Remarkable

In medicine, being “unremarkable” is good. We want our brain scans, nights in the hospital and test results to be unremarkable. Unremarkable means no tumor, code blue, or infection. It sounds boring, but when your child is medically fragile, unremarkable becomes your dream.

As I shared in a previous post, Juliann’s fourth birthday was difficult for me. 2014 was an all out battle against her disease, and we were failing miserably. We tried so hard to do everything right, but we simply could not keep her out of the hospital.

On her fourth birthday, I made a white Hello Kitty cake with pink sparkles. It was my attempt to give Julianna something “normal.” She took one bite, choked and threw up. We shifted from celebration to oral suction and worrying about another hospitalization. We had been battling this all summer. Julianna was losing her ability to swallow, and she wasn’t tolerating feeds through her G-tube (catheter into the stomach that delivers nutrition.) In that moment, I felt defeated: I couldn’t even give Julianna a taste of birthday cake. Was it really too much to ask?

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Fall 2012. Before all her hospitalizations, she could eat cake.

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Aug 2014. The calm before the storm.

Fast forward one year. Things are so different now. In some ways, they are worse: Julianna has lost all ability to swallow, and she is weaker. Her disease still has the upper hand in the physical realm. Her spirit, though, is thriving. She is strong, fearless and happy. It was this way all along, but because I focused all my efforts on attaining “unremarkable”, I lost sight of what is truly magnificent.

Julianna turned five this week. There were a few cakes, but no expectation of achieving what is “normal” for others. This is what happened during Julianna’s remarkable fifth birthday week.

  1. Julianna told her own story, and, in her mind, she has no limits.

When Homie is in town, she sits with Julianna at night. This week, Julianna told a story about their perfect day together:

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Summer 2012. A perfect day with Homie would start with fabulous outfits. I think that this is the inspiration for Homie’s flower dress in J’s story.

  • They share a bunk bed. Homie gets the top bunk, but she is scared of heights, so J must help her up the ladder.
  • In the morning, J puts on a blue dress with a bow around her waist. She wears a blue tiara, feather and bow in her hair. Homie has on a flower dress.
  • They go to the shoe store and each buy two pairs of shoes. J gets pink high heels and blue “low heels”
  • They go to Red Robin for lunch, and J has mac and cheese.
  • Last stop – chandelier store!

In real life, Julianna cannot scratch her nose without help. She has never worn “normal” shoes and it has been at least two years since she has been in a restaurant. In her world, she wears high heels to Red Robin, helps Homie climb a ladder, and she has managed to find a store that stocks just chandeliers. Her world is pretty great.

The future belongs to those who believe in the beauty of their dreams.

— Eleanor Roosevelt

2) She got to play in the sand.

J isn’t able to get out into the world, but she has people who bring it to her. E, her wonderful nurse, brought back some sand from the Oregon coast.

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Oregon coast in a box

You don’t need to be the tide to rise and fall,

you don’t have to be a wave to touch the shore;

just be a little sand-grain and feel them all

― Munia Khan

3) She was extra fancy and completely fabulous

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Aug 2015. Birthday. This was her evening outfit. (Yes, she had an outfit in between her day clothes and PJ’s). 

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Aug 2015 – Day after her birthday. Princesses can have tattooes, as long as they are sparkly and temporary.

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The inspiration of this birthday bouquet was Fancy Nancy. Because all floral arrangements should have feather boas. (Courtesy of J’s incredible palliative care volunteer).

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Aug 2015 – Day after birthday. Princess casual includes rose colored specs.

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Aug 2015. For the party, she had tiara, sash and statement necklace.

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Sometimes it’s enough to just have your cake. I know it seems strange to get cake for someone who cannot eat it, but J appreciates fabulousness.

More is more and less is a bore.

— Jean Arpel

4) She brought people together.

Social lives and travel are casualties of J’s condition. We’ve accepted it, but we miss spending time with loved ones.

