A few nights ago, I sat with Julianna on my lap.

J: Mom, I think I know more about life than you.

M: Oh really?

J: shakes her head. No, I’m just kidding.

I pressed her for details, and this is what she shared.

• “You have to be born for your mommy to see you.”
• “They let you sleep a lot. I know that really well.”
• “Cinnamon: if you sniff it, you will sneeze.”

Surely there’s more? There is, but she thinks of it in “bits”. She has promised to divulge her discoveries as they come.

One thing I’ve wondered about – and asked on several occasions – is how she stays so happy.

Perpetual brightness, like clichés, can be annoying. After all, clouds don’t have a lining, (silver or otherwise) and not everything has reason for happening. Our Julianna is almost always happy, but here is nothing false nor cloying about her. She’s a five-year-old who knows suffering yet is utterly unfamiliar with bitterness or self-pity. I don’t think she’s choosing joy or happiness – she just is.

And when I ask her, the answer is always the same. Why are you so happy all the time? She shrugs and says “God made me that way.”

And I believe it. I believe that her capacity for joy that is independent of circumstance is a gift from God. It’s humbling, and thankfully, it’s infectious.

 

These are the things that brought us joy this week:

A moment off BiPAP, that smile, and her cute little nose. I’m used to and grateful for the BiPAP, but I miss this.

Sunrise, sunset. Julianna slept through the sunrise (with her favorite colors!) but caught the sunset.

And, as always, the funny. 

M: What is this picture from?

J: Mom, it’s when I went to Alex’s school…

I look at again, and she’s right. About ten months ago, we took Julianna to Alex’s school play. She’s been out of the house just a few times this last year, so of course she remembers. But it’s still remarkable, how quickly her mind works…

M: You’re right! How do you remember things so well?

J: Mom, when you get older, you start to forget things….

M: Are you saying that I’m getting old and forgetful?

J shrugs.

M: So at what age do people start forgetting things?

J: Twelve.

Julianna is playing with her grocery store. Its shelves are lined with about a hundred tiny little items that require slender fingers and a good deal of manual dexterity. Steve doesn’t have the former, and keeps knocking things off the shelves. J is uneasy.

Steve picks up one of the items – a cheese wheel.

J: Can you put that down please? It’s expensive…

A few minutes later, she’s had enough. Steve comes to the kitchen and tells me that Julianna is requesting my presence. She knows my fingers are skinnier.

 

And lastly, J had a visit from some colleagues.

She loved it, of course. I wonder if any other Anna has ever been asked to read Calvin and Hobbes? Afterwards:

M: So I guess princesses are real, huh?

J: nods. Did you not believe me before?

M: I guess I didn’t…

J: pauses — But I did see some brown hair. (This Elsa is really a brunette…)

M: You did? (laughing)

J: quickly — But it’s OK!!

 

 

 

 

 

 

 

A few days ago, Julianna received a wonderful gift…

…and the mermaid tail begat a mermaid tale.

Julianna, sitting in her pink cart, declared “ Hello! I’m Julianna. I’m a mermaid. “

Like all good stories, the tale of how Julianna the mermaid came to land was revealed layer by layer. My thoughts in italics.

J: I was blown in from a storm. I was at the surface, and then the wind took me and BLEW me right onto your deck! And I said “Oh, what’s this? Where am I?” And then I opened the door and I was shocked – there were humans! I’ve never seen a mom and dad to live with before…

M: Really?

J: I’m just an orphan from the sea. (Seriously, where does she get this stuff? So dramatic!)

She continued

J: And their last name was Snow, and my last name is Snow. It’s like I was meant for this family. (Notice how she’s talking about us – to us.)

We adopted her, of course — even though the details of her orphan status were somewhat murky.

M: And what happened to your parents?

J:  Her voice is grave and quiet — Mom, mermaids die sometimes.

M: Oh, so your parents died?

J: nods

M: And you were all alone?

J: Yes.

M: You poor thing. How long have you been alone?

J: thinks for a few seconds —  22 days.

 

As bedtime approached, the conversation shifted.

J: Do you have any rooms?

M: Yes, we have several rooms.

J: Do you have any soft, pretty rooms?

M: Most of our rooms are plain, but we do have one pink, sparkly princess room. But you’re a mermaid, not a princess – would that bother you?

J: I’m a princess mermaid! I like pink!

 

We wheeled our princess mermaid into her new room.

J: Ooh, I’ve never seen pink sparkly walls like this before!

 

We put her in bed:

J: Ooh, this bed is so soft. My shell bed in the sea was hard.

What’s that black thing? (looks at the TV)

M: A TV.

J: A TV? What’s that?

