Sunday, July 30, 2017

Lots of Big News!

So I'm sure many of you have seen that we have had a lot going on lately!  Good news all over!

First of all, Elle had her first infusion here in Memphis at LeBonheur Children's Hospital!  June 20th, we were able to drive about 20 minutes to downtown, get Elle's enzyme infusion and were home by 2:38pm.  A.M.A.Z.I.N.G.  We are thrilled.  No packing, no coordinating childcare for Ann Carlyle for 3 days, no flying the day before infusion, no rental car, no killing time (with a very restless 5 year old) in Columbus, no overnight stay in the hospital, no flight home when Elle is feeling the worst of her post-infusion symptoms.  We all slept soundly in our own beds and we didn't have to recover from all the travel/being away from home/sleeplessness.  And because of that, we had a wonderful weekend that was restful but fun too instead of just trying to keep it together for one more day.  (Don't get me wrong, I still have plenty those days of just trying to keep it together, but we have removed a big and regular cause of that insanity, and for that, we are just so thankful.)

A huge THANK YOU to the LeBonheur team that pulled off Elle's first infusion off without a single hiccup - and all the while, celebrating every bit of it. We had so many people that came to see us that day to wish us well and to offer congratulations and at one point, sparkling juice in champagne glasses was passed around to mark the occasion!  It was a wonderful and (good) emotional day. 

A special thank you to Karen Butler at LeBonheur who worked tirelessly (not exaggerating here) with Frazer and Biomarin to get insurance approval in order to make this happen at our home hospital. She and Frazer spent countless hours everyday for at least 2 months - on the phone, going in circles, jumping through all the hoops and finally, finally their hard work paid off.  To say this is changing each one of our lives is an understatement.

And if that wasn't good enough news, Milla's House is officially open!  The celebration and open house for the newest location of the Kemmons Wilson Center for Good Grief - Milla's House - was yesterday.  So much hard work and support was and is still being put into Milla's House.  And we truly are honored that our girl's life is being celebrated in this way and we are honored, too, that we can be a part of helping the Memphis community.  Heaven knows, there is so much brokenness and we all need Jesus.  

Frazer and I were asked to speak at the ceremony, and here is what I said...

I wish that everyone in the world knew Milla.  If I could, I would talk about her for 2 days straight.

Milla was a firecracker from the day she was born. 

She did everything big.  

She played big, she laughed big, she got frustrated big, she yelled big, she dressed up big (most of the time, she had on no less than 3 tutus and amazingly could sprint around the house with my highest heels on), and she climbed and jumped off of everything she could.  

Big.

She also was a big-time introvert and gave everyone she met the stink-eye when she was little.
But when she was at home, she was a complete ham.

She just wanted to laugh and make us laugh.  

(Unless of course, she was frustrated and then everyone went down with her.)

I used to say, if Milla was in the room, we were all having a ball or we were all having a really bad day.


But then she had a seizure.
And then another and another and all of a sudden she was having to interact with new people constantly…mostly in the medical setting.

And her little self, although forced by circumstance, blossomed into an extrovert - looooving to be around people and charming everyone she met.


Her heart grew and grew.
And her laughter grew.

Even as her body stopped working. 

She would scrunch up her nose and smile so big - to her face’s fullest capacity.  


And even when her eyes grew dark and she couldn’t see anymore, she loved the sound of laughter…the sound of other people having fun in the next room would make her smile and giggle.  She glowed.

And so she became a bright, bright, bright light for Jesus.  

If Milla was near, you could feel the warmth of Jesus’ love radiating out from her.


We are so thankful to honor her life by helping to open a new Center for Good Grief.
Our prayer is that through Milla’s House and the incredible people that work there, people will feel the warmth of Jesus’ love during perhaps their darkest of days.


Thank you again for supporting us and Milla’s House!!



Thursday, we go back to LeBonheur for Elle's 24th enzyme infusion.  Please pray that all goes well.

We seem to be at a good place with night-help for Elle so praise God for that!!

Please continue to pray for us all as we miss and grieve for Milla.  Our family is just not complete without her.  We miss her all day every day.

Thank you for being with us and carrying us through this journey.  

