Thank you for all of your prayers and good thoughts and love. I know I don’t respond, but we read every single one – and it helps us more than you know.

Updates:

• We’re still pretty sure J had a small aspiration event. She’s on antibiotics, and seems to be responding well with a bit of extra respiratory support. She hasn’t been able to tolerate aggressive respiratory treatments, and that’s OK – sometimes it’s better to not stir things up. The fact that she’s comfortable and stable is amazing. We have definitely seen it go the other way (unstable despite aggressive treatment), and this is much, much better.
• Not sure what caused all of this in the first place (the throwing up part). J seemed more comfortable after we held her feeds yesterday. She got some Pedialyte to help prevent dehydration, but she got very little in the way of calories. Her feeds started up again this morning (at a lower rate), and so far, so good!

Now the more important (and interesting) stuff – how is J really doing?

Though she possesses a body that is unable to produce much volitional movement, our Julianna has a ton of energy. The best kind. You can hear it in her voice (always confident), see it in her eyes (sparkling) and feel it in the things she says (astonishing, funny, right-on). When we try to coax her to sleep, she resists – often by shaking her head back and forth to show “my energy.”

Yesterday, she definitely had less physical energy. Instead of enacting her own toy drama, she watched it on youtube. (Would you believe that there are dozens of youtube videos with Elsa/Anna/Barbie/Ariel figurine drama? They get kidnapped, fight over ice cream, learn to swim…I think J’s dramas are more entertaining, but I’m probably biased.)

In all the important ways, though, her energy – what makes her Julianna – was still there. For example,

1. J’s Pulse ox (machine that checks O2 levels) goes off. Her sensor has come off. It’s LOUD, and it brings me back to PICU days.

M: I hate that stupid pulse ox!

J: Mom….

M: what?

J: It’s not nice to say “hate.”

M: But I do. I hate it when it beeps at me.

J: It’s not beeping at you. It’s beeping at everyone in the room.

 

How can I argue with my calm, reasonable five year old who knows it does no good to waste precious energy on meaningless noise? A girl who has no concept of self-pity? I can’t, and that’s what helps me go on.

 

2) Julianna is half asleep.

J: “Mom, exercise”

I start moving her legs.

J: I’m walking…

M: Yes, you’re walking. What are you walking on?

J: soft grass…there are flowers.

M: What color?

J: Pink…I kick them.

M: you kick them?

J’s eyes open. She is surprised.

J: Not kick – pick!

M: Oh right, you pick them.

J: I would never kick flowers….

She closes her eyes again and we talk about the beautiful bouquet of flowers she picks while walking on the soft grass…

I forget sometimes, how fragile Julianna is.

The morning after I wrote my last post (the one that celebrated a cold-free winter that has allowed Alex and Julianna to spend precious time together), Alex woke up with a cold. A very mild one, but enough to make us employ the usual infection control protocol. Since I also had the tiniest of sore throats, I got Alex and Steve got J. From Sunday to Wednesday evening, I had no physical contact with my Julianna. I gave her air hugs and blew kisses, and I grilled Steve about their nighttime conversations.

Wednesday night, things were looking promising. My throat felt fine, and Alex was feeling better too. Maybe we could come together as a family again soon.

Then it all changed. Steve called me to the room just after I got Alex down. J had thrown up/gagged/retched/whatever it is you do when there’s no food in your stomach but stuff comes up. We think she has some sort of stomach bug, but it all happened so fast. She was breathing fast and hard and her oxygen levels were low – which means that she probably aspirated.

The night was OK. She had a fever but it responded to Tylenol. We’re back on oxygen and increased BiPAP settings. We’ll start antibiotics again today and watch her like a hawk. It’s all very familiar – and I hate it. Last night, I felt it again, the physical pain that comes when your heart hurts. She was doing so well.

Julianna is happy and playful except when she has a wave of nausea. I gave her a new baby Elsa doll as a bribe to finish a neb treatment, and she was delighted. Breathing hard, she still had the energy to comment: “She’s gorgeous! I love her headband…”

Our Julianna…the strongest and most fragile girl we know. We’re reminded again that we can take nothing for granted.

