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 think today was Kingsley's eighth MRI, but I could be wrong. I've honestly lost track. They're just checking things out, for once. We don't have any obvious concerns, but neuro wanted a follow-up after last years virus, and to check the syrinx, the possible retethered cord, space for the Chiari, etc.
This was the first time I've talked to him about it before hand. The Pre-Admit Clinic gave us a print out of pictures to talk to kids about and give them a visual description of what would be happening. Last night, we chatted about it and I showed him the pictures. He started crying. He said he didn't want the mask, didn't want to go to sleep, didn't want to go to the hospital. I was able to talk him down a bit and let him know that I'd be there and I'd be with him when he went to sleep.
This morning, I opened his door and his first words were: "Be-tuz I don't want to have a nap and I'm scared of the mask!!" Oh, buddy. :(
He did amazing though, truly. He was scared. He cried when the nurse put his bracelets on and kept asking for chocolate pudding. We spent most of the morning snuggling and reading books. When it was time to go in, I carried him and he told me how scared he was again. I laid him down on the gurney and the anesthesiologist was so wonderful, talking to him and trying to keep him calm. When they put the mask on, I reminded him that this was just sleeping medicine and it was going to be okay. He just nodded, held my hand, breathed slowly, and drifted off to sleep.
Crush my heart. Three year-olds should not have to be this brave.
He woke up in a great mood, making the nurses just fall in love with him. We watched Curious George and shared a popsicle until he was allowed to go. While we were there, one of the nurses showed me his hospital file. It was about three inches deep. This kid is going to need his own shelf by the time he turns ten.
Everything is fine. We saw Kingsley's neurosurgeon last week and she decided that the growth in his ventricles was not that severe and likely due to him growing. He is showing zero signs of a shunt failure, his shunt is all in tact and he's a happy kid. She is interested in his eye, which I now notice he's not using as much, sort of overcompensating with his right eye by turning his head everywhere to see things out of that eye. He's been referred to an ophthalmologist. And just to be sure, he's on the list for an MRI and should get in for that in the next couple of weeks.
In the meantime, he's doing great. Happy, healthy, good ol' Kingsley. :)
Our house is going on the market this week. It's been a little bit intense getting to the point where we are ready for this, so pardon my lapse. Hey! Here's a cute picture of Kingsley to make you happy:
Yesterday, Kingsley and I spent a lovely day in emerge. All. Day. We went in about 10:30 after taking our time getting up and ready, spending a bit of time with the girls, then we were off.
It started as a pretty quiet day in the ER. He got a bed right away, the resident came right over. He appeared to be quite baffled by King and the story I told him, as if his instinct was to label me as crazy, but that I was so convinced of it and there was that wonky eye, he had to follow through. The next doctor knew about shunts and asked all the right questions before paging neuro.
Tricky thing here is that this is our Thanksgiving long weekend, so both of the neurosurgeons who have done Kinger's big surgeries are away until Tuesday. Bad timing, buddy!
Our first trip was X-ray to check the physical structure of the shunt. It's all there, in one piece looking like it should. Phew. Neuro sent down the message that it didn't sound like shunt, but go for a CT just in case.
Hours later, we attempted a CT scan. It was King's first and it did not go well. Lie perfectly still on a bed with your head restrained? No thanks! That was about 6 hours into our visit and the first point where I almost cracked. We were so close to answers!! Fortunately, the nurses decided to give him some meds to make him sleepy.
I may have mentioned before that Kingsley is a rockstar. At this point, we had spent the whole day in emerg, not eating as much, not drinking as much, not napping, and not really going anywhere. I had 4 books, 1 car, and the iPod to keep him entertained. Kingsley, however had his wheels and his smile, which kept the nurses entertained and flocking to help out, which meant he had popsicles, bubbles, stickers, movies, and a hallway to race up and down. It wasn't a bad day.
Then he was given this sleepy med, right around shift change and the kid turned into an angry, drunk toddler. It was a bit amusing, to be honest, though not that much. He slurred, swung his arms wildly, and kept yelling about getting on his wheels and finding his own stinkin chocolate pudding since we were holding out on him. A topped up dose finally knocked him out and off to CT he went.