Much, much love!
Dana

Elle after her first infusion at LeBonheur

Frazer's parents, Kent and Kathie, are celebrating their 50th wedding anniversary this year!!

Elle and 2 of her AWESOME nurses at Nationwide in Columbus, OH as we left the hospital after her last infusion there.  Bittersweet.  We were wrapping up her 22nd infusion there. 10 months.  Needless to say, we love these people.

Elle and Ann Carlyle snuggling before bedtime

Me and Elle during her first LeBonheur infusion - wiped out

Dr. Boop accessing Elle's ventricular port at LeBonheur

Celebrating Elle's first infusion at LeBonheur!!!

Good times at dinner time with Elle :)
Milla's House is open!!


These two :)

Friday, July 21, 2017

Grand Opening Celebration of Milla's House July 26th!!!

It’s time to celebrate the grand opening of Milla’s House! 

We are very excited and would love for you to join us next Wednesday at Idlewild Presbyterian on Union at 9:45am. 

See the official invitation below.  

Thank you for your continued support! 




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You're Invited
Children and Family Enrichment Center at Idlewild
&
Kemmons Wilson Family Center for Good Grief
Milla's House
Wednesday, July 26, 2017
Idlewild Presbyterian Church
28 S. Evergreen
Memphis, Tennessee 38104
9:45 a.m. - Blessing of the House (church grounds)
10:00 a.m. - Opening Ceremony (sanctuary)
Enjoy refreshments after the event.
Join Baptist leaders, the Idlewild community and friends as we celebrate the opening of The Children and Family Enrichment Center at Idlewild and the Kemmons Wilson Family Center for Good Grief – Milla's House.
Designed as a place for children and families, this house symbolizes a commitment to foster emotional health through increased collaboration and specialized services.
Children and Family Enrichment at Idlewild | Baptist Kemmons Wilson Family Center for Good Grief


Monday, July 3, 2017

It's July!

First of all, THANK YOU for aaaaallll the prayers that you have said for us.  Insurance has approved the enzymes and we will start to have the infusions in Memphis starting on July 20th!!!  So we head back to Ohio just ONE more time this week, and then Elle's next infusion will be here at home in Memphis.  I can't even!

So Elle and I head to Ohio Wednesday morning while Frazer and Ann Carlyle fly to Pittsburg Wednesday for the annual Batten Disease conference. Elle and I will join Frazer and Ann Carlyle in Pittsburg Friday after her infusion.  Busy week but we all fly back to Memphis together on Sunday and then no travel for the foreseeable future!

Please continue to pray for health for all of us.  Frazer isn't 100% and I have had a sinus infection and bronchitis of some kind for more than 6 weeks now.  I think I have finally turned a corner with it, but still coughing a bit.  Also, I found out my thyroid isn't working well.  I started taking some medicine for it a couple of weeks ago...hopefully, I will feel some positive effects from the medicine soon.  I am feeling beyond tired, lots of headaches and just generally feel awful all the time.  The last 3 years have been unrelenting stress for us with no end in sight, really.  Although, having Elle's infusions in Memphis should certainly help a little (or a lot!) with this chronic stress-storm we are trying to weather. 

Milla's House is open!!  All are welcome to the ribbon-cutting ceremony July 26th at 10am!  I'm attaching the invitation for the details.  We had our the first ever counseling session at the House last Friday.  :)  We are so excited to be a part of this organization that serves our community so well.  Here is a link for more information about The Kemmon's Wilson Center for Good Grief and their newest location in Midtown called Milla's House...

http://www.baptistleader.org/baptist-news/baptist-to-expand-grief-services-to-midtown-memphis-and-jonesboro/

Also, Caitlin Lockerbie, a reporter from a local TV station did a story on our family and helped us get insurance approval for Elle's enzyme.  We think they did such a great job and are thankful that they wanted to tell our story.  Here's the link...

http://www.localmemphis.com/news/local-news/local-health-alert-family-fights-for-coverage-for-new-fda-approved-drug/t751068979 


May the Lord bless us and keep us and may His face shine upon us and give us peace.

