A midst the craziness of the first day of school yesterday for Rachel and Cordelia (grade 2 and SK), Kingsley had his follow up appointment with his neurosurgeon. The MRI showed nothing of interest! Everything was stable - his ventricles, his (potentially tethered but asymptomatic) cord, his (also asymptomatic) Chiari Malformation, and his syrinx which is all the same size, no bigger, no smaller and not causing any drama.
Such. A. Relief.
The girls had great first days of school. They both love their teachers and were excited to see their friends. No tears!
except Kingsley. he cried. smiling for pictures is pure torture before 9am.
I realize I'm dreadfully overdue for a Halloween report, to the point where no one even cares about Halloween anymore! But, some big things happened on Halloween, so trudge through it.
Firstly, Kingsley rocked his boat. He has been asking at regular intervals to go to a Halloween party and anything remotely resembling a pirate his him growling: "I'm a piwate! AARGGH!!" He was a huge spectacle (like that's new) and everywhere we went on Halloween he was fawned over. I'm telling you, wheelchair costumes are unheard of here, to the point that a lot of people did double and triple takes before they realized that it was actually a wheelchair and not just some insane mom putting a huge box on their child and making them walk around with it.
Halloween morning, King had his follow up with his neurosurgeon. The MRI had shown nothing that would cause the eye issues. Great news! Especially since his eyes have gone back to normal...?? We're all a bit baffled. I have photographic evidence, this crazy eye thing was witnessed by a lot of people, it wasn't in my head. Now it's gone and there was nothing to cause it in his brain. His ventricles were a tad bigger, but she has decided that it's because he is bigger and thus, his brain is also. September (and October) were a bunch of mostly-unrelated, flukes that sum up Kinger. Good enough for me!
Except one little blip where she said that at some point he is going to need another decompression because there isn't a lot of space back there. She said we will wait until he needs it, to watch for the signs. Insert knife directly into my heart and twist. Ouch. I'm not thinking about this one. I will deal with it when I need to.
Anyway, next up was a party at his playgroup, home for a nap, and then we got the girls and were ready for Trick or Treating! It was a dark and stormy night, unfortunately. Ever since Rachel was old enough to go out, we have been going Mall'oweening. This has worked out perfectly since curbs, driveways, walkways, and porches are terrible obstacles when you're a wee one on wheels and our malls are accessibility dreams come true. My charming little pirate cleaned up at the mall, to the point where I felt badly for the other kids ;) He got double candy at pretty much every store, despite my protests that a two year old really didn't need *that* much candy. We did attempt a few houses when we got home, which Kingsley was all for, but I was cold and wet and was pretty Halloween'ed out. All in all, a successful day.
Lastly, for those of you who have an interest in wheelchair costumes, Cassie from Beyond Measure has started up a blog to share costume ideas. If you've made a wheelchair costume before and want to share it or if you want to attempt to make one or just like stalking random things, check it out!
Yep, it's September, the month of doom around here. The month where Kingsley freaks us out and we get bad news. It's kind of getting creepy, if I'm being honest. September 1st hit and I was reminded that my nieces birthday was the next day. It hit me like a ton of bricks. Pre-Diagnosis Day. The day we got the results of the blood test that said something was wrong. I stopped my brain from going down there. It's been three years.
September curse is in my head. So what if September 2009 was diagnosis month, September 2010 was syrinx-not-gonna-walk month, September 2011 was deja-vu-scare-month? Don't get upset about stuff that wasn't happening.
The days ticked by. The curse loomed.
OK, I'll cut to the chase. Strange things are happening and I'm about 5 minutes from rushing him to the ER at any given point. I don't know if something is going on with his shunt or if I'm over-thinking. Either way, he's not himself and something really is going on.
Right now I'm in wait-and-see mode (which I now embrace, for those of you on the early end of this SB journey ;)). He's been grouchier and grouchier, eating less and less since the girls went to school. I have been excusing it as 'he's two' and 'he misses his sisters' and 'his entire world just flipped'. We've also had people in our house doing renovations all week, so it's been chaos. Totally justifiable to be grouchy.
Then he threw up (because he was crying and upset).
Then he threw up, again (for no apparent reason).
So, what's a shunt-fearing mama to do? I called the paediatrician and the neuro nurse.
Kingsley has had two days of total lethargy. He eats very little and didn't drink for nearly 24 hours (he is ok with that now). He doesn't have a fever, isn't fussy like he's in pain, won't tell me something hurts, no sign of UTI, ear infection, his eyes track normally and open fully (when he can keep them open), and his nose is not running at all. Just wants to sleep or snuggle all day.
I'm giving him 18 more hours and then we're trucking it to the ER if he doesn't get worse or better.
