Brown Paper Packages Tied Up With String...

Yes, in this picture, those are convenient cat-sized holes in the blinds. Loki doesn't like anything getting in the way of his view, so when we moved into this house he politely waited until we went out of town for a while and then fixed the problem in every room of our house, probably using his teeth. I look forward to replacing all of the blinds and possibly also substantial portions of our beige carpet (little tip for you: when you have a child with severe reflux, never feed them dark chocolate Pediasure unless you really didn't like your carpet anyway) whenever we move.

Connor's cardiology appointment went very well. Dr. P took a good look at him, did an echo and an ekg, and then declared Connor's blueness to be probably related to a combination of super, super fair skin tone and mild temperature regulatory issues that his increased activity have aggravated-- nothing to worry about, thank goodness! He said Connor's heart function looks fantastic. He also cleared us for g-tube surgery, so we're good to go. We've scheduled an appointment with the general surgeon in March, and during that appointment we should be getting Connor's surgery date at long last.

Now that one issue is resolved, we have another one to worry about. Doesn't it always seem to happen that way? For about the past week, Connor has been pulling his right hearing aid out. He won't leave it in for more than five minutes at a time. He's usually really, really good about keeping his hearing aids in, and he's leaving the left one alone-- it's just the right one he's pulling out every time. I asked him today if he could hear out of it, and he signed "No. No hear." Now I've checked the hearing aid out, and it's working the way it should be, so I'm wondering if maybe he's had some more hearing loss on that side. To check that, we'll need for him to have yet another sedated ABR. Joy of joys. We're waiting on a call back from his audiologist to see what she wants to do.

That's his better ear too, dang it.

We did have one more big event today. We received a package in the mail, addressed to Connor. I opened it up and took out Connor's long-awaited communication aids and switches! We've been waiting for these things for over a year, people. Bad insurance juju caused some major delays, but finally they are here, and we can't wait to try them out!

Connor is now in possession of two LITTLEmack communicators, one Vertical Wobble Switch, and one Powerlink 3 Control Unit. The LITTLEmack communicators are basically a button attached to a tape recorder. When you hit the button, the recorded message plays. We can use them to help Connor make choices; instead of having him eye-point, we can record two messages and he can push the button for the one he wants. They aren't anything hugely complicated-- nothing like the Vantage Plus the very talented Schuyler is using over at Rob's blog (you should all read his book by the way, as it's fantastic) but they are a start.

The wobble switch helps Connor do activities he wouldn't otherwise be able to do. It's kind of like a giant "on" button shaped like a joy-stick. We can plug it into a bunch of his toys that we've converted to be switch-activated, and he can turn those toys on and off by swiping his hand across his switch. It helps him be more independent as well; he can turn his nightlights off and on himself, as an example.

The Powerlink 3 is kind of like a giant switch board. It can change the function of Connor's switch. With the Powerlink, we can make his switch turn an electronic device on for a certain amount of time instead of just having it be a "one swipe on, one swipe off" set-up. We can also plug things into it that aren't switch adapted, so we don't have to convert them. This is so I can put my son to work. With the Powerlink, he can turn the mixer on for me while I'm cooking, turn on the vacuum to scare the cats away from my potted plants-- the possibilities are endless. I'm sure we'll use it for things other than my personal convenience, too.

We took a class a while back and have converted a ridiculous number of toys for Connor to use with his switches. We really, really like our soldering gun and will jump on any excuse to use it. Our current project is to convert a Powerwheel Jeep so that Connor can drive it with a switch. This has been our current project for the past six months or so, because we only work on it about 15 minutes a week, and in the meantime it has taken up permanent residence in the middle of my office floor in about 800 pieces. I'm sure we'll finish it about the time he outgrows it and can't use it any more. Oh well.


~Jess

We Got The Call!!!

I heard a terrific banging noise coming from the direction of my entryway today, and discovered Loki engaged in an epic battle. He was attempting to break his enemies "neck" by picking it up in his teeth and slamming it into the wall repeatedly. Then he'd throw it on the floor, bite it in the "neck," and kick it with his hind legs to "disembowel" it. See how diligently he defends me from his fierce and vicious foe?