Much love to all - Happy July 4th celebrating!
Dana

Pictures!

Elle at the Miles for Milla's House Bike Race

Elle and a giant unicorn floaty that she wanted to take with her on a walk through the neighborhood (naturally)

Elle post-infusion feeling a little puny

On our way to the beach Memorial Day week!

Ann Carlyle in Florida

Elle loved playing in the sand and the water

A storm blew over our fence and took out the power for almost a week while we were in Florida - not cool. Thank God for sweet friends and neighbors that propped up the fence and cleaned out our fridge.

A rare night out on the town in Florida

Ann Carlyle photo-bombing the picture I was taking of Frazer and Elle kayaking in the ocean :)


A family picture at the beach - everyone is looking and smiling!

The last couple of hours on our way home from the beach were pretty rough -  Elle finally passed out and gave us 20 minutes of peace

Ann Carlyle and Lady

Lady riding shotgun - she insists on jumping in the car any chance she gets!

Ann Carlyle at Camp Good Grief

A spontaneous morning nap - sometimes life catches up with Elle and she crashes - I'll take those snuggles any day of the week.

Face paint, pjs, Guess Who game-playing and mommy's fuzzy slippers in the backyard as the sun goes down - just a day in the life of Ann Carlyle this summer (and does anyone else think she looks like a teenager in this picture?!)

This girl had suuuuch an amazing time at Camp Good Grief

Elle in her playhouse

Me and my sister, Dawn at the U2 concert that she and her husband, Jeff, took us to in Louisville, KY a couple of weeks ago - such an awesome gift and good time together for a couple of days

A day in the life of Elle - multiple costumes at one time - a superhero pumpkin that day

Elle during an infusion

Elle feeling yucky after an infusion

Another day in the life at our house - Frazer trying to catch a cat-nap and Elle (dressed up of course in her ballet leotard) doing her best to NOT let him sleep (and howbout those legs that are miles-long...)


Invitation for the Milla's House ribbon-cutting ceremony

Milla's marker was finally placed - we are thankful to have it there but it cuts deep into our hearts.  Oh how we miss that little girl.

Sweet sisters at bedtime

Sunday, June 11, 2017

It's Summer-time

Hey everyone!  Hope you are all well. Thank you, thank you, thank you for your continued prayer and care and love and kindness.  Truly, God moves through people and holds us up through you all.  There is no prayer too small, no kindness or generous act too small.  It is all stored in our hearts and brings constant encouragement to us as we often are just trying to keep our heads above water.

The waves have been hitting extra hard over the past month or so.  Our trips to Ohio have been full of difficulties and we have seen a steady decline with Elle throughout the last 4-6 weeks.  Her seizures have increased in number and severity - although, the seizures are still way 'better' than they were back in the fall.  She has lost more strength and coordination and muscle control.  She has lost more of her speech - both receptive and expressive.  She is not complying with taking her medication as well.  Her rages and frustrations have increased a bit...some days are better than others.  Her sleep has been bad.  And on and on.

However, we have seen a little bit of a good rebound in her condition after her infusion last week.  Friday and Saturday were really great days and she was so happy and vibrant!  We love seeing her playful and silly and giggly and funny - her true personality coming through.  Today, she seemed a bit more tired and irritable and I noticed a lot more seizures than she's had the last couple of days.  

So we just never know what her days and nights will look like,  There is no pattern to any of her sleep, seizures, tiredness, crankiness, strength, speech ability, etc.  And while we are utterly thankful for these enzymes that she gets every other week, we have no idea how long these enzymes will keep her disease from progressing and we have no idea how quickly her disease will progress.  

We know that God knows this, though. We know that Elle's days and nights were laid out and planned before time began.  He has always known her.  He MADE her.  He formed her and loved her long before we knew her and loved her.  

And so when our faith falters and the frustration and anger come barreling into our souls, when despair threatens to undo us, this is what we have to preach to ourselves.  This is what we have to remember and this is what we have to lean on.  How deep the Father's love for us!  How vast beyond all measure!  And it doesn't matter if we feel lovey-dovey about it all.  Our God is steadfast.  He does not love us conditionally.  When we stumble and fall apart from the weight of all of it, He remains ever-present, ever-loving.

