Hope — and the CMTRF

This week was the official launch of the CMT Research Foundation (CMTRF).  Its single focus is to deliver treatment for this disease, and I have agreed to serve on its Board of Directors.

The decision to join was easy, because the founders of the CMTRF, Susan Ruediger and Pat Livney, share my vision: find effective treatment now.  They are passionate but also pragmatic. They know that it will take a lot of money, plus collaboration between the worlds of research, biotechnology and pharmaceuticals. And I think that they have the resources and drive to get it done.

My motivation for joining is professional and, of course, deeply personal.

As a neurologist, I know that CMT is really not that rare, and it’s becoming less and less mysterious. Breakthroughs are coming through for other neurological conditions – so why can’t it happen for CMT?

Take multiple sclerosis (MS), for example. It affects about the same number of people as CMT (2.3 million patients worldwide vs. 2.6 million for CMT). It was also discovered around the same time– and by the same person. (Dr. Charcot, in the 1860’s).

When I finished neurology residency ten years ago, we had three disease modifying medications for MS. Today, there are ten. With CMT, it’s zero and zero.

The progress made with MS treatment is wonderful, and it is inspiring. We need the same for CMT, and in this era of genomic medicine, there has never been a more promising time —  but we need the research and we need the studies.

On the personal side, it’s about hope.

I hoped for many things as Julianna’s mom, but I couldn’t hope for a cure. We were too many steps behind on the science, and I knew that ours wasn’t going to be that kind of story.

Today, my hope is for other families affected by CMT, now and in the future: may your story be very different from mine.

We must accept finite disappointment, but never lose infinite hope.

–Martin Luther King, Jr.

 

Moon-161-2

photo by Aubrie LeGault

Eight

On the day she would have been eight, I wonder if It was all a dream.

Did I really have two babies and lose one?

 

img_4637

The beginning:  Julianna, 2 wks. Photo by Stacy Newlin Nyikos.

 

Surely, it’s not possible, not when we wanted her so much. It didn’t really happen, right?

In my head, it’s simple. I was there for her beginning and for her end. She was here, then she was not – and I was there for all of it. It was my privilege, but also the source of my greatest pain.

 

IMG_3062

She was herself until the very end. On her last day, she received a unicorn and deemed it awkward…

 

My heart knows it too, but in a way that’s not easily expressed with words. Imagine the most oppressive blanket. It’s heavy enough to constrict your breath, but its weight falls just shy of crushing the life that’s left in you. It’s a continuous stream of blazing hot tears (sometimes a trickle, sometimes a torrent) or the searing pain of an open, angry wound.

Oppressive heaviness and exquisite pain: this is how my heart knows she is gone. It’s too much, and that is the source of my disbelief. When it’s this painful, a little disassociation is not a bad thing.

My heart knows something else too: if someone is a part of you, they never really leave. She’s in vivid pink sunsets and in the stories her brother tells, the ones that make us remember and laugh. She is behind every colorful item of clothing and sparkling accessory that I own. I think of her every time I see a baby, because nobody has ever loved them more. She’s in flowers, on mountaintops, and part of everything beautiful, bright and lovely — because she was everything beautiful, bright and lovely.

 

This slideshow requires JavaScript.

 

So, on the day she would have been eight, I am blessed and bereft. I am thankful, but so sad.  I celebrate and I mourn, because her presence and absence are undeniable.

 

img_4656

Mt. Hood is the best part of the landscape here, and is visible on most days. Earlier this year, we put some of Julianna’s ashes on “Mountain Hood”

 

A Good Day

She’ll be part of every good day. No matter what.  — “The Book of Polly”

 

I’m not sure what you’re supposed to do on the anniversary of your child’s death. It’s not just another day, and it’s impossible to ignore.

It’s like a great, looming cloud. It feels unsettled sometimes, and you wait for the storm. There should be a massive storm, you think — something that breaks things open and shakes the earth.  Something vital was ripped from the deepest part of me, and it needs to be acknowledged.

But there is no Zeus, and I am not God. I don’t control the weather, and I couldn’t cure her disease: acknowledge that, and everything changes.

Sometimes, the answer doesn’t come in words. We lack the language, I think, of a higher truth. So we listen, and we wait. We plan, but we do not control. It’s maddening sometimes, but it’s the best we can do.

