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Today, as I was getting Lizzie dressed, I was running a commentary as always.  I was telling her what a wonderful day it was and that since the weather was so nice that I definitely had to get her outside.  Yesterday we used her gait trainer outside, and she did really well with it.  Today I wasn’t sure if we should use her gait trainer again, or use her bicycle.  I mentioned this to her and I was finishing getting her dressed, and she started making “Buh,” sounds.  Now since the beginning of time for us, we’ve called Lizzie’s feedings babas since they started as bottles.  I looked at her smiling and saying “Buh, buh,” and I thought that’s what she was doing.  Never mind that b’s aren’t a common sound for her. I asked her, “Are you hungry, do you want a baba?” and then I saw her smile fall and I said, “That doesn’t make sense, you just had one.”  Then something occurred to me and I asked, “Bike?” she looked at me, and I asked, “You want to go on your bike?”  Her face lit up.  Now I don’t know if this was a fluke, or she was trying to say bike.  I’m praying it’s the latter. Image

So, a little while later after I pulled out the bike and cleaned the dust off of it from storage, I put her on it, and we did our usual walk around the circular street where we live.  Miss Lizzie was really pulling on the handlebars today, which was causing me to have to hold tight to the push handle and battle to keep her from veering too far.  I had just decided to walk around the front to flip the handles out of her range of grasp, when she started moving!  Lizzie was pedaling all by herself!  With the exception of a couple of times that I had to give her a nudge to restart her, she must have gone about 30 to 40 feet on her own.  I am completely amazed.  My eyes were tearing up.  I was so proud of her.  This is the first time she has ever done it alone.

I did not realize how long it’s been since I’ve done an update on Miss Lizzie.  Lizzie is growing and improving every day.  Lately she has learned how to “give five”.  She will put her hand up to receive five if you say, “Give me five.”  She also is standing more and trying to walk along her bed railing.  Recently she gave me a little scare when she lost 5 pounds.  That’s right, 5 whole pounds!  Then I went to her parent teacher conferences and it shed a little light on why.  It turns out my girl is walking up a storm at school.  They informed me that she walks from her classroom at one end of the building to the gym at the other end of the building where she participates in a half hour of gym and then walks back again, in her gait trainer the entire time.  It’s no wonder she lost weight, she’s burning a lot more calories than she was, and we hadn’t added any calories to her diet.  So, after some adjustment to her feedings I am happy to say that she has started gaining some weight again.

Lizzie has followed up with some of her doctor’s this week.  Her neurosurgeon said that she looked good and that he doesn’t need to see her again until next year.  That was a pretty standard visit for us.  She gave him a high five, with which I think he was properly impressed.  She wouldn’t give the nurse five though, because by then she was too happy pulling the paper off of the table.

Her weight check appointment went off without a hitch.  It actually was so short that it made our venturing out in what was blackened skies, torrential downpour, and a vicious electrical storm almost ridiculous.  But it’s done, and now we know that Lizzie is gaining back the weight she lost.  She is back on the right track.

Today we got some interesting and awesome news.  Lizzie went for a cardiology follow up.  Lizzie has always had to follow up yearly with a cardiologist because of something called a bicuspid aortic valve.  The aortic valve is a valve on the heart that is supposed to have three parts, but a bicuspid aortic valve only has two.  This is a cause of some concern because the parts of the valve are working harder than normal to compensate for the difference, and because of this it is something that needs to be watched closely.  Because we only go once a year, we seem to have some bad luck with keeping our cardiologists.  Every year we call and have to see a different doctor.  Today we saw another doctor for the first time.  He came in and was so good with Lizzie.  He talked to her.  He let her play with the curly cord on the blood pressure cuff while he manually took her BP after the automatic machine wouldn’t take her reading because apparently it doesn’t like wiggly kids who won’t hold still and try to lick and bite its cuff.  He told her she could play with that cord.  I thought, “Great. Here’s something that might actually keep her occupied during the echo.”  The echo tech came in and immediately took it away.  Lizzie wasn’t amused.  She also tried to pocket Lizzie’s measuring tape, something that I’ve found to be a good distraction for her, because “I’m sorry but it looks like one of ours.”  Probably because I got it at the same hospital as you, Lady.  At this point neither Lizzie nor I were amused.  I had to wrestle Lizzie the entire test because the lady was not allowing her to have the things that worked as distractions for her.  Needless to say I’m exhausted.  ANYWAY.  When the doctor came in after the test, he told me that they actually were able to see the third branch of Lizzie’s aortic valve.  In other words, it’s been there the whole time.  They just had never been able to get a good enough picture to see it.  She doesn’t have that worrisome defect after all!  How awesome is that?  She does have a more common, less worrisome issue with her mitral valve, but that is something that according to the doctor rarely develops into a bigger issue.  Because of Lizzie’s other health issues the doctor still wants to see Lizzie yearly, but the heart issue itself is so much less serious.  So all in all it’s good news.  I am so happy.

