White Elephant in Hannah’s Room

Hannah had another breathing/gasping episode Sunday night requiring us to call 9-1-1 again. We were at a restaurant when this happened, and by the time the paramedics came, her oxygen saturation was at 82% on room air (should be 98% to 100%). The paramedics started her on oxygen immediately so she went back up to 100% rather quickly. This time in the ER, she didn’t fall asleep afterwards as she did the first time. Within about 15 minutes, she was completely fine again, having caught her breath and relaxed.

We didn’t bring our oxygen with us to the restaurant – last time that will ever happen again. We are getting our pulse oximeter (portable) today so we can monitor her next time.

My first thought was this was a bit different than last month because she wasn’t eating anything at the time it happened, so I thought Hannah was having the dreadfully feared “laryngospasm,” which is common in many GD type 2 children. Honestly, I don’t know exactly what it was, as I have never seen a laryngospasm before (I even looked on youtube!).

I do know that laryngospasms lead to the one thing I have been hoping to avoid — the trach. Hannah’s ENT, Dr. Roy, and I have discussed the need to eventually have “the discussion” about it, and I was convinced even before hearing from him this morning that this episode is going to be the precipitus for it. At what point, how many episodes, etc., do we say “now is the time?”

The problem is we just aren’t sure exactly what these episodes Hannah is having actually are.

I talked to Dr. Goker-Alpan at the NIH today, and she also isn’t completely convinced yet these are laryngospasms and instead think they may be seizures and wants Hannah to have a full seizure workup including sleep-deprived EEG, BAER (brainstem auditory evoked response), and even a 24- to 48-hour EEG if regular EEG is negative.

She also wants to see if there has been any change in Hannah’s vocal cord weakness via larynscope, so when we see Dr. Roy next month, we will have him do that as he can compare it with the video from her scoping done about 4 months or so ago.

We also need to start keeping a log of these episodes, regardless if they are big (requiring 911 and ER visit) or small, so we can see if there is a certain trigger or not. Also, it will help us determine the frequency of these episodes which will eventually help us face the dreaded white elephant in the room – the need to have a tracheotomy for Hannah.

I guess for me, the trach represents the next phase in this disease…progression, deterioration, and that the disease is winning. If it is a laryngospasm, it is another piece of “evidence” that Hannah is following the type 2 path, which I just don’t want to happen.