 

And it went on and on:

J: What is that thing? It’s Hello Kitty, you say? Why does she not have a mouth? (It’s true – HK doesn’t’ have a mouth)…Have you heard of something called a book? I tried to make one out of seaweed in the ocean, but it did NOT go well. I’ve never had anyone read a book to me before…Is this a ponytail in my hair? I’ve never had a ponytail before! I couldn’t do this at sea… Can you turn on the black thing? So this is what TV is!

When it was finally bedtime (turning on “the black thing” pushed things back a while – clever mermaid trick), J tried to convince us to let her keep her tail on all night.

J: But mom, I’m a mermaid!!

M: Sorry – you can’t wear this all night.

After a few of these exchanges,

J: OK – Let me tell you something. I have a secret

M: What?

J: whispers — I can turn into a human

M: How?

J: shrugs her shoulders – I just do.

M: OK, so you can be a human for the night, and you can switch back to being a mermaid in the morning?

J: Yes!

 

The tail came off. I tucked J in and settled into the chair next to her bed. After about 10 minutes,

J: Mom, come here.

M: What?

J: I have to tell you something.

M: OK. I walk over to her.

J: I’m still a mermaid even though I have legs.

Bonus Features

 

Can you guess what this is?

Animal stickers on green foam? Yes, but the J-inspired version is better:

The green foam is J’s “Handy-Pack” (which is her version of an iPad). She asked us to put the animal pictures on her Handy-Pack and told us that she was FaceTiming with the animal shelter.

CMTA fundraising update:

Thanks to your generosity, we have raised over $2000 in just a few days. Our total is now just shy of $17,000!! This is amazing. Thank you, thank you.

The number should go higher soon. A few weeks ago, World Class Martial Arts in Camas, WA held a fundraiser for CMTA in Julianna’s honor. This is Alex participating in the “Board Break-A-Thon.”  (video editing by Steve). WCMA raised over $3000 for CMTA.

Help our Julianna make a world without CMT  — please donate to CMTA.

 

 

 

 

A few weeks ago, we received some of the pictures that were taken at our photo shoot for People. Sincere thanks to the photographer, Charles Gullung, and People for this gift. The photos are colorful, bright and happy. It’s just the way I want to remember things.

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Now that we’ve been in People (never our goal, but life is unpredictable…), there’s just one more publication that I’d like to make: the green journal.

Neurology is a weekly publication from the American Academy of Neurology. For neurologists, it’s kind of a big deal to get published there.

I don’t do research, so the chances of me publishing in Neurology are zero. I think I have a decent chance of getting into Neurology, though – as part of a case study.

Our family is interesting, medically-speaking. I’ve known this for a while, and it’s actually awful. It means that you have something bad and no one can figure it out. Julianna has one of the worst cases of CMT – ever. Steve’s is practically asymptomatic. To quote my high school self, quoi is the deal?

We may be getting close to an answer. We have gone through the latest round of genetic testing, and there’s a candidate gene. It’s something that is known to cause problems with peripheral nerve cells, but it hasn’t yet been identified as a gene whose mutation leads to CMT. If it pans out, Julianna could be the first case.

Why is this important? It will lead to a better understanding of what causes peripheral neuropathy, and it may pave the way for treatment. It could help other families who are watching their child decline and searching desperately for answers. Maybe – just maybe – it will identify something that we can try soon.

These types of medical advances require an enormous amount of resources. Our genes have been under analysis for over three years. It’s a collaborative effort, and research labs all around the world have contributed. Recently, I learned that our family’s gene analysis identified eight million “variances” (abnormalities) – which is actually a normal amount. We all have gene variances, and almost all of them never amount to anything. Only one (maybe two) is responsible for causing J’s awful disease. One out of eight million — this is just one of the things that the researchers have to sort out.

So far, we have raised over $14,000 for CMTA. This is a national organization that funds research for CMT, and they are the best way we can fight this awful disease. Our goal is to raise $60,000, but in truth, I would like to raise more – a ton more.

I’ve never tried to raise money for anything before, and I’m not a great fundraiser because I hate asking for money. I hate CMT more though — so I’m asking now. As usual, Julianna helped me put things into perspective.

M: Julianna, what should I do? I want to raise a lot of money for CMT, but I hate asking people for money.

J: But why? It’s for CMT…

M: I know. But I feel bad. What if they don’t have much money?

J: — spoken as if she’s stating the obvious — Mom, if people don’t have much money, they can give just a penny.

Julianna and Alex both know that a lot of money is needed to find a treatment for CMT. I asked them what they would like to say, and they came up with this:

CMTA’s vision is “a world without CMT.” To be honest, I try not to think of this too much. I got glimpses of this sort of world before the diagnosis, when I thought we were dealing with just a bit of motor delay that physical therapy and determination could defeat. Picturing our Julianna running, laughing out loud (CMT makes her laugh silent) and dancing with her whole body (not just her head) makes me sad, because the chasm between this dream and our reality is too great right now.