 

Please pray/send good thoughts. We want Julianna happy and comfortable.

Julianna’s CMT likes to throw curveballs – to the gut. I call them “BTW’s”. As in, By the way, she has scoliosis too. And paralyzed vocal cords. A dislocated hip. And that right diaphragm?…..not working. These are all the lovely gifts that CMT gave J during our hell years of 2013-2014.

When a disease takes so much away, you start to go numb. It’s a way of bracing against the pain of another loss — maybe if you expect the worst, it won’t hurt so much when it happens.

It doesn’t really work that way, of course. Expecting the worst doesn’t cushion the blow. It takes you to a dark place, and if you stay there long enough, the losses are much greater.

You see, numbness and love can’t coexist. If you love someone with a terminal illness, you will have pain. Reigning in your love to protect yourself just leads to more pain (and guilt). So the only answer is to love them and take the pain as it comes.

When you choose love over fear, you are able to see the beauty that was there all along. The CMT curveballs are still there, but they are surpassed by the wonderful surprises that come only through grace.

Wonderful Surprise #1: A&J

This week, Alex’s second grade class learned about Martin Luther King, Jr. and his fight for equality. They were asked to write a sentence about what freedom meant to them. This was Alex’s answer:

 

I wasn’t sure what he meant by this, but I knew I liked it.

I asked Alex about it the next day. Did he feel free in Julianna’s room because she doesn’t have the responsibilities (homework, hanging up her towel, etc) that he has? Because he can watch TV in her room? It wasn’t any of those things.

“I feel free in Julianna’s room because I wasn’t able to go there before,” said Alex. “And — I just like being with her.”

Up until the last month or so, Alex spent very little time in Julianna’s room. Their style of play (J is a director, Alex is a small tornado) doesn’t match up, and he’s not allowed to be near Julianna when he has even a hint of a cold. For the last few winters, he seemed to have a perpetual cough. When this happens, one parents is devoted to Alex, and the other is devoted to Julianna. We are careful not to cross contaminate. Strict infection control measures like these don’t exactly foster family bonding, but we don’t have a choice: the stakes are too high.

So far this winter, Alex has been gloriously healthy, and he has wanted to be with Julianna. After too much time taking a back seat to CMT and medical fragility, these two have finally been able to spend some time together as brother and sister. I wasn’t expecting it, but, oh, I am savoring it.

 

Alex wrote a few notes to his sister.

I believe that Julianna loves this more than anything.

 

Wonderful Surprise #2: Naughty J

It’s a novice parent move, leaving a baby unattended with markers.

This week, we left J unattended for just a minute — and came back to this.

 

With the help of lots of pillows and creative use of a hair elastic, Julianna was able to be a little naughty. She was so proud, and I was so happy.

 

Wonderful Surprise #3: Holding My Baby

I haven’t been able to hold Julianna in my lap for years. A combination of bad scoliosis, low tone and fear of making her aspirate kept me from trying.

The other day, I decided to give it a shot. It’s a bit tricky, and involves (again) a lot of pillows. Sometimes I get it all wrong, but sometimes I don’t. And when it’s like this, I’m in heaven.

 

J: Will I always be your baby, even when I am a queen? Or a giant?

 

 

Let me tell you something…

Julianna says this at least a dozen times a day. Her “brain is always going”, and she’s not one to keep things to herself.

We tease her for being bossy, but really, we are grateful that she is able to express herself so clearly. She knows exactly what she needs and wants. She tells us how her leg needs to be moved and where her back needs to be scratched. It is our job – and privilege – to make these things happen.

We are much more than the sum of our physical needs, though, and this is perhaps even more true for our J. Often, Let me tell you something is the beginning of a journey into Julianna’s world.

In J’s world, kisses are collected and placed into jars. She sends them out and puts “a little bit of love” in each one. She always blows a few to me as I leave for work and reminds me to keep one in my purse – for later. If I stub my toe as I’m wheeling her around in her cart, she instantly sends out more to take the sting out. Her kisses never run out, and they make everything OK.

