping...so we cut that too (that was hard for me.  having the monitor helped me sleep better, knowing his oxygen was in a safe place...but he hadn't had any issues in 6+ months, he was no longer "noisy sleeping", and he was at risk to hurt himself with his shenanigans).  I emailed his doctor and he actually called to pull one of our C-Pap machines.

So this sleep study was to be the for-real-this-time end to the C-Pap.

Well guess what?


IT KIND OF IS!

This last sleep study was the best Isaiah has ever had in terms of results.  His oxygen stayed up in a safe place whenever he did sleep (he didn't sleep so well at this one....actually ended the study early because of how much/loud he cried dealing with it)...he still has very mild apnea, but everything (his oxygen/CO2) stayed normal when he slept.  He officially has the blessing to stay off the c-pap and monitor.  And he doesn't need another sleep study until June of next year!

Can I get a WOOT WOOT?

We are hoping to keep his c-pap and monitor for the winter (tucked away of course) in case Isaiah gets sick and needs the support, but yep, I'd like to call this chapter OVER, my friends.  We're not ordering any supplies at this time since we have them on hand if they are needed (but they will hopefully not be needed).

And just like that, Isaiah's room looks like it did in my dreams....free of machines and their supplies...because he's strong enough without them.

We feel blessed and grateful that this study solidified what we've known for the last few months, Isaiah is strong enough right now to not need support....he may need it again some day.  He may need his tonsils/adenoids removed at some point if the apnea worsens. But for now, the chapter ends.

I do want you to know that I appreciated what the machines did for Isaiah.  They were the perfect support to help him grow strong. They are a huge part of why/how he is here today...but this is a time I have been dreaming about for two years! It's a big cause for celebration.

Excuse me while I go kick my heels... ;-)

Our First OI Clinic Appointment

Oh hey, there's a ton of information in this post.  Although I do talk a lot, my posts are usually less wordy, so excuse this one :-P
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We're at AI DuPont Hospital for Children pretty frequently, but as Isaiah gets older, the amount of time we're there continues to get less and less.  Pam treatments were supposed to be every 8 weeks until he turned two, now we're stretching them out to every 9-10 weeks and by the time Isaiah is three, I believe it'll be every 12 weeks (woo hoo! No offense Dupont family, we love you!)

Something else has changed since Isaiah is two, he's now ready to go to OI Clinic.  OI Clinic involves seeing his geneticist, orthopedic surgeon, and a physical therapist with OI experience, all at once!

Since we live a few hours away from the hospital, the doctors and schedulers did me a huge favor and squeezed us into the schedule when we were there for his latest Pam treatment.  It made for an exhausting day (especially because we also did a sleep study the night before, yup.), but was worth it.

We were grateful to have clinic the same day as pam for more reasons than just convenience.  The PT we'd be meeting with had a chance to stop in while Isaiah was relaxed and getting treatment.  When she arrived, he was rolling around playing on his bed in Day Medicine so she was able to see him comfortable...(he wouldn't be comfy later at the clinic appointment, because it all looks like a doctor's office, and spoiler alert, he actually fell asleep while we were in clinic, which really would have wrecked her chances of seeing him moving).  Her meeting us in Day Medicine gave us the chance to really talk about Isaiah from a PT perspective, what we're doing with him and what she'd like to see him do.

After Pam was done that day, we headed right to the sign in area for clinic.  From there, we made our way to X-Ray, where Isaiah had his spine and legs x-rayed.  They also included his right arm to check for the fracture we suspected (this was mid-September).

Once we were finished with X-Ray, we were brought to a room to wait for everyone to arrive.  They had been meeting with other families and then checking out Isaiah's X-rays together.  We were expecting to talk about rods with the doctors, so we knew that was a big conversation they were having over the xrays.

What are rods?
Rods are something many with OI get placed in their limbs if they are fracturing a lot (they basically act as internal splints) and to help straighten the bowing in the limbs if that's there.  There are different types of rods.  Isaiah's orthopedic surgeon works with what we hope he can get- FD Rods aka Fassier Duval Telescopong Rods.  Telescoping means they grow with the person, which means less replacing, less surgeries, as compared to other types of rods.  The rods are surgically placed inside the bones....and honestly, I don't want to go too much more beyond that right now, because it scares me.

