And a very, Merry Christmas to You!

Hey guys!  Thought I'd pop in for a long overdue update.  :)

This season is a tough one for us as many of you know.

We celebrated Milla's birthday November 2nd (it would have been her 8th birthday) and celebrated her meeting Jesus on November 26th...it's been 2 years.  I cannot begin to tell you how much I miss her.  That loss seems to grow daily and sometimes that void is so profound that it literally takes my breath away.  

But as you have probably heard said, the pain doesn't ever go away, we just get better at living with the pain.

I still have moments when it all just seems like too much.  I can't bear to go on.  I have no more strength to carry on.  

But I do.  Not because I am a tower of strength and determination.  Not because I can do anything I put my mind to.  But it's because of Jesus.  I can't talk about Him enough, y'all.  He sustains me through the darkest times, through my weakness, through my whininess, through my anger, through my pain, through my failures.  He is the One holding me up - carrying me.  

I think of how Elle needs us to do everything for her - she cannot walk, she cannot stand, she cannot feed herself, she cannot swallow liquids, she cannot speak the words she wants to say, she cannot use the toilet, she can't even hold her head up at times.  We hold her and carry her and feed her and listen patiently to her and wipe her bottom and use our hands to hold up her head so she can breathe and see.

This is an amazing front-row view that I get to live out - how God loves and cares for me.  All praise to Him Who carries us but Who also came and humiliated Himself as a baby (who needed to be fed, wiped and held) so that we might have life.  

Also, how amazing is this...He understands just how Elle feels.

It's been a hard season for all of us.  Ann Carlyle has even seemed a bit melancholy at times which normally isn't even in her playbook.  We all miss Milla so much and we all remember the precious and the painful of November 2016.  Thank you for praying for us and our families during this time.  We are truly blessed through that.

Elle is doing okay - in fact, after a couple not-so-great months, she seems to have plateaued for a few weeks now.  Her seizures are pretty stable these days.  A month or so ago, she gave us quite a scare when she got some kind of respiratory infection. We were able to keep her out of the hospital due to her amazing palliative care team but she was on oxygen and a monitor and was just really, really sick.  But she has rebounded really well and while we see a little diminished strength, overall she is doing well.

A big, huge, titanic thing has happened!  We finally got her wheelchair/stroller, her bath lift aaaaannd...a wheelchair accessible vehicle!!!  So now we can put her in her wheelchair (inside where it is nice and dry and warm) and then just roll her outside and roll her up a ramp and into a Ford Explorer!  It's awesome.  We can even start the car up from inside the house and have it all warm when it's time to go.  And I have heated seats which is just a Christmas miracle for me.  Elle's wheelchair rides in-between and just behind the front seats so she basically feels like she is riding up front.  I'm able to reach her and see her well and she loves her new car.  :)  Ann Carlyle loves it too and is super excited to help lock the wheelchair down and help with all that.

Our backs are rejoicing.  And shoulders and necks and arms and everything.

The bath lift is great too - it is a chair that you can raise and lower down into the tub so it's muuuuch safer for the bather and the bathee.

Thank you for all the love and support as always!  I can't ever say enough thanks for the all the hundreds and hundreds of meals we have received over the last 4 ½  years and now I am trying to cook some meals myself during the week. It feels good to be able to cook for my family again.  It's only been a week and Elle is not the biggest fan as it takes me away from her, but I think this will give us some help with some dietary changes we've had to start making due to some health concerns.  I've never loved cooking but I have truly enjoyed accomplishing these dinners this week.  Thank you to those still providing meals a few days a week and for doing the chopping and prep work (which is often the most time-consuming part!) so I can speedily cook a meal.  It takes a (big) village and you guys are the most amazing village!!

Thank you also for those that help us with childcare!  These times are much-needed and much, much, much-appreciated.  I can't express how helpful this is to our marriage and our chore-list and our tired bodies and minds.  Thank you from the bottom-of-my-heart to all you warriors that give of your precious time to give time back to us.  

Special shout-out to my mom...who is one of the most self-less and gentlest and amazing women I have ever known.  I could and should send a special shout-out to her every week.  She regularly drives to Memphis from Birmingham to help with Elle.  Elle is tall and strong-as-an-ox 60lbs and my mom is 5'4".  My mom is a superstar.  And she not only cares for the physical well-being of my girls, but for their souls.  Through her life and through her words and actions, we are all witnesses to Jesus' light and love.  Oh and she loves on me and my soul too.  :) We may be 6 months away from Mother's Day but she deserves to be celebrated every day.  I love you, Momma and THANK YOU.

These 2 things, help with meals and help with Elle, may seem like smallish things but they are massive things.  And we have had consistent help with those things since 2014.  Just wow.  And thank you again.