This is why I will not be buying shoes from Neiman Marcus any time soon.

Crazy cat.

In other news, we heard back from Connor's cardiologist today! He was very courteous, listened carefully to all of Connor's symptoms, and said that because Connor wasn't experiencing any change in "state of mind" during the episodes-- no confusion, loss of consciousness, etc. that they probably weren't something we needed to worry about, but that he wanted us to come into the office tomorrow to get him checked out, and he wrote us in. He also gave us a way to reach him more directly if we ever came across another situation where we needed to get a hold of him. I'm relieved-- didn't think it was anything big, but it's nice to hear it from a doctor. A polite doctor.

For the folks at home, here's a picture of Connor's "happy" toes, and a picture of Connor's "Popsicle" toes-- or I should probably say "Popsicle foot," as the whole darn thing turns blue, along with his fingers and the skin around his mouth. The picture on the left was taken after he'd been exercising for a little while, and the one on the right was about five minutes later after a rest and the reemergence of the pink "happy foot." Dramatic, no?
















Anyway, this will also be the last appointment we have to get out of the way before we can finally report back to the general surgeon to make Connor's g-tube appointment. This makes me very happy. I have Rocky Road ice cream to celebrate. Yum.



~Jess

In Which I Feel Sorry For Myself For Being Alone On Valentine's Day, Despite My Husband Sending Me Yummy Things

So the potluck went pretty well, as well as anything that's meant to be a replacement for a romantic dinner with your husband can go, anyway. The deviled eggs were a big hit-- I made half plain and half with smoked salmon and green onions. They disappeared pretty quickly.

I did manage to stop by Target today to indulge in my personal favorite holiday-- Half-Price Chocolate Day. I've eaten entirely too much chocolate this evening than is good for me, but oh well. I figure since I won't be getting a real Valentine's day next year either I'm entitled to indulge. He's going to miss my birthday, too. Poor me.

But enough whining: back to the potluck. Connor's friend C was there. C is six and he loves to make Connor laugh. He does this by pretending to hit himself in the head and then throwing himself on the floor. Connor thinks it's hilarious. Boys.

We left after about an hour-- Connor started saying "All done" about forty-five minutes in, and then he proceeded to wave "bye bye" to everyone before looking at me expectantly. I can't say I blame him-- the party was indoors in a room sort of like a high school cafeteria, and the acoustics were terrible. Add about 15 children with high-pitched, very loud voices into the mix and with all the echoes it was probably impossible for him to make out anything anyone was saying. He pulled his hearing aids out about five minutes in. I was trying to interpret for him, but it was hard to do-- everyone talks so fast and when there's four or five people involved in the conversation I just can't keep up. I didn't want to push him, so we went home.

Today Connor and I went out to lunch with some friends and then headed off to sign study group. Connor acted kind of like he wasn't feeling very good-- he was really, really quiet today and clingy. He didn't eat very well. He also asked for his daddy repeatedly. I don't know if he's just missing his daddy or if he's feeling poorly, but we'll take it super, super easy tomorrow. I still haven't heard anything from Connor's cardiologist-- and I don't expect to hear anything tomorrow as it's a holiday, but I'll keep a really close eye on him and call first thing Tuesday morning.

~Jess

Medical Monday: Cardiomyopathy (Updated)

Doctor Connor is in the house! Dr. Connor declares my glasses and stethoscope to be the height of hilarity. Also he demands chocolate pudding.

Because Connor has so many conditions and I refer to them all the time without proper explanations, I figured I'd declare Mondays to be Medical Mondays from now on. I'll blog each Monday about a different condition Connor has and then post a link to it on the side bar as I go. This way people can get a better idea of what Connor deals with on a daily basis and his family and friends can understand what I'm saying when I call and spout ridiculously long medical terms.

