How J Fights

For the last week, Julianna’s voice has been a tiny, high-pitched thing. She’s not sick, so it’s probably just the CMT doing its thing — again.

The other day, I told her that she sounds like a newborn kitten. Her eyes lit up with delight when she heard this, and it’s an accurate comparison. She communicates volumes in her little squeaks. She is a cute, curious and articulate little kitten.

IMG_1643.JPG

Dec 2015 — Julianna and kitten George.

 

The kitten voice hasn’t changed anything. She still has high standards and challenges me to be my best.

 

J: Mom, you’re a queen.

M: I am?

J: You should were a tiara.

M: Oh, well… maybe I’m off duty.

J: avoids eye contact — You’re off duty most of the time.

M: Julianna! Well, I don’t really know how to be a queen. How do I do it?

J: sighs. It’s complicated.

Snow-449.jpg

Dec 2015, Capturing Grace Photography. Flora, Fauna and Merriweather.

 

She continues to combines imagination with — a little mockery?

I knock over one of her lamps. There’s always too much stuff in her room!

M: Argh!!!

J: What’s that? An avalanche?

She’s also kind of a superhero. (The superhero world is a new discovery, but I think it’s always been part of her persona.) Recently, she told me how she would deal with ridiculous comments.

IMG_2844-3

J: For important things that are bad, I’ll kick them, and say “Uh-huh.” Sarcastically. For things that aren’t important, I don’t say anything because they can think what they want.

Examples of “important things that are bad”

  • people who says that J is not important
  • people who don’t understand that a mermaid’s tail needs to stay wet

And the stuff that’s not important? “People who say I’m not pretty.”

(Sarcasm for the important things, silence for things that don’t deserve a response: perfection.)

“I miss my old voice,” she said. “But I like this one too.”

This is how our little girl with her little voice fights, with imagination and spirit. Against these things, CMT has done nothing.

Happy Easter!

2011

IMG_0739

2012

 

DSC00213

2014 (not sure what happened in 2013…)

IMG_1580

 

2015

FullSizeRender-23

We have hope because the battle has already been won.

“Mom, I’m OK.”

This clip is from February 2013. Julianna was two, and she had just gone through her first PICU admission for respiratory failure a few weeks prior. It was “only” eleven days (and the mildest of all her PICU admissions) but it was my introduction to hell on earth.

A few hours before this clip was taken, Julianna had woken up with a runny nose. It was nothing — but in our new world, it was terrifying. The trauma of the PICU was fresh, and I was afraid. Should I stay home from work so that I could watch her like a hawk and worry more thoroughly?

My mom was visiting at the time and told me to go – she’d watch Julianna, and it would be fine. She sent the “I’m OK” clip later that day to reassure me.

Julianna ended up getting sick again. I couldn’t believe it – again? Couldn’t we get a break? Why did a two-year-old have to go through something like this? It was not OK.

Fortunately, she wasn’t hospital sick this time. We went through some scary, sleepless nights, but she recovered. So it was OK — in the end.

And that’s the way it is: not OK, but ultimately…OK. Not OK that Julianna has suffered and lost (and she knows it). OK, because she acts like it doesn’t matter. Not OK that a child has to be stoic for her mother. OK (and such a blessing) that she has taught me to worry less and live more.

Take this as an example:

J: Can we visit Ellie?

M: She lives really far away, sweetie. I don’t think so.

J: But we can go on an airplane…

M: It’s a little hard for you to go on an airplane now, Julianna. I’m sorry, but I don’t think we can do it.

J: Because of my mask?

M: Yes.

J: I wish I didn’t have to have my mask.

M: I know.

J: So will you and Daddy and Alex go?

M: And leave you here? No way. Do you really think we would do that?

J: shakes her head

M: I’m sorry we can’t go on trips. I wish we could take you places…

J: I have problems, but I’m OK.

M: Oh Julianna…we all have problems. Do you feel bad about it?

J: No. I’m OK…whispers. Let me tell you something

M: I lean in. OK – what?

J: whispering: let’s trick the CMT and give it poison. And then it will go away.

IMG_1288

Dec 2013 — J’s last airplane trip.

 

 

I have problems, but I’m OK.

Tell me, how do I respond to this with anything other than abject humility and the promise to try, to never lose hope? I have to be OK too, for her and because of her.

Today, she tells me that she’s OK in the softest of voices (the softest it’s ever been.) It’s in her silent laugh, and in the way she insists on playing even though it’s getting harder to sit up (“Body break, schmody break,” she said yesterday.)

It’s OK, though, one day, it will be so very wrong.

Ultimately — in the end — it will be OK.

This is from April 2014. Julianna had just spent two weeks in the PICU and was finally home. Alex had decided to welcome her back by decorating the house with streamers and balloons, and this is J’s first look. Her voice (much stronger than it is today) was weak from the hospitalization, so it’s hard to understand:

The words don’t matter. The look on her face says it all:  pure joy, loud and clear. After another hard battle and heartbreak, things were OK again.

Pictures from the week:

J was determined not to be pinched.

IMG_2818

Enjoying the view with a few dozen friends.

20160316_132416

Selfie

IMG_2825

 

Get your copy of “Julianna’s Adventures” today and help J raise money for CMTA!!
Support independent publishing: Buy this book on Lulu.
IMG_2819

Julianna’s Adventures

Today, Julianna told me that this is all a dream.

I was standing over her bed when she said this. I was a dream, our conversation was a dream – even the hug I gave her was part of her dream.

“So silly,” I said. “If this is a dream, where’s the real Julianna? What’s she like?”

“She can walk. She can breathe without a mask,” Julianna answered.

My first thought (fleeting): This is feeling like the St. Elsewhere series finale – you know, with the snow globe.

My second: She’s right.

There is something more true than the reality we know, and I believe that Julianna is closer to it than anyone I’ve met. She told me today that she always sees herself walking and going on adventures. I believe that this is more than her imagination: it is a vision of what is to come.

And speaking of her imagination and her adventures….

We are excited to announce that Julianna’s book is finally available!!!

 

IMG_2822

 

Julianna’s Adventures:

  • 66 pages, full color
  • features 3 adventures: stories by Julianna, illustrations by Christine Stalie
  • $20.52 (plus tax and shipping) on lulu.com
  • all proceeds will go to CMTA

 

This book was a labor of love. Christine spent months bringing Julianna’s stories to life. (J, of course, had distinct and precise ideas about how things should look and what colors to use.) We tried to use Julianna’s words as much as possible.

Julianna imagines a world without CMT. This book gets us closer to that reality. Please buy a copy and spread the word!

Buy your copy of Julianna’s Adventures here:

“Does the CMT make it hard to talk?”

 

Before I dreamed of becoming a mother, wife or a doctor, I wanted to write.

I didn’t have anything to write until my heart was broken, and I didn’t have the courage to share until I latched onto hope. I am grateful to finally be writing. It is one of the most satisfying but difficult things I’ve attempted.

One of the things that I struggle with sometimes is deciding on the tone of my posts. Our story is painful; Julianna is a riot. I’m sad; I’m hopeful. We are not a normal family; we’re just like every other family.

Moon-335

June 2015, Capturing Grace Photography

For example, the other day, Julianna asked me if she will one day lose her ability to speak. She was lying in bed on one her “body breaks.” Her spoken thoughts are like little arrows sometimes, unexpected and piercing.

J: Will I not be able to talk?

M: I don’t think so, Julianna.

J: Does the CMT make it hard to talk?

M: It has in you, sweetie.

J: Why?

M: Because you need muscles to talk, and to breathe. And that affects your talking. Your voice is softer now, but that’s OK. I understand you perfectly, and I think I always will.

J: My voice sounds the same to me, but softer to everyone else.

M: Yes, that probably right. It sounds the same to you. Julianna – do you worry that you won’t be able to talk one day?

J: Yes.

M: I don’t think that will happen. God knows how important it is for you to talk. I think He’ll make it so that you can always talk. I just believe it.

J: It’s up to God.

M: Yes – it is.

 

An hour later, we had this conversation as I was getting Julianna ready for bed.

J: Here – I made lunch for you.

M: Thank you.

J: It’s a sandwich, an apple and a banana. And for a drink, I gave you milk. It’s in a brown paper bag.

M: Thanks – that’s so kind of you.

J: Eat your sandwich.

M: The sandwich says “Don’t eat me! Don’t eat me!”

J: Just eat it.

M: But it’s talking to me.

J: Mom, if you just eat it, it will stop talking.

M: But then I’ll feel guilty…

J: Don’t worry. Christmas is a long time away.

M: What? So I’m only supposed to be good for Christmas?

J: OK, ok.  I’ll make you something else….mac and cheese.

M: OK.

J: And it doesn’t talk. It doesn’t have eyes or a nose or a mouth. It just sits there.

 

That’s the way it is here. We have a five-year-old child who has experienced too much, and she thinks about things that most of us cannot (and do not want to) fathom. She’s also a little girl with a delightful imagination and rapier wit. She breaks and fills my heart.

DSC00985

Age 2, after a “poke”

And this is the only way I can write it. It seems disjointed and contradictory, but it’s our life. Joy exists with sadness.  Bittersweet is its own flavor. Right now, that is enough.

_T7A6977

Dec 2015, photo by Charles Gullung

 

“My Nerves Don’t Let Me Walk”

_MG_6357

There was a time that I couldn’t look at pictures like this.

We were living in Arizona when this picture was taken. Julianna was two and Alex was four. Julianna is sitting up straight, and there is no adult hovering or lending a steadying hand.

Today, I can look at these pictures, and it’s a good thing. Every mother should be able to look back and see her babies when they were actually babies.

The most obvious manifestation of Julianna’s CMT is weakness. These photographs show how CMT has steadily and cruelly taken Julianna’s physical strength. It’s painful to see what once was, but I am comforted by the knowledge that CMT has not been able to touch Julianna’s spirit. The pictures prove it.

IMG_0535

Ten months old — and in a regular, non-medical high chair.

 

 

Fifteen months, in her first pair of orthotics (left) and in a physical therapy session (right — even then, she liked the bling.) We still didn’t have a diagnosis, and hoped that she was simply a late walker. I spent a lot of time thinking/praying “Please let this be an orthopedic issue (i.e., fixable), not a neurological one.”

 

DSC00267

At around eighteen months, she took her first steps with a walker. We made her do “laps” up and down this hall and bribed her with gummy bears. It felt like a miracle, and I longed to see more gains.

 

DSC00286

She’s almost two in this picture, and was at the height of her physical strength. Here, she is able to stand easily with one-hand support. I kept waiting for the day that she could stand unassisted (and maybe even walk down the aisle by herself) but it never came. She made a beautiful flower girl anyway.

 

 

She is two in these pictures. She plateaued in her physical progress, but  was able to get out and do “normal” kid things. One of her favorite memories of St. Louis (on a visit to Homie)  is this merry-go-round. She always chose the elephant. The picture with Alex is during our first month in Washington state. She is almost three, and we were able to get her out in just an umbrella stroller.

 

Left: First day of school! Preschool for Julianna, kindergarten for Alex. J had just turned three, and was able to walk short distances (i.e., 20 feet or so) with her walker.  She usually had a helmet (right) on while walking, because she wasn’t very steady. She went to school only a few months, and it was then that we started noticing a decline. It got harder and harder to coax her to walk, but we kept on her.

 

IMG_1345

Julianna never made it back to school after the winter holidays that year. In January 2014, our world changed with a runny nose. She would be hospitalized three times that year for respiratory failure.  The January admission was the worst one. She was in for four weeks and lost a lot of strength. She was never able to use her walker again after she got out. Here, she’s still in the hospital, but is on the mend and doing physical therapy. At that point, I was happy that she survived: that she was still able to stand was bonus.

 

IMG_1396

Summer 2014 — almost four. Her walker went into the garage, and J’s physical therapist let us borrow one with torso support. She wasn’t able to cover much ground with it, but it was better than nothing. She was having difficulty standing with assistance at this point.

Also that summer, we got a stander. This let Julianna bear some weight while supported. She is using it to paint (right). When she needed a break, she could recline. She didn’t love it, but the large “desk” was a good surface for playing and projects.

 

This was also the summer that scoliosis came into the picture. We tried a few different braces, but she hated them. (We can’t find any pictures where she looks like her happy self in a brace — this is the best one.) Worse still, they pressed on her abdomen and made her throw up, which led to aspiration. This was probably the hardest time for us. She was losing the ability to even sit up (right).