My daughter never ceases to amaze me.  There are times that this girl really blows my mind.  This morning was one of those times.  I have never gone with the assumption some people make, that Lizzie doesn’t understand things.  If there is something I think she needs to know, I will explain it to her.  This morning, for example, I woke up with a very unusual flare up of my rheumatoid arthritis in my left wrist.  I usually don’t have hand or wrist issues, and today it was pretty severe.  There have been days, where Lizzie has grabbed my arm hard and unintentionally injured an already sore hand, so I decided to let her know what was going on.  When I got up, I had put a muscle rub on my wrist and put the neck of  a black sock over it, so that it won’t get on anything and to keep the heat in.  I explained to Lizzie that the wrist with the sock was very sore today, and if she could avoid grabbing it that Mommy would appreciate it.  While I was getting her dressed, Lizzie grabbed that socked hand above the sock, and pulled on it.  I gently removed her hand and said, “No.”  Lizzie looked at me, tilted her head back and stuck out her tongue in her kissing position.  I was the one who was confused in the moment.  I looked at her, and held out my wrist.  My daughter leaned in gently and kissed my wrist!  She was kissing my boo boo.  I wanted to cry.  I asked her if she was kissing my boo boo, and she smiled at me.  Don’t tell me my girl doesn’t understand.  Then when I gave her my hands to pull herself to sit, she pulled only on my good arm, and barely held on to the bad one which is the only side she usually uses.  I was floored.  My girl is awesome!

For the past few months we have been weaning Miss Lizzie’s phenobarbital.  For those who might not know, phenobarbital is a drug that controls seizures.  Lizzie has been on phenobarbital since her first seizures in the hospital when she was almost 4 months old.  She is now eight and a half and this wean has been a long time coming.  We have tried many times over the years, always with the same result. Every time we weaned more than two or three milliliters she would have seizure activity and we’d have to go back up on her dosage.  Most people would think that this would be a reason to stay on it, but for Miss Lizzie we had even bigger reasons to want her off of it.  You see, every time we would wean Miss Lizzie’s phenobarbital, we would see amazing things.  At the age of almost 3 years old, Lizzie sat up unassisted for the first time.  Before that, we had wondered if this would ever happen or if it was even a possibility we should hope for.  The fact that this magnificent milestone immediately followed a reduction in this medication did not escape us.  After that we noticed that Lizzie would gain skills every time we reduced this medication.  It was easy to see that it was holding her back developmentally.  The problem was we had to find something else that would control her seizures if we were going to get her off the phenobarbital entirely.  We started with Trileptal.  It helped for a while, and we reduced Lizzie from 22 milliliters to 15 milliliters over a couple of years.  However, we then went through a year of more frequent, less severe seizures that no one could find the cause of.  So we added Keppra.  Then it was finally determined that a side-effect of the Trileptal, low blood sodium levels, might be the culprit in Lizzie’s new seizure behavior, so it had to go.  And it went.  Rather rapidly actually, because with that medicine Lizzie did not have the effect of more seizures with reduction, but rather less.  Next we added Topamax.  This has been our wonder combo, Topamax and Keppra.  Lizzie has now been almost a year with no seizure activity!  Don’t worry, I’m finding something to knock on as I type.  Which brings me to the reason for this post:  I am both thrilled and terrified to say, that tonight is the first night I am not giving Lizzie phenobarbital at all, in over 8 years!  I am thrilled because I can not wait for the possibilities that this may open up for her.  I am terrified because like that ratty old security blanket some kids have, we have never been without it since her epilepsy began.  I honestly think this is the best thing for Miss Lizzie.  Hopefully, this is the end of our phenobarbital journey.  Please keep Miss Lizzie in your thoughts and prayers as we complete this process.

Today we saw our orthopedic surgeon for a follow up.  This is the gentleman who handles Lizzie’s AFO’s (ankle foot orthoses), her TLSO (thoracolumbosacral orthosis or back brace), and her follow-ups to her hip surgery which he performed a couple years ago.

The news today was all good.  He told us that he was impressed with Lizzie’s hips.  A couple of years ago, he performed a surgery called an epiphyseal arrest on Lizzie’s hips.  Lizzie has a hip condition called hip dysplasia which means that her hips were in danger of dislocating, the main reason in Lizzie’s case being her low muscle tone.  Her muscles just were not strong enough to keep those hips in the sockets as they were growing.  Our doctor had told us that if they got worse we would need to do a major surgery which involved cutting and shaping the bones and would be followed with six weeks in a spica cast.  This is a cast that goes pretty much from arm pits to knees and holds the legs in an outward position.  I couldn’t imagine how hard that would be for Lizzie to go through or how hard for me to move all that extra weight every time she needed to transfer from bed to chair and back.  But our wonderful doctor had another thought.  There was another surgery possibility.  It wasn’t usually used for Lizzie’s condition, but he thought in her case it might work, and help us avoid the more invasive procedure. He could try it on Lizzie, and thought that it was another way to go.  Essentially he put screws into the heads of her leg bones on the hip end causing the bone to stop growing, and preventing the ball of the hip bone to grow out of its socket.  The way it was explained to me is as the pelvis continues to grow, the socket would recover the hip bone.  So far so good.  He told us today that her left hip is great, and her right is better since he saw her last.  He did say that down the road he may need to do another procedure to put screws into the other part of the bone to prevent that from continuing to grow, but not yet.  We will explore that down the road.  Her hips are doing well for the time being though, and it looks like no major hip surgery will be in our near future.  Woohoo!