Our Milla-willa has been gone for 6 months and 2 weeks now.  We miss her so much, so deeply.  I can't even begin to describe the longing we have to see her and hold her and listen to her belly-laugh again.  Please pray for our hearts as we grieve and try to find our way down this road.  Oh Jesus, how we hope You would come back soon.

As you know, Elle's enzyme medication has been approved by the FDA, which means we can have her infusions done here in Memphis.  And this is sooooo exciting!!  BUT, we have to wait for insurance to approve it.  So this is our biggest hurdle and biggest prayer right now - please pray for this approval!  And pray that it will happen quickly.  It has already been about 7 weeks since the medication was FDA approved and we need things to move along quickly.  Our hospital here in Memphis (LeBonheur Children's Medical Center) is ready to go - which is amazing and we are so very thankful for them working so hard for us.  So please pray, pray, pray for our insurance to approve it!!

We are surviving summer so far - we kicked it off with a beach trip to Florida Memorial Day week which was exhausting but really good.  We are a family of beach bums and the ocean soothes our souls.  :)

With the exception of this coming week, Elle will continue to have school throughout the summer - it is a very good thing for her to have this activity and stimulation for her brain.  (And good for her momma to have a little break from her constant activity and constant stimulation she needs.)  Ann Carlyle is going to a couple of day-camps this summer and has a pretty full schedule of activities to keep her busy.  

Ann Carlyle also just finished up 3 days of Camp Good Grief and it.was.wonderful for her.  She loved it and learned so much. She was able to be around a bunch of kids around her age that had all lost someone close to them...she was able to talk about Milla and all the complexities of her grief concerning both her sisters...she was able to have FUN and laugh and dance and see that all those things are okay too...she was able to comfort other kids and help them with their grief as well.  What an amazing camp it is!! And y'all, the people that run the camp and the people that volunteer to help out with these kids are unbelievable.  We have this treasure of a place (The Kimmons Wilson Center for Good Grief) right here in Memphis!!!

Speaking of, The Center for Good Grief's newest location - Milla's House - will open be opening soon this summer!!  Stay tuned for more details about the ribbon-cutting ceremony that will take place in July.  We would love for anyone that can make it to come out to celebrate Milla's life and this wonderful organization that is serving Memphis so well.

Okay, so I could type forever - I feel like I am leaving out so many things, but I gotta go get some sleep.  (Please continue to pray for our health - all of us - Frazer and I are so worn down physically and emotionally - life is definitely taking a toll on us.)

Much love to you!!
Dana

Friday, May 12, 2017

Miles 4 Milla's House is just one week away!!!



Miles 4 Milla’s House is one week away!  Please consider supporting us in raising awareness and funds for Milla’s House, which will open later this summer.  You can ride or show up and party at 11 am May 20th!  Sign up and/or donate here:  www.miles4milla.org  
There are “register” and “donate” buttons on the far left of the page.

Sign up by Monday, May 15 by 10am to be guaranteed a T-shirt at the event!

Thank you for supporting this wonderful event and cause!!!  You can find more info on Milla’s House at www.miles4milla.org

Friday, May 5, 2017

March and April and May (Buckle up - it's a long one)

I know it's been forever since I've written.  Part of it is because it was a busy, busy April.  Part of it is that I just didn't feel like saying anything.  We continue to travel a hard road, but some days/weeks/months seem to be harder.

My heart feels ruined at times, so broken.  But I cling to the only hope I know.  And I will praise Him through my tears for He came and lived among us and suffered and loved and died for us and rose from the dead so that we may live in perfect glory with Him.  So that Milla can be with Him.  And Elle.  And Ann Carlyle.  And me and Frazer.  

We miss Milla so, so stinkin' much.  Last week marked 5 months since she died - my heart breaks all over again to think it has been so long since I have held her, touched her, comforted her, kissed her, smelled her, fixed her wild curly hair...since I have heard her sweet voice, her giggle, her gut-laugh, even the way she struggled but was able to say 'mom' through her tears.  I long to be able to take care of her again.  There is such a void there for me.  I feel like I should be doing something with her, for her.  But she is not here and while I know with utter certainty that she is healed and is happy beyond comprehension, it is so hard to wait to be with Jesus, to be with her, to wait on this side of heaven.