Julianna isn’t afraid to dream, and she isn’t sad. She imagines a world without CMT. All the time.

Last night, during a rare quiet moment:

M: Julianna, what’s in that big brain of yours right now?

J: I’m walking. On the sidewalk.

M: Oh?

J: I’m walking, and nothing is bothering me – except for Alex. I’m wearing my colorful skirt and colorful shirt.

M: Where are you going?

J: The playground, of course!

June 2015. J is in her “colorful skirt and colorful shirt.” Alex is not bothering her. Photo by Aubrie LeGault, Capturing Grace Photography.

 

Later that evening, close to bedtime:

M: What now?

J: I’m walking…

M: And?

J: I get out of bed and go to the kitchen for a snack.

M: What do you eat?

J: Ketchup – and milk.

M: Are you sure?

J: Yes. And then I get out some toys and play.

M: All by yourself in the middle of the night? Aren’t you lonely?

J: No, I’m happy.

I sometimes wonder how mischievous J would be without CMT. The ketchup and milk story gives me my answer. This was taken when J was three and was still able to eat. We left her alone with a smoothie and came back to this.

I am grateful for Julianna’s huge imagination but I wish that she didn’t need it to walk.

If you are able, please help Julianna fight CMT by giving to CMTA — for Julianna, for a world without CMT. 

http://www.cmtausa.org/donate/julianna-snow/

We appreciate each and every donation!! And, if you know how to fundraise and have ideas on how to help, please contact me.

 

 

 

 

 

Except for ten scary minutes, Julianna had a great weekend.

The good: J is getting good sleep, and her heart rate is normalizing. It was too fast before, a sign that she was uncomfortable and working hard to fight her latest battle. We were able to restart her feeds, albeit at a lower rate. She played and played.

The bad: A few hours into her full rate of feeding on Saturday, J threw up. Her feeds go into her small intestine, so there’s not much in her stomach to come up. A little bit can be devastating, though,so we were alarmed. On Sunday, we kept her feed rate down, and things were fine.

Julianna got her g-tube (feeding tube to the stomach) placed in June 2014. It was supposed to make feeding easier and safer, but for some still unknown reason, it didn’t work. J couldn’t tolerate g-tube feeds and we lived with the constant fear of her throwing up.  We tried everything – different formulas, rates, body positions, medications. Nothing worked: she still threw up every single day. It was a difficult time. We knew that she was at high risk of aspiration but were helpless to stop it.

After four months, it happened and J ended up in the PICU again with aspiration pneumonia. The feeding issue was addressed with a G-J tube (a tube that lets us feed her through her small intestine, or jejunum). It’s not a permanent fix, and we were warned that it may not work at all —  but it was really the only option. Fortunately, it has worked beautifully. Her G-J tube has been in for fifteen months now (I think that’s some sort of record), and until a few days ago, we had no problems with feeding.

I’ll admit, the thought of going through more feeding problems makes me nervous. It is scary when a vital act (feeding your child) is dangerous. We have to watch her even more carefully now, because these events occur suddenly and silently.

I learned a while ago that control is an illusion. Julianna’s care is highly regulated. We talk about BiPAP settings, heart rate, liters of oxygen, millimeters of water, how much saline to put in her nebulizer. We aim for perfection, but nothing in this world is perfect. We will never be enough – but we have to keep trying.

There’s a temptation when writing sometimes to shove things into a nice package and put a bow on it — Here. The stuff inside isn’t so pretty, but I wrapped it up for you. Does that make it OK?

 Our reality is more complicated. The ugly and the beautiful are all mixed up. She fills my heart, but also breaks it. The status quo is tenuous, and I’m scared of change. On most days, the beautiful and the delightful are stronger than the ugly and the fear – and that has to be enough.

For the last few days, she has talked a lot about food – she wants to eat pretzels, biscuits, ice cream, salt, crunch: “Does anyone have some food here?? I LOVE pretzels.” She hasn’t gotten her usual volume of tube feeds recently, so I thought this explained all of the food talk. She told me in her matter-of-fact-without-any-self-pity way that she is always hungry. This is the strength that breaks my heart.

As always (thank GOD!), she wants to play. This is not really a pretty bow. It’s her world, and it has constant hunger mixed up with courage and magic. If I let her draw me in, it is enough.

On Friday, Julianna was an Asian princess with PJ’s from Singapore (Thank you, W!) and a silk fan blanket.

On Saturday, Julianna decided that her toys needed a spa day. Her unicorn and giraffe got pedicures. She insisted that they take naps afterwards, because she knows that spas are supposed to be relaxing. Afterwards, she defended them valiantly against the unicorn/giraffe “friends” who disapproved pink and blue hooves.