Back to ... Normal?

Yesterday, the Medical Supply company came and took out the bed we had put in the Dining Room for Ciera. It was a rental, and we hadn't been using it for some time anyway.

After everyone went to bed, I was putting the table back together, and it was strange. I would have thought it should be a good time, getting our dining room back. But somehow, it was a very melancholy feeling.

I'm not sure why. I think it's because it is supposed to be a sign that things are getting back to normal, but are they? Yes, Ciera is sleeping back in her old bed. But she's not "back", and who knows if she ever will be?

She's doing great, for someone who had a stroke and then brain surgery. She's happy, eating like a horse, and always wanting to move around and play. But the left side weakness hasn't really improved in a while. She can use her left hand, but often forgets about it and we have to remind her to (or make her) use it. When she's not using it, the tightening up of her muscles is causing her arm to curl up, looking kind of like a dog's front leg when he is sitting up and begging.

Her left leg is ok, but definitely weaker than her right. She can use her gait trainer, which is awesome, but her left foot always drags when she is being picked up or moved around, and when she climbs up on something she sometimes can't get that left leg up without help.

I knew this could happen, and in fact was likely. It's really only been about 3 months or so, and we have been told it could take 6 months to a year to get everything back (if ever). I just can't help wishing things were different. I have long been used to her "normal" due to the Zellweger Syndrome, but this is different, and will take it's own getting used to.

Just put one foot in front of the Other.....

So we just got back from Boston, and are getting ready for the weekend. We were at Boston Children's hospital for some tests related to Ciera's Moyamoya disease, and it went well.

We went down Tuesday for some pre-op work, paperwork, etc. Wednesday was the actual angiogram (sp?). That's a test were they go in with a catheter camera through an artery in her hip, all the way up to the neck and head. They shoot some dye in her blood vessels, take some pictures, and get out. The procedure only takes about 45 minutes, but there is prep and finishing, and then she had to lay flat for 4 hours so that she didn't open the area where the cath went in.

Like I said, the procedure went great, but man, I don't know how anyone can live/work in Boston! I guess we're just small town folk :-) It took us 2 hours to get from the hospital to our hotel - about 16 miles away - the first night (only 30 minutes to get back in at 5 the next morning). We ended up staying a 2nd night, because the Doctor thought a 4 hour drive in a car seat might not be the best choice for Ciera. The 2nd night, though, we were able to get a room at the hotel right next door to the hotel. Nice hotel, plenty to eat within a very quick walk, and relatively cheap for inside the city- I think we got a good rate because we were having stuff done at the hospital, and they have special rates for that.

It was also nice to see my brother Ray and Sister-in-Law Ruth. Kind of them to brave traffic to come to us, because we were burrowed in and not going anywhere!

The results of the test confirmed what we already kind of knew, that her case has progressed to the later, more serious stages of the disease. The Doctor told us that without the surgery, she would have about a 70-90% chance of more, possibly fatal strokes within the next few years. With the surgery, that drops to less than 5%. There are risks, of course, of the surgery causing a stroke or worse, but the percentages are very low, so we have opted to go ahead with the surgery.

Sometime in October or November we will have to go back for about a week straight for surgery and recovery. It's actually much better than I had pictured, so that is kind of good.

SKIP TO THE END IF YOU'RE SQUEAMISH! Basically, they will have to cut about a 1 inch hole in each side of her skull, and run the big blood vessels from outside of her skull into the hole and back out, leaving much of it under the skull when it is replaced. The brain will make new blood vessels from these "transplanted" ones, allowing more blood supply to the brain and reducing the pressure on the moyamoya vessels. It will be an all day affair, taking about 8 hours for the surgery

Here is a picture showing a person with moyamoya on the left, and a normal person on the right. Notice how many of the large blood vessels are missing. What we have seen of Ciera's scan look more advanced than the picture on the left (I will post some pictures when I can)

This picture shows the progression of moyamoya. Figure A is a "normal" scan, B shows narrowing just beginning. C shows significant new "moyemoya" vessels - you can see in the center how is looks a bit like a puff of smoke, which is where the name comes from. Figure D is the closest to Ciera (she is probably somewhere close to D, but a little like C as well). It shows the advanced stages where the new vessels have started to breakdown.

Not trying to freak anyone out here, but I hope it gives you and idea about what we're talking about. I may have posted this one before, but here is a description:

CLICK HERE FOR DESCRIPTION

There's a link on the left hand side of this page to a video of the actual surgery that will be performed. The Doctor talking in the video, Ed Smith, is the same Doctor who will operate on CC

CLICK ON THIS PAGE THEN FIND VIDEO LINK

Could be worse, I guess

It's been almost 3 weeks since Ciera had her stroke, and we are just beginning to settle in for the "medium" haul. We have a bed set up in our dining room, so we can be near Ciera while we are cooking or in the kitchen, plus she's near me while I sleep on the sofa at night. We have a temporary wheelchair that tips back, and hopefully will be getting a better, more permanent one shortly. We have modified her car seat to sit back at an incline as well. (Luckily for us, several months ago we had gotten a special needs car seat from one of our GFPD families, who unfortunately no longer needed it. But it already had the extensive head and trunk support that - at the time - wasn't as much of a necessity)

She's actually doing pretty good, all things considered. She can sit up almost fully, but we need a bit of a recline because she still can't really hold her head up. She is moving more and more, but her left is still very weak, especially her arm. It's like her brain lost the connection to whatever she used to do to move her arm. She can sometimes, when she's very motivated, but that's rare. She also still gets tired very easily, taking a nap around noon time every day. She has to work really hard to do 1/10th of the movement she used to do.