Limbo is a blissful place to be, in a way. Shunt malfunction is not fun. That means surgery, a couple days in the hospital and my poor kids having even more trouble adjusting to September this year. There's always the chance that this is related to Chiari in some way I don't know about, which is far worse. Then there's a chance that he'll wake up ravenous and ready to play. Or with a cold. Either way, we'll get through it like we always do.
Tomorrow is the true Diagnosis Day anniversary, which is still far and away the worst day of my entire life. Whatever this September brings, we can handle it.
I don't live in a state of constant worry. I thought I would, especially when handed the list titled: Signs of Shunt Malfunction. Fortunately, Kingsley doesn't give me a whole lot to worry about. There was his first autumn that caused me a huge amount of anxiety, obviously. Then there was this past autumn where I lost sleep a few nights. Then there have been a smattering of why is he crying?? moments where the the big bad shunt monster crossed my mind. But otherwise, no worry.
Last night, I didn't sleep. It was one of those nights where I did worry.
Kingsley wasn't sleeping. Again.
He's been off, not himself, grouchy, whiny, tired, miserable. My happy little boy is anything but happy.
Last night, as I rocked him in his room, the evil thoughts started creeping in...
Shunt. Cord. Chiari.
Oh, the dread that can creep into your mind at two in the morning.
Kingsley eventually went to sleep, after a dose of Advil and a long hour of rocking. In the morning, he was grouchy again, but I was able to run through all of the tests I could think of to make sure he still had all of the same movement and functioning in his legs that he had a month ago.
I mentally went through the Chiari warnings while we went about our morning and didn't find any that raised red flags.
I went through the all-too-familiar shunt watch list in my head and didn't check enough to warrant concern.
Then I stuck my finger in his mouth and rubbed his gums while he practically purred.
It's so much easier to think clearly when the sun is out, isn't it?
It's Valentine's Day!! What better time to think about the things that you love, right? ;)
Right now, I love...
My artistic little boy
I'll confess, introducing him to crafts didn't even cross my mind. So many things just don't seem to occur to me! He didn't show any interest, but again, that was probably because he hasn't had the same experience with exploring that the girls did. Once I (*ahem* or maybe his OT) brought the craft supplies to him, the floodgates opened. Now he's constantly rolling up to his sisters' craft table and causing mayhem.
There have been so many points along this journey where I pause, panic, and wonder: "Is he EVER going to learn to..." and eating non-jarred, non-pureed food was very top of that list. Because of Kingsley's Chiari, he didn't mouth toys or really anything. He had an extremely sensitive gag reflex which meant he easily tossed back to me anything that I tried to get him to eat. Once he had his decompression, we finally began making very slow progress with him eating food. Eating puree's was an accomplishment. Donating all of these leftover jars to the foodbank is an even bigger accomplishment. I'm thrilled to be saying good bye to puree's once and for all.
I zoomed in as he was opening wide to shove more of his cupcake in. Yesterday morning, he actually got mad when I tried to feed him his oatmeal. It was the first time ever that he wanted to feed himself a food that he wasn't one of his favourites. He wants to eat now and he wants to feed himself. It's been a great year.
He's fascinated with the stairs. Neither of us really knows where this is going to go or how he's going to finally master these things, but he is enjoying testing his limits and I'm enjoying watching him. So far, when he's at the top he just pulls himself forward until his torso is off the step and his arms are on the step below. Then he doesn't know what to do, so he pushes back up to the top. Repeat, repeat, repeat. At the bottom, he's just aching to hoist himself up. He's too small right now, but it's only a matter of time.
(don't freak out - he's not allowed near the stairs unless I'm there! the baby gate gets locked at the top unless I'm sitting on the stairs ready for him)
My sweet date
Kingsley and I are taking a break from some of our activities right now and just hanging out more. Last week we went on a date to the mall. He used to hate elevators, but now he loves them. He also loves timbits, people watching, spinning in his wheels and blowing kisses to all the older ladies that stopped to say hello. He likes to check out the wheels that other people are driving. He also likes to show off how fast he can go. He's a fun date.
The MRI is booked for December 15 with a follow up appointment on December 21, temporarily. They're still working out times since they want to do a full head and spine and that will take two appointment slots instead of the one they had originally planned on.
In the meantime, I have at least a dozen points in every day where I am convinced that we cannot possibly wait that long, something is wrong NOW. And then I have at least a dozen points in every day where I am convinced that I am overreacting because Kingsley is clearly as awesome as ever and not a thing is wrong with him.
