Yes, we're STILL waiting....

Sorry, I know some of you have asked, but no, we STILL do not have a surgery date. The two neurosurgeons had their chat about Boeboe, but nothing's decided just yet. The receptionist phoned me to tell me that we can relax, it's not urgent, and that we must go on our holiday in March and then come and see the neurosurgeon afterwards to discuss everything. That would be mid-April. If we wait for that, we'll only have a surgery date in May or June!!! Can you believe that?

I told the receptionist that this may not be a medical emergency, but we're dealing with the issues DAY AFTER DAY. Each and every day. This is urgent, in OUR eyes at least. So she made me an appointment as soon as there was an opening, so we'll be seeing the neurosurgeon 23 February. In the meantime, she made a note for him to phone me, which he'll hopefully do over this weekend or so. Hold thumbs! I'll update as soon as I've heard from him.

In the meantime, I bought the biggest pull-ups available in our country (which is still very small - it's made for children age 2 or 3). Fortunately Boeboe has such a slight build so she kinda still fits in it, though it's stretching quite a bit. Anyway, I bought the pull-ups to take with to school for bad days. But the moment she saw them, she jumped up and down in joy and immediately grabbed one to put on. I was blown away. So later on I went to talk to her, and I asked her why she is so happy about the pull-ups. She told me because she had an accident at school and the classroom was locked (it was breaktime) so she couldn't get her bag (with clean clothes). Since then, she's been wearing the nappies non-stop. :(

I'm a bit confused about it all. On the one hand, I feel so much relief for her sake. It's as if a weight is lifted from her (and my) shoulders. She doesn't have to stress about accidents anymore. And she doesn't have the embarressment of it anymore. But on the other hand, I feel like she's embraced it too well. Too easy. Is this it now? Is this what it has come to, after 4 years, to be put back into nappies again? It saddens me. But it also free's me. Does that makes sense?

Gosh, if I'm so full of conflicting emotions about this, how must my poor little girl feel? Though, to be honest, she still looks and sounds and seems just excited and happy to have a "good" plan. Something that can help her. And even when another boy noticed the nappies at school and called her a baby, it didn't phase her in the least. I tried to give her tools on how to handle the situation the next time it arises. But she just shrugged me off and said "Mommy, I do not mind being called a baby. But next time I'll just tell him about the problem in my back". What a big, wonderful and emotionally strong little girl I have!

Boeboe (around age 8 months)

Philosophizing....

That's the first time I've learned how to spell that word...:)

I've been tobbing over a few issues the past few months. I do not have any answers yet. I don't think any of the issues really has any answers. There's no right or wrong answer. In the end, every family will have to do what they feel comfortable living with.

My first issue is (I'll discuss the others over time, in different posts): When is it acceptable to abort for medical reasons?

Before having children, I never thought about it much. When I was pregnant with the first 2 children, I was led by the gynae through a few tests. I didn't even fully comprehend what they were for. I knew one looked for Down Syndrome, and one for Open-Back (as it is called in my home tongue, in layman's terms). Aka...Spina Bifida. I never made any decisions about what to do if one of them turned out positive. I was mildly worried, like any would-be-mommy, and very relieved and happy when all tests were negative. Thereafter, I never gave it another thought. Why would I?

When I was pregnant for the 3rd time, I didn't even repeat all the tests. Because by this stage, I've realised how very very precious life is. How amazing it is. What a miracle each and every life are. We wanted this tiny life, no matter how perfect, or not so perfect, it turns out to be. Of course, when it turned out to be a beautiful, perfect little boy, we were joyous and humbled.

Ignorance is bliss they say. Yes. I can attest to that. For 5 years, I've ignored the facts. I lived in ignorance. And it was bliss. I didn't want to acknowledge that even after having a perfect little girl that conquered her difficult beginning, there could still, after all, be a problem. A defect. I decided she's just naughty. She's just difficult. It fitted in with her personality, so that must be it. She'll come all right. At some point, she'll just miraculously heal. She'll understand how things work. How to control one's bladder, bowels and feet. Like any normal, perfect little child.

When that didn't happen, after months, years, I realised I cannot ignore it any longer, and I took her to a doctor. That has been 2 years ago. Two long years of figuring out that my perfect little girl, has a defect. Something that has been with her, forever. Since she was just a tiny little speck. Barely bigger than an ant. Even from before any of the tests were done to determine if there were any defects.

