To stop a leak at night, upsize or cuffed trach.

[Samswonder]

Hi all! Samantha is being vented at night again. Mostly due to energy issues. I will try to sum up as best I can to further explain without giving you all a novel.

Sam has Leigh's disease (a progressive neurological disease which also impacts her energy levels, the more energy she needs ie. when sick, the more likely brain damage will progress) It is a mitochondrial disease--again she is most impacted neurologically, and her illness impacted her center for respiratory function, requiring the trach last year--ironically a year ago today was her surgery.

Amazingly, Samantha became less and less vent dependent and we were able to wean her completely for a few months. (Was totally vent free by September) A subsequent MRI showed the lesions near her center for respiratory function had shrunk a bit. That sometimes happens with Leigh's disease (things get better, and then worse again and then a little better again etc and so forth). Also, she is on an experimental drug which we think is helping.

We also cut her night feeds because she was gaining too much weight and eating so well during the day--and replaced with water in her feed bag as hydration is key with mitochondrial disease.

We were considering removing the trach in March at that time.

A few months after stopping both the vent and tube feeds we noticed a distinct change which reminded us of a month or so before her going into respiratory failure last year. (didn't want to "walk" or move much, crankier and more fearful, just seemed tired all the time) As her disease is progressive, we were worried that by expending more energy to breath (especially at night), perhaps we were setting her up to fail again. And, as we still have the trach in--why not use it to give her body a rest at night to improve her quality of life during the day?

Soooo we decided to add the vent back in again at night. The day after we made that decision, she got sick and it wound up being RSV and she needed to be hospitalized.

Now we are venting and tube feeding at night and see a distinct positive change. More energy, happier, just seems more alive again. We are happy with the changes, but as she has grown a lot over the last year her trach is obviously too small for a vent at night. She cycles a lot (has a rate of ten currently just to prevent apnea alarms), but as she is not completely vent dependent I wasn't too worried about it. Weirdly she has started desatting even while on the vent about an hour after she falls asleep (when she is really tired I guess) It only happens a few times within an hour or so and then she is fine the rest of the night. I have been toggling between raising the sensitivity to prevent cycling, to then needing to lower it because the high sensitivity is "locking her out" of the vent and it's not catching her breaths. And then sometimes I increase the breath rate to prevent her from desatting too. Something really unusual happened last week. I went to check her CO2 level while she was sleeping--right after a desat. And didn't put the vent back on as again, she doesn't seem to be vent dependent. The CO2 monitor wasn't catching any breaths, and she immediately desatted down to 70. I felt her back and it seemed to me she had stopped breathing on her own. She came right back up when I put the vent back on. And she seemed her happy perky self the next day.

That is the strangest part, that these new desats are starting at a time where I feel she has been the best she has been during the day and vent free.

We have an ENT appointment in a few weeks and I am sure they will let me make the decision on wether to upsize her trach (she is a 4.0 right now), or get a cuffed trach and only put it up at night. Her team seems to always scratch their heads with her and usually leave these types of decisions to me and my partner as her needs seem so unique right now.

I hesitate with the cuff as that is one more thing to worry about. On the other hand, I'm not sure I want to go up big enough with the size as it may prevent her from talking (she doesn't wear a passy and we can hear her just fine now).

The great thing about Sam is that it is always subject to change as her needs are always changing....for better or worse.

Just wanted to get some feedback on what other parents have done when faced with upsizing vs. cuff. And if anyone has any feedback or insight on the novel I did wind up writing just now, please let me know.

Thanks so much everyone!

[jamie]

i prefer to have Evan with a cuffed trach instead of an upsize. one reason is i want to give his airway all the room it needs, make trach changes remain easier, and um make sure he stays as vocal as possible. he is only on the vent at night and when he has a leak we get LMV alarms all night long.

[Samswonder]

Despite him being vent free during the day they won't let you shut off the LMV? They shut it off in the hospital after her trach sugery (it was always a bigger leak than it should have been)--a week before discharge they tried to turn it back on and it alarmed constantly. I complained loudly enough that they allowed us to shut it off.

That being said...thank you so much for your comment as I didn't consider the trach change aspect. At 3 1/2 but very aware, the trach change is the one thing about her trach that S still doesn't love--actually she hates it. So upsizing would definitely make it that much harder. For that alone I'd prefer a cuff.

You can't boil the cuffed trachs, correct?

[Alex's mom]

Hi Monica

My daughter is vented for about 23 hours a day. We inflate the cuff on her trach each night. We have had a few times when my daughter will do something similar to what yours does. The first hour or so of deep sleep, her sats are lower than her normal. Her normal is 97-100 and what we see for that first hour is 92-95. Inflating the cuff really helped this.

I would avoid taking her ability to vocalize away as best as you can. My daughter is not able to communicate but makes some noise. I've always hated how upsizing takes away her 'voice'. You can always try using a cuffed trach inflated at night. If it's not successful, you can fall back to upsizing later.

We cleaned the cuffed trachs the same way we cleaned the uncuffed trachs. Wash thoroughly, rinse. Boil water, remove from heat. Set trachs in hot water until cool, then air dry. We use Bivona flex tend TTS trachs and have never had a problem reusing multiple times.

[Big Al]

The situation your describing sounds perfect for a cuffed trach that would be inflated at night, especially if night time is the time where your noticing the most cycling. You can adjust the amount of water in the cuff until the leek is no longer triggering the vent, and then no further allowing for maximum airway (for talking) and adequate control (limit the leak so the vent can understand whats going on). I would especially recommend this over upsizing because it can be more easily adjusted and wont have the effects on vocalization your worried about. A word of warning, if the LMV alarm is off the vent may not detect a decanulation. Please check by putting your backup trach on the vent and seeing if it will trigger the LP alarm. We lost two children locally this year due to parents and caregivers turning off the LMV alarm.

[jamie]

I have been scolded by our pulm and RT not to turn off the LMV, but turning up the sensitivity a notch did help. ask your RT

[Christamae]

I recommend a cuffed trach (which is what I have). A warning, though, the cuff slowly deflates and needs tightened occasionally. When the trach is too old or been in too long, the cuff won't stay up.