Happy 2017

Happy New Year! I cannot believe it's 2017 already...where did 2016 go?  If you only follow here on the blog, we kind of dropped off the face of the earth back in September....

Blogging, parenting two littles, and juggling the lemons life has been throwing my way has taken over my brain.  I used to find time to blog when Isaiah would nap or go to bed for the night....but he no longer naps regularly (BIGGEST SIGH EVER) and Jacob is still working on his nap schedule.  In those rare moments when they are both down, I totally sit in the quiet...

and just sit.

If you follow us on social media (you can see my instagram account on the right side of our blog), you know we've been chugging along and keeping busy.

For Halloween, Isaiah dressed as his favorite Disney villain- Captain Hook!


and of course Jacob was his trusty sidekick Mr Smee...



I threw the costume together myself.  Because of Isaiah's size, most of what is available in stores is really babyish looking...so thanks to the Dollar Tree (the hook, hat and sword), Amazon (the ruffled shirt), and what we had around the house, we DIYed a pretty fun look for him.  His daddy created the "Jolly Isaiah" (Hook's ship is called the Jolly Roger).  It was even on little caster wheels (you know those wheels that come with plastic drawers?  Well I never use them and yet continue to hoard them.  Glad I've kept them around! It really made the ship "sail" smoothly.

Before we knew it, it was Thanksgiving.



We spent the holiday with family and Isaiah cracked up at his shirt the whole day... "turkey. pie. nap. rePEAT!"

We went right into celebrating Isaiah's favorite holiday on the day after Thanksgiving...



and then we hit a snag...a week before Christmas, Isaiah caught the dreaded stomach bug which hit him HARD and so quick.  It started at 10pm, when he had been sleeping for hours...and thank goodness his daddy heard him choking because I had been so exhausted that I didn't wake.  Isaiah can't yet independently sit up so when it hit him, he couldn't get up to get it out and he was in such a panic that he didn't think to turn on his side.  Dave luckily picked him up....it was a mess....but the alternative would have been much worse.  He ended up waking about 6 times total overnight to throw up.  It was just awful.  We started laundry at 3am because we ran out his bed protectors and had to get creative....the next morning I gave him some Tylenol and it helped perk him up a bit.  He hung out with us in the living room watching Christmas movies on the couch and falling asleep for the rest of the day.  I didn't force him to eat or drink, but I constantly offered.  When he asked for anything, he'd throw it up as soon as it was swallowed (no matter the amount).  Awful. His daddy had the stomach bug just a week before and all he wanted to do was sleep....so we allowed Isaiah to do the same, thinking it was good for him...but we soon learned that dehydration sets in much much faster in a child than an adult.  When he awoke in the morning, he was still refusing to eat or drink, very sleepy, and whenever we'd pick him up, he's talk in crazy jargon.  I got panicked that maybe we were wrong on thinking it was a stomach bug and that maybe it was his shunt malfunctioning (Isaiah has hydrocephalus and a shunt in his head to alleviate the pressure.  Shunts can malfunction and cause pressure on the brain.  Symptoms vary person to person which makes it very hard to guess when they are malfunctioning.)  This was a Sunday morning and I truly didn't know what to do...but thankfully Isaiah's favorite doctors responded to my texts/emails, I spoke to the neurosurgeon on call at DuPont, and and was advised I take him to DuPont (I considered taking him locally but we thought that the hospital that had previous images of his brain would be best)....so I did.

Thankfully, since I had spoken to the on call neurosurgeon already, Isaiah was put on the list to be seen in the ER so he was taken literally right away.

Taking a medically complex child to an ER is difficult.  OI is rare.  Because of the severity of his OI, he breathes differently.  Everything felt "worse" because of his being sick on top of his every day issues.  I had to be confident in my answers to questions so the staff didn't over-react (that sounds rude....but what i mean is, Isaiah's breathing looks labored to the medically-trained eye, but it's not it just looks that way because of the shape of his rib cage.  If I sound unsure, they are unsure and it just snowballs...).  Thankfully they got blood work right away and swabbed his nose to test for sickness.  The nurse tested his glucose level.  It wasn't even registering on her monitor- that's how low it was.  She sent it to the lab with a "stat" label for an exact number and asked if he's had issues with his sugar levels ever before....no.  He gets blood work done regularly before pam treatments.  It's never been dangerously low, but he hadn't been able to keep anything down in over 36 hours.