So if these tests were able to see that defect, if it was as obvious as Spina Bifida. Would I have aborted? I like to believe No. I like to believe that I would not have been able to. You may say, but she doesn't have an open back. She doesn't have full blown Spina Bifida. Why worry about it? My answer to that... What difference does that make? Full blown Spina Bifida, or another neural tube defect. What difference does it make when the symptoms are so similar?

When a child is diagnosed with Spina Bifida, they determine the level where the back is open. The level where the defect is. That level, in many respects, would be an indication of what difficulties the child may face. Generally, the lower the defect, the less leg involvement. Bowel and bladder is almost always involved. So for a child with a very low lesion, you may find just a few symptoms involving the feet, as well as bladder and bowel.

Just like my daughter.

Should I have aborted my daughter? What if they said she had Spina Bifida, when I was pregnant with her. Should I have aborted then? No. A thousand times no. A million times no.

So if she shouldn't have been aborted, why should other babies with Spina Bifida be aborted? What if their only symptoms ever, would have been similar to my daughter's? Is that worth aborting a baby for? Because they're going to be like my daughter?

My daughter.... vivacious, spirited, beautiful, happy. My laughing little girl. When I think of her, in my mind's eye she's always laughing. Always. Her eyes are shining, her head thrown back, her whole body full of mirth, hair in disarray. This, is my daughter.

What if her defect would've been open, naked, visible to the eyes looking at the sonogram when she was a baby in my tummy. What if she, like so many, many Spina Bifida babies, was aborted? How empty would my life have been, without this beautiful, loving, kind being in my life. How much I would've missed. Her laughter, her warm hugs, her kisses. How sad would it have been, to have missed out on everything me and my daughter has shared together. How very, very sad. How sad for all the mommies who do have abortions. How very sad for what they miss out on.

Yes, they would've missed out on learning about catheters, and urodynamic studies, and physical therapy, and bowel programs and drynites and pull-ups and incontinence pads. They would've missed out on learning about AFO's and braces and other assisted-walking devices. They would not have to learn how to fit a wheelchair in the car. Or how to walk slowly so that your daughter can keep up. Or how your heart contracts when they have an embarrassing accident in public. Or how you want to cry when they fall and hurt themselves. The mommies that abort would never know about the heartache you experience when they wheel your child into surgery. Or when the pain medication hasn't taken effect yet.

They would also not know how strong these children can get. That they can smile at you when the nurse wheel them away from you. That they can lie so still during their umpteenth MRI that the images are near-perfect. That they can cry through-out another painful test they have to go through, just to afterwards smile at the nurse and say thank you, because the nurse have been kind and sympathetic.

Mommies who abort would never know how unique that baby was. How unique and special that smile can be. How precious those giggles and laughter can be. How excruciatingly beautiful the first time can be when they say "mommy". How many lessons such a little thing can teach you, as an adult. Lessons in patience. In love. Lessons in learning with what you can cope with. So much more than you believed of yourself.

Those mommies who abort, would never know how good it can feel to cuddle that tiny body, smell the baby smell on them, and realise that God trusted you, and only you, to take care of this very very special human being.

I feel so sorry for those mommies. So very sad for the lost moments. Moments of pure happiness. Moments of contentedness. Peace.

And then I realise how lucky I am. How grateful I am. That my daughter did not have an open lesion. A defect they could see on the sonar. That hers was hidden. So that I didn't have to make that awful, awful choice. How lucky am I. To be able to hug my child. That I can see her laugh. That I can get to know that beautiful, kind heart of hers. That I get to see her grow up. How lucky am I? :)

And so they grow up...

Monkeyman started school today. It's only playschool, and he'll only be going once a week. But I can't believe my baby is now grown up and a little boy...:( Too soon!!! Much too soon.

He enjoyed it so much. He had so much fun. I stayed the whole time, to familiarise him with the school, teacher, environment, routine, friends, everything, without him feeling I've thrown him to the wolves. Without just leaving him at a strange place, not knowing if mommy is ever coming back, and wondering why she's done this. But, he didn't really need me much, except for one time when he got all shy and scared. It only lasted a couple of minutes and took one hug from mom, to get him off my lap again. I'm sure after a few times of sitting in, I'll be able to leave him for a bit, stretching to longer and longer periods until he will be just fine on his own. He immediately took to his teacher and even spoke to her and hugged her. So much different from Boeboe at the same age, the same school, the same environment.

So my baby is growing up and I'm sad. But I'm also proud, and I think by giving him what he needed the past 3 years (lots of comfort from mommy when confronted with change or something unfamiliar), he was able to build up his confidence. I'm very proud of him, and very happy that I took that approach, and not the one advised by some (getting the child to cut the ties/force him to leave the apron/etc.).

