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.

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.

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.

June 2015, Capturing Grace Photography

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.

Drawing by Steve

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.

Dec 2015: photo by Capturing Grace Photography

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.

Nov 2014 – We took J to a lake. It was one of just a few outings over the last year.

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!

My husband of nearly ten years is all action and very little talk. This week, he had a lot to say, and it’s all important. 

This blog entry is a message that Steve posted on Facebook. 

June 2015. Photo by Aubrie LeGault, Capturing Grace Photography

First off, a heartfelt thanks and appreciation goes out to all of the incredible support we’ve received from friends, family, and perfect strangers. Now, to the reasons I’m breaking my usual silence:
Never, ever, in my life did I ever think I would be giving an interview to a CNN reporter. Let alone doing a follow-up taped interview to air very soon. Crazy…
What I have learned is that, while I do not find the general tenor of the media to be impartial, you really have to examine and judge the individual doing the reporting. So, when this individual, a medical correspondent, reached out to us, my wife Michelle (neurologist) examined her stories in detail. She struck us a person who does great research, writes well, and displays an unbiased presentation. My hats off to Elizabeth Cohen and her respectful treatment of my family’s journey. She started working with us in July, and our story just came out last week.

I realize that you can open quite a can of worms when you let someone into your home with a camera and bear your soul, pain, and suffering. I trust Elizabeth, even if she may not get final say on what is shown to the world.
For the record, I think my wife Michelle did awesome in the interview. I can’t say the same for myself. Let’s just say that my training to act calm and collected in the heat of battle (which requires disconnecting from my thoughts and emotions) was useless when I started talking about our daughter.
These interviews represent to us an opportunity to reach out to other families who are suffering through their loss, or staring at the imminent danger of losing their loved one; and of putting our trust and faith in God, just like we have with our daughter.

This is what we have learned:  this is our “Fact Sheet”:
1) You are not alone. Well before this story broke, I was surprised to learn how many people have gone through a similar situation.
2) Life really sucks sometimes.
3) It hurts, a lot.
4) Love is powerful, and rises above the pain in ways you may not even grasp yet.
5) Only you can make the right choice for your child…
…but if you have questions, don’t be afraid to ask, and keep asking until you get a straight answer. What does this procedure entail, what are the risks? Just because something can be done doesn’t mean it should be done. Care conferences help. In the end though, the choice is yours. And sometimes you don’t have the luxury of time to explore all the options to the depth you need to. (Reference #2, #3, but most importantly #4. )
6) Only you can know the true depth and complexity of the issues at hand.
7) Make a moment (We learned this from our grief counselor. She has been working — playing, really — with J for the last several months.)

I want to expand on this last bullet. Making a moment means stepping away from the battle (to keep that person from dying) and simply enjoying something together. It is creating a moment in time that you can cherish, regardless of the outcome. Why? Love is Powerful.
Cliché but oh so true: stop and smell the roses. Hold their hand. Play a game. Read them a story. Make an art project. Go for a drive. It’s horribly easy to get so caught up in the daily battle, but if you never stop fighting and struggling, you may miss the beautiful person you are fighting for. And before you realize it, there are no more opportunities to be had.
I’m not saying this is easy. It’s been an entire year since we changed our battle plan against Julianna’s condition. We did it because we realized that the harder we tried to fix her through our own actions, the worse off we all were. Every day, I still have to remind myself to stop being so focused on basic care plan stuff and to make time for fun things that she enjoys. When she wants to look out the window at the city lights, or deer wandering through, or the stars, or the moon; I have to squash the task master in me, remind myself that a few minutes diverted is okay, and start reconfiguring her equipment for a trip to the window.
8) Lastly, if you really can’t break away from the routine, then try having some fun along the way. Try singing a favorite song while you work. Something different. Special.

——
If you’ve read this far, here’s a bit more to chew on.
When you’re stuck in basic care survival mode, you’re not really living, and it is draining. It may seem relatively easy at first, but it wears on you. You eventually cling to it. You don’t want to stray off the path lest that be the tipping point where you lose your loved one. Then you turn around and hate yourself for it. Or maybe hate someone else for not helping you prevent this situation. This is a horrible position to be in, but God forgives you/them. Many people have done far worse things and been forgiven those sins.
You have essentially been living not to die, instead of just living (living to love). It’s similar to a basketball team which plays not to lose, instead of playing to win, or even playing for the love of the game. They usually lose and then wonder what just happened. So, instead of playing not to lose, instead of staying locked in the battle of the present, take a moment to take a deep breath, step back from the situation, and accept that sometimes the deck is stacked against you. Maybe you’re not really in control like you thought you were. Maybe it’s time to start blowing bubbles in your drink, make a replica of the Parthenon out of your chips, and build a house of cards (which is what you had already established in life) so that you can blow it over with a simple breath. Take a moment. Show your love, not through some menial but necessary task, but something silly, or different, or tender, or recreate a moment you know they cherished before through word or picture or pantomine.
Make a moment.

During this moment, Julianna danced with her dad while Homie looked on. J is too legit to quit!!