 

SONY DSC

A bright spot amidst all the decline was her power wheelchair. It gave her freedom, and she liked to go FAST!  (She would always “forget” to switch back to the slower indoor speed when coming from outside…) This is from January 2015. She drove into her princess room for the first time during her Make-A-Wish party. I am so grateful that she was able to do this on her own. Soon after this picture, her worsening scoliosis and arm strength made it impossible for her to use her power wheelchair. It sat in our dining room for over a year. It was a painful reminder, but we didn’t want to give it away. I also didn’t know how Julianna would feel. Last month, we asked, and her answer was simple: “Yes. Give it away.” No hesitation, no self-pity — it’s her way.

 

Moon-307

capturing grace photography

And now, we rely on the ubiquitous TumbleForm chair. It’s the only thing that is able to keep Julianna upright. She needs her BiPAP and suction with her at all times, which makes movement (even from room to room) difficult. She gets around on a custom made cart from Grandpa.

And speaking of that cart:

M: Did you know that if I could snap my fingers and take your CMT for you, I’d do it? Then I would have CMT and you wouldn’t. That would be so great.

J: But then you wouldn’t be able to walk.

M: I know. But it’d be worth it.

J: Mom, I don’t think that’s a good idea.

M: Why not?

J: Because you’d break the chair cart. You’re really heavy.

M: I wouldn’t have to use that, silly. I’d have something else.

J: Like a wheelchair?

M: Yes.

J – Oh! Laughs silently

 

 

 

The Onion

Almost two years ago, a family member (who will go unnamed) asked me put an onion in Julianna’s room. It would supposedly fight germs and keep Julianna healthy. He/She had read it in an e-mail from a friend, so it had to be true.

I mounted my usual protest (Where did you read that? That’s ridiculous!), but only briefly. I didn’t see how an ordinary onion could act as a microbial air filter, but what was the harm? It was better to keep the peace. And besides…what if it worked?

Confession: I’ve dabbled in a bit of superstition during this medically fragile journey. When you want something so badly, you’re willing to indulge in the irrational thought that a simple act can yield the desired result: the onion is akin to wearing your lucky shirt.

And it’s worked. We are on our third round of onions now (every time J gets sick, we change the onion), but she has gone up to fourteen months between illnesses. The onion is here to stay.

IMG_1893

J has 2 onions currently — they stay under her bed. They keep her princess room from being cliché. 

The superstitious side of me is also reluctant to talk about a wonderful and unexpected development: Julianna seems to be in a stable period. She’s gotten sick, we didn’t take her to the hospital, and she recovered – twice!

My logical self reasons through it. It’s not that her CMT is getting better. There are still signs of progression, but the rate has decline has slowed. And when she got sick, we put palliative care — and J’s comfort — at the forefront. Past illnesses were treated very aggressively. It was appropriate and life-saving then, but we’re at a different stage of her disease now, and we are making different choices. So far, it’s worked beautifully.

Julianna, of course, wastes no time or brain cells on superstitious rituals and thinking about how she can control her future. She is too busy playing, loving and having fun. She doesn’t obsess about how long it will last.

This childlike faith is an antidote against fear and past wounds. It is stronger than terminal disease and my imperfect faith. It is proof that our future can be better than we ever imagined, even as it brings something we don’t want.

This week:

Julianna channeled Princess Diana, circa 1985. “Do I look like a runner?” — J

IMG_2780

J made strawberry muffins with pink paper cups and colorful umbrellas.

IMG_2794

“Treasure this forever. I made it for you.” — J

IMG_2791

 

Physical humor, J-style

We are reading Calvin and Hobbes. The last few pages are always negotiated.

M: OK, it’s bedtime. 2 more pages.

J: Five

M: Three.

J: OK. Let’s shake on it.

I take her hand to shake, but J has other ideas.

******

An update on fundraising for CMTA:

We have raised just over $20,000 for CMTA!!! Thank you for all your support.

Julianna’s CMTA fundraising page has been moved to Crowdrise. It’s a bit of an experiment, but the CMTA and I are hoping that it generates more exposure and donations.

Please note:

– CMTA will receive 97% of funds donated through Crowdrise

— Donors are asked to contribute to fundraising costs – but this is OPTIONAL. If you wish to opt out, hit “Edit” at checkout

Check out the new page HERE:

PS- The new page an illustration from Julianna’s upcoming book, Julianna’s Adventures.