Okay, I'm going to wrap this up :)

Love to you and yours.  May your Christmas be filled with true peace and true joy.  Emmanuel - God with us.

Dana

October is Here!

Does anyone else feel like September lasted foreverrrrr?  It was so busy but it just seemed to drag on and on.  I digress...

First of all, thank you all for prayers and meals and words and thoughts and flowers and things to put a smile on our faces.  We continue to feel lifted up spiritually and physically - my heart treasures this community God has given us, near and far, stranger and friend, so deeply.

Second of all, thank you for your patience with me in getting out some updates on what's going on.  I know, I know, no one expects me to write and I don't feel an unhealthy obligation but I always feel a need (a healthy one, I think) to keep everyone in the loop as much as possible because you give so much to us!  But the summer was busy and exhausting and honestly, I just was trying to be present in it half the time and just survive it the other half.  Writing out what was going on with life seemed too much of a mountain to climb.  It made me more tired to think about putting it in words.  But I am feeling like writing again so here we are.  :)

I'm thankful too for Frazer stepping in and taking a seemingly huge weight off my shoulders last month and catching everyone up on the summer.  He just randomly offered to write and I said yes, please!  He says he's no good at it, but he is!

So we are hanging in there getting through the days that seem so, so long.  Ann Carlyle's homework/studying issue was a bear the first month or so of school but she's doing better with it the last week so maaaybe that will cut down on the daily drama.  A mother can dream...

(Her school and teachers are wonderful and Ann Carlyle is wonderful and she has many wonderful traits but self-discipline is not on the top-10 of her strongest traits.)  

I was able to spend TWO WHOLE weekends back-to-back in September with dear, dear friends.  Those times with those incredible women that I am absolutely honored to call friends (including my sister) were beautiful and hysterical and precious and filled my cup to overflowing.  Thank you, girlies, for loving me and loving each other and loving Jesus in ways that challenge me and inspire me.  I think y'all are just freaking awesome.  Just so you know.

Also, I have started meeting weekly with a small group of women that are in my Sunday school class at church and these women also are filling my soul's tank.  

It's so hard to be intentional with my time with other women when I'm not distracted by a million other things but it's so important.  God can use those relationships (new and old) and those times to encourage, to challenge, to teach and to see the beauty, brokenness and laughter in life - so many things.  It's something that I have not made a priority much the last few years and I was feeling that void greatly.

So all that to say, that is one of the things that God is using to set me feet back on firm ground.  He has reminded me that we need others to point us to Jesus and to help us trudge through the mire, crying and laughing as we go.  That is how He created us to be - in community.  We were not meant to be alone.

As Frazer mentioned in his update, things are never stagnant and there can be extreme ups and downs, often all in the same day.  It's a battle not to live in fear everyday.  It's a battle not to constantly wonder, what's going to happen tomorrow, next week, next month, next year?  It's not for me to have all figured out and praise God it's not up to me!  It's actually very freeing to let go of (my sense of) control - but it goes against my personality so I am constantly having to re-center myself around God and not what I fear tomorrow will hold.  Why are we all here?  Why do we have to suffer?  I don't have all the answers but I do know we are here for God's purposes, for His glory, for the beautiful story He is telling even when we don't understand it and we don't know the end and sometimes it seems that all is lost.  But it is not all lost.  It's not.  All the brokenness and tears and fear and anxiety and suffering and death will someday be no more.

Jesus died so that we may have life with Him - not so that we may have life through our kids, jobs, things, experiences, friends, relationships, etc.

Elle had a relatively good week last week but her seizures got bad again Sunday.  She had her infusion yesterday but we aren't seeing any improvement with the seizures yet.  We increased one of her meds a few weeks ago and I was hoping we could sit tight at that dose for awhile but we may have to make another change.  She's a bit fussier these days and sometimes seems confused or just unable to communicate her needs.  So that's hard and exhausting.  She's 6.5yrs old and 55+lbs and has legs approximately 6 feet long.  So that's hard and exhausting too.  We should get some new equipment in the next few weeks to help with getting her in and out of the car and bathtub and things like that.

One more thing I want to mention, we've had some health scares and grief in our extended family the past few months so please pray for them when you pray for us.  There have been and continue to be some heavy loads to bear.  We love our family so, so much.  (And thank you, family, for loving us through thick and thin!!!!!)

Thank you all again for your continued prayers and support. I just can't say it enough!!

I'll leave you with the words from a song by Sandra McCracken that has meant a lot to me the past few years.  Jesus' love and peace be with you all! 