Our concerns are that he had some sudden bowel and bladder changes in late September, followed by some rapid tightening in his hamstrings and odd twitching in his calf (tethered cord). He's also banging his head and grabbing/hitting his ears (shunt). He's waking at night, crying and he's much more irritable than his usual cheerful self (shunt). He's sleeping less and is eating less (shunt/Chiari). His language peaked and then has regressed, yet again (no clue). He's arching backwards in his Bumbobile and stander (Chiari).
On the other hand, he's teething and has been for about a month. His molar FINALLY broke through last night. He grabs both ears equally (his shunt is behind his right ear) and hits his head when he's frustrated, not really out of the blue (common in kids without language). His bladder stuff is all back to normal, his bowel stuff resolved when I dropped his PEG dose a little bit. He's fascinated with exploring his world now in both sets of wheels and seeing it from all angles - now that that is finally possible. When I'm with him or the girls are home, he's his usual self. When I go check to see why he's crying at night, he's all smiles and snuggles the second I get him in the rocker with me.
I just. don't. know.
While I am not remotely looking forward to putting him through another MRI, I am anxious to get answers. My house is benefiting from the stress, but my wallet is not. ;) I'm blazing through my list of Things We Need Done Before We Move. Christmas shopping is nearly done and if all goes well, the Christmas tree/decorations will go up in the next couple of days. I need the distraction or my mind takes me to places I can't function in. It doesn't matter. Either he's fine or he's not fine, but he will be.
Tomorrow, we meet Santa. I predict a lot of screaming. :)
After putting my baby on a bed and watching him go rolling through these doors
I was holding my breath, heart in my throat, not thinking, not thinking, not thinking about what was happening.
Watching the clock tick on, minute by minute, hour by hour.
Waiting to run and see my baby.
It's strange how much this anniversary has been on my mind. Although we obviously are not celebrating, it's so fitting that the one year anniversary of Kingsley's decompression surgery is on the American Thanksgiving, because I do not have words to say how thankful I am that one year ago his surgery went the way that it went; that I didn't think on that day about what could have happened, what almost happened, and what would have happened. Those are all the things that I'm able to start thinking about only now and it terrifies me.
It wasn't Kingsley's first surgery and it wasn't his last. There's no guarantee that he won't need another decompression at some point. He'll need to have surgery for his shunt again. Maybe many shunt surgeries. There's a good chance he'll need more detetherings. This is our reality. Not having those surgeries would mean not having Kingsley, which is something I can barely wrap my head round. And so we go through it.
But right now, today, he doesn't have to. Today, we went swimming and he did amazing. He paddled with his arms, floated on his back by himself, and blew bubbles. Today, he asked for (and received) hug after hug after hug. Today, he had two candy cane cookies for lunch, because he wanted them. Today, he smiled and played and pulled his sister's hair. Today is one more day that SB takes a backseat to life. We are so grateful for that.
For a long time I didn't want to admit this because I thought it was kind of weird, but then one day chatting with other moms like me I realized I am not alone. I collect hospital bracelets. OK, maybe not collect, but I keep them. All of them. Not just the new baby ones with BABY GIRL BELL written on it, but also the big red ALLERGY ones that Kingsley gets and anything else they put his name on.
I have quite the collection. Cordelia has only 4 bracelets from when she was born (including mine and somehow the one that is supposed to set off the alarm that managed to come home with us). Rachel has her birth ones, plus one trip to the ER when her whole face swelled from a mosquito bite. Kingsley has his collection from his birth, his two MRI's, his two surgeries, one ER visit for a shunt scare and now a second ER visit from last night. Lucky boy.
No worries, everything is okay, but we had a big scare! Just as I was about to head up to bed last night, I heard the strangest strangled cries from upstairs. I had to stand in the hallway for a few minutes before I could figure out whose bedroom to go into, as it didn't sound like any of my children. It was Kingsley.
My first thought was that he was choking. He couldn't breathe and was making the most horrible noises as his whole ribcage struggled to get in air. I scoured his crib, looking for any signs of what he could've been choking on. Nothing. Raced him downstairs and out the door for cold air. No change. Panic.
He wasn't crying, seemed totally relaxed about the fact that he could hardly breathe. I think that was what scared me the most. He just wasn't sick. If this was croup, wouldn't he be sick? And if it wasn't choking and wasn't croup, what was it?
That's where the mind flies to 'complications'. Things related to SB. Stridor is a symptom of Chiari. You know, that big bad thing he had that big bad surgery for last November? My mind went there. Jeff's mind went there. When I woke up my poor mom in the middle of the night, her mind went there. I don't have a clue how or why he would suddenly have Chiari symptoms again, but in the middle of the night it doesn't really have to make sense - I raced him to the ER.