I also asked him about Lizzie’s AFO’s.  I don’t think in all the time we’ve had them if I’ve ever asked if they were a permanent thing.  He told us that if Lizzie continues to weight bear on her legs and build up her muscles, and she is not turning her feet out when standing on them, that there is a chance that down the road she may outgrow the need for them. More good news!

Finally, he checked her back.  I was happy to hear him describe to his residents a slight scoliosis.  It’s not perfect, but it’s slight.  We’ll take it.  As long as it doesn’t progress or become aggressive, we should be okay with just her back brace for now.

So for now we are holding tight.  And we do more stander and gait trainer at home to build up those leg muscles.  For now we wait.  We got new orders for her AFO’s and TLSO to be made in a couple months because she is outgrowing hers.  We see him again in June.  All in all, a good day.

Tonight as I was getting ready to do Lizzie’s last feeding, I went into her room and found her standing in her bed. She was in the back corner, and as I walked into the room, she looked at me and smiled. I told her she was my pretty girl and she giggled. Then the most amazing thing happened. By now I was standing at her front rail, and she was still in the back corner. Holding on to the side, Lizzie coasted her railing to get to me. Now I have seen her go a step or two to reach a little further for a toy or so, but never like this. She was determined. It took about four or five steps and required her changing direction from side rail to front. And when she got there, she gave me a big hug, kiss and smile. I am so stinking proud of that kid. 

She also seemed to laugh at a joke I made today. That may just have been coincidence, but it is what it is.  She was being changed and put
her head in her back brace which was laying next to her. I quoted a line from the Princess and the Frog about having your head stuck inside a tuba, and Lizzie bust out giggling. Too cute. If it was a coincidence it was damned good timing. 🙂

In the last year, Miss Lizzie has learned how to give kisses.  If you are close enough, and she’s in a good mood, she will tilt her head back, smile, and extend her tongue.  She will then lick your cheek or mouth.  Usually these have happened in her wheelchair when I am leaning across her to hook up or unhook her feeding or if you ask her for one.  This is Lizzie’s kiss.  Or “smooches” as we call them.

Within the last 2 weeks, Miss Lizzie has decided that she wants to give me a bedtime kiss.  After her bedtime routine, if her railing is down, she sits up to give me a kiss.  If her railing is up, she will pull up to stand, just to give a kiss.  I love this new showing of affection.  She is so cute.  I always tell her that she gives the best smooches, and I will take all that she will give.  No wonder she’s my favorite girl in the universe.

For a couple weeks they have been talking about moving Lizzie up one classroom at school.  Apparently there was an influx in the number of students in Lizzie’s class and since Lizzie was on the cusp of moving up anyway they decided it was time.  When they told me, I went through some mixed emotions.  I was excited as most parents are at graduation times, but I was also afraid.  I was questioning if she was ready for this move.  I have been getting many updates about the progress, and they even tried her in the new classroom this week and she did well.  She is also with a teacher that she’s had in the past for summer school, so I wasn’t concerned about the teacher.  But we’ve had Miss Laura for the last few years.  Her classroom and parties and activities are comfortable and feel like home.  So while I look forward to the opportunity for Lizzie to grow, I worry about changes.  What else will change with a new classroom?

Then I got an e-mail from her physical therapist today.  We are in the process of trying to get Lizzie a new Rifton chair for home, since she has outgrown her old one.  She told me when I follow-up about the chair to contact a different therapist, because as of next week she will no longer be working with Lizzie.  I realized that Lizzie’s new placement must be happening next week.  But it wasn’t until she got home and I opened her backpack that it really felt definite.  This is what I found:

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These are from Lizzie’s classroom.  There are laminated photos from her activity board, the birthday cake with her birth date on it, and the school bus with her bus number on it.  I got teary-eyed as I pulled them out.  I guess it’s official.  Miss Lizzie’s moving up.

Today is the beginning of hydrocephalus awareness month.  Hydrocephalus is a condition that is defined by cerebrospinal fluid (CSF) building up in the ventricles of the brain.  Hydrocephalus can be caused by an over production of CSF or by an inability of CSF to drain due to blockage or inadequate drainage of the fluid from the brain.  In either circumstance, CSF building up can cause pressure on the brain that can cause seizures, headaches, vision issues, balance issues, or damage to the brain itself.

There are two main treatments for hydrocephalus.  A medically implanted shunt that takes the fluid from the brain to either the chest cavity or the abdominal cavity, where the fluid is then reabsorbed.  Or an edoscopic third ventriculostomy (ETV) which is basically drilling a bigger hole where one is too small for the fluid to pass, allowing it to pass along its regular cycle.  Neither of these is perfect.  Both can and do sometimes fail.  And neither is a cure.  Many people with hydrocephalus end up with multiple brain surgeries in their lifetimes.  And even when it is under control, hydrocephalus is still a life threatening condition.

We need to raise awareness.  We need better solutions, better treatments, and a cure for this condition.