Last year, I beat back the awfulness by writing something big (if you haven’t seen my CNN piece on hospice, please check it out. It’s so dear to my heart) and by taking a big trip.  

On Julianna’s second heaven day, I tried not to overplan. My goal was to see something beautiful and to honor her.

This was my something beautiful:

img_4706

Even on a cloudy day, the Columbia Gorge is breathtaking. Julianna saw this river almost every day. (There are mermaids in there, you know. Her mermaid friend told her.)

 

I honored her by making something sparkly,

img_4695

When yarn unravels, it’s easy to become a tangled mess. I spent a lot of time working out the tangles to make this piece. Metaphors abound.

and by going to her favorite place

img_4718

slide3

Harper’s Playground, of course!

 

 

Along the way, there were a few surprises.

img_4702

A lovely ice cream shop with fresh strawberry ice cream and a patio adorned by purple flowers.

 

 

img_4725

A free postcard in an art store one block down from the lovely ice cream shop.

 

 

img_4726

My family remembered her…how she would have loved all those babies!

 

img_4711

Julianna’s dogwood tree turned even pinker. 

 

img_4722

Alex’s piano teacher picked these flowers out of her garden for us.

 

img_4723

J would have liked this…

 

A friend (J’s best friend, in fact) shared a memory — this is one of Julianna’s “secrets”

J — My legs can come off.

J’s BFF — Really? What do they do?

J — They’re wild!

And her eyes just sparkled…

 

And another shared a picture that I forgot existed.  Pure treasure.

img_4714.jpg

 

There were tears —  believe me, many of them. But it was a good day.

…And someday, there is going to be a good day that we are all part of. I’m not sure where that is, or what it looks like, but I believe in that day. — “The Book of Polly”

Two Years

The second year is harder than the first.

I heard it many times during that awful, surreal, first year, from many different members of the “club.” I didn’t believe it, though. How could I? How could it possibly get worse?

But it was. For me, year two without Julianna was most definitely worse.

The new normal has become…life. Our house is quiet. There’s no reason to walk through the girl’s section at Target. I make travel plans for a family of three.  I’m getting used to the fact that she’s gone – and I hate it. ( I recognize that it’s necessary  — “healthy”, perhaps — to accept reality, but I don’t have to like it. We live in a messed up world, and it hurts.)

I miss her most in the evenings. She needed someone at her bedside at all time. It wasn’t a burden: how I loved sitting there with the familiar lullabies playing in the background.  She was usually chatty and often profound: the best conversationalist I will ever know. She didn’t like to sleep (“God says Julianna is not tired.”), but no one can defy physiology, not indefinitely. So she’d drift off and I would watch…my lovely girl, finally at rest.

It was the best part of my day. I’d be exhausted but grateful. We had gotten another day and she’d be there again in the morning.

Two years ago, she drifted off for the final time. The mornings have come – 730 of them –without her.

On that first morning after she died, I wrote this:

Today, I just want the world to know that there was a girl named Julianna.

Such a strange, simple thought.  But it has stayed in my heart, even as words have failed me.

Julianna.

She made me better.

I miss her – so much.

She asked me about angels, and she walks among them now. No, she runs, and I think she even soars.

Her imagination was magical.  It was surpassed only by her heart.

She asked us to remember her always.

Remember her — please.

Screen Shot 2018-05-30 at 5.58.12 AM

j2

2013. Photo by Jennifer Rialtos

The Pillows

Hello again.

Thanks to those who reached out (and kept reaching out :), and I’m sorry for the silence.

I haven’t been able to write, and thought for a while about taking down the blog, because the idea of a fading or stale anything  associated with Julianna is not acceptable. But, then someone reached out and told me that Julianna’s story helped them during a low and desperate point. And then I heard it again from someone else…

I always thought that she needed to be shared, and her story is not over. I’m not sure how,  when, and via which medium…and on many days, I’m just overwhelmed by grief and deficient on hope.

But today — there is this.

Thank you for reading, and for remembering  Julianna.

Michelle

 

Grief fallacy #1: Time heals all wounds

It does nothing of the kind.

So far, all time has done is create distance from the happiest time of my life: eighteen months of love and life and celebration and intent, the kind of life you create when you’re not sure how long it will last.