About halfway there, his breathing quieted. I could still hear it from the front seat, but I had to listen for it. Thankfully, the nurses and doctors we saw did a good job of not laughing at my panic as they diagnosed him with very mild croup, gave him a dose of steroids and sent us home. Turns out, you can have croup without being sick. Who knew? They did say that if the stridor continued longer than croup would to come back and we'd investigate other stuff. He's doing great today. Some normal coughing, but his breathing is fine.
Kingsley is hitting another big number: today he is 18 months old. How fitting that my little Potter boy has a big milestone like that on the day the last movie is released! ;)
It sort of snuck up on me. I may have been blocking it out, if I'm being honest. I feel like we are hitting some kind of Point of No Return here, where babyhood is ending and the last few days of this SB thing being incognito are zooming by quickly.
Kinger is no longer a big baby. He's just shy of 23lbs, lost some of his chub, and on the low average side for height. When people look at him, it's easy to assume that he's closer to one year old than two. People look twice when I say his age. The not-walking stuff stops being a 'late bloomer' thing at this age. I'm hearing less about the stories of So-and-So's Baby Who Just Took So Long to Walk and getting more of the awkward smiles. It's a good thing he's so darn cute, or people just wouldn't know what to say!
Really though, the walking thing isn't bothering me much. I'm (mostly) over it. I'm getting better at judging when to toss the SB diagnosis into the conversation and when to just accept the awkward smiles.
What is starting to nag me, way back in the back of my mind is the rest of his development.
Today, at his check up with his paediatrician, she pulled out the Nippissing Checklist. I get them in the mail every few months and I don't sweat it when I check NO, NO, NO, NO... down the list for all of the gross motor and a lot of the fine motor or eating things. But today was different. NO, he can't stack blocks. NO, he doesn't know at least 3 body parts. NO, he doesn't follow basic instructions to get things or give me things without a visual prompt....
Up until now, I have been able to brush off any developmental gains he's not making. Cumulatively, he has spent nearly a whole month of his first 13 months of life in the hospital. He has had 4 surgeries, 2 of them on his head/brain. And then the ever-complex way that one developmental skill is so intertwined with a dozen more: he didn't know how to eat finger food because he wasn't interested in picking up and mouthing toys thanks to the Chiari issues, for example.
So, part of me is saying he doesn't know how to stack blocks because the only time he'd ever have practice is when I put blocks in front of him, which I don't always think to do. He can't take his shirt off because he's using his hands to sit up. On and on, I can excuse it away. However, I don't know if I should be anymore. Is there a point where I have to stop excusing small delays before they become big skill deficits? Or will he eventually catch up in his own time?
I don't have answers. It isn't causing me too much stress, truly, just starting to nag at me a bit. I talked to his PT about it yesterday and she's going to put in a referral to another team at the centre... I forget their title, but they specialize in assessments of development sort of stuff and will be able to tell me to either relax or start pushing for more. He's also having an assessment with a SLP in 10 days, so we can see what's happening there as well.
Hey! It's wait and see time! My favourite! I'm really good at this stuff. ;)
At least I have Harry to keep me occupied in the meantime. I might be a little bit busting-out-of my-skin excited about that.
Christmas Eve marked one month post op for Kingsley. So much has changed in the last month! So much is different than it was three months ago and is light years from where it was just six weeks ago. But there is still so far to go. With every day that goes by, I find I'm still holding my breath, waiting, praying that we see more changes. I feel like that clock is still ticking, counting down to some invented deadline of March 1 when everything that is going to happen will have happened and anything that hasn't returned is lost forever.
Where he is now though, is pretty amazing. My little rockstar has been showing off the past month.
Starting at the top: his shunt is doing fine. No problems to date. We are closing in on his one year shunt-iversary - less than a month to go. That's a big milestone. His hair has grown a lot lately. I'm tempted to get it cut. It will no longer go into a cute faux-hawk, so I've been settling for the classic church boy (or Senator Kingsley, for my sister), which makes me laugh. It's starting to grow back over his new scar, which is also continuing to heal nicely, or so I'm told. I don't have a lot of experiences with scars. His still seems angry red and huge to me, but what do I know. Soon, half of it will be covered by hair and it won't be so obvious. I feel like I'm hyper-aware of it when I'm out in public with him. I don't want freak-show stares and I don't want sympathy stares. I realize he has a lifetime of awkward stares coming at him and this is probably a really stupid and futile thing to be wishing for. So... there.