He was deadtired afterwards, poor thing. Slept very well this afternoon, and has been a bit topsy-turvy tonight. Which I realised was because of being tired, shame. I'm grateful that he doesn't have to go everyday, or full day, when only 3 hours of playschool has tired him out this much. So tomorrow...me and him get to sleep late...:) Can't wait!

Waiting...

I hate waiting. I'm not just saying that. I know, most people doesn't like waiting. But I really, really despise it. When I stand in a queue or sit in a doctor's reception room, I start counting, in my head. Count count count....anything to just let the time pass. And to keep my mind busy. I hate it when my mind is blank. I can't do it. I need to keep it busy. This is probably why it's difficult for me to switch off at night, to fall asleep.

Back to waiting. So ja, we're waiting. Waiting on the doctors. And it ain't easy. I phoned both the neurosurgeons' offices. The old neurosurgeon is back from holiday, so we're waiting for the new neurosurgeon to speak to him about Boeboe's case. Then I phoned the new neurosurgeon to get an update. The receptionist told me that the hospital's neurosurgeons all meet on Wednesdays. So only next week Wednesday will they (hopefully!) discuss Boeboe. I just hope that we'll have an answer SOON thereafter. I'd love to get a surgery date then, but that may be asking too much? Thing is, we're planning a road trip middle of March. I can't expect Boeboe to have a back operation end of February and be fine in the car for hours on end a few weeks later! So I really, really want to sort this all out ASAP. You'd probably ask...why not postpone the road trip? Well, that would be a bit problematic to say the least. First, it has to be a school holiday, since Mr N is now grade 4 and writes exams from this year. So, our options for holidays are March, June, September and December. We plan on going to the Southern coast of our country, which has a winter rainfall season. With rain sometimes falling for days and weeks at a time. So going down in June (mid-winter), would be just silly. September holiday is only 10 days long. We need much longer than that (it's about a 4000 kilo round trip we're making). And December is just too expensive for us to go (being mid-summer holiday season). So that only leaves next year March if this year doesn't work out. So, as you can see, we really, really want to go. We've been planning it for 2 years, and would be so sad if we have to postpone with another year, because the doctors couldn't operate in time.

So everything is on hold. I hate not being able to plan. I want to know what happens and when it's going to happen. Now, I can't even plan my boys' birthday parties for February, because we don't know if and when Boeboe will have the surgery. I can't accept other people's invitations, because again, we don't know when Boeboe will be operated. We can't make appointments for Boeboe's care (phsycologist, occupational therapyst, speech therapist, etc.) because of the unknown surgery date. We can't even plan any weekends away.

So in the meantime, we're hanging in limbo. I need to sort things out for Boeboe. She's having accidents at school, and we can't have that happening. The botox hasn't worked it's way out fully yet, so there is still some "coverage" from that. But unfortunately it's not going too well otherwise with her. So what now? What do we do until surgery? Friends have suggested some very nice options/products which I want to consider...but what if surgery will happen in a few weeks time? Is it worth doing anything before that? Spending money on something that may never be needed? If we're lucky, of course. Then surgery will take care of the accidents once and for all. If we're not as lucky, we may need to asap find something to help her cope better at school, until we're sure where we stand after surgery. Then we can get a repeat botox session. I don't see that happening for at least some months after surgery! Since it can take nerves months to regenerate. In fact, they say you could see improvements even up to 18 months or even 2 years after detethering surgery. But I don't think we'd wait that long. After some months, I'd like to repeat the botox, if of course, it is still necessary. I guess we'd need to have a urodynamic study again first, to see what real improvements was brought on by the surgery.

So, now we're hanging in limbo. I can't help Boeboe much at the moment, I can't make appointments, I can't organise our lives. And I totally hate it. I hate not knowing. But what choice do we have? None. So, now we wait...

Not such a good a day

So poor Boeboe is having up and down days. That's pretty normal, I'd guess for anyone with a medical condition. When she has a few good days in a row, I tend to wonder...am I leading my daughter down the wrong path? She's so normal today, everything is well. So the self-doubt sets in.

Then she'll have a bad day, inevitably again, and I'll take a deep breath. We're still on the right path. I'm not putting her through major back surgery for nothing. It will be worth it. It IS necessary.

Today was one of those. A bad day after some good ones. The bladder spasms is causing not only accidents, but temper tantrums. I'm starting to get a pattern here. I'm not excusing her or condoning her behaviour. I still believe that some (if not most) of her tantrums are from a behavioural point of view, and I'm trying my best to watch her closely to see if it's caused by the bladder, or behaviour. Today, the bladder was at fault, most of the times.