-Dana

(Here is a link to it if you want to listen to it...)

https://www.youtube.com/watch?v=0YO4fZUCQAw

Rock of Ages, when the day seems long 

From this labor and this heartache, I have come.
The skies will wear out, but You remain the same 
Rock of Ages, I praise Your name.

Rock of Ages, You have brought me near 
You have poured out Your life-blood, Your love, Your tears 
To make this stone heart come alive again 
Rock of Ages, forgive my sin. 

Rock of Ages. Rock of Ages. 
Bind Your children until the kingdom comes. 
Rock of Ages, Your will be done.

Rock of Ages, when in want or rest, 
My desperate need for such a Savior I confess.
Pull these idols out from my heart embrace. 
Rock of Ages, I need Your grace.

Rock of Ages, broken scorned for me. 
Who am I that You would die to make me free? 
To give me glory, You took the death and pain. 
Rock of Ages, my Offering. 

Rock of Ages. Rock of Ages. 
Bind Your children until the kingdom comes. 
Rock of Ages, Your will be done.

Rock of Ages, "It is done!" You cried. 
The curtain's torn and I see justice satisfied 
Now write Your mercy, on my heart and hands. 
Rock of ages, in faith I stand.

Rock of Ages, my great hope secure. 
Your promise holds just like an anchor to my soul 
Bind Your children with cords of love and grace. 
Rock of Ages, we give You praise.

Rock of Ages. Rock of Ages. 
Bind Your children until the kingdom comes. 
Rock of Ages, Your will be done.

Milla's House fundraiser with Holland and Birch jewelry this month!

Exciting news!!!

Milla’s House fundraiser with Holland and Birch is back for the month of September! Check out the new cuff, necklace and beaded bracelet - they are beautiful! Grab some jewelry for yourself and/or for a gift and know that you are also giving the precious gift of compassion and hope to a grieving family through Milla’s House.

hollandandbirch.com/collections/millas-house

Finally! An Update!!

Frazer is awesome and wrote a loooong overdue update for us... :) See below...

Wow, Dana has not provided a long update since April and only a couple brief ones since then!  I hate to disappoint, but this is not one of the famed updates from Dana.  I wanted to provide a brief update on the family and I hope to follow up with a few additional and more general thoughts in a day or two.

Since Miles 4 Milla in May, we have cruised through the summer and are rolling in the new school year.  We love you all and will follow with more details in the future.

Just kidding.

Seriously, the summer was good and bad, like much of life is for all of us, right?  I tell people that it seems like the dust has been kicked up in the air and never settles. 

-          We went to the BDSRA conference in July in Nashville and it was great to be there and see many of our friends in the Batten community.  We were thankful that Dana’s parents were able to join us.

-          We tried a beach trip, but the first day, Elle got a fever of 103 and high heart rate and generally was not well.  We decide to bail and came home the next day, which was the right call.  Traveling is so hard on Elle Belle and this trip really drove that point home.

-          Ann Carlyle did a camp for a week, her first away from home, and LOVED it!

-          Dana, Ann Carlyle and I got to go to Camp Good Grief for an afternoon in June and serve snow cones to the campers and volunteers, that was a lot of fun!

-          My work has been good and no big news to report there, which is a great thing.

-          AC has started 4^th grade at CMDS and is loving it so far…except for homework, which no one loves.

-          Dana is in a good place.  She would not tell you this and I am not saying things aren’t hard, but she really is coping and moving through our days, weeks and months with strength and peace.  There are a number of reasons, some known and some unknown, but at the end of the day, she is placing her foot down for the next step with boldness and the whole family benefits from this gift given to us!

-          We had a trip to Ohio to see Dr. Emily for a six month checkup.  No big news, great to have her team see Elle.

-          Elle Belle, well, that’s a hard one.  In some ways, she really is doing good and in other ways, we see the disease progressing.  The effect travel has on her or when she gets sick, reminds us how fragile she is.  Her seizures seem more frequent and lasting longer, so we are considering changing one of her meds later this fall (waiting on an FDA approval of a label).  The disease progresses, slowly, but not slow enough.  It’s amazing how much more she looks like Milla Willa every day, and sounds like her from time to time (see pics).  She is generally a happy kid as long as you are not bathing her or changing her clothes.  Night help has been better and more consistent and we are grateful for that.

We are cared for, provided for and loved well by so many and we thank you for that! 

Please consider joining us on September 30 for the Good Grief 5k at Memorial Park.  Here is the website: https://campgoodgrief5k.racesonline.com/home

Lastly, We have been blessed to have AC and Elle to receive horse therapy sessions from our wonderful friends at Southern Reigns through a scholarship they provided for us.  Elle and AC love the horses and the time, but we have recently come to the conclusion that Elle is just too weak in her core to continue doing this right now.  We are on break and maybe someday down the road, we can rejoin.  Southern Reigns is such a wonderful organization and horse therapy is such a blessing to those fortunate enough to have it.  May I ask that you consider supporting their “Hay for a Day” campaign?  Info is below and following that are a couple of Elle pics from our Ohio trip.