More head stuff... He had his third tooth pop through this morning! Top right. He's congested, but otherwise is taking it in stride. I have really good teethers! He is also chatting so much, which I can't get enough of. His little voice is adorable! He says buh buh buh buh bah bah all day long. In the past week he's added in puh, pa, and now MAMA! I think he means it, too, not just random mamamamama babbling. He does it when he's mad or when he's upset about something. He'll look around and sort of half-whine, half-yell it until he sees me and then it's all yell until I come rescue him. That's my boy. ;)
One more head thing. I have read about kids with SB having reactions to loud or sudden sounds. I can't recall if it was attributed to the shunt or the Chiari, but I've noticed Kingsley definitely has this problem. He hates when I use the handheld mixer on any level above one - instant screaming. The vacuum is also feared. The Magic Bullet is a nightmare to use when he's anywhere in ear shot. Christmas has produced quite a number of toys that seem to be on the same decibel. He got this cool car race track thing from Grandma that sends him into hysterics. The girls are in love with it, unfortunately.
(one of the scary toys is in the background. you drop a ball down and it makes a noise on each level. evil)
An exciting development for me is that Kingsley is beginning to eat more. Yay! He eats three meals a day, about 1/2 jar or 2-3 cubes for breakfast and lunch, a full jar or 4-5 cubes for dinner. He doesn't always eat it all, but he is getting there. He's currently into the meat/veg combos and not really interested in anything fruity or sweet. Definitely Jeff's kid. I've also caught him putting something in his mouth three times in the past week. That seems like nothing, but for a kid who gags on his own fingers, this is a big deal. He opens his mouth when you put things in front of him. Whatever you put in there, he will automatically spit out, though. Baby steps.
("You want me to do what with this cookie thing?" he didn't eat it)
Kingsley used to rock at tummy time. He would push up into a baby upward dog, pre-surgery. He could roll from his tummy to his back going left and right. He could do Superman's with his arms out, his chest up. He was just on the brink of army crawling. Since his surgery, this has been the biggest area of regression. It has bothered me a lot that he lost this area of strength, although it's for obvious reasons. I'm sure it didn't feel good, considering where his incision was. He's regaining the strength quickly now. He gets up on his elbows and tolerates being on his tummy without too much complaint. He tends to lie sort of sideways with his head down, then push up for a bit, then lay back down for a break. He is able to roll tummy to back again.
(down, up, down, up, down... this is as high up as he goes now)
The B and B stuff (bowels and bladder) are just fine. Nothing exciting going on there, which is just the way we like it. His urodynamic study in mid-November was fantastic.
So, moving along down the line we are at the big stuff. Looking back now, I'm able to think more objectively about what he was actually able to do by the time we got him in for surgery. The truth is - he couldn't do much. His PT had told me in September that he was functioning at L1, but I would be surprised if he was doing even that well by mid November. He rarely moved his legs at all. They mostly just were there, still. If they moved, I would get excited. It was a rare event. He also was losing some strength through his torso. He slumped in the highchair and couldn't hold himself up in the Bumbo very well. I had to put extra padding in his booster seat for when he played on the tray. When I would hold him, he'd snuggle into me, which I thought was sweet. But, now I realize it's because he wasn't able to hold himself up. Bittersweet.
Where is he now? Well. Realistically, probably still around L1/L2. But, so much stronger. He holds himself up beautifully. He has come further in the past 3 weeks toward sitting independently than he was even in the summer. It's so close. Maybe it'll be his birthday present to himself. :) He moves his legs a lot, I've mentioned that previously. He is just always moving them. They move from the hip, if you can imagine that? He tucks them up when he's on his back. When he's on his tummy, you can see them starting to pull up under him a bit. He uses them to push himself over, which he didn't before - previously it was all upper body and arching his back. A bit more strength there and he'll be able to flip from back to front. He hasn't shown quad functioning to his PT or my mom yet (I have no clue), but he has medial hamstrings (still, no clue). I can feel things working in his upper legs. If I hold his legs down straight when he's lying on his back, I can feel resistance. When I push against his feet, I feel resistance. I have so much hope for the next few weeks and months. I'm so anxious for more.
We have a lot more help lined up for the new year. Just before Christmas, we met with the PT from our SB clinic and she is setting up regular PT for him, in addition to his visits with his Parent Infant Therapist (who is an OT). They'll come out to our house, which is wonderful. He also has a post-op appointment with neurosurgery next week and clinic with the developmental paediatrician on the 10th. I have to remember to call and book his one year check up with his paediatrician. In February, he will see ortho for some baseline assessments, and then in early spring we start rounds all over again with neuro, uro, and dev paed. That's also when he'll go for the follow up MRI to show what surgery has done.
He was supposed to be measured for a standing frame in late November, but surgery has postponed that. I'm excited to get him standing. I'm trying very hard to look past the accessories that are coming and just focus on where it's going to take him.