We've been discussing bladders on the one forum I chat on, and I thought maybe you would be interested to know as well, what the urologist told us about Boeboe's bladder. It's all very technical, and I'm sure I'm getting some things wrong or mixed up. So please don't take this as medical advice, this is just how I understood the explanations given to me by several of Boeboe's specialists. And, this is unique to Boeboe. Not all tethered cord bladders works the same. It really is a highly individualised thing, it seems.

Boeboe should hold about 250ml volume in her bladder, for her age. Bladders that doesn't hold enough, or too much, is usually severe cause of concern for a urologist. Boeboe had what is called a Urodynamic study, a VCUG, 2 cystoscopies and several sonars, and this is the information given to us about her bladder.

When a baby is born, the nerves in the sacral level of your back (around your buttocks) fire off impulses to the bladder to contract. These contractions expell the urine. A baby does not have control over this process. But around age 1-2, the brain and spinal cord starts to mature and links up. The brain are then learning to control these contractions by inhibiting the nerves from firing off. The brain sends a signal to the nerve to inhibit it. The bladder are then in a state of rest, and it can be filled with urine. Once it reaches a certain point, it lets the brain know (via the nerves and spinal cord) that it's full. The brain then waits until the person is in a comfortable place (preferably the bathroom, of course!). It then stop the inhibiting signal that was continuously send to the sacral nerves. Thus, allowing the nerves to fire off their impulses to the bladder to contract, expelling urine.

Think of a bladder as a small, fluid-filled balloon. When the bladder contracts, it does it gently. Like pushing your fingers into the balloon, about a cm or so. That's all that's needed to expell the urine. Nothing hasty, nothing painful.

Unfortunately not so for my daughter. When her nerves fire off a contraction/spasm, it's done swiftly and harshly. Like pushing the balloon so severely that your fingers nearly touch each other through the balloon. This is painful. And worst is, this does not only happen when she is safe and sound in a bathroom. The signal travelling from the brain down the spinal cord to inhibited the nerves firing off, gets interrupted. So the sacral nerves never gets the message that it must stop firing contractions to the bladder. She has what is called an uninhibited neurogenic bladder.

Thus, her bladder nearly constantly contracts, or spasms. When she had her urodynamic test, she got the first extremely severe spasm when there was only 30ml of fluid in the bladder. She had continuous severe, painful spasms, until at 50ml she couldn't take it any longer. (That's about a fifth of what she should've hold in her bladder.) The spasms measured at about 110cm when it should've been around 20-40 at the very most.  Here's the results of her urodynamic study. It's a bit difficult to make out, but the originals was taken by the neurosurgeon, so I only have a scanned version at the moment to share. 

Another worry with her bladder is that it's thick-walled. A bladder is supposed to be a certain thickness. But it's a muscle. The harder you put it to work, the thicker it gets. So, Boeboe's bladder has these extreme spasms every so often throughout the day, but she tries to keep control (try to keep from having an accident) by closing the sphincter. Now imagine that you're pressing that balloon severely, while holding the opening closed. That's what her bladder does, so it excersize the bladder muscle, resulting in a thick wall. A bad sign. She also has trabeculi (little knobs) all over the bladder on the inside. And diverticuli, which is like little nooks and crannies, another bad sign.

The problem with a bladder that is closed off at the bottom during such severe spasms, is where will the urine go? The only place left when downwards is closed off, is upwards! That means, back into the kidneys. This is a very very bad situation. It's called urinary reflux, and it will, eventually lead to kidney damage. And damaged kidneys stop working. And you all know that even though the human body has 2 of the things, it unfortunately cannot function without at least one working kidney. So better take care of those vital organs while you still can.

Fortunately, all Boeboe's tests showed that she does NOT currently have urinary reflux. Because, she has accidents. She tries to control it, but fortunately fails. Better the urine out (even at uncomfortable times!) than up into the kidneys. The urologist believes that Boeboe has about another 3 years, if we don't get rid of her bladder spasms, until she's going to gain sufficient control to have less accidents. But, this would lead to urinary reflux and kidney damage at the end of the day.