By this time, the ER was a very busy place. You aren't given a lot if privacy, really, given that kids are often loud and dramatic and only a curtain separates you. I kept catching myself feeling badly for the other parents... Aw, that's rough, their kid is in so much pain, that's so hard, I'm lucky King's not in pain and we're just... WAITING TO FIND OUT IF HE NEEDS ANOTHER BRAIN SURGERY. Nevermind, I win. Oi.
Angry, drunk toddler returned as the meds wore off, just in time for me to meet the Sr NS resident and find out that King's ventricles were enlarged - an indication that his shunt wasn't doing its job and that part or all of it would need replacing. He wanted Kingsley admitted and to have more tests the next day to figure out exactly what wasn't working and where.
So, here we are.
He was finally settled in his room and went to sleep just before 2am. I've had a very short sleep on a very uncomfortable chair-bed, but he's still dozing. I think today will be a sedated MRI and a shunt tap/shuntogram which I'm too tired to explain right now. I'm glad we're here, thankful in a weird way that this is 'just' his shunt, and looking forward to getting it dealt with and behind us.
We're cursed. Unless there is some miracle and Kingsley's eyes are doing what they are supposed to do, I will be taking him to the ER in the morning. I've just had enough of the worrying and the wondering and the second-guessing. He has too many things going on and I need to be confident that they are not all related to some bigger thing. He's seen a handful of doctors, specialists, therapists, and I've been on the phone with nurses and pestering my SB peeps online and all anyone can say is: "If it gets any worse, take him in." I don't know what 'worse' means! I've started asking them to define 'worse', because every call I make seems to be to describe something worse than before.
At the start of September he had a virus of some kind.
Followed by constipation for weeks and he was the grouchiest kid in the entire planet.
At the same time, his eyes started doing funny things.
Then, he started getting urinary issues.
And he had a tooth break through.
The constipation ended and he returned to the cheerful, funny kid he usually is - absolutely no sign of discomfort or illness
... except for the urinary stuff and the eyes.
Then, his neck got stiff and I just threw my hands up and called/emailed nearly everyone on Kingsley's list. The answer was, "If it gets worse..."
Otherwise, neurosurgery has bumped up his regular check up to this coming Wednesday.
This evening, I noticed his left eye was getting 'stuck'. It won't move left of centre.
This is new. Does this count as 'worse'?
Then his urinary issues completely resolved themselves. That's better.
I can explain (or the doctors we've talked to could explain) the cause of each individual issue on their own. I can't shake the feeling that they might be related, though.
Through this whole thing he hasn't had a fever or any other indication that there's an infection anywhere in him. He hasn't shown any of the classic signs of shunt failure... except that constipation can cause one and sudden onset of eye issues could be a symptom of one... but I'm not sure which came first: the constipation or the eyes. They were literally the same day. His gross motor and fine motor functioning has not changed.
So, now I'm done torturing everyone around me with my constant whining about not knowing what's going on with Kingsley ;) Tomorrow, there will be one of three things happening:
1. we'll find out something bad is going on inside Kingsley and find out how to deal with it and I will feel crazy for not taking him in earlier;
2. we'll find out I'm completely paranoid and he's got a bad case of ToomuchiPaditis and I'll feel crazy for wasting Kingsley's good mood at the ER; or
3. there will be a story on the news about this wild mamabear who attacked an ER resident after being told 'wait and see' for the billionth time... in which case I'll feel a little crazy for being on the news. ;)
I'm ready. Wish us luck.
In the meantime, distract yourself by looking at this gorgeous shot of King. We had a photoshoot with the very talented Stacey Hanlon yesterday and the cuteness was overwhelming.
Haha! September did not let us off that easy. Kingsley is doing so so so great. He's feeling great, he's back to his super happy, sweet self. We went to the paediatrican yesterday and everything is great.
Except for this eye thing. Do you see it? It's not the angle. It's sporadic, not constant, and it's brand new because we watch this carefully for it. Off to call the optometrist...
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?
I almost missed the fact that yesterday was Kingsley's two year Shuntiversary. This is a very exciting thing in our world.