I think that's it! Now, I have a birthday party to start planning, don't I? I can't believe my baby is almost one year old. It's so silly, I swear I wasn't this emotional when the girls turned one. Maybe it's because he's (probably, likely, maybe??) my last baby. Oh, my sweet little boy.
I can explain now what led Kingsley to having the surgery he just had and what the procedure actually entailed. He's in a ward room right now, so there are other parents there with their babies. I found myself telling his tale to another mama yesterday and halfway through describing his surgery I just stopped and started laughing. She was in there with her 6 month old daughter who has CP and just got a shunt put in. She just said, "Yeah. It's sort of crazy what our kids go through." It's hard for someone who doesn't have a kid with these sorts of special needs or medical needs to fathom this sort of thing. It's hard for me to fathom most days. It's literally so far outside my realm of normal that it makes me laugh to think that this is actually happening to my chubby, happy, healthy little boy. Not that it's funny, just that I don't really know how else to digest it all.
Well, Kingsley wasn't doing so well with his leg functioning. It wasn't just a matter of him not moving as much as expected, but more that he used to move more and now he moves less. We took him for an MRI to find out what might be going on. There was a large syrinx (like a cyst) right down his spinal column, top to bottom almost, putting pressure on his nerves. There was a larger part in the lumbar area (below your ribs) and a larger part in the thoracic area (mid back). His NS thought that the syrinx had been caused by this other problem he has called Chiari Malformation II. That essentially means that part of his cerebellum is not tucked up inside his head like ours is, but instead hanging down in his spinal column.
The CMII is most often asymptomatic. Kingsley's is asymptomatic - he's never had trouble with it, it just is. However, it's filled up the space at the top of his spinal column and not allowed CSF (the fluid that moves around in your brain/spine) to move from his head to spine and thus caused pooling in the spine, aka the syrinx.
So, the goal of surgery was to create more space there that would allow fluid to move freely and hopefully, in time, allow the syrinx to drain it self or something and ease up on the nerves. To do that, they had to a procedure called a decompression. It has a fancier name - something like posterior fossa decompression, but I may be saying that wrong. If you find this stuff gross, stop reading.
.... okay, I warned you. They made an incision from the bump on the back of his head, down his neck. Then *lalalalalalacoveringmyearslalalalala* they took out part of his skull and part of two vertebrae (C1 and C2) and half of a third, I think (C3) and then put a patch (Jeff likes this part - says they took something from a cadaver's thigh, which may very well be true) of some sort of dura tissue stuff and put it over the place where the bones used to be, but not on as tight. That was sewn in, then everything else (muscle) sewn back up over top. Ta-da. Big franken-line scar for Kingsley. :( I hope I explained that all right.
The MRI on Thursday night showed that there was indeed fluid now passing through around the cerebellum. Not a ton, but it's there. He also has a lot of swelling happening right now. Hopefully, when the swelling goes down, there will be lots of room in there for the fluid to flow. In three or four months, he'll have another MRI to see how successful it was. Hopefully, between now and then we will see a return of some of the leg movement he used to have.
And that is why my baby is sad. Although I did get a smile out of him today! Just one, but so worth it. He's off the oxygen now and other than seepage from his incision (not CSF), he is doing fabulously well. He's very swollen all over from the drugs he's on, but that is going day by day.
Saddest face ever. He mouth looked distorted from the breathing tube and he had red pressure sores on his forehead
His 'computers' as Rachel called them. He was well hooked up.
All his tubes and wires
Day after - looking a little better
Rachel is getting over a cold so she had to wear a mask when she visited
She thought she was the bees knee's. Was sure that everyone was going to think she was a doctor.
Can't go without popsicles! King had nice nurses.
Kept trying to tear off his mask, so he was switched to the prongs. Also better for his MRI to see how thing are going (they are going well).
This morning. Soooo puffy!! They say he'll get better over time, but he'll get pretty bloated from the meds before he'll get better. He's like a little stuffed sausage.
Free hands! No more IV's stuck in everywhere. Getting unhooked and ready to go up to the nursery wing, out of PCCU.
The top part of his incision. Bandages off and getting some air. It's about 4-5 inches long, I think. There was a bit of leaking at the bottom, but they don't think it was CSF, just blood and yummy stuff like that.
New room up in the nursery wing. A lot less monitors and a lot louder. He's sharing a room with 3 other babies. Single rooms are reserved for all the respiratory kids in this time of year. Booooo.
Snuggled up in his new room.
