Elephants and Fighter Pilots

The worst and most serious problem with neuromuscular disease is that it weakens the muscles that help you breathe. Julianna is with us today because of BiPAP.

BiPAP Demystified

BiPAP (bilevel positive airway pressure) is a form of non-invasive ventilation. This means that it helps Julianna breathe without requiring an artificial airway (i.e., tube down the nose or into the throat). It assists her breathing, but it cannot breathe for her.

It works like this:

  • Julianna inhales
  • Her breath triggers the machine to push pressurized air – positive pressure – into her lungs
  • Julianna exhales
  • The pressure from the machine drops so that it assists her to exhale.
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Trilogy

This is the Trilogy, it’s kind of like the Cadillac of BiPAP. It has 6 hours of battery life, which means that J isn’t confined to her room, and that power outages are not fatal. (We also invested in a power generator – it is worth every penny).

The air is pressurized and is pushed out into a water reservoir. This reservoir humidifies the air, so it’s less irritating. Another hose connects the water reservoir to the BiPAP mask.

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The setup is called a circuit: BiPAP to humidifier to Julianna. The circuit is connected by hoses.

A word about BiPAP masks:

  • There a few different types. Full face masks cover the nose and the mouth. Nasal masks cover just the nose. “Nasal pillows” seal just the area around the nostrils.
  • Fit is critical. The headgear has to be tight enough so that the mask seals, but it has to be comfortable.
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Jan 2015. J uses a mask called the Wisp – it’s a light, low profile mask. It took a few tries to find the right one, but it made all the difference.

Julianna and BiPAP

Jan 2013. We have tried our best to not be defeated by all of the hospitalizations -- but the truth is, every single one of them were hell.

Jan 2013. First hospitalization. She has on high flow nasal oxygen. It wasn’t enough. 

Julianna was two when she started using BiPAP. At the time, she was in the PICU with pneumonia. She was getting as much oxygen as she could through tubes in her nostrils, but she wasn’t turning the corner. The only thing that stood between her and intubation (tube down the throat and being on a ventilator) was BiPAP.

Like most, J hated the mask when she first put it on. She accepted it, though, and it kept her off the ventilator. She wore it continuously for several days, and then we were able to wean it off. By the time she left the hospital, she needed it just at night.

With each hospitalization, J’s need for BiPAP has increased. Last fall, she was able to spend up to 2 hours without it while awake. This February, she started using it around the clock.

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Jan 2013. This is one of the first nights at home with BiPAP. She was much happier once we found a mask that let her use binky.

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Fall 2013 (I think). To find the best settings for BiPAP (i.e., amount of pressure the machine uses to push in the air), J does a sleep study. She has one every year.

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June 2015. I’m grateful for BiPAP, but I miss seeing that face. We put a protective barrier on her nose (Duoderm). Constant use of the mask can be hard on the skin.

Many kids (and adults) never get used to BiPAP. We are thankful that J tolerates it so well. Her life depends on it.

Fun Things to do with BiPAP

  1. Pretend you’re an elephant. (We played this game with Julianna to make her like the mask more.)

2) Accessorize

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Summer 2015

3) Blow Bubbles

There’s an exhalation port where the mask meets the tube. It blows a good stream of air. (It is also good for drying hands and blowing out birthday candles.)

4) No explanation needed

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Aug 2015. Just awesome.

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See #2.

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