Monday, April 20, 2015

Uncertain territory

It's troubling when the one thing that used to save you, free you even, is now the last thing that you want to do.  Because truly how can one write the painful truth of the every day when it's so heavy that the words fall short?  They are far too flat. Even the most exquisite and descriptive vocabulary couldn't explain the pain, fear, sadness and downright cruelty of what happens in the hours that make up our days.  I no longer need to try to understand it and I'm very close to not being able to fully explain it.  The details are all a blur.
She woke from the long sleep with one small smile.  Forced, but there.
And then she moved swiftly into 6 days of the most wretched, gut turning cry you have ever heard.  It seemed as if she was in pain, but typically she doesn't feel or respond to pain. Long ago I had to stop driving myself mad trying to figure out what was wrong and just treat the crying.  But nothing 'treats' the crying.  Nothing in the world stops this child from crying. 
Her brain causes the cry. 
And her brain and only her brain stops the cry. 
We did notice that she had thrush so we treated it.  A little numbing of the mouth and some good meds seemed to take it away after about 2 days.  But the crying went on.
I, too, cried every single one of those days. 
I have never seen her body so stiff or rigid.  I have never seen her muscles be so flexed, seem so strong.  And yet I have to look at her and reckon that on the other hand, I have never seen her look quite so weak.  Quite so very different and weak. 
Not long after she stopped taking her bottle, her swallowing ability decreased. The amount of saliva in her mouth has increased because she isn't able to adequately swallow it all.  There's a lot of drool and a lot of choking, all of which is new and scary.
Last week sometime it occurred to me that she has also fully stopped giving me any hunger cues.  So now I am feeding her strictly by schedule, along with reading what her body needs.  I'm keeping close watch on her gastric residuals as to not continue feeding her if at any time her stomach stops processing food. 

For several weeks my stomach has been in a 'knowing knot.' 
I have watched the physical signs of her decline and I have noted each of them closely in my heart.  I have yet to fully write it all.  I have yet to fully express the depth of it all or the immense and utter turmoil it causes my soul. 
All I know is that I do not want my baby to suffer. 
And this disease causes nothing but suffering.
Mabel herself brings so much joy and is purely peace.  But what I see when I look into her 6 day screaming eyes is a wicked disease running wild throughout her body. 
It infuriates me and there are times I think I might physically vomit at the very knowing.
But the knowing is what keeps me going.  The knowing of the disease, the extensive and detailed knowledge of this thing inside of her is what pushes me forward. 
I know what it is doing in her because I am an expert on this disease and Mabel.  I watch her breathe so frequently that I believe if she stopped and I was nowhere near, my heart would beat out differently in that moment. 
But I am near.  I am always near, almost breathing the very breath that she is breathing, I am so close.
  I know when she has an infection because I know the exact smell of every inch and every part of this little girl's body.  I know when her cry means that she has to poop because I am the one who assists her in doing so, each and every single time.  I know when she has thrush because I know the typical color of her tongue.  When something is abnormal, I am so attentive to this little girl that I will know it. 
And I will catch it because where batten disease rages in the realm of uncomfortable, I am in the business of making my baby comfortable.  Where it acts, I counteract.
I am not about earthly healing for Mabel.  I am not about fixing her.  I am even beyond treating the symptoms that are so untreatable. 
But I am about making sure that while there is breath in her lungs, she will feel rest and comfort and peace.  She will lay in my arms and look up at me with eyes that cannot see and know that she is loved beyond a doubt. 
I'm at the most helpless part of the journey. 
I can't articulate whether or not I think she's dying and yet I cannot fully say that I don't think so.  I just know that we have all noticed the change and we all feel a little uneasy, slightly unsteady with each day.  It all feels like unknown territory.
I am not scared or sad about the prospect of Mabel's death. 
It is everything leading up to her dying that is terrifying.  All of this that we're already living and walking through is what is most horrible about this disease. 
It takes away the abilities that she once had and by doing so it reminds you that it is also slowly taking her.  It reminds you that this brain inside of her is wasting away while she sleeps and is stealing from her everything that keeps her alive. 
At least scientifically and medically speaking that is what's happening.
But every day we depend on Love and we are reminded that God created us all perfectly in His image.  That He is the creator.  He alone numbers not only the hairs on our heads but also the days in our lives.  I look at my beautiful girl sleeping and I don't see the thief, batten disease.  I see the giver of life, Jesus.  I know that she is far too perfect for this world and that He has a plan to heal her fully in eternity.  So I just hold her, and in the quiet pray for mercy. 
Thankful for every day-good and bad, that she is here. 
She is our greatest gift. 
Being her mom is the most humbling and I would gladly do it for 100 lifetimes, if He would so choose to let me. 
I am so grateful.   