Kingsley has hydrocephalus - which means that there is too much CSF (cerebrospinal fluid) in the ventricles in his brain. This is a problem as it would just make the ventricles grow and put pressure on his brain causing damage. Years ago, people with hydro would've passed away or had severe cognitive impairments, but in the last 30-40 years, a shunt has changed the lives of people with hydrocephalus. It's like a little valve/pump device that is behind his ear, through his skull and into his ventricle. It drains the fluid out of his brain and through a long tube (that you can see under his skin if he's topless) down into his abdomen where it is reabsorbed by his body. No pressure on his brain, no damage caused.
The one downside of this shunt is that it is not perfect. It can malfunction, become clogged, get infected or even break apart. It's quite common for people with shunts to need a shunt revision - in fact about 50% seem to need at least one surgery in the first year with a shunt. I think most kids need a revision by the age of five, but more and more I'm hearing about kids passing that marker just fine. Regardless of when/if there are problems with the shunt, at some point Kingsley will need the tubing changed as his body grows and gets longer.
For now though, going two years without shunt problems is pretty awesome and worthy of recognizing. So, yay for Billy the first! Here's to many more years in harmony. :)
Kingsley is borrowing some more new equipment! Up here in the cold, not-so-snowy north, we skate. By 'we' I mean most of the population except for people like me who just stay inside and make hot chocolate.... up until this year anyway. Jeff has been itching to start the family skating ever since I mentioned that I saw a mini-sledge at our centre in the fall. We all got skates for Christmas and now Jeff is even more excited. So, today, Kingsley and I picked up the sledge.
Sledge hockey (called sled hockey in the USA) is a pretty major sport. It's essentially the same as ice hockey, except it's done on a sled (metal contraptions with blades) instead of skates. The other big differences is that the sticks they use are about 1/3 the size of a regular hockey stick and they have picks on one end. To move, you use the pick end, then flip it over to hit the puck. I actually know very little about the rules of hockey and thus, sledge hockey, nor am I even sure if I'm using the right terminology here!
I have no idea if this is actually a 'cool' sled, it just looked cool when I googled ;)
Kingsley is going to be on the toddler-skate equivalent of a sled - a platform with a seat and two skate blades underneath. We hemmed and hawed about giving him picks, but once I had him strapped in and just showed him the picks, it was pretty clear that he was going to want them. As soon as his belt was done up, he started rocking back and forth as if he was trying to get the sledge to move. I handed him the picks and he immediately held one in each hand and poked the ground for leverage to get moving. He's a natural!
The local sledge hockey league doesn't accept kids until they're 7 years old, so Kingsley has a few years before he's old enough to really get going, if he decides he actually likes it. In the meantime, I'll take lots of pictures... you know, just in case I ever need to do a Paralympic bio talking about how he's been on the ice since the age of two. ;)
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. :)
To recap a bit - in the first few months after Kingsley was born, he was able to bend his legs at the knee, move his ankles slightly and twitch his toes, which meant that for all intents and purposes, he was going to walk. Enter the syrinx, a horrible winter, two huge surgeries, and finally 4 months of recovery, calm and progress. There are currently no plans for Kingsley to walk; he can move his legs from his hip, a bit of hamstring and random twitching down to his toes.
That's where we sit now. After watching him lose functioning twice and not even notice it one of those times, I am hyper aware of every kick and twitch his legs do. Every few weeks, I videotape him moving and watch them all to see if there are any changes. Every other day or so, I will tell him to move his legs and watch to make sure he is still doing it.
Last night, he did not move his legs. At all. I begged, I pleaded, I tickled, I ordered. Nothing. This morning, it was the same. I was absolutely heartbroken. My mind started working overtime, trying to count the weeks and see if we could get surgery over with before my sister and nieces come to visit, wondering if it would the start of a neverending series of cord releases or (no! no! no! no!) another decompression, and whether it would even work.
I made one final attempt to get him to move and...
You hear that desperation followed by the huge relief?