Fraze

CLICK HERE TO MAKE YOUR DONATION THROUGH PAYPAL TODAY.

You can also send your donation to Southern Reins: 

916 Billy Bryant Road

Collierville TN 38017

Your gift is 100% tax deductible, EIN number 47-4647784

For more information, CLICK HERE to visit our Hay for a Day page.

An Announcement from the Director of The Center for Good Grief, Angela Hamblen Kelly...

Today is a special day!

Thursday, July 26, 2018 marks the one year anniversary of the opening of Milla’s House and in one year, we have been busy. We have always known the need to provide grief counseling to our community was essential, and we have tried to keep up with the increasing demands from year to year. Expanding our reach to the midtown area has allowed us to serve more grieving individuals and families in our community.

Because of your support, Milla’s House has been able to provide over 2,000 individual grief sessions in this first year!

This is incredible work you helped us accomplish and we would not be able to do this work without you, our gracious friends and supporters. We sincerely thank you for supporting Milla’s House which serves countless individuals and families in our community.   

Ann Carlyle Gieselmann (pictured below: center, and her cousin Vance to the right)  attended Camp Good Grief last summer and had a wonderful time with new friends, learned a lot about feelings, emotions, and coping skills,  and worked really hard on her grief. This summer, she was thrilled to come back to Camp (alongside her parents, Dana and Frazer) to serve sno-cones to campers at our annual carnival afternoon. As a special surprise to Ann Carlyle, she was inducted as an honorary member to a special group of people called Team 1520. Not just anyone can be a member of this elite group – Team 1520 members are former Camp Good Grief Campers who come back to volunteer and serve at our camps. They are teenagers committed to working on their grief daily and when they are ready, have the unique ability to share their common experience with other children who have lost a special loved one. We are so proud of Ann Carlyle!

While Milla is no longer physically with us, her legacy impacts families in our community in big ways. In honor of the one year anniversary of Milla’s House, we encourage you to celebrate with a donut, share your favorite Milla story with someone, or share Milla’s House with someone in need. 

Most sincerely,

Angela Hamblen Kelly	&	Melissa Surles
Executive Director, Baptist Centers for Good Grief		Development Officer

THANK YOU!!!

On behalf of Milla's House and the Baptist Centers for Good Grief, I wanted to thank all of this year's registrants, sponsors and donors for a fantastic event at Wiseacre on May 19!  With your help and many more we raised just over $104,000 for Milla's House!  We had 56 sponsors, 73 donors and 490 registrants for the rides and party!  We distributed 543 T-shirts, ate a bunch of tacos from El Mero and around 55 pizzas from Dominos!

It is such an honor and privilege for Dana and I to see Milla's legacy through Milla's House and it means a whole lot to us to have so many support the work being done and joining us in honoring Milla's legacy.  We are humbled by the continued support of our community!

Thank you for joining us and we look forward another fantastic event in 2019!

Frazer & Dana

5 Days Post Surgery Update

I just wanted to send a quick word out that Elle is doing well!  She seems a little weaker in her core and neck strength the last few days and she's been fussy and restless a little more than usual.  But overall she is doing great and her incisions are healing well.

The surgeon had to shave Elle's head - a lot - in order to have a clean surgical field where he took the old port out and also on the other side of her head where he put the new port in.  After much brainstorming on how to fix her hair, I trimmed about 6+ inches from her super long hair and we are doing a lot of 'Milla buns' and headbands to cover up the incisions/scars/shaved head.

(For those of you who may not understand what 'Milla buns' are :) it's what we call pig-tail buns on the top of the head because I put Milla's hair in those buns much of her last 3 years as her hair was so curly and wild and that kept it out of her face.)

We are so thankful we went ahead and changed the port out with a new one...the old one had a sizable hole in it. It definitely would not have been good to have another infusion with the old one.

We are still on schedule to have Elle's 45th infusion tomorrow morning.  We don't know if we will see a difference in Elle' condition with the new port but anything is possible.

Also, we have a new night nurse starting this week!!  For now, we will still have no help on Saturday nights but 6 days a week is sooo much better than 3 days a week!  

One more big reminder!  Miles 4 Milla's House is THIS SATURDAY, the 19th at Wiseacre!!  Feel free to ride bikes, walk, run or just come for the party!  The family ride (I'll be walking) starts at 10:15 and the party starts at 11.  Rain or shine, it will be a blast with good people, good music, good beer and good food.  Please help us support the Memphis community by supporting Milla's House Center for Good Grief!!