Tuesday, April 7, 2015

The long sleep.

It was the second day of the long sleep when I found myself curled up in my large bed, covered and weeping.  The first day of her sleeping caught me off guard; worried, anxious and sudden, my mind ran wild.  People came and went and all the while I said nothing.  I pretended, to them, as if she were taking a nap or I had already put her to bed for the night.  The reality, however, was that she had fallen asleep after a day of crying and she woke very little after that.
For 56 hours in fact.  
On day two of the sleep, the worry and anxiety turned to simple sadness. 
That this is our reality.  That she could in fact go to sleep and maybe never wake.  That I am going to have to bury my daughter.  That this disease is changing her and will take her from me. 
I repeated over and over that her actual death is not what worries or even saddens me.  It is everything leading up to that point that is heartbreaking and terrifying because it is so very unknown.  Not only to me, but for everyone. 
I called my sister on the first day of the sleep.
I explained my frustrations, not being able to articulate what I think the slumber meant and yet knowing somewhere inside of myself what it must.  What it probably does.
What it must mean?  What it probably does mean?
Well all of the symptoms and the signs point to chemical, and physical shut down of her body.  "If I were on the outside looking in at someone else's life while this was happening to their child with batten disease, I would form an opinion about it based on the symptoms," I told her. 
  She stopped eating by mouth 5 weeks ago, she has stopped giving me hunger cues altogether, and now she has fallen into a long sleep.  Her body is entirely still, something out of the ordinary.  And yet, I have no conclusive proof that her brain is, in fact, shutting her body down.
The only proof I have is in my gut. 
The wrenching and non-digestive type of feeling. 
But as a mother, the ONLY mother and caregiver to this child, it is infuriating to me that I cannot and maybe will not be able to know when death is near. 
Jeni expressed that she didn't want to go to work if we thought it was more serious than just sleeping.  I explained that we had to be able to live. We have lived so fully with her and I want  us all to carry that through.  I want us to live, breathe, and wholly feel it all.  I don't want us to sit for weeks at a time and hold vigil for her.  We have lived and allowed her to live and I want us all to continue to do just that.
So what does it mean that she came out of 12 hours of screaming only to go straight into a 56 hour period of sleep? 
I do not know.
For some children, this is part of a very noteworthy decline, one that leads to a somewhat peaceful death.  Not shocking, but just shocking enough because it is 'gradual and yet sudden,' as a good friend quoted to me this week. 
For other children, this is just another phase in this disease.  I remember at the conference last summer being amazed at the number of children who were sleeping and were so still, with little to no movements like Mabel has, and I was in shock to learn that many of the were, in fact, not sedated.  That was just a phase that they had moved into. 
And I'm not sure if we are there, or if Mabel's disease is moving in that direction.  All I know is that for 2 1/2 days straight I could barely get her to open her eyes.  She was not medicated.  She was simply sleeping.  She was peaceful.  She was calm.  I was able to feed her through her tube, keep chapstick on her lips, change her diaper and even put her on my chest, all without her waking. 
All of this for a child whose brain has never prompted her to sleep on her own.
So while the rest was welcome, and while seeing her peaceful was an amazing sight, indeed, it was incredibly worrisome and all new territory for all of us.  The entire house felt different; heavy.