 

There’s a code. It goes like this.

M: The “code” is the hearts — then what?

J: And then love, joy, happiness, nice words, no bad tricks. Good tricks. No tests. Joy. And words that have – you say “please”. And what else?

M: Tell me what the code is. Where is the code?

J: In here — J points to her chest

M: In your heart?

J; In my chest.

J: Turn that back on – it’s getting dark  — the iPhone is going to sleep

M: And how do you open the code?

J: With a lock

M: With a lock, with a key. And what happens when you open it

J: The happiness goes on your shoulders

Like her kisses, the code never runs out.

“I keep ordering it,” says J.

In J’s world,  princesses are real — the ones in Disneyland, anyway. She takes this point very, very seriously.

J: Let me tell mommy this too. So, all the Disney princesses are real. Seriously. I mean, I’m telling you the truth. What are you doing?

S: laughing

J: Why did you laugh?

S: Why did I laugh?  — laughs some more

J: Why are you laughing?

S: Because the whole thing is pretty funny.

J: Yes it is. I agree.

 

 

The latest character in J’s world is the Julianna Tiger.

Like her big brother, Julianna is a Calvin and Hobbes fan. Recently, we read the strips where Calvin uses his Transmorgifier and turns into a mini tiger.

“What do you think a Julianna tiger would look like?” I asked.

“Let me tell you something,” J said. And in about fifteen seconds, she had it all laid out.

Stripes: pink and purple

Eyes: sparkling, with hearts in them

Wearing: a feather boa

Roar: only gentle ones

Is all about: LOVE. “She’s not a ferocious tiger.”

Fun Cool Fact: The J tiger can aim her tail and shoot flowers into the ground whenever she wants. She makes fields of flowers wherever she goes.

This is the Julianna tiger.

We found an appropriately pink tiger and presented it to Julianna. She was delighted.

 

She named her tiger Rosie, and introduced her to Hobbes.

 

And there was a wedding

 

J dressed up, of course.

“And now they’re waiting for the babies”, says Julianna.

 

When you spend time with Julianna , she draws you into her beautiful world. Her world is brighter, sweeter and more joyful than ours. There’s a bit of drama and some strong opinions, but the overwhelming and overriding theme is love (sprinkled with a call to fabulousness.)

In my former life, it was important to travel and see the world. Some part of me still misses it, but for now, I am beyond content to stay here and explore our Julianna’s world. I can’t imagine anything better.

Dear Readers,

This post is going to be different from all the rest. Next week I’ll get back to the heart of this blog: celebrating the life of Julianna. Today, I have to share something that’s been on my mind.

mmm

In the next few weeks, our family’s story will be featured in People magazine.

The story is from our point of view, and it will (I hope) show how our love for Julianna and Alex drives everything we do while facing a difficult situation.

We share our personal (and sometimes painful) story for lots of reasons, but here’s the one I want to focus on today: we are really not that unique. There are thousands of families around the world who are caring for and loving a child with a terminal illness. We don’t often hear their stories, because it’s not easy to talk about what our lives are like.

So much good has come out of sharing our story, but we’ve also had some criticism. This is a risk you take when you share. I knew that from the beginning, but I was also naïve then. We are loving parents whose daughter has a terminal illness. Every decision we have made about her care has been with the help of medical experts and has been guided by Julianna. How could any of that be controversial?

I know better now. With this new article coming out, the debate about “can a 5 year old decide?” may be renewed. (For those who have difficulty reading beyond the headlines, note this: It’s really the adults who are making the medical decisions. Julianna guides us, but the decisions are ours.) Some will say that we are teaching Julianna that life is not worth fighting for. With this comes the implication that we don’t believe her life is worth fighting for.

I’m anticipating more of this noise, because it’s happened before. These types of comments have appeared on Facebook, other blogs, published articles, and even my Instagram feed.