So this is why we're doing what we're doing. Going for the neurosurgery (back operation). We've tried the medication route (meds that supposedly stops the bladder from contracting so severely). Unfortunately, it failed. Accordingly to the urologist, because her spasms are just too severe. :(  We also tried the botox route. We had more success, and maybe with some dossage modifications, we'll have more success. But, this isn't a permanent solution. At best, we're looking at 6-18 months of less spasms. It doesn't fix the cause, or stop progression. I just paralysis the bladder somewhat. We even tried a combination of botox and medication. Alas, the side effects of the medication wasn't worth the tiny, tiny bit of gain we got.

Ultimately, we're doing the back operation to try and keep her symptoms from worsening. Ultimately, to keep her kidneys healthy. I'm hoping that even if the operation may not be a 100% success for her bladder, that it will at least allow SOME signals of the brain to reach the damaged nerves, to inhibit those contractions. To stop firing off the spasms.

And then, maybe, we can really celebrate the good days, and they'll become more regularly, and the bad days will become less and less.

A GOOD day!

Before the holidays, I phoned the urologist's office to tell them of the neurosurgeon's diagnoses (of an occult tethered cord) and to ask if the urologist think we should continue with the botox (scheduled for 22 January) or not. They only came back to me today, to let me know that the urologist says no, we must cancel the botox! (Yay!!!) I'm very relieved! I was dreading the whole botox thing, since it is such a schlep. We have to ask the in-laws over to help (which they are more than willing of course, bless them) so that we can leave 2 sleeping boys in their care while we're off into the city (about 40 minutes drive) at 5:00am. By 7:30 she would've gone to theatre, since it's done under general anaesthesia. Out of sympathy for our fasting daughter, neither me nor her daddy usually eat or drink anything. Which, given my low blood sugar problem, is just terrible for me. I get all confused, jittery, anxious, feeling ill. Put the stress of your daughter under anaesthesia while feeling like that, and it's a bad combination. So, I'm very relieved we're postponing indefinitely! Hoping that we'll never need it again, but knowing that realistically we're just postponing for a few months. I'm very good with rather postponing the inevitable, than facing it head-on.

Second good thing of today...As you can see, I made smileys work in my blog!  :))   =D>

I feel so good about it! It's the one thing of work that I really miss. Feeling that rush of satisfaction when you figured something out while programming. I miss that.

So, I'm really happy today. :)  I know, it seems like something simple. But I've gotten used to having emoticons convey my feelings over, more than words can do. I participate in a parenting forum where I'm a very active member (hmm...something tells me that's THE understatement of the year :p )  In this forum, we regularly use emoticons, so since moving the blog over to blogger, I missed being able to use smiley emoticons in my posts. Well, after a little google, a little programming (REALLY little...hahaha...it only consisted out of copy and paste!) I can now use emoticons in my posts. I'm not sure if this will work on all browsers and platforms though. So let me know if anyone has a problem with them?

To be grateful, or to be angry....

The hardest arithmetic to master is that which enables us to count our blessings.  ~Eric Hoffer, Reflections On The Human Condition


To be grateful for the blessings in life, or to be angry for what has gone wrong...Is that the choice in life? How we look at things? Maybe that's the lesson we need to learn. To change our attitude, to change how we feel towards the things in our life that's not in our control. It's just so difficult, when dealing with everyday life, to be always grateful. But is that really expected of us? Is our God not a God of love, who understand when we get upset? That knows how difficult it is to be always grateful? Grateful that your babygirl only has the very very mildest form of a Neural Tube Defect. Not full-blown Spina Bifida. It could've been so much worse.

So in the same token, I should be grateful that Mr N only has Juvenile Myoclonic Epilepsy, and not one of the progressive types of epilepsy where the seizures attacks all cognitive functions. At least my boy's seizures will only attack his memory or something small like that. Not the areas in the brain which he needs to eat, to dress himself, to count, to even talk.

For that token, I should be grateful that we all have GERD. It could've been cancer. And we should be grateful to only have Gilbert's disease. It could've been a life-threatening liver disease. And I guess I should be very grateful that I have the slow form of Glaucoma. I could've been blind already.

Glaukoma.
Endometriosis.
Gilbert's Disease.
Juvenile Myoclonic Epilepsy.
Occult Tethered Cord Syndrome.
Irritable Bowel Syndrome.
Gastro Esophageal Reflux Disease.

How many more diseases can one family take? My husband told me, after Boeboe's problems started, that we must take our baby son along to be checked out by all the specialists as well. Since our oldest son already has a diagnosis (epilepsy) and our daughter is in the process, why not cut to the chase and find out NOW what will be wrong with him. Hahahaha. It's funny. It's funny with a tear in the eye.