His level came back: 20.  The lowest they wanted to see the glucose level is 60.

I dont know how he wasn't seizing.

There was a swarm of doctors and nurses.  They accessed his port (he didn't even make a peep where he'd normally scream his head off).  The nurse very very quickly loaded him with fluids and what she called "a sugar shot" and just like that, he woke up, started crying, started talking.

Results from his nasal swab came back and he had tested positive for rhinovirus....which sounded scary to my panicked mom brain but turns out that's just a cold....(cough cough just call it that cough cough).

Isaiah was admitted because he was so very severely dehydrated.

We spent the next 2 nights inpatient.

I learned that a person who is 18 pounds typically drinks 24 ounces in a day....but my son is not typical.  Isaiah was getting that amount through an IV in his port.  After over 24 hours, his face and feet started to swell.  His daddy and I got concerned that we were overdoing it with the fluids....yes he needed them, but 24 oz a day just seemed like it was too much for his body.  Trying to explain that to doctors (when i am just a stay at home mom, no medical background)  is not easy, but thankfully,  the doctors heard me out and agreed to back off.  It was a bit of a tug a war over it, back and forth....because I lacked confidence on Isaiah's typical fluid intake....but eventually Isaiah agreed to eat and started to drink more like his normal and I asked to discharge.

I've now observed that Isaiah typically drinks between 18-20 oz per day. :)

The few days left before Christmas and the holiday itself were a whirlwind.  We let Isaiah rest as much as possible and thankfully he got his appetite back (and then some).  He went right back to singing Christmas songs and watching movies (He took a liking to Elf while in the hospital...I was watching it when I thought he was asleep ha...so we watched that quite a few times.).



Christmases of the past usually involved a lot of travel for us, but with losing my dad earlier this year and everything that occurred within just that week, we decided a quiet Christmas at home was just what we needed.

Santa, and quite a few sweet family members, spoiled all three of our little loves.


Isaiah loves his very own dollhouse and Jacob loves his activity table...and Santa never goes wrong with treats for Carl lol...

Dave and I felt extremely grateful we were able to be home together with our little family.

The rest of the break was recovery mode for the entire household...with all that togetherness and time of sharing, Isaiah spread his cold to ALL of us. I can laugh about it now...ha

and now it's 2017.  Geez I still can't believe it.  Along with the hustle and bustle of life with a baby and a toddler, my mom has been sick and in and out of the hospital....that's taken over a bit of my brain.  I don't so much want to share her medical issues without her permission but it's been so hard on her and my sister and it is difficult for me bc we don't live as close as I'd like us to.

On top of everything, toddler life with OI....this year should bring BIG changes to Isaiah's life in reference to his OI- namely, RODS.  Isaiah is due for his next dose of pamidronate soon and along with that we have a Dexa scheduled.  The Dexa will show his doctors Isaiah's bone density and will give a clearer picture of how well Isaiah's bones will hold rods.

I also want to focus on Isaiah's teeth this year and getting them protected with caps...he has his next dentist appointment scheduled for February, so we'll hopefully work on getting that ball rolling then.

I hope your holidays were magical and your new year has started off wonderfully. Hopefully I'll pop back in again SOON! <3

Pam + OI Clinic

Right before Isaiah officially turned 3 years old, we had a quick trip to duPont/Nemours for Isaiah's pamidronate treatment.

We made our way to the Ronald McDonald House on Sunday, since we were expecting to be at the hospital bright and early for treatment.  We actually spent a lot of time enjoying what the McDonald House has to offer this go round....usually we stick to our room, dinner, and the play rooms.  This time we checked out the library, did a craft one night, visited with some therapy dogs, celebrated Isaiah's birthday after the monthly meeting....we made lots of fun memories this trip!