We've been working on a plan with school, and thankfully they've been very helpful so far. We are going to have her Intervener come to the house some next week, and we will also bring CC into school for small stretches and see how it goes. If she does well, and builds up more stamina than she has right now, we'll try to get her back in school as much as we can soon. Her school therapists will also be coming to the house on occasion. Again, I guess we are lucky that we previously had all of these services, so that they are in place already at school.

Both Jenn and I have started back at work. It's hard to sit at work and concentrate sometimes, because all I want to do is be at home with my family. And it's been tough on Bailey, too. She's having to deal with all of this stress about her sister, losing even more of the time she had with Mom and Dad, and starting a new school year at a new school.

It's amazing how different our lives are then they were a month ago. I used to be jealous of other families who had "normal" kids, and who didn't have to worry about having a kid with a disease like Zellweger's. Now, I am also jealous of ourselves from a month ago. I would love to have Ciera back to where she was, signing and talking, crawling everywhere and doing amazing things with her wheelchair and gait trainer. I miss that life, and we just don't know if she will ever get back to that. We'll keep working towards it, of course, but it is a far from certain outcome. If I had a stroke and got back 80% of my skills, I could probably walk and stuff. It took everything she had to use her wheelchair and gait trainer, and I am just afraid 80% - or even 90% - won't be enough.

And then there is still the black storm on the horizon - what to try to do to help the moyamoya disease. There's not a lot to go on, when she is the first person to have both of these conditions that any of the Doctors - including the Zellweger specialists and the moyamoya specialists - have ever heard of. Medical treatment of moyamoya just doesn't have a great success rate, and some of the treatments actually go against things we are doing because of the ZS. Surgical treatment is the best option by far, but we need to find out if she would be a good candidate, all things considered. There's a LOT to consider, even if we find a Doctor willing to do the moyamoya surgery.

And yet, as the title of the post says, it could be worse. Since she had her stroke, 2 of the children from our GFPD group have passed away. What we are going through right now would be a blessing for them. It's a very strange feeling to be so numb and upset, and relieved at the same time.

So if you know what the image below is, you'll know how we are taking things right now :-)

You've got to be f'in kidding me

A number of years ago, I wrote a book detailing the choices my wife and I made to use Donor Insemination to have a child, through her birth, and finding out she had a terminal metabolic disorder called Zellweger Syndrome.

At the time of that book, Ciera was 5 years old, an achievement we never thought would happen. As she closes in on 10, I was trying to start writing a follow-up. I had gotten as far as this sentence in the forward: Happily for us, Ciera is now approaching 10 years old! “Though she still has daily struggles to deal with, she has been fairly stable and continues to grow, learn, and experience life.”.

Now, as I sit in a hospital room with Ciera, hoping to get home tomorrow, I can’t believe how much has changed from a couple months earlier when I wrote that. The new sentence should be “Though we have gone through so much, and thought we had become accustomed to our life, sometimes life just stands up and kicks you in the balls with steel-toed shoes.”

For those who don't know, Ciera recently had an aneurysm burst which caused a stroke. It turns out that besides the Zellweger Spectrum PBD she has been dealing with her entire life, she also has something called "moyamoya" disease http://www.childrenshospital.org/conditions-and-treatments/conditions/moyamoya-disease

About 10 days ago, we were enjoying a nice Sunday morning. Ciera was crawling around, and I noticed she was having trouble, not able to support herself on her right side. She fell a couple of times, and looked really tired crawling up on to her mattress on her room. When she tried to crawl off, she fell on her left side, pretty hard. I picked her up, and she was acting as though she had a seizure. She was really tired, and not holding her head up well.

We were keeping an eye on her, and she started to feel better. After I while, I started to put on her DMO - a special "west suit" that was designed to help her stability. I had it on backwards, so took it off and gave her to Papi (her Grandfather) while I turned it around and got it ready. Ciera started acting all sleepy and limp again, then, and we decided to take her to the E.R.

Damn good thing we did, because what we found out was that she had an aneurysm, and was bleeding in her brain. Thankfully, the Neurosurgeon was able to go in and stop the bleeding. She spent about 5 days in the PICU, and has been in the Children's Hospital here since then. But what we found out floored us just as much as when we were told about her Zellweger diagnosis. They told us she had MoyaMoya - the main carotid arteries and blood vessels in her brain were malformed and shutting down, and to compensate, her brain had developed all of these tiny blood vessels to do the work. The problem is that these tiny ones are not strong enough to do the job reliably, and things like this stroke will continue to happen.

She is doing ok now, but today was a bit of a downer after the last two days of amazing progress. She was tired pretty much all day, and had a small fever during the evening. We even had another CT scan done, but luckily there was no new bleeding. These up and down days are just to be expected when recovering some something like a stroke, from what we understand.

So right now I am sitting here, having no idea what the next several months will hold. We have to figure out if she will be a candidate for corrective surgery, but she is the truly unique position of having these two conditions - possibly the only one in the world. Doctors from Vermont, Boston, Salt Lake, and even London are looking at her records and getting together to try a figure out the best course of action.

Thankfully we have a wonderful group of family and friends who have been really helpful - taking care of Bailey, cleaning our house, getting ready for Ciera's new equipment, and just helping us emotionally and helping keep an eye on Ciera so we can breathe for a moment. But still, it's going to be a while before we know what the NEW, new "normal" is going to be.