 

_T7A6977

Magical. (notice all the pillows). photo by Charles Gullung

 

Distance creates numbness, but numbness is not the absence of pain. It just masks the pain, and in some ways, that’s worse. A certain amount of numbness is needed to live in a world that my daughter has already left, but too much is dangerous. I know the numbness has been too deep when pain feels like a relief.

It takes my breath away, this pain, and it hurts in ways I cannot describe. I feel it in the weariness of my bones and in the heaviness of my spirit.

And there’s sharpness too, exquisite and intense emptiness when I realize (in yet another way), that she is really gone.

The other day,  a bag of pillows  did me in.

Julianna had about a dozen pillows on her bed at any given time. They were all grandma-made, mostly pink and came in a variety of shapes and sizes. The little flat rectangles cushioned her heels when she sat up in her Tumbleform chair. The long cylinders cradled her back and kept her in a less aspiration-prone side position as she slept. The medium rectangles propped up her arms when sat up in bed (only possible with the hospital bed and a plethora of more pillows, of course).

They were so important, these bespoke little pillows. They supported her small body and prevented bed sores and propped up weak limbs so that she could wave her forearms about and delight and direct. They had to be just right, and it was something that only the experienced Julianna caregiver would know.  How lucky was I to be one of them!  It was my privilege to know these things and to do these things. I miss these little acts, the thousands of tasks that were a manifestation of love for the girl with the loveliest of hearts.

img_2326

A five pillow arrangement. Her right side always needed support. (“stupid scoliosis!!” –JYS)

 

Memories flooded and tears flowed freely at the sight of these pillows. I held them close and took in their scent, but I  smelled nothing but a faint staleness.

I’m not sure what I was expecting. The pillows have been hiding in a closet corner for almost two years, and Julianna never really smelled like anything to me. Maybe that’s what happens when someone is so deeply a part of you: I can’t smell her any more than I can smell myself.

 

img_4528

The pillows — and other things I can’t give away.

I sat in my puddle of tears and imagined what she would say…Put them in the washing machine…let’s PLAY!!..I smiled as I ugly-cried.

 It always comes back to love. It’s greater than the pain; it’s what remains.

 

img_4225

A Talk

This week, I partook in two of my greatest loves: public speaking and flying redeye.

(To quote Julianna, (who was quoting Calvin and Hobbes), sarcasm will get you nowhere.)

About that…Julianna came across this phrase while reading with Steve. She asked what it meant, and he explained. For some reason, he thought that she’d have an opportunity to use this phrase on me.

S: So, when mommy says something sarcastic, say it. Wait for the right time – there’s no rush. Wait for the perfect moment – it will be funnier that way, OK?

J: OK.

seconds pass.

J: Mom, come here!!

I run over.

J: Mom, SARCASM WILL GET YOU NOWHERE!!

(I digress, but it’s a funny memory. And I think that sarcasm is pretty great.)

 

Anyway, after work on Wednesday, I took a redeye to the east coast so that I could talk about Julianna.

The event was the Tenth Annual Pediatric Bioethics Conference at Wolfson Children’s Hospital in Jacksonville.

This was the title of my talk.

img_4315.jpg

 

I came prepared.

img_4312-1

 

And this was my name tag.

img_4313-1

 

I was asked to share our story and talk about how we let Julianna participate in our medical decision making.

I had three main points.

Screen Shot 2017-11-06 at 6.42.25 AM.png

Listen

One of the reasons I never wanted to go into pediatric anything is because I thought that babies and young kids couldn’t tell you what’s wrong. Adults, I thought, were much easier to figure out.

I was an idiot.

(The other reason I never liked pediatrics is because I don’t think that kids should get sick. That worked out well, didn’t it?)

Kids communicate with us all the time — sometimes verbally, often nonverbally.

Julianna was incredibly articulate. She told us — with words — that she did not want to go back to the hospital. But all along, she was communicating with us in other ways. Her body spoke to us in with its decline. Her eyes sparkled when we went into her magical world (“Let’s play!”) and filled with tears when we asked too much of her body. They expressed everything we needed to know.

Hospice

It’s not giving up.

It gave us more time, and better time.