Isaiah decided to torture us party all night Monday night (from about midnight to 4am)....and yet was still in ok humor for his first day of pam treatment....it was a long morning (especially for Dave and I) but was made so much fun by getting to hang with our pal Gavin, his family, and our new friend Maddie and her parents.  She was so sweet with Isaiah! After pam was done for the first day, we headed to OI Clinic for x-rays and discussions with Isaiah's geneticist, his orthopedic surgeon, the PT we've worked with since Isaiah left the NICU, and more. 

Isaiah fell asleep in my arms for some of the discussions, but ended up waking and showing off how he can scoot when laying on his back and how he can sit up on his own.  His x-rays also did some showing off for him....his bones look beautiful.  They are looking better than ever before.  Guys, his body is looking ready for rods!!!! Last year, his bones just weren't ready; if rods would have been surgically placed in his bones, they would have likely migrated right out, which would have meant more surgeries.  But now, his bones are longer, stronger, and should be able to hold those rods...and he is showing interest in getting moving, which is something he wasn't showing last year.  

We're expecting to schedule his first rodding surgery (his orthopedic surgeon does 2 bones at once, so it'll be a few surgeries over the years) for the spring, unless Isaiah fractures badly before then.  If he does, it'll be done asap.  Those little leggies will be straight before we know it!  Until then, we're going to continue to push Isaiah to develop muscles in his legs by having him weight-bearing in his new gait trainer (that finally came!) and continue to give him incentives to get sliding and scooting on the floor.  His arms have really developed some beautiful muscle thanks to pushing himself in his chairs, so if we can help develop his leg muscles?  

The rest of our trip went very smoothly and quickly....Isaiah had an adorable therapy dog and his owner come and visit us during day 2 of pam and Isaiah just loved petting him.

I spy Gavin! :)

He thankfully napped and slept well Tuesday into Wednesday which helped all three of us really enjoy the rest of the trip...

Sitting next to the August birthday cake! Isaiah was the only birthday kid there for the month of August....and he sure enjoyed that cake!

Working on a craft after dinner on Tuesday at RMH

And now we are switched to the 12 week dosage schedule since Isaiah is officially 3!  This trip was so positive and full of good news!  Hopefully they can all be this good. :)

An Update

Where has this summer gone? Oh I know, to doctor's appointments, therapies, and chasing after Isaiah.

I wanted to share a big detailed post about our Disney trip but time has been flying and my ability to sit down anymore (without falling asleep) is so limited.  I did however wake up at 3 am the other day and couldn't fall back to sleep for the life of me thanks to pregnancy-induced carpel tunnel (I didn't know that was a thing! but now I can tell you it is and it stinks) which enabled me to finish putting together a photo book on Shutterfly (with my left hand because my right is out of commission thanks to the carpel tunnel)...

Click here to view this photo book larger

Photo books are the perfect gift for any occasion.

It was an amazing trip!

Since our trip, we've had a lot going on! Isaiah had another pam treatment (directly after we came home from Disney. not sure what we were thinking scheduling like that!) and sleep study.

Dave took him to his sleep study which gave me an opportunity to get a good night's sleep over at the Ronald McDonald House.  The study went well...so well that Isaiah's pulmonologist told us that Isaiah no longer has obstructive sleep apnea. WOOOOT! It could definitely reemerge at some point, but for now we celebrate....and call the oxygen company to check if the doctor discontinued our script for everything so they can come take the backup cpap that we've had around "just in case".

We've spent the summer doing lots of summery things like swimming whenever we can, playing in the water table, and blowing bubbles...but as it's gotten hotter, it's gotten tougher for me to be outside, so we're also getting crafty and finding ways to have fun inside.

I've also spent a bit of time trying to get Isaiah's gait trainer.  We ordered it two months ago and now finally have an approval from insurance! There was quite a bit of miscommunication that I had to figure out once I had all the information on the company that Isaiah's PT had gotten the ball rolling with...but once I had the information and touched base with Isaiah's doctor, the approval came less than a week later. (If only I hounded two months ago?)