A dear friend and fellow Batten mom from California (they lost their oldest son last September and their youngest son is receiving the same enzyme treatment as Elle) reminded me the other day to look at it this way - each day that goes by is not a day farther away from Milla, it's a day closer to when I get to see her again.  We preach this to ourselves over and over and sometimes it reaches our hearts too.  The deep pain is sometimes too much for words.

April is always a crazy busy month because Ann Carlyle's birthday is April 6th (8 years old!!) and Elle's birthday is April 20th (5 years old!!).  I know every parent says this and I know I say it every year, but I can't believe how old my babies are getting.  5 years seems especially old to me though...Elle is officially not a baby anymore...and we feel she is doing measurably better with the enzymes than she would be at 5 years old without the enzymes.

So between birthdays and birthday parties and a new canine family member and Ohio trips and big announcements and just regular life, the weeks have flown by.

Ever since our dog, Maddy, died last August, Ann Carlyle has been begging (and begging and begging and begging) for a dog.  Back in August and through the end of the year, I could not imagine getting another dog.  Of course, Ann Carlyle insisted she would take care of it but we all know who ends up taking care of the dog after the first week or 2.  But sometime in February I admitted to Frazer that I was thinking about thinking about thinking about the possibility of getting a dog.  It was a sloooow process.  But we finally decided that we would surprise Ann Carlyle with a dog for her birthday.  After extensive searching, I had picked out a possible rescue dog but we weren't able to meet/get the dog by Ann Carlyle's actual birthday, so we surprised her first thing in the morning with a card that told her we were getting a dog.  Y'all, she was so cute.  She had to read it a few times, did some double-takes and asked 'seriously??!! for real??!!' with the biggest smile on her face.  It was awesome.  

A couple days later, Frazer and I went to meet Lady and decided to bring her home.  And while we thought we were getting a 1 or 2 year old dog hound-mix that was house-broken and just chill enough to make it okay to add a dog to our circus, we ended up bringing her home, falling in love with her and then learning that she was at least 5 years old, is a Rottweiler mix, has major anxiety issues and a bad case of heart worms.  Our special needs family has found a sweet special needs dog and she has been such great therapy for all of us.  I knew she would be great for the girls, but I have been truly surprised by how much she has helped me too.  (Despite her incredible anxiety and health needs.)  Oh and Ann Carlyle is still doing GREAT with her Lady-responsibilities.  I'm so proud of her!

So, one big announcement that was made last week - the FDA approved the enzyme medication to be used commercially!!  Kind of a big deal. :) This means that we will (hopefully) soon be able to get the enzyme infusion here in Memphis.  I can't even remember what it was like not to go to Ohio every other week.  It goes without saying that to be able to simply drive downtown and get her infusion will be life-changing.  It is such a big interruption for everyone in the family to travel so often and creates a burden on so many people that help us do it every other week (although, they would never call it that because all you people helping are awesome!).  It will take a bit of time before insurance gets on board with approving coverage of the drug and for the hospital to get all their ducks in a row, but we are hopeful that sometime this summer, we will get to start getting Elle's enzymes in Memphis.  (Please pray that this all proceeds quickly and that insurance will pay for the enzymes.  It is about $700,000 per year (yes, $700k) to get this drug - so, as you can imagine, insurance approval is imperative.)

Also, we have announced the opening of Milla's House!  The Kemmons Wilson Center for Good Grief is expanding to a second location this summer in Midtown and it is being called Milla's House.  We are beyond honored to be a part of this community service in an area with great need.  We will be holding a couple of fundraisers this year - I'll get to that in a minute - but please consider helping us fund Milla's House.)  If you want to know more about The Center for Good Grief and the important work that they do, here is a link to an article about it...