I'm tired. I'm tired of being grateful. Being thankful that Boeboe doesn't have full-blown spina bifida. Tired of being grateful that Mr N's medication is on such a light dosage. Grateful that Monkeyman's bloodtests only showed an iron deficiency. I'm tired of being grateful we ONLY give them each 2 types of medications each day. Grateful they're not dying. Grateful they have all their cognitive functions. Grateful. Thankful.

When do you stop being thankful, and just cry out at the injustice of it all?? When are you allowed to say WHY. WHY do they have ANY of these diseases? WHY my children. Why MY children? What did any of them do, to deserve this? What life will they have, with these diseases? Will it be good? Or will they cry out one day and ask WHY ME?

Sometimes, Boeboe already gets upset. When I tell her to come clean up the mess. Or go take a bath (for the 3rd time in one day). Then she'll moan, stomp her feet and scream "WHY"? Yes, why. Because you were dealt a raw deal, my child. Because life isn't being kind to you at the moment. Because I can't take the smell, the mess, the awfulness of it all, and you need to clean it. Because I don't want to. Yes, most times I have to go and help, because little 7-year old hands are still dumb and uncoordinated and overhasty.

Sometimes, Mr N also gets upset. He doesn't want to drink a little pill EVERY morning, and EVERY evening. Never, ever a break. Never. Even when he was vomiting with the scarlet fever, we had to make him drink it. Because he can't skip a day. When he was smaller, we struggled. He was trying his utmost best not to have to drink the pill. Bargaining with us. We took him to a child psychologist, and she gave us tools. It worked. We had to show him that there's something wrong in all of us. Sometimes, the human body isn't perfect. Daddy wears glasses everyday, because his eyes doesn't work so well. Mr N's little head doesn't work well in one little place inside in his brain, and he needs to drink that little pill. Now, we've told him, that Boeboe's back has a problem and it influences her bladder and bowels. Do not tease her. Do not laugh at her.

So today I'm not feeling thankful and grateful. I'm tired, and I'm angry. And I want to ask WHY us? And I know, you'll excuse me asking that. Because indeed, why US?

But then....why NOT us?

Tethered cord explained...

First I wanna say, do not take this as medical advice. This is just how I understand what my daughter is going through, from my research on the internet.

When a fetus is formed, it goes through some pretty amazing processes. From a few cells that forms into a little body with all the organs intact, limbs attached, a head with a brain inside, and, of course the back with the spinal cord. These processes are very delicate and a certain number of things have to happen in the right order, at precisely the right time. If anything interrupts this process, there could be a defect in the fetus. Sometimes the defect is big enough so that a natural miscarriage follows, and sometimes the defect is so small that not even an MRI can pick it up.

One of the very early processes, is when a certain layer of cells (which later also forms the skin) forms the top part of the spinal cord. This process is called neurulation, and happens on the 18^th to the 22^nd day after conception. Thus, in around week 5 of pregnancy (14 days is added before pregnancy that leads up to conception. So at 6 weeks pregnancy, the fetus is 4 weeks “old”, and at 20 weeks pregnancy, the baby is 18 weeks “old”).

The bottom part of the spinal cord is formed by a process called canalization, starting around the 28^th day. The 2 parts of the spinal cord, formed by neurulation and canalization fuse together, and a 3^rd process starts. Around the 45^th day, the regression process takes place, forming the conus medularis (the pointed tip of the spinal cord) as well as the cauda equinae (the nerves that dangle from the spinal cord tip). The regression process has one last part. The lower end of the spinal cord degenerates, to form a fibrous band that extends from the very tip of the spinal cord down to the sacrum (very low back) where it is attached. This band is called the filum terminale, and the formation of it happens during the 45^th to the 48^th day. The 9^th week of pregnancy.



Source: http://en.wikipedia.org/wiki/Filum_terminale

 Because the degeneration that forms the filum terminale isn't a precise process, defects are more likely to happen than at the other processes. When degeneration fails, the result is a tight filum terminale.

Tight filum terminale means that the spinal cord is tethered to the bottom of the spine more tightly than it should've been. The spinal cord needs to float freely in the spinal canal. When the cord is tightened, it cannot float freely and this influence the nerves sprouting from the bottom of the spinal cord. These nerves serves the bowels, bladder and legs. As the person bends during normal daily activities, the cord is stretched tightly, instead of the filum terminale that should've stretch like an elastic band. When the cord is stretched, nerves are being damaged.

There are different pathologies that can cause a tethered cord. Tight filum is just one such possibility. Others include lipomas (fatty growths), scar tissue, bony spurs, dermal sinus tracts, etc.