And we've just been getting ready for Isaiah's baby brother to arrive and get Isaiah ready for preschool! Details on that in the near future (I'm attempting to get some posts written over the next few days so I can stop being so neglectful. ;)

Breathing Test

Yesterday morning, we ventured to DuPont for a breathing test in their PFT Lab.  (PFT stands for Pulmonary Function Test.)  Isaiah's been doing remarkably well in terms of his breathing....he hasn't needed his C-Pap, he no longer sleeps with his pulse ox (unless he's sick) because we don't need to monitor his oxygen levels....but last year we did this test and found out that Isaiah would benefit from oxygen when flying in an airplane.

Last year's test was traumatizing...for everybody involved.  Isaiah cried.  I cried.  Techs teared up.  The doctor held Isaiah's iPad to no avail....Apparently after we left that day, their team sat down and said "how can we do this better?" (I would have thought they would have chalked it up to a grumpy and overwhelmed one year old (and a crazy over-emotional mama)....but nope, they are awesome and decided to reevaluate things.  They now test one less aspect than they did before.  The mask isn't as invasive and intimidating (last year's mask was covered in extra sensors and tubes)...

When we arrived yesterday, it was like they were waiting for us as this was the real test of their changes.  We were a bit more prepared this time...Isaiah is a year older, and although he does still cry and fight things, he handles them better than last year...even if it is just slightly better.  He has his wheelchair and thats what I try to keep him in when he sees his doctors and does tests as it gives him a sense of being in control of the situation.  Daddy came along to give Isaiah a second, familiar and loving face as he sat during the test.  I also let him do as he wishes on the iPad, which is his favorite thing in the world...and that's really what got us through this year's test. 

The test is just ten minutes.  They basically lower the oxygen concentration in the mask and have him on a pulse ox to monitor his oxygen and heart level.

and once he was calm, he did beautifully.  They were able to get accurate, fantastic readings.  Isaiah sat mostly at 97%.  He did blip down to 93% once but it was so quick and not a concern.

and so we are so excited because ISAIAH DOES NOT NEED OXYGEN when we fly to Florida this summer!  Last year, along with his stroller, travel highchair, car seat, car seat base (which we should have checked...lesson learned), and our regular carry ons for us and Isaiah (cough cough diapers, toys, SO MUCH EXTRA baby needs), we had to carry an oxygen concentrator (which was much bigger than we were anticipating) and a bag along with it (which held our pulse ox machine, extra probes for that, oxygen cannulas, tubing, etc etc etc).  I am exhausted just thinking about it.

This time we are cutting the carry ons down, cutting the high chair (well that'll go into our checked baggage) and car seat base, and trying to go lighter....except well, we are bringing his wheelchair along with the stroller (and both will be gate-checked).

"Hello? anyone there? My parents will probably still overpack."

I keep seeing bringing both as a positive....At the airport, he can travel around in his wheelchair and we can use his stroller to carry our carry ons and car seat. ;-) We weren't planning on bringing his wheelchair, but with Isaiah's independence and our comfort level with Disney World (yep, we're going back!), we want to have it for evenings here and there at the parks where we'll plan to just have dinner and meet a few characters.  We also want it for around where we are staying so he can have some down time in it.  (Last year we'd put him in the crib to unwind midday and he would just roll around and yell like a maniac.  SO MUCH ENERGY from so much to see! The chair will get that energy out so he can get a good nap...hopefully....in my dreams.)

Seriously though....can't you just picture him wheeling up to Mickey? (ahhh my heart is bursting)

Honestly, I was not happily anticipating going through everything to get to Orlando and home....but now that we don't need to bring oxygen because our baby is so strong??? Let the count down to summer begin...Bring on our Disney trip!

and thanks to the awesome team at DuPont/Nemours for changing that breathing test to make it less scary for our Isaiah.  You guys rock.

Pam Time and MRI

The older Isaiah gets, the tougher it is to get a moment to sit down and post to the blog! Sorry to those that only follow the blog, we're doing fine, but last week was a BUSY one!

Isaiah and I made a trip to Delaware for Pam (pamidronate- the medicine he gets to help his bones).

Honestly, it all went great!  He was such a big boy and handled having his port accessed (basically got an IV going but instead of in a vein, they use the port he had placed when he was 6 months old) like a champ...his medicine was flowing before we knew it and he was rolling around Day Medicine like he owned the joint.