Go here to sign up or donate:

www.miles4milla.org

Much love and THANKFULNESS for all your prayers and encouragements!

Dana

Quick Post-op Update

Elle’s surgery went well! We are in a room and she's sleeping right now.  She is running a little bit of a fever and having a lot of seizures but all in all, she is doing fine.

We will stay the night at LeBonheur and should get to go home tomorrow barring any complications.

Thank you for continually lifting us up!

PS - for those of you that didn't know, Elle's surgery was cancelled yesterday and scheduled for today because the hospital had some emergency cases come in.

Surgery Bumped

Hey everyone - I just wanted to let everyone know that due to an emergency that came up, Elle's surgery was bumped to tomorrow at 2:30.  

Please continue to pray for her and that she will have patience and understanding as much as she possibly can tomorrow!  And of course for safety and her doctors and nurses, etc.

Thank you!!

Dana

Surgery Next Week

Hey there, all!

Elle had her 44th infusion Tuesday and we ran into a glitch. Her port (it goes into her brain to deliver the enzymes) started to leak.  Thank God they figured out a way so that she got most of her enzyme infusion but we are going to have to replace the port.

So next Tuesday, she will be having surgery to take out the bad port and put in a new one on the opposite side of her brain.  We aren't sure why it stopped working - it should have lasted another couple of years.  They did a CT scan Tuesday just to make sure nothing had broken off or dislodged and the scan looked fine - so there's no emergency in needing to get the port out.  And there are no signs of infection.  Whew.

Her surgery is at 11:30 so please pray for fortitude for us as Elle can't eat or drink after midnight.  She usually eats 2 or 3 breakfasts between 1am and 7am so this is going to be very difficult for her as she can't understand why she won't be able eat or drink.  She is cognitively around a 2 or 3 year old level - with a 6 year old body - and the strong will and feistiness of a wild animal. :)

Tuesday, she will get a CT scan and then the surgery and then will stay in the hospital for 1 night.

Please also pray for good wound healing and healing in general and no infections in order to be able to get her next infusion on schedule (the following Tuesday).  Also, as you all know, any surgery for any person is not without risks, but for Batten kids, the stakes are a little higher because of their weakened immune systems and weakened muscles among other things.  There is always a nervousness with anesthesia and breathing tubes - having trouble breathing on her own after they remove the breathing tube.

Please continue to pray for a night nurse as we still don't have one for 4 nights/wk.

Thank you all so much for all the kindnesses and love and encouragement!!!  Much love right back!

dana

April

Long overdue update - but thank you for faithfully praying for us and loving us as always!  God is certainly sustaining us through you!

Not much has changed with Elle until really the last week or so.  We are still working to control her seizures.  We started another new medication about a month ago after trying 2 others that failed horribly.  We have to slowly increase (or decrease) the doses of these medications and currently we are still increasing the newest one.  I will say that for the first time in a long time, we may be seeing some consistent improvement in her seizure frequency and without the dreaded behavior problems that usually come along with seizure meds.  She is still having some hard and long drop seizures but we are seeing less of them!  And her mood has been mostly good!  She has been more giggly and happy and she is having a bit of an easier time with transitions.  AND slowly but surely, her falling-asleep patterns have gotten so much better!  Her overall sleep is still awful but she is not restless and thrashing and moaning for 45 minutes as she is trying to go to sleep.  She typically will fall asleep on me and I'll hold her for awhile and then put her in her bed.

So for now, we have seemed to solve the going-to-sleep problem...now if we could just get her to stay asleep...

The only good thing about her sleep pattern is that there is actually a bit of a pattern to it.  Milla never, ever had a pattern to her sleep (other than it was bad) and Elle hasn't either until the last couple of months.  So at least we pretty much know what to expect.  We still don't have a night nurse for 4 nights every week and that has been absolutely brutal.  Please continue to pray that the staffing company finds someone soon to fill this spot.  (On a positive note, the night nurse we have for the other 3 nights is absolutely wonderful - she is fun and full of energy and Elle loves her.  :) We pray that she will be able to stay with us for a long, long time.)

We are so thankful to finally be seeing these little bits of (good) change in Elle's condition as she has been on a downward spiral since mid-December.  Her little body has been suffering so much.

April brings lots of rain and lots of birthdays around here!  Ann Carlyle turned 9 earlier this month and Elle turned 6 last week!  Both had wonderful parties to celebrate their sweet lives and they both had a blast (even though Elle's party was rained out) - and lots and lots and lots of treats as the whole month has pretty much been a birthday celebration!  As so many have said before and I continue to see in my life, the days are long but the years are certainly short.