This year, we were far from alone. All week, people blessed Julianna (and us) with their presence. In this busy, increasingly technology-dependent world, they gave her what is most important: time, love and attention. And, as always, good conversation. I believe that these are the things that Julianna lives for.

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Aug 2015. J and D, our favorite PICU nurse. She helped gets us through some of our worst days.

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Aug 2015. J and one of her handsome baby cousins. His onesie says “Tough guys wear pink.”

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Aug 2015. J with C, her “best friend” from the PICU.

Friendship is unnecessary, like philosophy, like art…. It has no survival value; rather it is one of those things which give value to survival. – C.S. Lewis

Happy Birthday, Julianna!

Will I still be your baby even when I am a queen? — Julianna, age 4

Julianna is 5 today!

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Aug 2015

We are celebrating Julianna’s birthday a few times this year. A few weeks ago, there was a joint birthday cake with brother Alex and her beloved cousin.

A look back:

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2011 – 1st birthday

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2012 – 2nd birthday

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2013 – 3rd birthday

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2014 – 4th birthday

Happy birthday to the most fearlessly happy person I know.

In your five years, you have:

Been a biker chick.

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Summer 2012

Gone blonde

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Winter 2014

Cruised around in the coolest carriage ever.

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Halloween 2014

Been very, very silly

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Summer 2013

Battled your way through way too many weeks in the PICU and, somehow, come out smiling.

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Feb 2014

Every day, you teach us how to live. We love you madly.

I love love. Love is real. — Julianna, age 4

“Not Sparkly”

If you think about it, singing is a complex activity. It involves the brain (verbal memory, pitch, sense of rhythm). You need your respiratory muscles to generate sound and volume. And, unless your singing is strictly a private affair, it requires a decent amount of confidence.

Julianna has always considered singing to be one of her talents.

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Summer 2015. J is in her Cinderella dress

Summer 2015

J and Steve are watching Cinderella. The step sisters are singing.

J: They are not good singers.

S: What about Cinderella? Is she a good singer?

J: She is a good singer, but not as good as me.

It has gotten more difficult. She needs BiPAP to breathe, and her right diaphragm is paralyzed. Her vocal cords do not open fully. She does, however, have a perfectly good brain and plenty of moxie.

(Note the dramatic arm movements. In this moment, she is Elsa.

Songs also let us be ridiculous. And, as always, Julianna has strong opinions. (Apologies for the potty humor…)

Feb 2015:

Julianna learned the song “Little Bunny Foo Foo” today. She likes it very much. This evening, while changing her diaper, I started singing “Little stinky poo-poo, you are not allowed here…”

J: Because you are brown and not sparkly!

“I’m Bossy but a Little Bit Polite”

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Summer 2013. Grandpa made this for J because it just fits. Says J: “I’m bossy, but a little bit polite.”

I hope that we’ve established by now that Julianna is wise beyond her years. Her remarkable words about heaven are what started my quest to share her with the world. She teaches me to embrace joy instead of giving into fear.

J’s wisdom is not limited to the profound. This girl knows that there is a certain way to do things, and she is not afraid to deliver her opinions. If Glamour ever needs a guest columnist to instruct (tough love style) and encourage, Julianna is their girl. This is what it would look like:

Be direct but kind

Julianna tells us she loves us at least ten times per day. Sometimes it’s tactical.

Background: Moving J from sitting to laying down is complicated – there are tubes and lines to watch. It’s one of the main things that a new caregiver has to learn.

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June 2015. Moving Julianna takes practice. (Photo by Aubrie LeGault, Capturing Grace Photography)

March 2015

Today I moved J from the chair to the couch. It was awkward.

J: Are you training?

Later that night, I ask her about  her comment. She avoids eye contact and says:

J: Mom, I love you!

Take care of your face

April 2015

I try to wipe Julianna’s face with a baby wipe. She refuses.

