“Will You Make Me Strong?”

Since February, Julianna has needed BiPAP almost 24/7. She takes it off for ten minutes each day during her bath.

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Sept 2015. Done with bath & back on BiPAP.

About a month ago, I walked into our bathroom while Steve was giving Julianna a bath. J was leaning back in the bath chair with her BiPAP on. She smiled at me, happy as a clam.

She looked so pleased with herself for sneaking some extra time on BiPAP. I couldn’t help but acknowledge the obvious.

“The BiPAP makes it easier to breathe, doesn’t it?” I asked.

She corrected me. “It makes it easier to talk.

In other words, Julianna needs to breathe because she needs to talk.

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Sept 2015. A rare photo without BiPAP. I miss seeing that cute nose.

Julianna’s words. I would not have had the courage to start writing had it not been for her words. They are funny, profound, encouraging, heartbreaking, en pointe. Her words reveal her enormous heart and sharp intelligence.

Just this week, there’s been a change. Her voice, already softer than it’s ever been, has taken on a high-pitched, forced quality. I don’t know what this means, but I suspect that it’s not good. Nothing about this stupid disease is good — it takes and takes and takes. What if it takes Julianna’s voice?

At another time in my life, I would have tortured myself while trying to come up with a solution. I would have stayed awake (even later, I mean), wondering what this would do to Julianna, and to us. I would have made a mental flowchart and researched devices not covered by insurance and shed bitter tears over yet another loss.

I’m not doing that anymore. The events of last year forced us to acknowledge that Julianna’s disease will ultimately win the physical battle. It’s a devastating reality, but once accepted, everything was lighter, sweeter. Julianna is so much more than her physical body, and these things are now our focus.

Julianna’s voice may be weaker and softer – but it hasn’t stopped. If fact, she talks even more these days. There seems to be a sense of urgency: she can’t keep quiet because there are too many stories to tell, too many opinions to state. There’s no time for sadness or self-pity, so we follow her lead.

I started writing so that I could share Julianna’s words with the world.  God willing, I will continue to share, regardless of what happens to her voice.

These are some of Julianna’s words. I want to remember them forever.

Sept 2015:

When I’ve had a bad day, I sometimes seek advice from J:
M: What would you say to someone who’s being mean?
J: “Go AWAY.”
M: What if that doesn’t work?
J: Then I would kick them.
M: You would?
J: Yes, although you would kick them for me.
PS —  She really said “although”

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Sept 2015. Relentlessly happy. The solution to all my bad days.

April 2015 — Note to Julie, dictated by J:

Miss Julie — Am I a little girl always? I love you. Wish for a unicorn. Remember Julianna always!

April 2015 — note to Homie, dictated by J:

Homie — I love you. I have a loose tooth. There is something weird. Do you know what is weird? It is glue. Love, Julianna.

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Summer 2015. Julie (our hospice angel/volunteer) with J.

April 2015 — J’s prayer:

Dear God,

I love you.

You are good.

You are very good.

Will you make me strong?

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June 2015. Photo by Aubrie LeGault, Capturing Grace Photography

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.

“I Can’t Walk, Remember?”

Julianna is highly quotable. She has the gift of finding the right words at the right time — and with the right delivery.

This is a collection of her best one (and two) liners, in rough chronological order.

18 months (approximately)

Like most kids, Julianna learned to talk by mimicking. She’s still really good at this.

Age 2:

We moved to Arizona when Alex was two years and Julianna was 4 months old. We hit gold with daycare providers again. For over two years, a wonderful woman named Margi cared for our children. Her home was as clean as it was warm, and it housed four generations of her family.  Her husband, Frank, also helped. As Margi said, J had him “wrapped around her little finger…tight!” There were many arms to hold and love Julianna. For a while, Margi worried that this was the reason that Julianna didn’t walk.

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Jan 2012. “Frankie” (this is what J called him. She is first and only person to call him this.) is sitting behind her. Just two days ago, J asked if she could go back to Margi’s house.

As you can imagine, J’s verbal skills did not lag. She was a chatterbox from the beginning. One day while I was driving her and Alex home, she said:

“Frank says I talk too much. That’s not fair!” 

Ages 3-4:

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The journal. It took a while to find one that matched her room.

I started recording Julianna’s words in a journal shortly after she turned 4. This is a list of one-liners that she used starting at age 3. I think that she learned most of these from her favorite TV shows.

— All right, all right, girlfriend.

— Relax, princess!

— Sing it, baby!

— Don’t freak out.

— What the heck?

This one is a Julianna original. She still says it a lot

“I love everyone – except for bad guys.”

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June 2015. For Julianna, “bad guys” are toys who do naughty things. Typical offenses include not sharing or rolling in mud puddles.  She puts them in “jail” — fancy ones, of course.

Nov 2014:

Julianna really hates bedtime.

Me: It’s bedtime, Julianna

J: No it’s not. Bed, with a circle and a line through it!

(She was verbalizing a prohibition sign for “no bed”. I was not able to find one on the internet – such is the stuff of her imagination).

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Drawing by Steve

Feb 2015

J is in her room. She sneezes.

J: I must be allergic to chandeliers.

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Jan 2015. J sees her chandelier for the first time. She is most definitely not allergic.

May 2015:

J does not like messy hair. Hair is not my priority.

J (to me): Your hair is unpleasant.

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June 2015. Photo by Aubrie LeGault, Capturing Grace Photography

May 2015

J is with Steve. She is being impatient about something.

S: Hold your horses!

J: I don’t have horses. I have ponies.

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Summer 2015. J and two of her ponies. And matching hair extensions.

Shortly before her 5th birthday:

J’s nurse: So Julianna, what are you going to wear for your birthday?

J: My birthday suit, of course. Naked!! (pronounced NAY-ked…)

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Aug 2015. Princesses wear birthday gowns, not birthday suits.

Age 5

(This is my personal favorite. She said it just a few days ago.)

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This morning. I can tolerate feather boas. Alex, not so much.

I know that it’s weird, but I can’t be around feathers because I am scared of birds. Julianna knows this.

A few days ago, I find some feathers in her room. I ask her where they came from.

J: It wasn’t me! I can’t walk, remember?