"Can you show me where the door is?"

Pamidronate is done a few hours each day over 3 days for kids Isaiah's age at DuPont. (That varies by doctor and protocol).  We normally stay at the Ronald McDonald House across from the hospital but since they are renovating right now, instead they helped us get into a local hotel about ten minutes away!  It was super convenient for us and the same rate as staying at the McDonald House.  They even had an amazingly generous welcome pack for us at check in at the hotel.  We were still able to have meals at the house if we wanted to and play in the playrooms...we had dinner and playtime both Monday and Tuesday night.

Here's a little collage of Isaiah at dinner, playing pretend in the Pirate Room of RMH,
and opening his Welcome box at the hotel.  He loves his new teddy bear named Steamer.

I think the only tough part was that the hotel didn't have any cribs available, only play pens...and King Isaiah does not sleep in a play pen...apparently....he had very rough sleeps when I tried to have him sleep in the play pen so instead he joined me in my bed.

Basically taking it over.  HOW does such a tiny little boy take up a whole queen size bed?  Like that. (Picture above.)

Day 2 is usually our easiest day (since he's already accessed and doesn't need to be deaccessed), but it proved to be a rougher day than normal...we had a late start due to some technical issues with the pump and I had a mini heart attack at the end of his infusion but it all worked out.

To make up for a later exit than expected, we grabbed Isaiah's very first Happy Meal through drive-thru...

He loved it.

Our third and last day in Delaware started EARLY (6:45. at the hospital for breakfast.), we had an early morning MRI to check on Isaiah's shunt and hydrocephalus.

This was Isaiah's third MRI.  His first he had sedated, and it helped diagnosis his hydrocephalus.  His second was an unsedated fast MRI; I actually went in the tube with him. He handled it better than I did! This third one was similar to the his last MRI, unsedated and and fast....but he's older now, more observant...more nervous.  this time he cried and jumped whenever the MRI made noise and I ended up having to hold his chin so he didn't move his head all around...this time it felt a bit like torture....putting Isaiah through these things is so tough sometimes....I know it's our normal, but it doesn't make it easy.

Anyways, after his MRI we started Isaiah's last day of pamidronate.  We were (luckily) able to schedule a follow up with Isaiah's neurosurgeon only an hour and a half after the MRI (in the morning during pamidronate)...normally you can't leave Day Medicine when the medicine is flowing, but a nurse from the MRI area was able to help us and tag along upstairs.

What'll happen if I "push da button"?

We met with the doctor who showed us the latest pictures...I made this little collage to compare...the first is not exactly the same view as the other two but it shows you all of the spinal fluid in Isaiah's head at the time (all the bright white).

We're pretty happy with the results.  His ventricles are smaller but not too small (you don't want them to shrink rapidly from what I understand) and they are more symmetrical than they were before.  Progress.  Isaiah's doctor seemed happy with the results and this time we don't need to see him for another year (whoop whoop! no offense Dr C).

After our follow up, we made our way back to Day Medicine and finished up with Pamidronate, Isaiah was de accessed and we hit the road!

Thanks to being there early, we actually finished the earliest we ever have...and were home by 1:30!!!  We had a nice lunch and Isaiah was actually able to nap in his own crib!  Home sweet home, for sure.

The rest of the week was full of a visit from another OI family from out of state...and it was awesome!  Updates on that soon. :)

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
___________________

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.  :)

Pam Recap

Sorry for the lack of midweek posting....some things got the best of my brain and I just couldn't bring myself to open the blog.  There's some confusion with our insurance and Isaiah's care this past week...and once things are done and settled I'll likely share what happened (if that's legally allowed), but for now, let's keep it vague so I don't go into a panic attack...

This is Isaiah's "Mom, can I have a ball or maybe eight of them?" face.

Good thing this cute kid is in our lives to distract us from our worries.  

We're home from DuPont. We got home Wednesday night after what was a wonderful trip in regards to Isaiah.  Truly, Isaiah was a rockstar.

Isaiah tried to take over the magic show at the McDonald House on Tuesday night.