So on day two, deep beneath my covers in the safety of my room, I let loose. 
I prayed and cried and prayed and cried.  Somewhere in the day I found strength between tears and I managed to follow my own mantra about continuing to live, and I was able to go for a run.  It was a 'grief run' for sure; the wind blowing and me knowing that it was, but not fully feeling it.  It was one of those days where the rest of the world looks so happy, without a care, free and light, and in my heart I wish I could have screamed to the mountains everything that was  happening in my beautiful, cozy little home with my beautiful, dying little girl. 
It's horrific really.
The whole thing is horrific. 
But on Easter Sunday, I was determined to at least get her out of the house, sleeping or not sleeping, to be with our family and to break up the heaviness of the situation.  She did wake up, though, not like her usual self.
And even today, she's just not.  She's awake, eyes open, but she is still. 
Calm.  Quiet.  Peaceful.
All beautiful things that I have long prayed for.  And yet my heart turns and aches at what it all may mean.  Each change is so difficult to manage, so frustrating to try and understand.  All I can do is lean on the ones who love me so greatly and the One who controls it all and trust that it will all be exactly the way that it is meant to be. 
Instead of trying so hard to figure it out, I'm trying so hard to not take her big, beautiful eyes for granted.  I'm trying to smell her a little deeper.  I'm trying to focus on being present with her while still living 'normally.'  I'm trying to accept it all and worship a God who is bigger than any and all of my feelings because He placed them in me and He created me for this purpose.
To be her mom. 
Life or death.  Here, now, then and always.
And it is the greatest gift I will ever receive. 
Today she is awake. 
So much is different and new, but she is awake and here with me. 
And for that very reason, I am thankful.

Monday, April 6, 2015

Easter 2015

Monday came.
Jesus left his tomb empty on Sunday, fulfilling His great promise and then, as expected, Monday fell upon the people.  I can't imagine what that Monday felt like; both hopeful and heavy, I can only assume.
Our Easter was beautiful. 
It was calm, light, colorful.
The days leading up to it were heavy and hard, scary and emotional. 
But Sunday came and our baby woke up and Jesus' promise was strong and steady in my heart.  It was refreshing to say the least.
The sun shined brightly over our family, giving us a day together that we will always remember. 
My sister started the day with a really great surprise engagement announcement.  I am so happy for her, and thankful the promise of all things new.  The hope of what our future holds is encouraging and uplifting when the rest of me feels like life will always be sprinkled with some sort of sadness.  And it might but there's beauty there too. 
Easter has always been one of my favorite holidays; both for it's spiritual significance and for it's very refreshing energy.  On Easter morning, when you wake up, things just feel differently.  The earth has made the last final turn from winter to spring and around that last little corner are yellow daffodils, red cardinals, blue jays and a whole lot of extra light.  
This Easter was no different. 
After a winter that was long and taxing for all of us with the worry of what it may hold for Mabel and for our family, spring has sprung and we woke with a certain knowing.  Though, the few days prior to Easter were nothing short of challenging for our spirits.  I learned in the quiet of our home just how comforting and beautiful this family is to one another.  There is a gentleness that will guide us through whatever the days ahead may hold.

What used to be babies ascending down the hallway with binkies and blankies are now really big kids, who light up my life.  They are so smart and articulate, gentle and kind.  Their hearts are good.  This Easter was incredibly easy and fulfilling for all of us and I could truly see the happy on their faces.  

 Family is family.
Sometimes it isn't blood or shared last names.  Sometimes it's a group text with all the women in your life that have always and forever stood by your side.  Sometimes it's the people who have chose life with you over life elsewhere.  Sometimes it is all the people who share the same blood but haven't always gotten along, coming together because it's important.  It matters.
And yesterday our family did just that.  We loved on everyone in our lives in the ways we could and needed to.  It was a really great and special day.  I'm so thankful and I will always remember it. 
Happy Spring! 
Now, please let April showers bring so many May flowers. 

Friday, April 3, 2015

Good Friday.