DISCLAIMER: I would like to emphasize for those people who stumble upon this blog that I'm not a doctor, and I have no medical experience other than the approximately 900 doctor's appointments I've been to in the past two years. I just know about these things in relation to how they apply to Connor and not anyone else's child, so if you want accurate info on this sort of thing, please please please ask your doctor or go to the medical library instead of looking it up on the Internet. The library is your friend.

UPDATE: As undeniable proof that you need to get your info from the doctor and not me, a very nice person from the HCMA let me know today that you can only have LVNC or HCM-- not both as Connor's diagnosis sheet from his coordinating care doctor reads. I went back through my files and then called Connor's cardiologist for a double-check. Evidently somewhere along the line one of Connor's coordinating care doctors read LVNC with symptoms of HCM in a report from one of Connor's cardiologists and interpreted it as LVNC and HCM, which then found its way into Connor's permanant records and will now be almost impossible to change. Thanks, HCMA. Post corrected.

So that's that. Today's topic is cardiomyopathy.

I was checking out the news today online and came across an article about the Williams family. Louise and her twin daughters, Katie and Lauren all have dilated cardiomyopathy: a rare heart condition in which the heart is enlarged and weakened. I'd encourage you to stop by their website and leave some words of encouragement.

While Connor does not have dilated cardiomyopathy, he does have a very similar condition-- left ventricular noncompaction. I'm no expert on this sort of thing, but here is how it's been explained to me by several cardiologists:

The condition called left ventricular noncompaction (LVNC) is very rare and little understood. The muscle of the heart, known as myocardium, starts out during fetal development as a very spongy and spread-out material. As the heart begins to develop further, this material draws closer and closer together, becoming the dense, extremely strong muscle needed to be such an efficient pumping mechanism. With LVNC, the myocardium stays spongy, especially in the left ventricle, and so the heart is weakened and can't pump as efficiently. The difference in the heart muscle causes very deep nooks and crannies in the wall of the left ventricle, known as trabeculations. Trabeculations cause issues because blood can pool and form clots in these nooks and crannies, greatly increasing the risk of stroke. Connor takes aspirin every day to reduce the risk of this happening, and also takes another medication to help his heart pump more efficiently.

Because of how hard it has to work, Connor's left heart wall has become thicker over time. This is a condition that can cause serious problems if the thickness of the walls cuts off the blood flow in the heart. However, in Connor's case, the atrial septum-- the wall that divides the left and right ventricles of the heart-- is not becoming thicker and so he has no problems with blood flow. Other than not being able to play any contact sports or be in the military (I'm just heartbroken over that, I tell you) his heart issues shouldn't otherwise limit what he can do as long as they're well-managed.

The nature of Connor's heart defects are one reason why he is considered to have a shortened life span-- we've been told somewhere between five to fifteen years. However, most people who have been discovered with LVNC are diagnosed because they are going into heart failure, and so understandably the mortality rate is pretty high. It's likely that there are many, many people walking around with LVNC who have no symptoms and live a normal lifespan-- they just aren't ever diagnosed with LVNC and so the statistics don't reflect them. It's also likely that if Connor didn't have so many other things going on, his heart defects would never have been found, as he shows no outward symptoms at all. Besides, the doctors don't exactly have the best track record on the whole Connor-kicking-the-bucket thing. They've already declared him doomed on four previous occasions, and he's still his happy little very alive self.

Like Louise, Katie, and Lauren, Connor's heart condition cannot be fixed by surgery. If he were to have issues with his heart, he would have to have a heart transplant. He is at risk for sudden death, heart attack, congestive heart failure, and stroke. Luckily, Connor's heart thus far has caused him no problems and we hope this will be the case for a long, long time to come.

Here's some good links involving LVNC:

The Cardiomyopathy Association

The Internet Journal of Cardiovascular Research

Left Ventricular Noncompaction

Phoebe Willingham Muzzy Pediatric Molecular Cardiology Laboratory

American Heart Association: Rare Cardiomyopathies

~Jess