No Judgment

Though most of the response to our story was positive, we were also criticized by some who felt that we didn’t make the right decisions, and by others who felt we gave Julianna too much input into those decisions.

We made our decisions out of love, and with the support of our medical team.

And unfortunately, we are not alone. For all the parents who have to make impossible decisions, please — no judgment.

 

And if there is:

Screen Shot 2017-10-14 at 11.41.08 AM.png

 

Remember:

Screen Shot 2017-11-06 at 6.41.57 AM

 

 

 

 

When Words Fail

Julianna was the most stoic person I’ve known. When she cried, you knew that something really hurt, and you wanted to fix it immediately.

The tears, you see, came from a little girl who had endured a lifetime full of tear-worthy events but shed very few. She simply did not have the time, not when there were stories to tell and fashion advice to dispense and people to love.

slide4

“Let me tell you something…” I am so grateful that she was able to see her words in print.

 

One thing that upset her greatly was when she was not understood.

As her CMT got stronger, her physical voice got weaker. Her voice got softer, and it had to fight against the noise of the BiPAP – which kept getting louder because her chest muscles needed more and more help moving air.

IMG_3050

A little microphone made all the difference. Along with the pink koala and happy manicure.

 

 

Through all this, her need to be understood never changed. She wanted us to understand her stories, agree with her opinions, and appreciate the nuances of language. She cherished words and insisted on proper enunciation (in one of her stories, there was an Ella, an Ellie and an EEliza. Different people, different names – and they had to be pronounced correctly.)

We did our best to understand her words and intent. When we failed, tears would spring from her beautiful eyes and she would shake her head in frustration.  This is how important it was for her to be understood.

 

IMG_2901

Sometimes she dictated, and we were just the scribe.

 

I share Julianna’s love for words, and I communicate best through written word. I can’t paint, sing, dance or emote. But I can put my feelings into words, and I need to put them into words – just like Julianna.

And now, words fail me.

I don’t know how many more ways I can say I miss her, or that it hurts, and that nothing feels the same.

I’ve used all the words I know, and they’ve expressed just a tiny portion of what I feel — and only the smallest, most palatable bit.

These days, I have no words – so I knit.

 

img_4303

 

img_4304

I have a lot of pink yarn

 

I learned via YouTube and I make a lot of mistakes – but life is far from perfect, and my knitting can match.

I knit with bright colors and soft yarn. I want her to approve of everything I make.

 

img_4286

She would like this. 

 

 

img_4299

This yarn is called “Unicorn Sprinkles.”

 

I knit to fill the quietness that is our “new normal.”

I knit so that I don’t have to think, and I knit to help me think.

I knit because life didn’t stop when my world did, and I resent that sometimes. Knitting slows it down.

I knit because I’m starting to forget things she said and how she was. I’m getting used to this life without feeding tubes and syringes – and I don’t like it. I don’t like the fact that I seem to be tolerating it so well.

If I knit until my hands are on fire, I feel something – and sometimes that’s better than nothing, even if it hurts.

img_4302

I have a lot to work out, apparently.

 

I knit because I miss her so much, but I seem to have used all my tears (they went away with my words, I think) and I can’t just go back to the person I was before she was here. Something has to be different — because she changed me fundamentally.

So in this new life, I knit.

(But I still need my words. I miss those too.)

Seven

She would have been seven today.

If only her nerves worked. (Not the metaphorical ones that are equated with bravery. Those, she had in abundance and they worked just fine.)

If only they were able to carry the signals from her remarkable brain down to her arms and to her legs. Where would she have gone? How hard would she have hugged?

If they had done their job, the muscles she needed to breathe would have been strong, and she would…not even notice.  Because breathing should be effortless. What would her laugh have sounded like?

If those stupid nerves worked, she would be here now. There would be cake, and it would be pink and she would be able to eat it with her own hands. She would swirl around in a flouncy dress and tear open presents (Or maybe not. She never let us rip up wrapping paper. She couldn’t hurt anything that she found beautiful, and to her, most things were beautiful.) She would run around, fueled by a little extra sugar and an abundance of love. It would be what every little girl should experience on the day she turns seven.