As I do each Easter week, I quieted. 
I told my spirit, 'ponder.  sit.  listen.'
And we did, spirit and I, together.
I reflected on this, Good Friday. 
I thought much about the crucifixion.  The garden of gethsemane.  The knowing in His heart and the knowing of the Father.  The helplessness of a mother, of all those that loved him.  The weeping he did over his friend Lazarus and his dead body; the friend he loved and loved deeply.  I thought long and hard about the weeping.  The sorrow.  The story. 
The utter and crushing truth of what this very day held for my One True Love, Jesus.
The heaviness always overtakes me. 
The days leading up to Easter are brutal for my spirit in the most convicting and altering ways.  I purpose myself to walk through it in my mind, over and over. 
I think about it.  I cry over it.  I wrestle with it.
Blood and wire and lashing and tears and sweat and yelling and hatred and longing and love and brutality and heroics and sacrifice and betrayal and judgment and submission and anger and peace and purpose and surrender and heaviness and yielding and life ending.
For life beginning.
Life everlasting.
Live eternal.
For you.  And for me.
It swallows me whole.  The entire portrait of the one true God-man who stole my heart.  His life story is beautiful and tragic and victorious.  I often wonder if He questioned, like I do, his life and the ultimate totality of what it meant for this world. 
I wonder if Jesus, the human God, ever thought to himself that while yes, He would die for the salvation of humanity, if given the option, He maybe would have chosen differently. 
But then I see Him. 
A striking 33 years old, in the prime of his manhood.  And He chooses the cross. 
He could have chosen differently. 
And even if He couldn't have chosen a different path or a different outcome, He surely could have chosen a different way to handle it. 
But He didn't.
Instead, He splintered His own back, carrying a heavy, rugged, scraping tree so that you and I could live free in a world full or torturous, horrendous, unspeakable sin.
And experience grace.  And truth.  And hope.  And life. 
They split open His body until the blood and the bones matched the pavement beneath Him.  Until everything inside was outside. He was negated to less than a shell of a man. 
He was humiliated, brutalized and tortured beyond recognition. 
His mother was watching. 
And on this day she stood beneath her child as they hoisted from the ground to the sky, her son on a tree above a crowd that was taunting and defiling Him in every unimaginable way. 
It is horrific. 
It is true.
And it humbles me to tears. 
A mother's love.  A child's sacrifice.  A crowd full of people whose spirits were wrecked; some holy-wrecked and some blind to the holy.  It troubles me and it's conflicting. 
But ultimately, it's a beautiful glimpse into the heart of our Savior.  It's a beautiful reminder that on a very typical day for the rest of the world, one man [the true God] gave His LIFE.
He gave it up.  And He did so at the promise that we would be set free. 
So that when I woke up on this Good Friday morning and walked into the room where my baby was seizing in her sleep, I didn't have to be overtaken by fear or worry.  He did it so that I could rest in the knowing that if she meets Him today, it will be forever. 
And I will join them in a Heavenly place one day. 
So that I could have hope of the restoration of her body, and her spirit with a Father who adores her.  So that this life on earth could pale in comparison to the eternal place of rest she will find in Heaven. 
And so that, in so many ways, I could long for that for her.
And for me.
And for you.

So that we could find total and full peace in life after this world. 
In a Savior's promise and sacrifice.
In a God who gave it all.
Who felt it all.
Who was, in reflection, so much like you and I.
The man who was God.
Who was everything.
Who is.
And is to come.
Today, I think about His dying. 
And in just a few I will think and reflect and be honored at his rising.
His love is so much more than enough.
Happy Good Friday, friends.
May you feel Him around you today. 

Thursday, April 2, 2015

home, at last.

"What is the goal?
A house that is like the life that goes with it, a house that gives us beauty as we understand it-and beauty of a nobler kind that we may grow to understand." -Elsie De Wolfe
One year ago, we were one year past the time when our family shifted and changed.  In the cold of winter Nora expressed the need to move from our big white house where we were once a family to a place where her memories would be new.  I heard her.  I watched her closely and decided that this little girl was onto something.  We needed a fresh start; a foundation where we could build our strength back up and therefore our lives. 
One year ago the kids and I lived moved into a quaint 3 bedroom house that was just the right size for us.  We had neighbors, the best front porch, a large yard, and a really great open field where they could run and play.  The move was hard initially.  When we left the big house, especially for the kids, it felt like we left an entire life behind.  And essentially we did.  We left the notion of what once was in that home; airy, cold and bitterly empty.  But we didn't look back either. 
I tried really hard to make the transition good for the kids.  And as the days passed, we watched as they got better.  Emotionally they just got better.  There were still really hard moments.  Really tough days.  There were crying breakdowns and fits of anger like I've never seen.  There were hard truths to talk about and really honest conversations about life that I wish never had to be had with my children, only 7 and 8 years old.  It was excruciating to walk through those days with them.  But we did.  We walked through it, together.
And we did what we set out to do.  We started fresh, building strength from a foundation of God, love, honesty, trust, and family.  We answered their questions truthfully.  We spent every day with them, never leaving and assuring them that we wouldn't.  We talked, talked and talked some more.  We hugged, danced, celebrated holidays, ate family dinner, enjoyed making new friends, tested our limits and really opened ourselves up to the possibility of a life that was far more grand that we ever could have dreamed.
A few weeks ago, the dreams that I once dreamed as a little girl came true. 
It's not at all how I imagined it would be with a cookie cutter family, shared last names, Sunday morning church, healthy children, and a home that was our home from the start of it all. 
Rather, God turned what could have been very broken and very imperfect into something very, incredibly, oh so extraordinarily good. 
As He promised He would.