If you are interested in donating to Milla's House, here is the link to help you do so:


AND, another BIG announcement - we are having a fundraiser for Milla's House in just a couple of weeks on Saturday, May 20th!  It's called Miles for Milla - it is a bike race (a 40-miler and a 6-miler) with a party at 11am with a band, food, beer and fun for the whole family. :)  We would love for everyone to join us (even if you don't bike - you can be a ghost-rider or just come to the party)!  We want as many people as possible to come out and have a good time (and the t-shirt is awesome!).
PS - when we announced to event last week, there was a hiccup with the website, so some of you may have not been able to register, but all is fixed now.
The link for information and to register is: 

(By the by, the registration and donation links are off to the far left side of the web page right now...it may be hard to find but we are working on changing that.)


So, there's a lot going on these days. :)

Please pray for all these things mentioned above and please pray for Ann Carlyle - this week, she has felt the weight of her grief a little more than usual, and we can see that affecting her when she slows down from all her activities.

Please pray for Elle - the last few days, she has been having an increase in seizures (even though we upped one of her meds a couple of weeks ago, which worked until recently).  And she's just been 'off' - fussier, more tired, the rages are coming back.  She doesn't have any symptoms of an infection, but it's possible that she coming down with something or fighting off something.  So please pray for her general health and that these neurological things settle back down as well.  Her seizures have been on the rise for about a month now.

For our latest trip to Ohio, we were there for 5 days so that Elle could get some extra tests run while we were up there.  It was a crazy week.  We flew to Columbus before sunrise Monday morning and went straight to the hospital from the airport to get admitted for a 24 hour EEG.  (That flight had mechanical and weather issues, so instead of less than 3 hours, it took us about 5 hours to get up there). Tuesday, we were discharged and then went to a physical therapy appointment where we learned she needs serial casting for her feet.  Wednesday morning, we had to be back at the hospital early to have her enzyme infusion (which includes an overnight stay).  Thursday morning, after leaving the research clinic, we went to the surgical unit so that Elle could have a sedated eye exam and MRI.  This unexpectedly took pretty much all day.  And anyone with a kid who has had anesthesia knows that kids get super crabby when they wake up - good times.  We were discharged and then spent the night in our Ohio apartment and flew out Friday around noon.  It.was.a.long.week.

The good news is that she did not have her usual fever after the infusion - so hopefully, that reaction is starting to subside.  However, she still feels super yucky and lethargic.  Also, we found out that her eye sight (it's 20/80) has not gotten worse in the last 6 months which is amazing!!  I am still waiting to hear back from the eye doctor with the in-depth eye exam report but we are thankful at least that she is still able to see.  

The MRI of her brain did show degeneration compared to 7 months ago.  But her doctor anticipated this and was unsurprised at how her brain looked.  Bottomline, she feels that Elle is right in line with the other patients that have been on the enzyme treatment for 3+ years.  According to her, the first year of the treatment, the brain degeneration continues and then they see a level-ing off of the degeneration on the MRI's.

Even though there were 'stable' reports from the tests, it is still hard to hear all the ways Elle's brain is getting worse.  And we are reminded that this drug is an ongoing treatment, not a cure.  It is a treatment to only slow the progression of her disease - the disease will continue to progress regardless.  And we will have to watch as her body continues to fail her.  We think she is actually doing better cognitively, which is unfortunately a double-edged sword.  I love seeing her comprehend things that maybe we haven't seen before, but she also is getting more frustrated as she comprehends the ways her body can't keep up with what she wants it to do.

Thank you for pursuing us and praying for us and caring for us!!  We continue to (very much) need all those things.  Some days we are able to carry on swept up in the fast current of daily activities/life, but other days, it feels like we are being sucked down into the muck and every little thing takes so much effort and seems to go no where.

One more thing, please pray for our dear friends, the Betts, who lost their cute-as-pie, precious son, Bentley, at the end of March.  We met them at the very first BDSRA conference that we went to a couple of years ago.  We have gotten to see them a few times in-between the annual conferences because they live just a couple of hours away and have come to Memphis to visit.  Like all the Batten 'babies', we smile to think of Bentley running around healthy and healed with Jesus, but oh how we grieve the hole he left here physically and in our hearts.

Lord Jesus, come quickly.

Much love (and thank you for enduring this enormous email!)
Dana
(I'll post some pictures soon.)