Still here and breathing but…

Hey everybody,

I‘m still here… haven‘t felt like writing in a long time… But I miss it. And I miss you. And I want to let you know what‘s going on. Well, what has been going on since June. It‘s pretty hardcore and sad, so… I don‘t know, be careful… if you have a pet, maybe have it near you while reading…

This was originally a mail I wrote in German to my doctor and osteopaths. Then the wonderful Katie Griggs translated it for me because I just couldn‘t go through the whole writing process again (But then I tweaked it a little to sound more like me…).

So there.

Dear All,

time‘s up: I have to make a decision about ventilation.

I‘m terrified.
I don’t want any of this.

But here goes: on June 10th I went to CABS (Charite hospital outpatient clinic for mobile ventilation and Oxygen… something) and I want to tell you about it. Plus it helps me organize my thoughts if I tell you.

The last time I visited CABS was 2 years previously when everything was pretty good. So they told me I didn’t need to go there regularly, just come if there is a problem. I developed a “problem”, that‘s why I made the appointment. My problem: About a week before the appointment I started keeping my eye-thingy on overnight, because my voice had become so weak, that the nurse didn‘t hear my calling right away. The weak voice is a sign of problems with air and breathing. Plus, I just had a feeling.

The problem with ALS is not inhalation, but exhalation. That’s why my oxygen saturation is always good, but I have too much CO2 in my blood because I can’t exhale enough. The CO2 value should be 33-43 (I don’t know the unit), mine is 49. CO2 is an anaesthetic gas, which makes you tired. I can confirm this, I am always tired, I only have to close my eyes for a few minutes and fall asleep. If I let this go on now without treatment, the tiredness will get worse and worse, I will sleep more and more and eventually fall into a coma and never wake up, which is the natural death of an ALS patient. Dr. Kim, who was very good by the way, could not tell me how long this would take, because I was not there for the past 2 years and so my course on this is completely unclear.

So it’s not good that I haven’t had my lungs checked for so long. It would’ve been better to have some regularity. Unfortunately that wasn’t clearly communicated and nobody paid attention. I often feel abandoned with my illness.

Fortunately, I can obviously rely on my gut feeling, which tells me when something really needs to be done. Like now: if I want to act, it has to happen quickly, depending on my decision. That’s why Dr. Kim had to give me some difficult information in a compact way. I have three options: do nothing (see above), non-invasive ventilation (mask) or invasive ventilation (tracheotomy).

For mask ventilation, I would have to be hospitalized for at least one week to try out and adjust the settings. This has to be done by a lung specialist. However, there is always the possibility that mask ventilation does not work for ALS patients. Pressure is exerted and sometimes the airways simply collapse. You have to try it, it‘s not possible to predict it from the condition of the patient. But you notice immediately whether it works or not. And it stays that way, the airways do not collapse sometime later if the condition worsens.

At this appointment on June 10, I tried something else that allows conclusions to be drawn about the possibility of mask ventilation: a Cough Assistant. Because lately I have to clear my throat all the time and I have a frog in my throat which I just can’t get rid of. I found the Cough Assistant quite horrible and could not stand the mask on my face very well, but it worked. So Dr. Kim made two cautious predictions: 1. mask ventilation could work for me, but 2. it would likely take more than a week. Great.

Once you have the mask, you’d probably only use it overnight. But over time, you would need it more and more until you were wearing it 24 hours a day. And: if I decide to use mask ventilation, it has to happen now. Like, in the next few weeks, not months. Like, I should take the next possible appointment. Dr. Kim repeated the that several times.

A tracheotomy was only explained briefly because I didn’t really want to hear it. But what I didn’t know yet, was that even with a tracheotomy, you can still breathe on your own and would use the ventilation initially only overnight and then increase it as needed until you are ventilated 24 hours a day at some point. But then all the breathing problems associated with ALS are over and you can live on for a long time. Conversely however, the ability to communicate will then decrease. So, you can move your eyes less and less, so that you can express yourself less and less by eye control. So you have to specify in a living will how long you want to be ventilated.

So these are my options.

I‘m scared.
I don’t want any of this.

Dr. Kim said a few more good things (I think): “There is no right and wrong. There’s only right for you. That’s all that matters.” and “A long life isn’t everything. It’s all about quality of life.”

Years ago when I first heard what was going to happen to me, I spontaneously thought: If I can’t breathe, I can’t live. Those are the “rules” in my world.

At the time it all seemed so theoretical and far away. Now it’s here. And it’s real. It was a shock. (Stupid, right? Like I haven’t noticed what ALS has been doing to me for years.) But after a few days of shock I realized that the “rules” in my world still apply: If I can’t breathe, I can’t live. Don’t want to live.

Whether I eat myself, or the food runs into me through some hole, whatever. I don’t care about that at all. But breathing… I want to do that on my own, I can’t give that to a machine.

And mask respiration is an imposition. Just the thought of a mask makes me anxious. Like claustrophobia. It’s a very unpleasant feeling to have such a thing on your face. I can’t imagine that, constantly or for several hours at a time. Not to mention that a week as an inpatient at the hospital is a nightmare. I don’t feel like it.

And I once said: What‘s another hole? But that doesn‘t apply to a tracheotomy. I don’t want that kind of hole.

Another reason against the tracheotomy is that I already have problems with too much air in my stomach. It causes nausea and burping is torture, and that won‘t get any easier. And Dr. Kim has said that ventilation will bring more air into the stomach, and unfortunately the air cannot be prevented from getting into the wrong place. So I would feel nauseous all the time, but I wouldn’t be able to communicate that? No, thanks.

And anyway, if I don’t do anything now, become more and more tired, sleep more and more and then don’t wake up anymore, there‘s something organic and natural about it. It feels right. Now a tracheotomy and then somehow determining when I don’t want to live anymore feels weird.

And the nursing crisis is a huge problem that won’t get any smaller anytime soon. It’s sooo hard to find good, qualified nurses. The only reason why I am now so well cared for and still have a life worth living is because of my parents. And my biggest fear is that they might die before I do. Which, of course, would be right. So I feel really bad about that and I’m so worried about my parents. Who’s gonna take care of them when I’m gone? But the other way round, I can’t imagine life in an institution without my parents by my side either. It’s all shit. Dr. Kim’s right, a long life isn’t everything.

So this is it. I don’t want ventilation and I‘ll let things run their course. Well, actually, I don’t want any of it. And I’m scared. But that’s the way it is now, and that’s okay somehow.

If you have an opinion on this, I would like to hear it. Above all, have I forgotten anything?

Best wishes


PS: The option of a tracheotomy remains for me for a while. If I want another hole. But I don’t think that’s very likely.