Parents like us have had their hearts broken a thousand different ways. We have wounds that will never heal, and we have gone through a form of hell on earth. There should be no judgment – but there is. This is one of the reasons why I’ll keep sharing: I want the world to be a safer place for families like ours.

Here are three things I want people to know about what it’s like for parents who are caring for a terminally ill child:

1. Assumptions create judgment.

Sometimes, the harshest judgment comes from those who think they know what we are up against. The truth is, even when people share the exact same medical condition, their experiences will not be identical. Sometimes it’s very similar, sometimes it’s wildly divergent. This is why medical care is individualized.

2) We make the best decisions we can with the information we have.

Like all parents, we don’t always know the “right” answer, but we have to do the best we can. The stakes are a lot higher for some of our decisions, and sometimes it feels like torture: If we do X, she may live longer, but Z may happen, and that would be worse. Is the risk of Z worth it? Could I live with myself knowing that Z could have been avoided? What if Z never happens and we’re too afraid to try X? We quintuple guess ourselves – over and over and over again. If you have not had to make these kinds of decisions, be thankful.

3) You want to know that someone else has survived this.

We knew that we weren’t alone, but we felt utterly alone. I desperately wished that I could find other parents who could show me the way. How do you continue to parent when you know you will outlive your child? How do you make these impossible decisions? Is there hope for us?

I didn’t find other parents like us until I began sharing our story publicly. And I wouldn’t have found them if they were not, in turn, willing to share theirs.

I still don’t know what made me send the “heaven” conversations to the Mighty last May– it was almost on a whim. I was clueless back then, and I’m grateful for that.  If I had known then that it would generate criticism,  I would have never submitted the story.

As they say, ignorance is bliss.

On Sunday, we woke up to a surprise:

Snow is kind of a big deal here because 1) it’s pretty rare and 2) we live on top of a huge, steep hill. Our Sunday plans (church and Costco — yes, we live on the edge) were shot and we stayed home. Not a big deal as we buy everything in bulk (Costco).

Sunday night, we had freezing rain. Alex’s classes were canceled, and I had to stay home from work. Our usual Monday helper crew (nurse E, grandparents) very reasonably wanted nothing to do with our icy hill, so it was another day at home, just the four of us.

We were also homebodies on Saturday, so this was our third day in at home, just the four of us. I felt some cabin fever today. It’s always good to be home with family, but it’s not a great feeling to be homebound. There are errands to be done, patients to see. The holidays are over, and we need to get on with our lives!

And then I remember our Julianna. She is homebound. Every. Single. Day.

In the last year, she has gone out just a handful of times – a few trips down the hill to grandma’s house, even a few times to school. When the weather isn’t nasty, we take her out on the deck. But most days, she is home.

There are lots of reasons for this. We have all kinds of mobility devices (power wheelchair, manual wheelchair, a rolling chair thing), but none have kept pace with the aggressive awfulness of her disease. To go out now, she would need a chair that would let her lie on her back — and BiPAP, suction, cough assist, and a vehicle to accommodate all of this. It’s a lot.

I know that J would love to go more places, but she doesn’t complain. As long as there are people around to play and love, she is happy. Her buoyant spirit and powerful imagination leave no room for self-pity or boredom. It’s yet another thing I can learn from.

Here’s how we spent our time this weekend, stuck on our icy hill.

For Christmas, J received a book that contained everything she needed to open up a veterinary practice. It gave some basic materials (signs, forms) and some suggestions. She and Alex came up with the rest.

This is Dr. J and her assistants, Snoopy and Hobbes.

 

Alex registered patients at check in. (Tylenol Hello Kitty had the flu)
He then brought them back to the Exam Room (aka J’s bed) and helped with the exams. I was the scribe. Their exams and plans were documented on the “Exam Checklist” – you can one taped to  Tylenol Hello Kitty’s wheelchair.

J’s window sill was the inpatient area/recovery room. Long Monkey (he’s really one of those microwavable neck wraps) is in a recovery cage, and Mrs. Hare is keeping him company while wearing a lampshade collar.