You see, two years ago when our world was rocked on a cold February morning, I set in my mind to go on.  I set in my mind to trust God with whatever it is He was going to do with the mess I was in.  I quite literally remember looking in the mirror one day and saying, "I'm done.  It's all up to you.  Whatever it is that you're going to do, just do it.  I'm all in."
I was. 
And here I am. 
Two years later.  
A few weeks ago the kids, Chris and I moved into a home that we hope will be ours until these kids are grown.  It sits on the outside of our little town, on top of the most perfect hill and rests in the center of 2 acres.  The sun sets directly behind our living room window where we just happen to have large sliding glass doors.  There hasn't been an evening since getting here that I haven't stood in awe.
Of this life.  That sun.  The beauty.  The bigness of this world and of His love. 

When I stop and really think about it all, I'm overwhelmed. 
Some of us bounce questions around to one another, asking things like,
"Was this God's plan all along or did He take what went bad and truly turn it around for good?" 
"Was this the ultimate end game?" 
"Was this what God intended all along and it just took life and choices and the mess to end up here?"
All I know is that out of the darkness, depth and ashes, God truly did form beauty.  It is still really difficult on most days but I can look at the faces of these children now and see that in it all, they are going to be ok.  Their foundation is sturdy and their hearts are pure. 
They are good.
This may not have been the original plan, but I have to believe in my heart of hearts that this was the plan God envisioned for us; that He wanted the goodness of this exact moment, this exact life to be our truth and our legacy. 

The last 4 weeks have brought amazing changes for our family as a whole; changes that will inevitably provide security, safety and stability for the kids.  In moving to a new home, I tried desperately to make it feel 'normal' for them right away as to help control what could otherwise feel very scary and overwhelming for them.  I also have this strong desire to make everything feel as normal as I can in their lives because they have so much going on that is so far from it.  

5 weeks ago Mabel had the biggest seizure that I've ever seen.  It lasted a very long time, what felt like an eternity.  Chris and I watched her and I felt the panic rise from my stomach into my throat.  There was nothing we could do except watch.  I began to sweat. 
We are wholly helpless.
She hasn't quite been the same since. 
She stopped drinking by mouth completely.  She's choking or more often than not, flat out refusing to swallow, some days even her own saliva.  I'm thankful for her tube but I'm just sick about it.  This is one change that I have always known that I wouldn't be ready for.  I could never have anticipated how hard it has been for me, more so because I know that any change of this magnitude is one that could mean so much more than just not drinking a bottle. 
She is far more lethargic than she's ever been, falling asleep at random times and some days not having enough energy to even jerk around like she once did.  Other days she still screams for 12 hours straight and seizes almost non stop.  The up and down from day to day is emotionally exhausting.  It's so sad and hard and unpredictable.  My mind and body are exhausted at all times.  And my heart? 
My heart is still beating but otherwise, it's hard to say.  I'm very sad and very scared.  Very worried and very stressed. But mostly sad.  

Strangely the sad only fills up part of my spirit.
I'm in no way taken over by it anymore.
Because truly, life is really unbelievably great.  Every day I wake up and wonder how anyone gets this lucky. 
Even on our hardest, saddest, most overwhelming days I can't help but look around and feel blown away by joy and love.  

The big kids are doing great in school.  Nora's doing speech and is in therapy, which she loves.  Braden had a couple of appointments this month as well, one ending with two teeth having to be pulled, another telling us he needs his tonsils taken out and another that will bring about a whole lot of relief for him in general.  Their grades are above average and they seem to be making really great friends.  Every single day I am in awe of how really great they are.  They handle life with such grace, such poise, such dignity.  I pray that these are traits that they continue to exude throughout life.  They are honest, kind, compassionate, empathetic and very intuitive kids.  Those things are incredible gifts to possess!
All in all, the view from here is remarkable. 
I can see things exactly the way they are meant to be seen. 
There is peace, hope, joy, family and love. 
So much of it.

Our days are, as usual, a perfect blend of calm and chaotic.  There is just enough of a balance that it all feels too good to be true.  I feel incredibly lucky, thankful and happy. 
I've never known the kind of joy that my heart knows right now, all of us together in a place that we not only call home but that feels like a home should truly feel.