He was pushing himself everywhere he could...we were able to visit the NICU and see some of his old doctors and nurses.  He wheeled laps around Day Medicine while his pamidronate was flowing.  He was all over the place at the McDonald House, and it was thrilling to have people who have seen him in the past tell us how impressed they were with his progress.

He even showed off for Dr B and his PA....lifting his head like this...

and um.....even showed them how he is for real trying to crawl.  ISAIAH is trying to crawl!

The last two months, Isaiah has made leaps and bounds...but the most have been within the last two weeks that he's had his wheelchair.  He's talking more.  He's moving more.  He's pushing himself physically beyond his own boundaries.  It's amazing.

Dr B seemed pretty darn impressed with him...to the point that he mentioned a magic word...RODS.

We think maybe maybe maybe it's time to start talking RODS.  For those that are reading this and thinking "uuuuh, what does that mean?", well, you know how the bones in Isaiah's legs are bowed?  Dr K will at some point place rods in those bones, straightening them and giving him something stable to (someday) possibly walk on if Isaiah is interested in that.  They are also basically internal splints, helping to avoid more severe fractures that can occur with extra movement/weight bearing.

Dr B has signed Isaiah up for a September OI Clinic appointment. OI Clinic is basically a cluster of appointments with Dr B, Isaiah's geneticist, Dr K, his orthopedic surgeon, and a PT at Dupont.  It's there that we will talk about rodding surgery, Isaiah's progress, and more (well, I don't know because it'll be our first clinic! lol).

I'm hesitant to get too excited.  Partly because of the issues we had this week with insurance, and partly because I don't know if Dr K is really ready to talk rods yet....

but gosh, RODS! AHHH! I won't lie, when I see Isaiah start to try to crawl, I hold my breath, worrying about the possibility of a fracture...

But I suck it up and help him in ways his PT has shown me...because CRAWLING IS AWESOME.  I make sure his legs are inward so his hips are lined up correctly, and we're working on helping him get that darn right arm out from under his body....his pushing his wheelchair should help with that too.  It'll really help him to build his upper body muscles, which will help him to hopefully lift his upper body before we know it.

Guys, there was a time I didn't think I'd ever be able to talk about Isaiah crawling.  And lifting himself with such strength!  Ahhhh!  He is a determined little guy, and we are very blessed parents.

Pam Time!

Yesterday we checked in to the Ronald McDonald House, our home away from home, for Isaiah's next Pam-cation ;). For those new to the blog, (hi there!) Pam is short for pamidronate, the treatment that Isaiah gets to help his bones. We come to DuPont every eight weeks for it.  It's a treatment that takes 3-4 hours a day, each day, for three days, so we stay at the McDonald house across the street to make it easier on us.  It's an outpatient treatment so we do it all together. As Isaiah gets bigger, the amount of days we do this will be less, so at some point we'll just be doing this one day every 16 weeks (can't wait!).

For now, we take advantage of having the McDonald house across the street and make a little vacation out of things. Usually when we arrive, Isaiah has a hissy fit, but this time he rolled in with pride. He wanted to check everything out.

We had a great evening with his buddy, Gavin, and headed to bed early to ready ourselves for the first day. 

The first day of Pam is always the toughest.  Isaiah has to be weighed, measured, and accessed. When I say "accessed", I'm referring to the nurses accessing the port in his chest and getting it ready so that his IV fluids (the Pamidronate) can flow. If he didn't have the port, they'd start as IV. 

We're adding to the long day with a check in with audiology late in the afternoon. 

I'll be updating Isaiah's Facebook and my Instagram (both of which you can get to, whether you are on either or not, by just clicking on them over on the right side of the blog so keep an eye out. :)

Photos for DuPont

A few months ago, I was asked if Dave and I would consider having a professional ad agency contact us to work on some advertising footage of Isaiah for AI duPont.  As you know, I feel very strongly for duPont/Nemours.  It's a scary thought, but when I think about if we would have stayed with our first path of care, I don't think Isaiah would be alive.

Clearly, I just had to say yes... ;-)

The doctors at duPont believe in Isaiah, and always have.  They never wanted to write his story for him before he was out of my belly and they've allowed Isaiah to sail the ship, be the captain, so to speak.