Rods mean a lot to us.  I believe Isaiah feels unstable.  I believe he feels the fragility of his bones and that's why he isn't more active.  I am hopeful that rods inside of his bones will give him the courage to move.  But....according to Isaiah's ortho, it's not quite time yet.  He'd like to see more to Isaiah's bones.  He doesn't feel his bones are right for FD rods yet, but believes they will with a bit more time.  In the meantime, we're listening to the PT we met with who offered us a laundry list of things to work on with Isaiah and his therapy team at home.  She wants him bearing weight on his limbs and although it makes us extremely nervous, we're working on it.  More details on that in the future. ;-)

Isaiah's geneticist is thrilled with the progress of Isaiah's skeleton.  Isaiah's bones look brighter/whiter than they've ever looked before.  He has no scoliosis and that makes my heart sing.  Remember how he had a moderate kyphosis and we were put on limitations on what to do with Isaiah?  Well guess what?  That's practically gone!  He has one vertebrae affected by the kyphosis, as compared to the four that were affected last year, so that's fantastic news.  That means the pamidronate is doing it's job and Isaiah's bones are getting stronger.  OI type III is deforming; there's no way around that.  It will be a constant battle to keep his spine as straight as possible.  His spine is very important, because if it curves too much, it can very much affect everything in his torso.  His lungs are already restricted by the space available, curving of the spine would restrict that space more.

Speaking of his lungs....we've also recently found out the results of Isaiah's latest torture sleep study!  Details on that later.

Gosh, if you actually read all of this, you deserve a cookie.  :-P

Nemours Perinatal Program

Hi there, lots has changed since the last time I posted to the blog.  September was a busy month!

For those that are new, welcome!  I started this blog when we were pregnant with Isaiah as a way to share what was going on once we found out that things weren't typical.  We have a big family, and it was hard for us to call each person to tell them, going through the emotions each time.  Once Isaiah was born, the blog blossomed into telling about life with him and his OI, and started being viewed by more than friends and family.

When we were pregnant, a number of doctors and medical professionals threw around the word "lethal".  We were told by some that Isaiah wouldn't even be able to cry, that when he'd go to take a deep breath, his expanding lungs could break all of his ribs, the pain would be too much, and he wouldn't be able to take the deep breaths he needed to.

But that was wrong.  He did cry.  His cry was loud and it was beautiful.

Thankfully we had found Nemours (AI DuPont) while pregnant.  When we found Nemours, the word "lethal" was never brought on the table.  Instead, Isaiah's geneticist, Dr. Bober, would tell us that we couldn't predict what would happen, that we'd have to wait and see once Isaiah was born.  In the meantime, we worked with the Perinatal Coordinator and the NICU to set up a plan for Isaiah's arrival.

You can learn more about Isaiah's beginnings here.  For now, I want to share a video with you.  Back in July, during Isaiah's Pam treatment (Pam is what I call Pamidronate, the medicine that Isaiah gets every 2 months to basically help strengthen his bones), we helped Nemours to create an advertisement for their perinatal program.  We are one of three families that were to be featured in the video.

Recently, we were able to view the final cut. Check out Isaiah passing out in my lap (ha) while we shared pieces of our story below:

We love Nemours. I mean it when I say if it weren't for them, that I don't think he'd be here.  When I talk to other families affected by severe OI, I often hear that the word lethal continues to come up (even after the child survives birth, doesn't need breathing support or if they do, only minimal support, etc).  Thankfully, the teams Isaiah has worked with at Nemours never let that word affect how they cared for Isaiah.  They only ever believed in Isaiah.

And for that, we're forever grateful.
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I'm attempting to get back to regular blogging this week for anyone who'd like to learn more details of our lives, from doctors appointments to play time.  I am working on posts for Wednesday and Friday about details of our first OI Clinic experience and his sleep study results.  :)