Note: I started this update this morning and then stepped away from it until late this afternoon.  And Elle's seizures were worse today again.  So we will see how she does tomorrow.  And we will be thankful for the few good days she had this week.  

So that's about all I have for now.  

Oh - please don't forget to sign up for the Miles for Milla's House Bike Race!  It is May 19th at 10am and you can even walk/run if you want to - that's what I'll be doing!  Go to http://miles4milla.org to sign up or donate.  (There's also information about Milla's House on the website.)

Much love to you all!  God's mercies never fail.  I read this quote from Joni Eareckson Tada recently...

"The weaker I am, the harder I must lean on God's grace; the harder I lean on Him, the stronger I discover Him to be, and the bolder my testimony to His grace."

God is good - on good seizure days and on bad seizure days.  God was good even when Milla died and He remains good as we continue to grieve.  God is good when we get to sleep and when we don't get to sleep.  He remembers that we are but dust and will hold us together despite our frailties.  Thank God my circumstances don't change Him.  

-Dana

Spring Break Update

Hi there to all of you!

I just wanted to send out a quick (hopefully) email while I have a second.  I mentioned some of this on instagram and facebook the other day but I know not all of you see those posts.

We are on spring break this week - which is in no way a 'break' for us.  It's hard to not have school for the girls, especially Elle.  She needs that routine and stimulation and socialization she gets from the classroom.  Anytime there is a break/holiday in the schedule, it is really, really hard.  We will get through it and it will be fine but it's going to be a long week.  I do have some sweet friends helping a bit here and there this week for which I am ridiculously grateful.  :)

Another reason for the oh-my-soul-how-are-we-going-to-get-through-the-week is because we are without a night nurse again for 3-4 nights/week.  So Wednesday through Sunday, Frazer and I will be getting about a couple of hours of sleep at night.  And having no relief during the day just compounds all of it.  The mental exhaustion.  The physical exhaustion.  The emotional exhaustion.  I am constantly on guard with Elle - for her physical safety - but also as I watch her seize so much and watch her have nerve pain/discomfort and as I fear the disease that is killing her brain cells, I can feel the effects that this constant stress is having on my body.  I mean, this stress has been a constant for the last 4+ years but there are times that it has been on overdrive and I feel that the last couple of months, that overdrive has been my almost constant companion.  

So Elle's seizures are still uncontrolled - we have been working on that but I will say this past week, for whatever reason, there has been a speed bump with those seizures.  We saw a slight decrease in the number of them but we are still not where we want her to be - which is in a (relatively) controlled state of seizures.  And today, the seizures have been really bad again.  Hundreds every day and even some while she sleeps.

The other thing we are having a hard, hard time with is her getting to sleep and staying asleep.  This has always been a problem, as most of you know, but it's been an extra-bad problem since the beginning of the year.  She writhes and twists and thrashes and tosses and turns and moans and cries when she is trying to get to sleep.  It's awful.  She is exhausted and has her eyes closed during all this movement and wants so much to go to sleep and her body just won't let her.  Sometimes, it seems as though she is just uncomfortable but other times, she is in pain.  We started a new med for her to take before naps and before bedtime to hopefully help with this. It was prescribed almost 2 weeks ago, but between insurance pre-certification and trying to find a drug store that had it in stock, we just got it Saturday evening.  The first night, we saw no improvement at all.  Last night, we saw a little improvement.  The jury is still out, but we are hopeful.  And with better sleep, maybe we will see improvement with her seizures as well.

Elle is still doing really well cognitively and is strong.  She is still pulling up to a standing position, bearing weight and scooting around on her hands and knees to get around.  She loses her balance or seizes and falls a good bit too, but we are thankful for the mobility that she has.  

Her vision is not great and her speech is not great.  She's having a harder time communicating what she needs or wants and that gets super frustrating for her and makes me super sad to not be able to understand her.  But how about this for crazy...she can still say 'rain, rain, go away'!  It doesn't sound perfect but for her to be able to string any number of words together is amazing.  (She's had a lot of practice lately with our weather.)  It's always fascinating to me the random things that hang on.  Milla had things like that too.  An ability will be pretty much all gone but then something within that ability that is relatively complex will still be hanging on that they are able to accomplish.  The brain is so crazy complex and amazing. 

We are truly fearfully and wonderfully made.  

Ann Carlyle is doing well.  She sees Elle's disease progression and she worries but she is talking about it and asking questions and processing it with us and that's what we want.  She is turning 9 in a few weeks on April 6th and she, of course, is ecstatic about that!  She's amazing and strong and beautiful and full of joy and quick to forgive and quick to laugh and has amazing empathy for others (including her parents!).  We are so, so proud of her!