What's that thing over there? Oh, yeah, that's a huge weight that just fell off my shoulders. I can't tell you what a relief it was to see that. I don't know where he was hiding it. Maybe this kid is already in the tempermental toddler phase? Maybe I should be worried about that sporadic showing. Time will tell, but for now, I'm breathing easy. Too bad shunt watch can't be resolved this quickly!
You know, you can go days and weeks and months without giving it a second thought and then you get a day like today and suddenly you begin to appreciate every moment, every hour, every day that this lousy thing leaves you alone.
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.
Three big things. One is that I had an MRI today. I'd never seen MRI images or anything before, so I had no idea what to expect, but once again my amazing OB outdid herself. She brought Jeff and I right down to the Doc that was reviewing the images and had him show us and explain everything they see.
It was actually a little bit breath taking. I got to see all of him inside all of me. So incredibly cool. It was almost like peeling my skin off and having a look inside, only less gory. You can see all of his organs and all of my (squished) organs. The placenta is right there. His feet were practically in my bladder. He is gorgeous.
Anyway, he showed us his spine and you could see plain as day how it was all normal up to a spot. He then did a cross section to show us vertebrae by vertebrae and we saw L3, L4, totally normal. Then L5 had some splitting or something going on, but not fully defect-ed. S1 and S2 clearly have the defect and are open. The sort of cool thing was that in cross-sectioning him, my spine is also in the picture and you can compare the two. Seriously, so cool. The Dr's said they don't think L5 is effected, but they can't be 100% sure until he's born. That would be amazing if it wasn't, so cross you fingers and eyes and anything else that he functions lower.
His ventricles (in his brain) are getting a little bit big (with fluid), but there isn't pressure, so they aren't calling it hydrocephalus. They expect them to get bigger, and anticipate a shunt, but not that there will be any problems with it.
The second big thing is the whole induction/c'section debate. All of the OB's at the hospital that my OB presented my case to agreed that an induction would be fine and cause our son no further complications or damage. So, full steam ahead. Then Jeff veto'ed it and my OB said we both have to agree, so it was back to a c/s. Then Jeff changed his mind and said it was up to me. Then (you may have caught), we discovered that our boy is breech again. Back and forth, back and forth. I'm leaning toward a c/s at this point, even if he flips. It's predictable, for the most part. I can plan for it. Even though it still makes me nauseated to think about.
One last exciting thing was finding out about a new spinal nerve re-routing surgery out of China being trialed in the States right now that our son could potentially get when he's 6yo+ that will allow him to have bowel/bladder control. Hopefully, they'll work it out in the next 6 years and it'll be routine by then.
And that's about it. I'm finding it better if I stop reading stuff about SB now. I know what I know, and I'd prefer that our son just teach me what else I need to know. Reading about other kids just leads me to panic about the 'what if's' and best/worst case scenarios. I'm half eager for him to just get here and get on with it, half prefering to make these last weeks drag on forever and not have to deal with it. I'm not sure I'm ready.
We had a neurosurgeon appointment today. We didn't learn anything new about the baby. She explained shunts and what would happen with the baby after he's born and while he's in the hospital, more information about his surgery and what she would be doing. We discussed the pros/cons of an induction vs a c-section. That's about it.
One thing that they are making very clear, both at LHSC and where I work at HHSC is that there is a very strict H1N1 policy for the duration of flu season, which will most likely still be in effect when we are in the hospital. No children under 16 are allowed to visit. Cordelia and Rachel will have to wait until he's home to meet their little brother and they won't be able to visit me while I'm recovering. There is also a very strict 1 visitor per patient rule. For me, that means people can only see me one at a time, not together or with other people. If someone (for example, Jeff) is with me, anyone else will have to wait in the waiting room for him to leave. Same rules apply to the baby. Unfortunately, parents count as visitors, so there is a chance that no one will see him, depending on how willing I am to be separated from him. Truthfully, I am not predicting that I'll be dragged away from him very often especially once he's allowed to be picked up/nursed.
We asked how strict they are going to be about the rules, especially in antenatal and where he is, since the visitor policy always seemed loose after the girls were born. They were adamant that those were the highest susceptible areas with all the vulnerable newborns and would be inforced the tightest. This flu thing sucks.
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!