J: They’re for bums. And trash cans.

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June 2015. Washcloths, not baby wipes. (Photo by Aubrie LeGault, Capturing Grace Photography)

And Don’t Forget your Neck

Dec 2015. Bedtime with J. I’m bent over her at an awkward angle.

J: Your neck is wrinkled!

M: I can’t help it. I’m old.

J: You have to help it. Help it!

M: How?

J: Don’t bend your neck.

Sometimes cute requires sacrifice 

Everyone in our house has a big cranium.

(High school flashback: It’s a few weeks before graduation, and I’m being fitted for my cap and gown, and it’s not fun. The principal tries to make me feel better. “Some football player” has a cap size bigger than mine.)

There’s no way around it: the headband and tiara industries are sizeist. Julianna refuses to be excluded.

Last week, I picked up a cute headband for J.

J: I love it!

M: You won’t be able to wear it for very long, Julianna. It’s tight.

J: C’mon mom. I’m tough!

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Aug 2015.

Accessorize!

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Dec 2014. Channeling Rudolph, not Bozo.

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Oct 2014. Numchucks elevate the dreaded hospital gown.

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July 2015. Who said BiPAP has to be boring?

Speak softly and carry a fabulous photo

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Jan 2015.

The bejeweled photo within this photo was one of the delightful touches that Make-A-Wish put into Julianna’s room. It was meant to stay on her dresser, but for a while, J insisted that the picture go with her everywhere. We laughed at her outrageousness — who carries around a fancy, framed picture of themselves?

Then again, good things usually happen when we follow Julianna’s lead. And really, wouldn’t we all feel better if we always had a fabulous photo at our side, reminding us of our happiest moment?

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July 2015.

I think so. (Tiara optional)

You can also go digital!

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Thanks to JSP, one of several friends who say Julianna is their spirit animal. (I love that she just happened to have a tiara…)

Chapter 2: What are the chances?

If a little knowledge is a dangerous thing, what happens when you have a lot of it?

“…what are the chances that a neurologist’s kid has a rare neuromuscular disorder??”

— email I wrote to H.S. (friend who is also a pediatric physical therapist), Sept 2012

One of the worst things that a young child can do to a neurologist mom is to lag on milestones. There are a lot of horrible diseases out there, and we know too much.

During Julianna’s first year, we dealt with colic, a move (different state, different jobs) and Steve’s three month long deployment. The signs of her disease were there, but for the first year, I was too busy to freak out.

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Winter 2012 – Arizona. J and A’s second state.

That’s what I told myself, anyway, and that’s what I tried to project. I saw things, but I didn’t want to overreact.

These were the early symptoms:

  • Feeding difficulty – this was the first sign, and we blamed colic. (See the Angry Burrito). Things got easier once she started solids, but she was never a fast or vigorous eater.
  • Coughing with feeds – I knew that this could be a sign of aspiration (i.e., food getting into the airway instead of the esophagus), but  it didn’t seem to bother her, and she wasn’t getting frequent respiratory infections.
  • Stridor – this is a high pitched, whistling noise she made with inspiration. It usually means that something in the upper airway is blocked. If it comes on suddenly, it’s a medical emergency. J’s came on gradually and was there most of time.
  • Weak ankles – whenever J tried to bear weight, her feet supinated (ankles rolled outwards).
  • Unstable with sitting. J started sitting up at around 6 months, but she would plop over without warning. She didn’t sit up securely until about 12 months.
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    Aug 2011. First birthday. My hand is behind her for support – just in case.

At her first year well check, we talked about all these symptoms. Overall, J looked like a healthy baby who was lagging in gross motor milestones. I had already decided that it was either a neurological or orthopedic issue, and her pediatrician agreed. Orthopedic issues are usually a lot more fixable (and therefore less scary) than neurological ones, so it made sense to pursue this first.