Today has been eventful. I've hardly had a moment to sit down. Monitors going off, other babies screaming and waking him up, nurses and doctors and specialists coming to poke and unpoke and do everything else imaginable. We got our first hint of a smile when we came in this morning. So awesome to see. But he's been pretty grouchy since. I also got to hold him and breastfeed him this afternoon. He was in heaven. Would not let me move a muscle or he'd just clench on with a definitive grunt of complaint. It's hard to see him so puffy and uncomfortable, but every day he's improving. He hates the nose prongs and is trying to pull them out now that his hands are free.
I'm going to go hang out with him for a few more hours before going home. He still has his ng tube in to feed him when I'm not around. They didn't want to trouble him with bottles since he hates them. The feeding team was fighting to get him an actual bed so I could side-lie feed him and not have to jostle him, but no winning that fight yet.
So... for an update. It's been confirmed and confirmed again. And then again. He does have myelomeningocele spina bifida. It's not going away, it wasn't a fluke ultrasound or blood test. I'm going to do my best now to explain what this all means for him and for us, since it's sort of a foreign thing for most people.
At about 24-28 days after conception (about the time the reality of pregnancy is setting in and you start wondering who to tell), the bones that make up the spinal column start to form. They are supposed to form a sort of tube with the spinal nerves all down the middle. What happens with SB (specifically the type our son has) is that the bones don't all form the tube. At some point, they just hang out in a straight line or arch instead of a triangle (which is a really bizarre thing to see on an u/s, let me tell you!). That leaves the nerves to stick out of the column. Then everything forms around the spinal canal, or in his case, doesn't form. He has a hole in his back where the spinal nerves are sticking out in a sort of sac. Because they are hanging out instead of tucked in all snug like they're supposed to, they get damaged by the amniotic fluid. The damage is not repairable and is permanent. And it happens pretty much right off the bat. Where the hole (or lesion, as they call it) is located makes a huge difference. It's like breaking your back. Where ever the 'break' is changes everything. This picture breaks it down with the different things that can be effected. http://www.spinalinjury.net/test2/as...cord_map02.jpg
Basically, where ever the lesion, everything listed below is a write off. So, lower = better.
Our son's lesion is either low lumbar or high sacral. Twice we've been told sacral only. We can go for an MRI, hope he lies still and not up against me too much and try and see exactly where. Or annoy the next u/s tech enough that they actually take the time to count vertebrae and find out for sure, but even knowing where isn't going to tell us much - the nerve damage can go up a bit higher than the lesion point. And the range of functioning is huge. As you can see in the picture, his bladder, bowel, and sexual functioning will be effected. The degree to which his legs are effected is what we don't know. Could be his feet, could be his ankles, calves, knees, quads... It's hard to face the unknown.
The other thing he has is something called Arnold Chiari Malformation 2 (ACII). It's a bit much to get unless you understand brain/brain stem anatomy, but essentially, the nerves hanging out his back pull his brain down the back of his head a bit. It makes for a wonky picture on the u/s that they call 'the fruit basket indicators'. He has a lemon shaped head, instead of a circle. This goes away by the time he's born, I'm told. This is what was flagged in our 20 week u/s as an abnormality, even though the tech couldn't see the lesion. The cerebellum is the part that is pulled down in the back, into a banana shape. The ACII likely won't cause many problems for him, it's pretty rare that it causes huge complications, it just is.
However, there is one more big complication he'll have. He'll likely (85%) develop hydrocephalus at some point. Normally, cerebrospinal fluid flows around the brain and spinal cord and then is reabsorbed into the blood stream. Currently, he has a hole in his back, so the fluid just comes out (which is how they screen you positive on the IPS - the fluid/protein in the amnio gets into mama's blood at unusually high rates. this is also how they know the type of SB, as myelomeningocele is the only type with a big ol' hole in the back). Hydrocephalus is when the body doesn't reabsorb the fluid and it just pools in ventricles in the brain, puts pressure on the skull/brain and gives you a big head. Left untreated, causes brain damage. Many SB babies develop hydrocephalus in utero. It's not as scary for babies to develop hydro as for, say, your or I bc their skulls aren't formed. There is less pressure on the brain, brain damage doesn't happen yet. BUT, better not to have it. Our son doesn't have it yet, his head and ventricles are completely normal. Yay! BUT (big but again)... once they close his back, the fluid will have nowhere to go and hydro will likely develop. If not before then.
Anyway, that big rambly leads me up to his birth. I was told that I'd have to have a c-section to reduce the chance of his sac getting damaged. However, my OB is now arguing this, saying I'm a prime candidate for a vaginal birth if she's ever seen one given that: his sac is small, his head is normal, this is my 3rd baby and the other two were both big headed chubbers. He'll likely be smaller than them, just because. But IF I go that route, I'd have to be induced. Which nearly defeats the purpose, in my opinion. And Jeff and I would have to be 100% convinced that we would never wonder 'what if' regarding the lesion/damage. And his neurosurgeon would have to agree. I'll likely have a c/s, but at least it'll feel like my choice, which helps for some reason.
