Neon Spandex

 

Nobody (except maybe morticians) likes talking about death. Certainly no one in their right mind likes thinking about children dying. Laura and I are no exception.

But sometimes we have to.

And we know that bringing it up makes people, probably even you, uncomfortable. Please hear me out as I explain why we have this most depressing "habit"...

From the outside, it may seem like we spend an inordinate amount of time talking about the threat of death from Cardiomyopathy. It might look like we're just wallowing in gloom and doom instead of looking on the bright side. This can be especially confusing when Simon seems to be doing great and we're still talking about death.

There is a reason for this.

Laura and I are two of the most optimistic people I know. The dictionary defines Optimism as " hopefulness and confidence about the future or successful outcome of something; a tendency to take a favourable or hopeful view". I'd say this definitely describes us.

We are also, what I would call, Down-to-Earth, which the dictionary defines as "practical and realistic".

Living with Cardiomyopathy means that our optimism must be tempered with what is practical and realistic, in order to maintain sanity.

We recently got more information from the Pediatric Cardiomyopathy expert, Dr. Towbin. A few months ago he reviewed Simon's echos and diagnosed him with something called Left Ventricular Non-Compaction, which is basically a more refined description of the anatomy that is leading to the poor heart function. Laura talked to him a week or so ago and was able to ask him specific questions and get more information. Dr. Towbin explained that kids with LVNC, more than other types of Cardiomyopathy, have a tendency to "go up and down" with their function. It's great that he's doing so well right now, he said, but that we can never let our guard down. And no one should ever try to wean him from his meds. He mostly echoed how we're approaching this disease (enjoying the good time right now but knowing things could get hard again) and suggested a few more tests we may want to consider.

It was both comforting and sobering to have our gut feelings about how this might look for the rest of our lives confirmed by the world expert. We've heard of kids that were deemed to have their cardiomyopathy"resolved",  told they had normal function, were taken off all their meds and then tanked abruptly and either died or needed heart transplants. I now wonder if those kids had undiagnosed LVNC.

When we mention this possibility to people I often get the distinct sense that they're thinking, "Okay Debbie Downer! Why don't you just look on the bright side and enjoy that he's so much better?".  This is particular true for people who have said, "How great that Simon is so much better!  I bet you're relieved" and gotten our somewhat depressing explanation above as a response. I understand that they're trying to be helpful and positive. We don't want to make people feel bad. AND, it feels important to convey that we're still living with this disease, it will never be over and it's not that we're just killjoy pessimists. We are enjoying this time of improved heart function and hope it will last forever, but we don't ever want to be that family, knocked on our asses by that sleeper wave again.

When I think about what Dr. Towbin told us, I combine my "optimism" with my "down-to-earthness" to crystalize into this:
I hope for the best quality of life possible, as few "lows" as possible, and that Simon does not die an early death from this disease.

I can not hope for a day that Simon is not on medications or a day that I can feel relieved that we are out of the woods. That day will not come. It's not a realistic goal and to pine for something I will never have is a recipe for madness, not happiness.

We are part of a listserv through the Children's Cardiomyopathy Foundation for parents of kids who have this disease.  There are a couple hundred families on it. We email each other constantly, checking in about how our kids are doing, asking questions about medication side effects, or funny symptoms or suggestions for how to ask our doctors hard questions. We share good news and hard news. We know each other. We are a community.

Every year a handful of children from this group pass away but Friday night I got news that * three* children from our Cardiomyopathy listerv had died within about 36 hours. This many, this close together is pretty unusual. As you might imagine, it's also pretty disturbing.

Someone close to us was with me when I got the news. She saw that I was upset and observed, gently, that Laura and I seemed to focus a lot on the death and dying part of this disease. I could tell that she was speaking to me as someone who loves us and doesn't want to see us suffer and wasn't trying to shut me down. But I still had to resist the urge to throw something at her.

I struggled to explain that when we talk about that grim aspect of Cardiomyopathy, we aren't trying to be dramatic, we aren't trying to shock people, we aren't trying to get sympathy. It's the threat we live with pretty much on a day-to-day basis. She posited that we all live with the threat of death every day, that any of us could be hit by a bus.  I inferred that she meant that we can't live productive lives worrying about death all the time, which I actually agree with. 

But...this is different.

As one parent on our listserv said, "yes, any of us could get hit by a car, but with Cardiomyopathy it's like we live in houses with a front door that opens onto a freeway". I'm not worried about Simon dropping dead out of the blue.  I'm worried about the delicate balance we've managed to gain over the last few years being tipped in an instant by something minor: a cold, a fever, something mildly concerning. It doesn't take much with our kids to knock things off kilter and we can suddenly lose all the ground we gained. I'm afraid of our lives being ripped asunder once again without warning, of the bug that will knock out all his reserves and leave us fighting a long, hard, awful, painful fight back in the ICU with the spector of death looming over us. 

Here's the analogy I came up with:

I said, "It's like we're gay men living in the 80's".

She stared at me blankly.

I realized she might be confused since we didn't have any neon spandex or cocaine in the house so it definitely wasn't the 80's and we have a severe shortage of testosterone or handlebar mustaches at Casa Fitch-Jenett.

"What I mean to say", I continued, "is that we live in a community that is staring death in the face every day, much like gay men in the 80's facing the AIDS crisis. The children of our friends on the Listserv are dying from this disease. This is not something that "could" happen or is happening to people "out there". It's happening to people we know, in our circle. And we have the same disease. It's likely that it could happen to us".

We live with this disease every single day right in front of us, worry about germs 10x a day, listen for a new cough every night, check for a sweaty forehead during naps, communicate daily with other parents who are also living with this disease, have relationships with parents whose kids are dying, pray for kids we *know* who are hanging on by a fingernail. There's something about warding off evil day-in, day-out that makes it feel a little different, a little closer than it is even for people who are so tightly in our lives.


It's so important for us to have people like this who can hold a focus point outside the storm, who can remind us that there are chirping birds and sunny skies out there. AND, it's also important that we keep a firm grip on a harsh reality so that we aren't caught off guard and spun off into the skies.

So sometimes we're going to focus on the hard stuff.  And we need to. It's our reality. It may not be pretty, but it's what we got. Thank you for bearing with us when we do. And if you struggle with this and try to tell us not to worry about Simon when we think we need to, we have a new code phrase that we will utter with love.

"Neon spandex".

Kinda catchy, right?