Possible symptoms of a tethered cord (caused by a tight filum or other cause):

• Outward markers on the back, like a birthmark, hairy patch, dimples, etc.
• Scoliosis (curvature of the spine)
• Back Pain
• Gait, feet or leg abnormalities
• Drop foot
• Leg length discrepancy
• Feet length discrepancy
• Abnormal gait (stumbling, lifting legs too high, difficulty walking, etc.)
• Leg weakness
• Sensory abnormalities/paresthesia (skin numbness, pins and needles, etc.)
• High foot arches
• Clawed toes
• Toe-in (toes point sharply towards other foot)
• Pain in legs
• Tight or stiff leg muscles
• Muscle atrophy
• Bowel problems
• Loss of bowel control
• Constipation
• Impaction
• Bladder problems
• Incontinence
• Frequent infections
• Urgency
• Frequency
• Renal reflux with possible kidney damage

The list probably isn't complete, as it's such an individual thing. Depending on which nerves, and to what extend, it is being damaged by the traction. I've highlighted Boeboe's symptoms. Some of it is small symptoms, like the toe-in, the leg pain, high foot arches, and leg length discrepancy. It could/would almost be normal, if she didn't have all the other symptoms as well. The biggest problems we have with her, is of course the urinary and bowel problems. Of which she has nearly every possible symptom. That is why we've fought so hard to get her diagnosed.

Once a child is diagnosed with a tethered cord, surgery is nearly always indicated. The main goal is to stop more damage to the nerves by releasing the cord (detethering). Sometimes, the child is fortunate enough to experience improvement in some symptoms. We are hoping that since Boeboe has an occult tethered cord (meaning, it wasn't stretched out so much that the conus was lower than normal), she would be lucky and will have at least some improvement. Usually, pain and bowel symptoms improve after detethering. Unfortunately, urinary symptoms has the least likelihood of improving, though some studies had between 60-80% of people with occult tethered cord that experienced at least some urinary improvement.

Best case scenario would be if Boeboe could end up continent, in both bowel and bladder, and not experience the severe constipation or bladder spasms anymore. As well as an improvement in her dropfoot, so that she doesn't fall so much. I know that this scenario is highly unlikely, but we can hope, can't we?

It's in the feet...or is it?

I took the photo of Boeboe's foot yesterday. I have no idea if that's just how her feet are supposed to look, or if it is influenced by the occult tethered cord. But here goes:

Boeboe's feet a few days after birth. Clearly flat as can be.

Boeboe's feet age 2. Still flat, though there's the beginning of an arch on her left foot.

Boeboe's feet age 4 (with Mr N here). Clearly arched by now. Her left toes are curled, though this isn't a permanent thing. She can curl it easily, but it doesn't stay like that. Oh, and don't ask me, I can't remember why she has the little plaster on her cheek! :-)



Boeboe's feet as it looks today (age 7). The left foot (the one on the right)'s arch is especially pronounced. It's also the left foot that mostly "drop" down, causing her to fall.

So is this normal? /hmm  I don't know. The doctors still seem to think so. To a degree at least. Maybe that's just how it is. It looks just very different from Mr N's feet. His have small arches, compared to hers. His are almost flatfooted.

So long, 2010...

2010. Over and done with. Where did it go?

I'm not particularly sad that this year is over with. But when I see how fast my baby is growing up, then I want to grab hold of time and force it to a standstill. He can't be turning 3 soon, can he?!

New Year also starts the count-down to the new school year. Something I loath, completely. But let's not dwell on that, there is still 11 days of holiday left!

So what did 2010 teach me? That I must, and can be patient with my daughter. With helping her, with her tantrums, with her differences. That was the major lessons I (had to) learn this year. It still doesn't come easy. When you're called for the 3rd time to the bathroom in 1 hour, you feel like screaming and stomping your foot like a 2-year old. Or when you deal with the 4th tantrum of the day, you feel like grabbing hold of the nearest flowerpot and throw it against the proverbial fireplace. Or when she for the umpteenth time forgot what she was busy telling you... you get my drift! So I needed those lessons. I still need them, to cope. Every day.

Though, it's been a marvellous 4 days without bathrooms and accidents and medications and tantrums. I missed my children something terrible. But in a way, it was good for me. I believe it was good for them as well. Mr N just wanted to come home. Part of it was his love for his home, and missing us, but a big part was missing the Wii, the computer and the satellite dish. I think it was good for this technology-crazed boy of mine to be without for a bit.