Here are some of our chart notes: (italics are Julianna’s words that I scribed)

Patient #1

Name: Long Monkey

Heart rate: still beating

Length: really long

Temp: 3 degrees

Eyes, Ears, Fur : good (checked)

Mouth: teeth and breath are smelly

Paws: Good – have been brushed and washed.

Treatment: Tylenol and Motrin

Plan: Stay 3 days and rest. And sleep.

 

Patient #2:

Name: Mrs. Hare

Heart: beating slowly

Length: medium

Eyes: checked bad (blurry)

Ears: checked good

Fur: checked bad (a little shaggy)

Mouth: checked good (she can still eat)

Paws: checked bad (it has a little bruise)

Treatment: Bandaid (circled)

Plan: stay 2 days. Rest and watch something that distracts her from bad things.

For most of the day, J had a strict no-shot policy at her hospital. Her only treatments were Bandaids, Tylenol, Motrin and rest. Then Alex insisted on giving shots.  They argued, and J compromised. She now gives shots, but they are gentle shots with “soft needles.”

Later on in the evening, while playing with Steve, she transitioned from vet hospital to K-drama. The bad Korean queen (Vivian) is in jail (formerly a cage in the vet hospital) because she has torn apart the good Korean girl’s (Young-Hee) house. I have no idea how this happened, but I’m not surprised. That Vivian girl is trouble.

 

J’s imagination requires willing participants and TOYS. We have way too much.

I look at the pile of toys in the corner

M: Julianna, this is crazy.

J: Don’t worry. They’re not alive.

M: But we have to do something about this.

J: They’re just toys. Don’t judge.

She’s right, of course. Who am I to judge?

New Year’s has never been my thing. It carries too much expectation for change and improvement. That’s fine if the year stank (2014), but when it was great (2015), I’m not ready for things to change.

2015 was wonderful. Julianna was healthy for all but one week, and the one unhealthy weak was miraculously benign. Our pink, sparkly princess got a pink, sparkly room. We shared our story more publically than we could have dreamed, and this has given some meaning to our past pain. We laughed a lot and shed very few tears. It’s hard to imagine that things could get any better.

There are some troubling signs, though. Julianna is weaker — this is undeniable. She got over her cold largely unscathed, but she needs more respiratory support now. Her scoliosis is a lot worse. It’s also been almost one year since she was able to use her wheelchair. The ridiculously expensive and heavy power chair (in “pop star pink”) is taking up space, but it’s hard to give away. Doing this is an acknowledgement that her relentless and evil disease continues to rob Julianna – and us.

Summer 2013. Julianna loved her wheelchair, and she was a good driver. She liked to go FAST – I smile thinking about how she always “forgot” to change it to the (slower) indoor settings after being outside.

I’m not sure what to expect from 2016. To face it, I need to remember the hard-learned lessons of a past that I usually want to forget. (I wanted to elaborate more on these points — this was supposed to be a meatier post – but the words aren’t flowing today. )

1) Control is an illusion. God is real.

I can’t fix Julianna – my best isn’t good enough for that. I have to trust God.

2) I can’t see the full picture.

My mom/doctor instincts were correct, and J has an awful neuromuscular disease. But there has been beauty and grace in this life, and it has sustained us. I could not have imagined it because my vision is limited. I’m only human.

3) Enjoy the here and now. 

This is all we have, and it is good.

 

J gets a new doll.

J: She is ravishing!

M: Julianna, where did you learn that word? Do you know what it means?

J: It means super beautiful.

A triple ponytail (J’s idea)

  
 

 This. 

Alex makes Julianna happier than anyone else, and he’s been doing a lot of it lately. In past winters, we didn’t allow this because he always seemed to have a cold. This winter, he’s been healthy and affectionate.

M: Alex, it’s so great that you’ve been snuggling with Julianna. She loves it.

A: Do you like seeing it?

M: Yes

A: (like he’s admitting a state secret) I like it too. She’s not an ordinary girl.

M: Why?

A: Because she has CMT — and she’s my SISTER!