We saw the orthopedic surgeon when Julianna was about 14 months old. He didn’t have a diagnosis, and thought that J would simply be a late walker. He reminded us that 5% of kids don’t start walking until 18 months. He recommended AFO’s (ankle foot orthoses, or ankle brace). When she started wearing them, we noticed an immediate improvement in posture and stability, and we hoped that this would make the difference.

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Jan 2012: J’s first pair of custom orthotics. (They got cuter when she began picking up the colors herself.)


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Jan 2012. First day with orthotics. We had high hopes.

I felt somewhat better. We had 4 months for J to start walking and still be “normal.”

Note:

Julianna’s first year was not all about missed milestones. Like I said, there has been a lot of worry and tears, but a lot more joy. This is also what we remember from the first year.

Happy, Bold, Confident, Commanding

In an e-mail to her friends, my mom had the perfect description for Julianna: she is “happy, bold, confident and commanding.”

HAPPY:

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May 2011. Happy, bald and beautiful.

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Winter 2014. She makes me so happy.

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June 2015. Alex has always been able to make her laugh. (Photo by Aubrie LeGault, Capturing Grace Photography)

BOLD:

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2014 – one of her hospitalizations. I swear, I don’t know where she learned to flirt.

Oct 2014: — To a male PICU nurse:

J: You are very handsome.

CONFIDENT:

7 June 2015 – (recorded in an e-mail to friends)

J learns that she is going to meet a new nurse soon.

J: Make sure you tell her about me

M: Ok – what’s the most important thing I should tell her?

J: I’m funny.

M: Uh huh.

J: Cute. Smart. Gorgeous

M: OK…

J: My eyes look like almonds. I’m honest. One day, I realized I like straight hair.

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Summer 2015. Almonds. (Photo by Aubrie LeGault, Capturing Grace Photography)

COMMANDING:

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Summer 2011.

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Summer 2015. Leg exercises.

Steve exercises J’s legs during body breaks. The TV is usually on. Whenever Steve pauses and looks at the TV, J says:

J: Dad, Dad, exercise!

And if he looks away again,

J: Dad, focus what you’re doing!

“The Claws are for Bad Guys”

Banner photo by Aubrie LeGault, Capturing Grace photography

Last December, Julianna told Make-A-Wish that she wanted 1) a white kitten named Snowflake and 2) a pink princess room with sparkle. Make-A-Wish came through big on the second wish:

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Jan 2015. Make-A-Wish princess room.

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Jan 2015. Kitty corner (Steve’s creation)

I was in charge of the first wish. I got the kitten part right: this is George.

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Dec 2015. Humane Society meet and greet.

Julianna wanted a cute and cuddly friend. He has turned out to be a good therapy cat.

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Dec 2015

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Dec 2015

George was just three months old when he came to us, and had a lot to learn.

Dec 2014 – journal entry

First day with George. He jumped on the couch while I was trying to do J’s treatment. I put him down. He jumped right back up. Down again. After the third time, Julianna turns to me.

J: Mom, I think George is stupid. Don’t tell Dad

(She really wouldn’t let me tell him for a while. I think she was afraid that George would be sent back. And she knows we don’t like that word…)

We tried to teach George through positive reinforcement, and asked Julianna to reward him when he was being good.

Jan 2015 – journal entry

George comes into J’s room and is calm:

J: Welcome, George, welcome! I love you. You’re so kind. Thank you. Welcome to my room!

Another time.

J: Welcome to my room, George! You’re so beautiful. I mean, handsome. Thank you. I love you.

As George become more of an established presence in our house, he showed up in Julianna’s imagination and role-playing:

26 Feb 15 – journal entry, conversation with Steve

J: I am a kitty. A brown kitty.

S: You are a cat with brown fur and cute eyes. And soft paws

J: I have claws.

S: You have claws? But you never use them, right?

J: The claws are for bad guys.

S: Oh.

J: The biting is for really bad guys.