In Which We Have An Extremely Busy Time

Our Internet lives!

Apparently someone from our Internet provider had been mucking about in our utilities box earlier in the week and had knocked something loose, which disconnected our Internet.  So they sent a very nice man out to fix it, who informed us that there would be no charge.  Which is good, since their company broke it in the first place. 

Oh well.

We've had an eventful couple of days!  Yesterday Connor and I traveled down to visit the Farmer's Market in Olympia; I needed to start my Christmas shopping and was also running low on yummy smelling soap, which is a dire situation.  So we restocked on frothy, soapy goodness and then trolled the shops along Capital Way-- including the fantastic little toy store Wind Up Here off of 5th Ave, which is where we found the beginnings of Connor's Halloween costume.  They had the most glorious white fuzzy monster hat, which the little guy not only didn't object to being plunked on his head, but he actually enjoyed carefully feeling the soft fuzzy ears on it.  So Connor is going to be a Yeti for Halloween; a perfectly respectable Pacific Northwest sort of thing to be.  I'll be getting him a white sweater and some light colored pants and trimming the cuffs with fur, and we might have to find him some fuzzy slippers.  I think it'll be pretty great!

We were stopped at a street corner waiting for the light to change so we could head back to the car when Connor began having a seizure.

Let me tell you, there is nothing that attracts attention like doing mouth-to-mouth on a small child at a busy street corner.  We literally stopped traffic.  A variety of bystanders rushed over to see if they could do anything to help.  The seizure wasn't very long-- only a couple of minutes-- but that was enough time for someone to call 911.  The police car pulled over next to us as we were walking back to the car-- I'd gotten flustered by all the attention and set off initially in the wrong direction so I was having to backtrack-- and the officer made sure Connor was okay before moving on. 

That was more than enough excitement for one day!

Then today Connor had two more short seizures: one at school and one in the bookstore I'd popped in to grab some reading material for Connor's afternoon cardiology appointment.  Neither lasted more than 45 seconds and he didn't even have to go home after the one during school, but three seizures in two days isn't so hot.  He goes up on the Lamictal tomorrow though, so I'm hoping that will curtail the issue.

I'm happy to say that the little guy's cardiology appointment went swimmingly!  His heart is functioning well and he's been released to yearly appointments instead of every six months, which is pretty fantastic.  He was extremely good for the appointment and when prompted held very still for the ECHO and the EKG both, which I thought was very mature of him.  He got a pinwheel as a reward, which I have attached to his wheelchair.  He spent a lot of time staring intensely at it as if he could make it move just by willing it to do so. 

So all in all, it was a busy couple of days!

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

Frustrating Day

So I made the mistake of bragging to Ellen over at To The Max about how I'd actually had a pretty good week. Sometimes I think God uses my life for entertainment value. "Did you hear that?" he says, elbowing Gabriel in the ribs. "She's bragging about having a good week again. Give her the chair!" Then Gabriel comes down and executes a pile driver on my day while all the other angels look on and cheer.

Anyway, yesterday afternoon after his evaluation Connor kind of got all quiet and started acting like he was tired. I figured he'd just had a hard day and didn't think much of it. This morning when he woke up, however, he was kind of blue around the mouth and at the fingers and toes. Any mom who has a kid with heart issues knows that this is not a good thing.

Connor usually doesn't have any cyanosis-- that's the blueness-- so I was a little bit worried. He didn't seem out of sorts at all, though, it obviously wasn't a seizure since he was still conscious and breathing, and he pinked up pretty quickly when I got him dressed. I figured maybe he'd just been a little cold. I debated with myself about whether or not I should take him to Family Conversations play group, but I ended up deciding to go ahead since he was pink and happy, and just to call and make an appointment with his cardiologist for the next week.

I told them at play group that he was looking a little blue around the edges and so to watch him carefully (we go off and leave the kids with the therapists for about an hour while we listen to presentations or get sign lessons) and when we came back, they said he'd had a couple of episodes and seemed to pink back up when they lay him down on the ground. He started going cyanotic again during the last song of the day, so we took him into the other room. By this point I'm thinking-- okay-- I really don't want to wait until next week to see someone. He probably needs his medication adjusted and that's all it is-- he's gained quite a bit of weight and he's more active now-- but just in case I'd better take him in. We debated on whether or not I should just call an ambulance, but by this point he was once again pink and happy, so I just put him in the car and drove down to the military hospital where our cardiologist works.

I called the appointment line on the way there and was informed that they could squeeze me in in early March. Riiiiiiiiight. I explained the situation and they said that I could either call the pediatric specialty desk directly and see if they could page the doctor or I could go to the ER. So I called the ped's specialty desk. The phone rang twenty times. No one picked up. I gritted my teeth and drive to the emergency room.

When I got to the ER, I explained the situation to the guy at the front desk. He looked at my currently pink, happy child and said, "Well, here's the deal. He's not blue right now, and we're pretty busy today, so it will probably be an eight to ten hour wait, and the Echo team will probably already be gone for the weekend. If I were you, I'd just walk in to the clinic. They should see you a lot faster." So we got back in the car and drove back around to the clinic.

When we got to the clinic, the person in charge of reception at the pediatric specialty clinic was still MIA, so we walked down to the general ped's clinic. They told me that they weren't able to make any appointments for cardiology, and not only that, but Connor's doctor was out of the hospital for the day. I explained the situation for the third time and they decide to find another cardiologist to take a look at him.

This is when things get really ludicrous. The other cardiologist comes out, and with him standing over me in the middle of the waiting room we have the following conversation:

Me: Hi! I'm Jessie, and this is Connor-

Man: He probably has arrhythmia.

Me: Um...well he's got left ventricul-

Man: I know. I read the nurse's note.

Me: (Refusing to be interrupted.) He's had some intermittent cyanosis, which is not a typical symptom for him, and it seems to cluster around periods of activity. He's also been acting unusually tired. His medication hasn't been adjusted for a while, and I'm wondering if we couldn't have you take a quick look.

Man: Probably arrhythmia. If it happens again take his pulse and see what it's doing. Then you can have me paged.

Me: Well, he hasn't had any arrhythmia before, so I was just thinking maybe we need to check his lisinopril dose-

Man: Well if he's going into heart failure there's nothing we can do anyway. Just take his pulse if it happens again. We'll have Dr. P (Connor's cardiologist) call you tonight.

And then he walked off, leaving me sitting in the waiting room in sputtering, incoherent rage.

It's not almost nine in the evening and Dr. P hasn't called-- leaving me to speculate that they probably put the page in through the computer system and not the pager system, so he'll get the message on Tuesday (it's a four day weekend because of President's Day). He's usually pretty good about calling me back, so I'll give him the benefit of the doubt. I didn't stay and insist on Connor having an Echo done for two reasons--

1) He is still being a pink, happy baby and he hasn't had any more blue episodes. We'll just take it really, really easy the next few days until we get in to see our cardiologist, and if things look any worse I'll immediately take him to the ER, which is literally a two minute drive from my house.

2) I never ever want to see that doctor ever, ever again.

Seriously. I could maybe, maybe almost sort of understand him being a little rude if I was in there, say, five or six times a day with imaginary ailments, but this is the first time I've ever done this sort of thing. Maybe things were really, really busy and he didn't have a lot of time-- then I could understand him being a little bit short with me. Fine.

But what kind of a jerk on being told that a kid with heart issues is potentially having trouble walks into a crowded waiting room, doesn't even bother to take the kid's pulse, announces "well if he's going into heart failure there's nothing we can do anyway?" and then walks off?

Bleagh.

~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