So what else did I learn in 2010? That I have amazing kids...:-) :-) :-)

No really, I do!

Mr N. Ah, my clever little boy. He really came into his own this year. He got certificates for academic achievement. Not only in the maths we knew he was good in. But also in Afrikaans. Wow wow wow. I was speechless and so very proud. But most of all...I was proud when he was chosen as one of the 4 best readers (out of 29) for his class. My boy...a good reader?! I was in tears! There's no greater gift to a child (okay, maybe some!) than teaching them a love for reading. I love words. I love books. I love stories. I dream stories, I live stories. I would die without words in my life. The worst thing of being diagnosed with glaucoma, is the fear of someday not being able to see words anymore. I can live without seeing a tv. But don't take my written words away from me.

I digress. Mr N...ah yes, he did so well in grade 3. But not only academically. He was on the 1st cricket team for his grade. He absolutely loved and adored the game and gave it his all and everything. He also grew emotionally this year. Getting a maturity and understanding that surprised me. I love getting to see glimpses of the man he is going to be one day. And it makes me proud! Also...health wise. He did so well. Nearly never had any illnesses, apart from gastro once and scarlet fever. And his clean EEG (no seizures)!!! That was THE best news. If it's clean again in 9 months time, he may be taken off the lamitrigine (epilepsy medication). That would be a wonderful, wonderful day. It's been 4 years now. Of giving him the meds every day. There were difficult times, where he refused to drink it. Or spit it out. Or hide it in his cereal or the dustbin! And the side effects. Horrible, horrible side effects. It would be just lovely, to throw it away and have our son back...unmedicated!

Next is our daughter. I can just say wow. How, after everything I've done to her, did she turn out to be this kind, loving, sweet, happy and amazingly strong little girl? What did I do, to deserve this beautiful little girl? She had to cope with more this year, than some adults even have to cope. And she did it beautifully. She went for test after test, doctor's visit after doctor's visit, without complaining. Without digging her heels in (accept on the rare occasion, LOL). She tried drug after drug, experimental treatments and never lost hope that THIS time it'll work. Her faith was and is still unwavering. That God WILL help her. At some point, when we've prayed long and hard enough, He WILL listen and answer all her prayers. She prayed for a rainbow around May this year. Just after the rainy season in this part of the country. So no luck. For months and months she prayed to see a rainbow. She never stopped praying. Never forgot about it. And then, one day around October. It rained and there was a beautiful, big rainbow. She was ecstatic. I, was humbled. She has given me hope this year. Hope for her. Hope for us. Hope for the future. Because she believes. And that's all you need.

She also worked hard at school. Between everything, she still had to learn how to read, how to spell, how to write, how to count, how to subtract, how to multiply and how to divide. And she did it. She did it all. I'm so very proud of her. For sticking to everything and taking it on, and overcoming the obstacles in her path. And to stay happy and kind and thoughtful and loving and a little chatterbox through it all. Never letting depression or heartache or sadness overwhelm her. Never letting life get her down. What an amazing little girl I've got!

And lastly (but definitely never the least!), our little Monkeyman. What a great little personality this guy has. He's kind and cute and loyal. He's also extremely perceptive, sure of himself and trustworthy. He's contend and peaceful and calm. And he is such a little boy. So typical boy. Give him a car in the one hand and a ball in the other, and he's happy as can be for hours on end. He entertains himself. He rears himself! He is so easy-going. Such a splendid little boy to have. He gives us so much pleasure, so much joy. We can't imagine life without him. It would've been seriously lacking! We can just stand in awe of how fast he is developing. He can do things at age 2 that I never thought is even possible (for a kid of mine that is!). And he do it all by himself. I do not sit and teach him anything. He just picks it up. From tv, from conversations around him, from following his siblings, from copying his dad. It's just absolutely astounding. The way he plays kinect. And Wii. Especially Wii. I don't even need to put the game on for him anymore. He does it all by himself. From step A until step Z. He just knows what to do. What buttons to press, what options to choose, what answers to give. Amazing.

So beside learning that I have awesome kids, what else did I learn in 2010? Hmmm...that SA should be given the chance to host the Olympics! And that if they don't do something SERIOUSLY soon about the farm-murders, food prices are going to skyrocket as we're loosing our farmers to other countries, resulting in us having to import more food. But that's a whole different conversation for another day. Not at midnight!

So I hope that everyone will have a great 2011. That it will be kind to us all. That everyone's prayers will be answered. And that we'll all stay healthy. And that Boeboe's surgery will be one big roaring success. Happy New Year!!!