They've always provided Isaiah with a "team".  For example, although he's seen multiple doctors from the pulmonary department during inpatient stays, he's only followed by Dr H, which offers us the consistency he needs.

I love the nurses, respiratory therapists, x-ray/MRI technicians, PAs,...everyone.  Everyone treats us kindly.  Everyone that we've worked with remembers our names and we are always greeted in the hallways, lobby, and cafeteria by friendly, familiar faces.  I especially love that Isaiah can see his old NICU doctors and PT and that they can see how much progress he's made.

I love that they listen.  I appreciate that Dave and I are respected as being the ones who know Isaiah best.  I am grateful that no one feels like I am stepping on any toes when I ask to be the one to position Isaiah for a surgery, or that when he was in the NICU that we asked to do everything but touch the machines.  I also love that I can ask a question ten times to be sure I understand and they answer with patience, wanting me to understand, not just saying to "trust them".

AI duPont Hospital has helped us help Isaiah since before he was born, so obviously when they asked for us to be a part of last year's radiothon, I had to say yes because I'd love to help duPont.  When they asked us to take photos and video for advertising, I didn't hesitate to say yes.  They are a big reason he is here today.

The photos in this post are just a fraction of what Ben Van Hook and his team from &Barr took.  (They also took video and even Carl hammed it up for them, curling up, belly in the air, on Isaiah's nursery carpet when Isaiah and I read a book.)  

They can be used for anything the marketing department of Nemours chooses to use them for, from brochures for the orthopedic department to a billboard on I-95.  I have friends that have friends in that department, so hopefully they'll let me know if Isaiah's photo/video is pulled for anything. ;-)

And we're not done yet, next week is Pam time again! (Can you believe it?  It's been 8 weeks already?) During/after our treatment, we'll be meeting with someone from the Marketing department who will be interviewing Dave, Isaiah, and I for Perinatal program at Nemours.  I'll be sure to share that interview with you once it's pieced together and live. :)

And if you see us in any Nemours commercials or advertising, let us know!  I feel like Isaiah advertising for them makes him an official "dupont kid" as I've always called him (and hey, they now have an official OI clinic label, which means he can be a "dupont adult" there too! That label gives them the ability to work with OI patients with OI until they are 35...aka Dr B isn't allowed to retired until Isaiah is 36. #kiddingnotkidding).

<3

Pam, Pulmonary, and HOME!

So, seriously, didn't Isaiah's MRI look awesome!? Yep, I'm still talkin about it. ;-)

Yesterday, we checked out of the McDonald House bright and early, and headed over to DuPont for breakfast, a quick breathing test (I'll explain more on that in the future, but all is good!), and our last day in Day Medicine for Pam until July.  

We find different ways to entertain ourselves in Day Medicine. But when you have about 4 hours in one place, waiting for medicine to slowly flow, you get creative.  I brought our house a ton of toys (and a few books) each day so thanks to them and our friends, we had lots to do.

Here is Isaiah playing with blocks on Day 2.

Isaiah was rocking sitting up whenever he was up playing and eating.

On Wednesday, we had an appointment with Isaiah's pulmonologist, Dr H.  We check in with him every 4 months to make sure Isaiah continues to do well in the breathing department.  He checks his CO2 levels, listen to his lungs, and ask me lots of questions.

Waiting for Dr. H.

All continues to go well and we were reminded to schedule another sleep study for September.  (boooooooooooooooooooooooo)

Last day of Pam!

The last day of Pam went smoothly.  I helped de-access his port when all was said and done by removing the tape myself; the nurse even showed me what to do with the equipment when de-accessing him (aka taking the needle out of the port in his chest)...I'm not sure I'm brave enough to ever do that, but if I did need to at some point, it's good to know.  I know they showed me when Isaiah first got his port placed, but I was too freaked out by everything to even look at it then.... all my friends/family that know my fear of needles should be so proud of me nowadays. ha

The drive home was quick, especially for Isaiah.  He fell asleep as soon as we were exiting the parking lot and didn't wake up until we were down the road from our house! lol  

It's wonderful to be home! Now I need to get us all unpacked and back to our normal.  :)

Happy Weekend!