My patient scared the hell out of me!!

[warrior woman]

I work private duty for a family with a 7 yr old child with Mitochondrial Disease. Well, my main job is to safeguard him from seizures and gagging as he has a G-tube for nutrition due to the fact that his swallowing is impaired. Well, for the last 2 months he hasn't gagged because I can usually help vent him(expel gas from his stomach manually) and the gas bubbles are gone. However, the other night, he was especially gassy and the bubbles were hard to break up, and he started gagging, turning red inthe face, and gasping. Well, I quickly unhooked the feeding tube from the G-button, and vented him out (the family vents him out with a syringe) All the while keeping his airway patent and open. Well, the gas finally expelled the distention in his belly went down, and I could start breathing again!! I swear, it's a wonder my hair didn't go all grey right then!!! Or fall out!! He settled down for the night, and I told the family what happened. They said it happens for them too, and not to worry. Whew!! What a night!!:

[Spidey's mom, ADN, BSN, RN]

WW . . can you give us a word picture of what this disease looks like? How does it manifest itself in this child's life? What is he capable of doing?

You are a brave woman and so is his family. Not being able to breath is scary.

Thanks.

steph

[warrior woman]

Thanks Steph. Well, the best that I can describe it is that the Mitochondria which converts sugar into energy is being slowly desroyed from within.It's a genetic disease that stems from 2 recessive genes coming together. But here's a puzzler. This family also has a daughter who's 13 and PERFECTLY FINE. I can't figure it unless it's just the mom's recessive gene that affects the fetus. Anyway the cells of the child are slowly dying due to the destruction of the Mitochondria. The family knows that he is basically terminal, but also need to have home care come in while they try to lead as normal a life as possible. Hope that helps.

[warrior woman]

The family knows that he is basically terminal, and accepts that. But he still needs watching and care. And you can never take for granted that his routine or condition won't change even though things seems pretty stable right now.

[Gompers, BSN, RN]

I've had this happen to me at work. In our NICU, sometimes we have chronic babies up to a year old, and most of them end up with G-tubes and fundoplications because of reflux and/or feeding disorders. The problem is that a lot of these kids are on continuous feeds at night, and the tubing we use has no air port! So all night long, the stomach doesn't get vented and by morning, sometimes they end up having some respiratory distress. We once trialed a tubing system that had an air vent, but for whatever reason we didn't purchase it. I try to tell people that even though we don't have that particular tubing, we can basically make our own "vent" by plugging in extra tubing and syringes, etc. I seem to be the only one who does it on a regular basis though. It seems like babies and children are more sensitive, respiratory-wise, to abdominal distention.

Maybe I'm a little sick, but it makes me feel better when I see or hear a big "burp" bubble gurgling up that vented tubing. The kids just look so much more comfortable afterwards!

[warrior woman]

No Gompers you're not sick!! I was so relieved to see those gas bubbles in the syringe, and see his belly go back to normal size, not to mention seeing him get his breath back!!