So, the day he's born, he'll be taken immediately from me, either way, and they'll put stuff on him to protect his back, make sure he's stable and then he'll go for surgery. They'll open the sac, tuck his nerves back in, close the bones, dura, muscle, skin, etc over the hole and patch him up. He has to stay on his stomach and stay still for awhile after that. Then, they wait/watch for hydro. Sometime in the next week, he'll likely get a shunt put in. It's basically a pump/valve that will go into his head, into the ventricles and is attached to a long tube thing that goes into his abdomen. It drains the fluid from his head to his body where it'll be reabsorbed. It's there for life. And has risks of infection, malfunction, blah blah, big scary stuff, but saves his life. Then, after all that fun stuff, I will finally get to bring him home, about 10-ish days after he's born. And basically go into hibernation until spring!
There is an SB clinic at TVCC which includes our neuronurse (who is wonderful), PT, SW, urologist (bc of his bladder/bowel issues), and some other folk. Some get involved with me now, the rest later with him.
What terrifies us is the unknown. We know the details, we get what happens. But I have no idea how much he'll be effected, how his surgeries will go, how his health will be...
It also bothers me that I don't know why this happened. My OB did the whole Good Will Hunting 'It's not your fault' thing with me the other day, which I know, but of course I doubt bc that's what mama's do. She said folic acid only prevents about 75% of cases, and she believes it reduces the degree of SB in the rest, so not taking it would've been potentially worse. Why it happens at all though is a big fat unknown. My new least favorite word!
We met with the neurosurgeon and neuro nurse this past Wednesday and had a whole bunch of questions answered and got some new information. The only really bad news that we heard was that the referral said the lesion was lower lumbar/high sacral. We had previously been told that it's only sacral, so hearing it was higher was not pleasant.
The nurse confirmed from the u/s pictures and the referral that as of 2 weeks ago there were no signs of hydrocephalus or club foot/feet. But the Chiari II Malformation (odd head shape that was flagged in the first ultrasound) is present, which means that hydrocephalus is highly likely, especially since about 85% of kids with SB end up with a shunt. It's just a matter of time, but the later the better. The feet being okay had something to do with the lesion being lower or smaller or that his feet are moving, but I forget exactly what. It just meant something good, so we'll take that!
The nurse was so helpful regarding answering questions regarding what would happen in the surgery and the days after. She explained how I'd be able to breastfeed still, how he'd need to be positioned and held when we're able to do that, whether we'd get to see him (briefly, hopefully) or hold him (not likely) before surgery, and what he'd be doing in the meantime and with whom. Even though my OB is at St. Joseph's, he'll be born at LHSC which is where his surgery/ies will be. If he needs a shunt we'll see about it after his back is closed.
She explained his bowel/bladder stuff. Not fun stuff to hear, but she explained how they would test his abilities and what they'd be looking for while still in hospital. She said that if he's sent home without a catheter it doesn't mean he'll never have one and vice versa. Like his leg functioning, it's all wait and see and nothing we can really predict or do anything about until he's born. She explained how toilet training would differ for him.
She was very optimistic though about his functioning and kept referring to 'kids whose lesions are so low like his' and things like that. It doesn't sound low to us, but she'd know better!
The neurosurgeon herself was also very nice and helpful. They spent over 2 hours with us! Our neuro seemed an awful lot like my OB in mannerisms and personality, which I liked. She has recently come from Sick Kids in Toronto and before that practiced in Europe, where she's from originally. They had never before been asked about banking cord blood (which we thought was weird!), but said they'd search for any research out there being done with SB/stem cells, if there is any, and let us know.
I think that's about all there was. I have an u/s and OB appointment on the 16th, where we'll probably hear that the hydro has started. Or maybe not! We meet with the neuro again at the end of the month. They've also put in a referral to the SB clinic at TVCC, so we'll get connected with them sometime soon.
Jeff and I are doing alright. We've been reading a lot of books, talking to people, and connecting with some other families online (although things are pretty different in the states - they're all gung-ho on the prenatal surgery which I wouldn't do even if it was an option, which it is not). I have found a forum which has been very helpful to read about a whole bunch of other parents' journeys. It's been a pretty heavy 2.5 weeks, but everyone keeps saying pregnancy is the hardest part and that once he's here and there is less unknown/more adorable baby boy, we'll be just fine. I can't believe he'll be here in 14 weeks or so! Keep him in your prayers in the meantime.