This blog is my record of my journey with my son who had a rare, and eventually fatal metabolic illness. It is the story of the last year and a half of his life, his death, and after. I have shared this journey this in the hopes that is will not only help me come to terms with the realities, but also that someone along the way may find it helpful, as they face a similar journey.

This is my place to comment on events, blow off steam, encourage myself (and maybe you), share frustrations, show my love, grieve my losses, express my hopes, and if I am lucky, maybe figure out some of this crazy place we call life on earth.

The content might sometimes get a little heavy. As an understatement..


People who are grieving may write sad or difficult things and bring you down. This blog may not be for the faint of stomach or of heart. Read with caution and at your own risk.

If you are new to this blog, I suggest reading it from oldest to newest. It isn't necessary, as what I write is complete in itself. But this blog is sort of the result of the "journey" I'm going on, and I think it sort of "flows" better from oldest to newest.

I do hope that in the end you will find, in spite of all the difficult and heartbreaking things, things that are worth contemplating.

Welcome along!

Saturday, July 10, 2010


Thought I'd write a bit here about our new CPAP and oxygen monitor. In case anyone is interested. Or maybe in a place where they are being considered for one.

These machines amazing me. I think this sort of thing is really the "pinnacle" of what science achieves. Something that helps people, and no ethical questions involved, unlike genetic engineering.

In my ignorance, I thought that the Oximeter, oxygen monitor, saturation monitor or "sat." monitor, as it may be called, measured the oxygen in the skin somehow. Silly me!

The probe actually is an infrared light that beams through a toe or finger to a sensor on the other side. As the light goes through, it meets hemoglobin molecules which can carry up to 4 oxygen molecules as they course through your blood. Based on how the infrared light reacts as it hits these molecules, the oximeter can read how much oxygen your blood carries, and what your pulse rate is. Amazing!

The CPAP is just an all around cool idea. And it works! So simple, so effective. Just blowing air into your nose (sometimes mouth too) creates the pressure to hold your airway open!

So, how is this new equipment working for us? Well, in all honesty, I'm not really getting much more sleep. Not yet anyway. We need to wait for about 2 hours after Joel's night meds before we hook up the machine. Because it holds Joel's airway open, if any tummy contents come back up his throat, they could just go straight into his lungs. Which isn't too good, and can cause something called aspiration pneumonia. You get the picture. So we have been careful with this, and so far, so good.

However, this means Joel is sleeping for about an hour and a half before we can hook up the CPAP. (I just can't seem to squish his feedings and meds any closer together.) Which is lost sleep for us. And right now, for some reason, Joel is waking up really early. Between 5:30 and 7am, instead of between 7 and 8 am which was his previous time to wake up.

I'm not sure how much of this is due to him needing less sleep as he is sleeping better, or how much of it is that the CPAP starts waking him as morning approaches and he enters a lighter sleep. I suspect it is a bit of both, especially as he is still napping quite well each day.

My hope is that as time goes on, we will all get more used to the machine, and Joel will start to sleep a bit longer. If not, it is not that big of a deal to me. It's more than worth the trade off to see him sweat-free with relaxed breathing in the morning.

For it seems to me that the CPAP really is working. It has only been just over a week, but I think it is making a difference. When I have seen his early morning breathing, it has remained relaxed.

To me the surest sign that Joel does obstruct and the CPAP helps, is when I connect the monitor and flip him on his back to hook up the CPAP. As soon as he is on his back, his oxygen level drops down enough to set off the monitor. I don't even have the mask to his face before it drops. When I hook up the machine, it slowly goes back up and the sat monitor has not once gone off with the CPAP on, even though Joel is sleeping on his back. Which is a position I have not dared to put him in since March. So yes, there really is obstruction, and the CPAP for sure keeps his airway open.

We have been awaked once, due to the mask slipping on his face and breaking the seal needed to maintain air pressure. But that's it. On the whole, I am very pleased with how it is going. Last time we had a single does of dexamethasone, it lasted a week. Now it has been two solid weeks, and he seems good to go.

Funny thing is, it is still a bit hard to relax and go to sleep. I just have gotten so used to being stressed about his breathing. So I lie there and think : What if the mask blocks his nose? Oh, yeah, the monitor would go off. What if the pressure isn't strong enough? Oh, yeah, the monitor will go off. What if the probe falls off his toe? Oh, yeah, the monitor will go off. What if the power goes out? Oh, yeah, it has a battery. You get the picture. I just can't seem to fathom that we have a system that is sure to tell me if a problem develops!

So falling asleep has not yet been easier, but I hope that as time goes on and things continue in the same way, I will eventually relax. And for sure, no waking up at 4 or 5 and listening to this heavy breathing and wondering, wondering, wondering if I should get up, or if it is still "ok."

There are not too many plusses to Joel's illness. But if you are going to have an illness that makes your tone so low your airway doesn't stay open, then it's a small blessing that you also don't move around much in your sleep. Which is one reason that Joel's monitor never goes off.

I knew this well, but I was strongly warned a few times that a monitor would only decrease the the quality of my sleep, as they tend to go off so much due to false alarms. Which I do know happens on active, healthy children as I have stayed in the hospital with these children as room-mates. And yes, I did NOT sleep well. (course, who ever sleeps well in a hospital anyway?)

Joel, I knew, would be a different story. I tried to tell them that, but you know... ? Still, I am grateful that it has been so "quiet" at night. I try not to take things like this for granted.

The big thing about all this stuff isn't even so much that I believe it might give Joel a bit more health or a bit more time. As much as I say that it is just so hard to let your child go, even when you know it is to a better place, it is even more basic than that.

I don't know how many of the doctors think that an inability to let go is why I push for stuff like a monitor or CPAP. In the name of truth and fairness, what really makes me do it is simply this: I hear my boy struggle to breath. Even if it were not life threatening, it hurts. Sure, I want him around for as long as possible. But when I wake up and hear him struggle on and on... well, I just gotta do all I can to help. If his breathing stops suddenly, well and good. He's let go. As hard as that would be, I think I'm going to be ok with it.

But when your baby is still TRYING so hard, you just gotta do all you can. I know any mom will understand this. What mother could just lie there and listen to your child in such difficulty? It's not about death prevention. It's about HELPING your child do what they are struggling so hard to do.

So that is how the CPAP has been for us so far. Maybe in a month or two, I'll put a little update on here, for those interested.


  1. I'm so glad that it seems to be helping! And, I totally agree with and understand the wanting to keep your child from struggling/suffering! I hope you can relax and get some more sleep soon.

  2. I'm so glad the CPAP is working for Joel. I totally agree that there should be no reason for him to struggle through breathing when there is a solution to help both him and you have more peaceful nights. I hope your confidence in the system grows and you can get some good stretches of sleep.