As for me and Frazer, we are grieving and stressed and tired and anxious...in various stages and places but nonetheless, this season has been a hard one.  We cling to truths we know in our hearts, even when we don't feel them.  We are leaning into each other even when we don't feel like it.  We put one foot in front of the other even though it seems impossible to just do that simple thing sometimes.  We miss Milla so very much.  We grieve not only the brokenness we have in our little family but also the brokenness we see in our friends lives and in the world.  How do we do this without breaking ourselves?  Jesus.  That's it.   I have been paralyzed many times at the prospect of going through yet another day and having to deal with everything - the grief, the physical exhaustion, etc.  Except.  Except for my hope and absolute belief that Jesus is alive and that He is making all things new.  Except for my hope and absolute belief that all of this suffering is for a more wonderful purpose than I can possibly imagine.

I hate to send out updates that are so down-in-the-dumps but that is honestly where we are.  Please continue to pray for us and thank you for already doing that so much and for so long.  Thank you for encouraging hugs and words and hand-squeezes.  Thank you for helping out wherever is needed, no matter how small.  Thank you for just being willing to help out.  You are Jesus' hands and feet, serving Him by serving us, loving Him by loving us. 

Our love and sincerest thank you,

Dana
Oh and PS - Save the date!  The 2nd Annual Miles for Milla's House Bike Race is May 19th!

2018

Hello!

Well - it's been a bit since I've written - but kind of by design.  I decided I would check out for awhile during December.  Life has felt pretty heavy as lots of birthdays and holidays and anniversaries are all clustered together this time of year.  Milla's birthday was November 2nd, the 1-year anniversary of her death was November 26th, right after Thanksgiving and then Christmas, New Years, etc.  Even though technically this was our 2nd Christmas without Milla, it kind of felt like the first.  I remember almost nothing from December 2016.  Pretty much after Milla's funeral, my memory is as foggy as can be.  So, much of this December felt new and empty-ish and hard and dark with splashes of numbness thrown in there.  

I think Ann Carlyle was in over-drive trying to keep us all happy and excited and full of the Christmas spirit in December which is sweet and awesome but trying to keep up with her almost did me in.  The long break from school and routine does not bode well for our family.  And here we are with an extra long weekend due to the snow in Memphis closing the city down and running into the MLK Jr holiday.  And I just heard that Monday night might bring 1-3 more inches of snow.  Heaven help me if we are homebound for another day Tuesday.  Heaven help us all.  I'm barely keeping myself on the right side of sanity today.

My teetering mental status is mostly due to the events of this past week (aka mental, emotional and physical exhaustion and lack of sleep).  Frazer and I took Elle to Ohio Sunday for a checkup with our doctors and therapists up there.  We spent the entire day Monday at the hospital as Elle was getting checked out and tested all morning and then she had an EEG that afternoon.  Tuesday, we saw an eye doctor all morning and then flew back home after lunch.  It was good that we went - we got lots of good information - but it was exhausting (along with very little sleep) and it was like a fire-hydrant of people and information about Elle's current condition.  There were some things we expected and some things that we did not necessarily expect.  

In general, she is doing very well for her age (especially cognitively and with her gross motor strength) but her disease is still progressing.  The enzyme therapy has definitely slowed down her progression but it has not stopped the disease.  Also, Elle's seizures were very well controlled last summer but the frequency and severity of her seizures have been on the rise all during the fall.  Over the last few months, we have increased the dosage of her 2 seizure meds but that hasn't seemed to make much of a difference.  So we are now starting a 3rd seizure med.  It will most likely take a few weeks to know if this 3rd drug will help and we have to watch Elle closely to make sure this drug doesn't impede her other activities/quality of life, which it could.  

Besides the seizures, our biggest concern has been her frequent coughing/choking with food and water but most often with her saliva.  We already thicken her water to make it easier for her to swallow and she's done great with any kind of food and her own secretions until about mid-December.  There is a possibility that this is happening because of her seizures.  But there is also a possibility that her swallowing abilities are weakening.  Aspiration (of anything: saliva, food or water) is a big concern as that can lead to pneumonia.  We will have to wait and see if we can get her seizures better controlled, if that also helps this choking.  We will be doing a swallow study at some point as well to see if she is actually aspirating even small little bits into her lungs.  And hopefully, the doctors can give us recommendations/medication to help with that.

As I said, Elle's gross motor strength (the big muscles doing the big movements) is so much better than it would be without the enzyme therapy, but she is still weakening and her legs/feet continue to turn in and lose flexibility.  We have a few things in the arsenal that we will try out to help this issue as much as we can.  Right now, we are going to try night splints.  These splints are basically hard braces that go over her foot and all the way up to her knee.  I'm worried this will affect her sleep (which is already awful) and also, please pray that the splints work so that we won't have to do things like injections or muscle relaxers that can exacerbate other conditions and/or have to do serial casting again.  Serial casting is where both legs are in casts up to her knees for at least a month - we did this last May and it.was.awful.  We really, really do not want to have to do that again.