Instead, she is five. She will always be five, except for the times I think about her at two weeks (the most wonderful peach fuzz baldness), four months (COLIC!) or two years (sparkling eyes). She will always be my baby and my big little girl; never a teenager, never an adult.

 

IMG_3242

I have always loved bald babies.

IMG_5636

In between rounds of colic. She looks deceptively sweet here…

DSC00286

Trying on her flower girl dress. Her gold walker is in the background. She is almost two here, and I was sure that she’d be able to walk down the aisle if we worked hard enough. We did — and then worked hard to turn a wagon into a cloud-like carriage fit for a princess-to-be.

Maybe it’s a waste of time to think about the “if onlys.” It’s like reasoning with a thunderstorm or asking gravity to take a little break.

Julianna body was her Achille’s heel.  We propped it up, outsourced what we could to machines and coaxed it along, but it was a losing proposition. The problem was in the DNA, and it could not be fixed by twenty-first century science: my heartbreak was guaranteed.

And so was my joy, as long as I let her in – just a little bit.

For a while, I tried to resist. Her disease was a beast. We threw everything we could at it, but it only grew stronger. At my lowest point, the little girl lying in the bed — the one who couldn’t even scratch her own nose without my help — terrified me. She would leave me one day, and I would be broken.

And yet, I couldn’t help myself. She was so easy to love.  You just had to take her hand and say “yes” — yes to her brightness and her beautiful way of seeing the world. She made me better, in so many different ways.

Love was her superpower and it overcame my fear.

It hurts — in a way I cannot yet describe – not getting to seven (or ten or forty five).  But love is stronger than the pain and bigger than the emptiness — every second of every day.

Even on the day that she would have been seven.

 

20131215_173130

 

Time, Wounds — and Healing?

The day after Julianna died, we got rid of all her medical equipment. We were indebted to the machines (they kept her alive, after all), but when they were no longer needed, I wanted them gone. Stupid machines; stupid disease. I hated it all.

So the machines went away, and our house went quiet. I no longer heard the whirring of Julianna’s feeding pump (it ran thirteen hours a day) or the constant whoosh of her BiPAP.

Of course, the sound I missed most was Julianna’s voice, her constant observations and ridiculous wit.

Let me tell you something…

Ooh, what’s that?

Let’s play!

And so much more…

Last summer, the silence of our house was overwhelming. It shouted at me, cruelly: JULIANNA IS GONE. SHE IS REALLY GONE.

One year later, it feels different. It’s still too quiet, but it no longer taunts me. We gave her the most beautiful life that we could. She was surrounded by love. She is free. There is peace in this kind of silence.

I don’t think that time heals all wounds, not when the wound goes straight through the heart. But the pain is not quite so exquisite. It’s less likely to maim. Is this what one year does?

And then, I see it. It’s pure gold, a forgotten photo:

 

img_4216

Sept 2013

My heart is filled with joy, and then it is crushed.

This is her pre-K class photo, her only class photo.

She was able to sit up – by herself. This was before the scoliosis destroyed her spine.

Her fingers aren’t that curled. Why didn’t we paint them that day?

Her jumper was from Costco. It seemed like a good back-to-school outfit. I often had a hard time getting out of Costco without getting her another frilly skirt or princess dress. Her eyes lit up and she always said “I love it!” I still can’t go into that section. I miss that.

Her pink tennis shoes. Finding shoes that fit over her orthotics was work. They had to be big but light, and not completely ugly. I usually bought ten pairs of shoes and was lucky if one worked. When I found these, I bought the next size up –but she never got to use them. A few months after this picture was taken, her CMT took over, and we started our year from hell. She lost the ability to stand, then sit.

Her hair is in her signature bob. Could it be any cuter?

And that face, that smile, those eyes. Her mischievous, knowing, beautiful eyes. What was she thinking? What would she have become? What would she be doing now?

There are wounds that cannot be healed on this side of heaven. This will not go away.

*******

This weekend, we attended a bereavement retreat. The theme was the “Wild West of Grief.” Each family made a craft project that depicted their grief journey.

This was ours:

img_4218

It was a bit too much, but I think that she would have loved it.

*********

This is your last chance to get the 2017 “Love is a Superpower” t-shirt. All proceeds will go to CMTA. Orders need to be in by 31 July 2017. Adult and children’s sizes are available.

Click here: