Friday, August 29, 2014

Hard day

Two children with Batten Disease died in the past 24 hours. 
At least two more (that I know of) are actively dying as I type this post.
Several others are dealing with hallucinations, terrible fear and anxiety, and hours of non stop seizures.
 
Today I climbed out of bed long enough to get the big kids ready and off to school, feed Mabel and scroll through facebook.  And then I took my girl and went back to bed. 
 
We didn't sleep, of course.  Once she's up for the day she is up for the day.  But I covered up and I held her close. 
 
On days like today the anguish that I feel is all consuming.  It's debilitating.  It's heart breaking and it compresses me.  On days like today when I am reminded of the sadness that is to come, of the very real loss that others are feeling in the exact moment that I'm living in-
I can hardly move. 
 
I've done my best to survive and even live with some valiance since Mabel's diagnosis.  Life has tried hard to stop me from doing that or to at least slow me down.  I have pushed forward, and charged through so many things that have led me to where I am today. 
 
But today, and days like today, I feel helpless.
My mouth is dry. My eyes are tired.  My cheeks are wet with hot tears.  I can't pay bills, I can't make phone calls, I don't want to return texts.  I don't want to shower or get dressed or function 'normally.'  
 
I just want to lay right here and hold my baby. 
Nothing, and I mean NOTHING seems important outside of these 4 walls today.  Nothing even compares to the amount of deep empathy and sadness I feel so far inside of my gut. 
---
For the past two nights she has cried and cried.  She started in again this morning but rather than my usual frustration that I feel, not knowing how to help her or how to make the noise stop-I literally got on my knees and thanked God for the noise that she is making.  For the breath that she is breathing.
 
I am at peace with Mabel's life and in turn, her inevitable early death.
But that doesn't mean that I don't hate this disease and that I don't long for as much time as I can possibly have with her.  I don't want her to suffer and so there are days that I pray for mercy.  I beg for her to not have to endure this disease for years to come and I beg that she would skip right over the horrifying changes that are probably to come for her. 
But on the days when she is 'good,' smiling, responding, even screaming-I am thankful.  Thankful for the time that she is here and the ability she has to do those very simple things. 
---
The decisions that batten families face at the end of this disease are literally too difficult to even comprehend, let alone write about.  They are painful and excruciating.  The days that lead up to a child's death after battling batten disease are torturous.  Some die peacefully, and beautifully.  Some suffer right up until their last breath. 
 
For the families, and especially the parents and caregivers there is a sense of relief after their loved one has passed away.  But with that sense of relief comes this intense amount of guilt for feeling that way.  It's all an awful cycle that doesn't end at death.  As I've been told numerous times, Batten disease takes everything from these families; their loved one, and so much more.
---
Please pray for our batten community today and always. 
I think what a lot of people forget is that just because I answer you that "Mabel is doing good," when you ask...it doesn't mean that it's all good.  The pain never escapes me.  What we feel for the other families that we love and are walking this journey is an unspoken amount of deep empathy; painful even.  They need our support and our prayers.
 
Wrapping you up in them today, friends, with so much love.
 
 
 
 
 
 

Tuesday, August 26, 2014

Reminders.

With the big kids starting school this week, I found myself at the kitchen table, laptop open and writing the usual "about our family" emails that I have sent to teachers, counselors, or basically anyone who enters their lives who may just need to understand Mabel and our situation better. 
 
I decided to do this when they first began school last year because I think it's essential that the people who see them on a daily basis understand their emotions better based on what could be happening at home.  Last year I enrolled the kids in school just 6 months after their dad left and after being homeschooled each year prior.  Mabel had just received a diagnosis one year earlier so there was a lot of information that contributed (and still contributes) to how my children behave, and respond on any given day. 
 
Most people would look at our family and understand without question that my children have a unique situation.  They have a sister at home on hospice with a terminal brain disease of an unknown gene.  All that we 'should' know about it's progression has gone out the window because we cannot find within her, the missing link that shows us what gene is responsible for batten disease in her body.  So my kids go to school every single day with the unspoken worry that they could, at any time, lose their baby sister.  My kids also carry several other heavy, much more superficial burdens on their tiny little shoulders.  It's incredibly sad and weighs so heavy on me every day that I don't think I could fully explain it. 
As I was typing my very personal, very detailed emails to the kids' new teachers, emotions sprung up in me that I have buried deep for a very long time.  Daily I am with Mabel.  Daily I take care of her basic needs like feeding her, changing her, cleaning off her spit, pulling her hands out of her mouth a trillion times so she doesn't bite through her skin, bathing her, giving meds, walking her, rocking her, brushing her hair and teeth, etc.  All of the caregiving comes second to the most important part of my days with her which is holding her, smelling her, talking to her, dancing with her, smiling at her and loving her. 
 
I am with her at all times. 
And so I have come to the conclusion that I don't always see the changes that she is making.  And if I do see them, I adapt to those changes so quickly and so efficiently now that I almost don't take the time to feel what those changes may mean for my little girl. 
But as I was writing things like "she cannot sit on her own, she is blind, she does not eat because she cannot swallow well without choking and even if she could she never learned to chew, she seizes almost constantly, her body moves without rest, she requires care at all times, her brain is shrinking and essentially she is a 4 year old infant.  Oh yeah, and she could die at any time..."
 
As I'm writing these things, it hits me once again; my friend grief.
knock knock knocking.
'This is my baby I'm writing about.  This is my child.  All of these things that I haven't gotten so good at explaining and even better at living with...they are affecting her.  My Mabel.
My baby.'
It's devastating.
And it's no less devastating just because I have learned to deal with how devastating it is.  It's heartbreaking, maddening, frustrating, sad, disgusting, heart wrenching, throw up at any time devastating. 
 
And yet here it is.
Here she is.
The truth. 
She is [still] actively dying. 
Her beauty is unmatched.  Her soul is perfect.  Her love is pure.
She smiles and giggles.  She responds to my voice.  She loves to be tickled on her right rib by my nose.  She loves baths and water of any kind, though Michigan water was cold and she made it clear that she didn't appreciate her toes being in it.  She loves her brother's noises and when her sister carries her.  She is our everything. 
Our everything. 
I can't explain it but grief can.
Grief can explain it all so very well.  He just creeps in and makes himself known as he has done from the very beginning.  I said hello and I opened the door. 
It was long over due for a visit, that's for sure. 
I'm so happy and life is so full and so good.  Every day I cannot believe how beautiful it has turned out to be.  Though I'm uncertain of my future days, aren't we all?  I have learned to embrace this unknown and very difficult walk with an ease.  Sometimes that makes me feel guilty and other times, like in the dark kitchen a few nights ago, I realize that it just makes me human.  God gave us a great ability to cope, survive and move forward.  But He also provided a comforter, the Holy Spirit, and in my case a visitor, Grief. 
 
It's all ok.  It's just all a little sad. 
She is still very much alive but she is still very very sick.  I forget sometimes and that's ok but this week I remembered, felt disgusting amounts of sadness about it and now I'm going to try and say goodbye to grief and sadness so that I can continue to live with her in total and full peace. 
For the teachers of my children and all the people in their lives who love them and are helping me keep them emotionally well, I love you and am grateful for you.  Truly. 
I cannot do this alone, but with your help I feel confident that they will be ok.  And that's a really good feeling...

Highlighting summer

I wanted to reminisce about summer before it is so far gone that I forget completely how wonderful it was.  The more time that goes on and the older I get I realize that it is by far, the little moments and the quick adventures that make up the story of our lives. 
 
At the beginning of the summer we embraced our new house and the neighborhood.  It was refreshing to start fresh and give the kids a place where they would create their very own memories.  Memories that I'm positive will last an entire lifetime. 

If I was asked to sum up the past couple of months in just a few words they would probably be:
love.  friends.  chaotic.  happy.
I feel like we accomplished everything that you should get done in one summer.  We had cookouts, campfires, s'mores, lemonde and vegetable stand, soccer, softball, late nights, late mornings, coffee on the porch, beach days, pool days, sunscreen, bike rides, carnivals, popsicles, sibling fights, sibling sleepovers, swimsuits, bare feet, stroller walks, sprinklers, trampolines, sunglasses, friends on friends on friends and so much more!


This summer we have made great strides forward as a family, embracing our life for the exact moment we are in.  Braden is the same as he's always been-steady, funny, fit-throwin and loud.  Nora is not at all the same as she once was, and though that saddens me, I have seen this little girl mature into a very bright, special young girl.  She is closed off and yet learning to be open.  She is scared of being hurt, of hurting others, of giving and receiving love, but she is incredibly wise and resilient.  I am so proud of her.  Mabel's summer has been beautiful and enjoyable.  A true gift from God.  














I have such sweet summer memories.  I have such a full heart. 
It's incredible how one minute in this life you can think that everything is over.  All the things you once imagined or dreamed for your heart, for your soul; shattered.  But then, suddenly, life resumes and your spirit breaks free from sadness.  You realize that you're standing right smack dab in the middle of a brilliant life.  The colors around you are rich, bright and vibrant and you can't help but realize that while we are here, on this earth, it is worth tasting.  It is worth loving.  It is worth the sorrow because as is the promise of a beautiful King, sorrow lasts only for a night.
JOY comes with the morning.
 
This is certainly my morning.
 
And though summer was incredible, I am bidding farewell to her now.  Waiting patiently on my favorite love--fall.  

Friday, August 22, 2014

School Days 2014

For the redheads, the summer has officially come to an end.  They both started school just 3 days ago and so far they are doing incredibly well!  They seem more brave, more independent, more sure of themselves than I ever dreamed they could.  These kids inspire me to be the same!
Summer has been brilliant. 
I have loved living in this hew home, watching the kids run through our neighborhood with their friends, carefree and child-like.  This is how it's supposed to be. 
 
The last two days have been emotional for me.  I look at just how much they've grown and my heart breaks.  Deep inside of them they have endured so much pain in the last 18 months.  Their hearts have been broken, they had to start school, meet new people, move to a new home, adjust to Mabel's needs and all the while they do it with grace.  They have struggled, absolutely, but I cannot fully explain how wise these two kids truly are.  They have a foundation of Jesus that lingers long after they speak.  They shine a light of God that is not only seen but felt.  They are deep reflections of the specific prayers that I diligently prayed over them and will continue to do. 
I am so proud of Nora and Braden. 
 
And then there's Mabel.
This summer has been a gift to me from her.  She has lit up my days with smiles, and giggles.  She smells delightful.  She has had a few seizures that look differently than they 'typical' but overall, she is doing well.  We started a new medication and though I have seen little difference in her movements or seizures, her irritability is less which amazing.  She seems generally comfortable and I feel like we have her at a really good place.  I've done a lot of thinking after the batten disease conference and have decided to make some calls to her geneticist to get some input on a few thoughts that I have.  But for the most part, I have just soaked in this girl as much as I can and it has been oh so good.  
Parenting can be so hard.
Many days throughout the summer I looked around and wondered how I would make it through.  Most days it's a thankless, endless job and it can be more than exhausting.  But at the end of the day when Nora asks me to lay with her and I rub her sweet red hair, or Braden wraps his arms around me to tell me that I smell good or when Mabel taps my face and lets out a sweet giggle-
the work fades away...
 
...I remember the purpose of it all.
I focus on the joy and the outcome of this beautiful life. 
I am raising humans.  I am responsible for them.  They are mine. 
And they are the greatest gifts that I could ever receive. 
So it's all, always worth it. 
 
Happy School Days!


Thursday, July 31, 2014

Happy 8th Birthday Nora!

Happy Birthday my girl!
Currently you are on a day date with Nanny.  She is sending me photos of you getting presents from Meme, visiting Uncle Mike's produce stand, getting your hair done by aunt Jeni, lunch together and shopping.  It's your kind of day and I'm so thankful you are being pampered! 
 
Speaking of being pampered, we celebrated your big day last week by having a huge birthday party.  There are so many things I want to say to you, baby, but I know that you already know.  Mostly I just want you to know how proud I am of you, how beautiful & brave you are, how independent, strong, and smart you are and how tender your heart is.  I am proud of you every single day; that you shine the 'light' of Jesus, as your name means.  You are an incredible gift and the only thing in the world I ever wished for was to be your mommy. 
8 years ago you made my wildest dreams come true!
 
Every year our lives are changing.  But when I look back and see you beside me I feel strong.  I pray every day that you look back one day and feel the same.  It's you and me, baby.  The same team always.  I will never leave you and I will always walk beside you in this life. 

I pray that you look through these photos and see joy, happiness and so much love.  Please know how special you are!  I love you more than life.
Happy Happy Birthday Nora Elaine!!!