Ultimately, it is her brain that controls all of everything so as long as the brain is degenerating, her body is not going to work like it is supposed to.  We can only do so much to combat all the issues without fixing the root of things - her brain.  The enzymes are not fixing her brain, but they are slowing down the rate at which her brain cells are dying.  I'm going to be honest, it is haaaard to live in the limbo of hope and keeping Elle here with us longer and quality of life and experimenting with her brain and this disease.  For example: we have seen amazing cognitive preservation and even tiny improvements the last few months.  Typically, Elle would have greater dementia at this age (without the enzymes).  So while it is awesome to see her understand things and do things that defy her disease, it is not awesome to see her so frustrated and sad and angry at the things she sees other kids doing that she can't do...or at the things that she remembers doing, but can't do now.  She tries to get up and walk on her own (we haven't seen her do that in a long time) but then can't and she's sad and frustrated.  So while dementia is not something I wish for, I see a certain mercy in some of that dementia.  Does that make sense?  It's murky stuff sometimes.

We have also been struggling with her sleep disturbances.  She's always had issues with sleep (as do most Batten kids) but those issues reared their ugly heads in a new way this fall.  Elle was waking up multiple times every night with screaming fits that we could not sooth.  It was absolutely terrible.  We don't know if she was seizing or hallucinating or something else but she was clearly having some kind of neurological problem.  We increased her one of her meds a couple of times (it helps with sleeping and also seizures and neuro issues) and that seemed to do the trick with the screaming fits.  She is still waking up a lot throughout the night and/or super early for the day (like 3am-early) but we are thankful the fits have subsided.  

We do have regular night help (night nurses) but for the last couple of months, we have not had help on Saturday nights and for the last 3 weeks or so, we have had many nights that our nurses have been sick and unable to work (or we have been in Ohio with no help).  Frazer has taken the brunt of this, staying up with Elle most of these sleepless nights but we are both so exhausted.  This carries over into our days, of course, and no sleep = everything else becoming harder.  (Hence, the question of my mental health this weekend and my grinchiness over the snow.)

Another issue Elle is dealing with is her vision.  We know her vision will continue to deteriorate until she becomes completely blind and it seems that her vision has taken a bigger hit over the last few months and even more so the last week or so.  So far she has compensated pretty well but this is another reason for her frustrations.  She's been screaming/fussy much more these days, and I know the lack of vision does not help.  (Her eye doctor in Ohio confirmed this Tuesday as well.)  

Whew.  It's a lot.

I was reading today that sometimes 'our emotions aren't in sync with our beliefs about God, and it's okay to tell Him.'  I've felt that much of the time lately.  God is the source of my hope, courage and delight but in these moments, I feel fear and hopelessness (in this world) and sadness and sometimes I feel nothing at all.  It's just hard.

On another note, Ann Carlyle is doing really well.  She soaked up every last drop of the Christmas season and is enjoying all her many lego sets she got for Christmas.  We talk about Milla a lot and Ann Carlyle misses her greatly and sees/has questions about what's going on with Elle - but overall, she continues to 'work her grief' very well and we are thankful that she is so open about all her thoughts and feelings.  Ann Carlyle is a big ball of energy and joy and light.  Unless it's time for homework.  Then she is a black hole of pouty-ness and unhappiness.  Like ya do.

Frazer started a new job in December and that has been a better environment for him and therefore better for all of us.  He was able to take some time off between jobs which was also great for him.  That time-off correlated well with all the difficult things in November so he had a little more time to process things than he would have had had he been going to work everyday.

We are slowly getting back into our routine with school and therapy appointments and doctor appointments and enzyme infusions (if we could just get through these snow days!).  Our infusion days are changing to Tuesdays instead of Thursdays which I think will help Elle tremendously in relation to all her other activities.  Thank you to our infusion doctors and nurses at LeBonheur, who are so gracious to accommodate us in this way!

Thank you all for your steadfastness in praying for us and helping us and loving us, for we need your prayers and your help and your love so much.  Our hearts overflow with gratitude for you all the time.  

Throughout my days, I am telling my eyes to look to the hills and remember from where does my help come?  My help comes from the Lord who made heaven and earth.  He will not let my foot be moved; He who keeps me will not slumber.  The Lord will keep my going out and my coming in from this time forth and forevermore.  I will continue to preach the truth to my heart in the midst of these hard days.

Much love as always,

Dana