A quick update from Dad

A quick update from dad.

Elle's seizures have increased and generally, she is doing worse, which is to be expected with a degenerative disease, I guess.  Her appetite is not great, her attitude is not great in that she gets frustrated easily and has a very short temper.  She seems to be close to adding morning nap as her sleep during the night is less.  She rarely seeps past 515, and typically she is up before that.  On the bright side, it is a fun challenge to come home from work and try to get her laughing and playing each day.

Milla is generally weaker and her energy level is down.  She seems to have some minor blood circulation issues which don't really need to be dealt with, just another thing we see.  Her appetite has held up pretty good, but her tremors have increased.  Also, we are noticing her vision seems to be worse.  Or, she has trouble controlling her eye muscles to focus on what she wants to look at, its hard to tell the difference.  Her spirits are good and she still loves to giggle and be silly.  Its fun coming home and getting her to laugh!

I was in the kitchen last week and it hit me (more than it usually does) how labor intensive our world is with the girls, which does not let up and is overwhelming at times.  Its also been pretty overwhelming this month to deal with the degenerative nature of Battens.  It never really settles, rarely are the girls ever doing better and we face a long road ahead, but with all that, we have come this far thanks only to God's love and grace He pours out on our family and we plug along!  We thank all of you for pouring out your hearts, time, gifts, prayers, love, meals, flowers and all you have done for us and continue doing for us.  Whether you know it or not, you are being used by God to strengthen and sustain us!  He provides us the strength to make it through each day and there isn't much more for us to ask for right now.  I typically seem to cope and plug along much better when I focus only on my next step.  Very short sighted vision seems to be very effective and we look for our manna each day.  When I provide updates to friends and family or here on email/blog, it forces me step back and assess and opens my eyes to the degenerative nature, but it also opens my eyes to all that God has done to bring us this far and what He does for us each day and week.

Thank you for loving us and caring for us.  I am sorry that I cannot write as well as Dana, but we wanted to be diligent about getting more regular updates out.  I  promise Dana will send the next updates as she is much much better at these than I am, but please know that we are grateful for the love and support that you continue to put out on us!

Fraze

What?? So Soon??!

Hello again!  Just 1 week and here I am.  And at the risk of exposing my self-pride - I am awesome.

So quick update...

Elle had a rough start to the week.  Last Tuesday, she screamed all.day.long.  It was rough.  She just wasn't feeling good and falling a lot and acting like she had just gotten off the tilt-a-whirl.  She's also been getting up around 4/4:30am most days.  So that is contributing to her feeling yucky as well.  She's done a bit better over the weekend although yesterday, she tagged her eye and has a nice cut above and below her left eye.  It's swollen and colorful but it's okay (in that we didn't have to take her in to get stitches or anything).

Milla is also doing better this weekend than she was last week.  Much of the week, she was out-of-it and although she had some great bursts of energy, we can see her overall strength and alertness continue to diminish slowly.  Also, her tremors were more exaggerated.  We are expecting to get some wrist/hand braces in the next week or 2 to help with hand-positioning and possibly her tremors.  She seems a bit more alert this weekend although she has had poor sleep off and on this week.  

We are seeing some extra neediness and attitude from Ann Carlyle, which is hard because, for better or worse, we expect her to be more independent and obedient.  

So while it was a rough week, things improved this weekend - probably because Nana and Granddad (my parents) were here!  :)  

We are hoping to get in for a checkup soon with the girls' neurologist  - possibly this week.  

Thank you to all who have bought the book (Voyage to the Star Kingdom) and for all your kind words!  

And that about sums it up!

Much love!

Dana

2016

So, Happy Thanksgiving, Merry Christmas and Happy New Year!

I know I've been quiet for awhile - part of that was trying to take things off my plate to slow down (if possible) and enjoy the holidays, to remember why we celebrate Christmas, to just be with my family.  November and December were a bit of a dark time for me.  My heart was heavy and I felt like a robot, just going through the motions.  I had emotionally shut down.  I've had these times here and there over the past year - times that have always come and gone - although this one seemed to last a bit longer.  Thankfully, I feel like I've been pulled out of that particular mire and feel like my footing is indeed secure again.

Honestly, we have struggled a bit with the whole "happy new year" thing.  Because we know there are more hard times ahead...probably even harder than we've known.  At first, I felt angry and bitter about it.  What is there to be happy about??  What is there to look forward to??  Which also made me reflect on the past year with the same heartache.  But then.  Then my heart felt a change - begrudgingly at first.  (Make no mistake, this was God changing my heart and reminding me.)  All the people and meals and gifts and time and service and prayers and encouragement that we have experienced!  The love that we might have never known had we not been in this situation.  The faithfulness of God and seeing Him show up again and again.

This is a perfect segue into telling you about the book. :)   The book that 2 sweet friends (the sister and cousin of one of my oldest and dearest friends, Laura Capitell Balzer) have written and illustrated, who were inspired by our girls and the community that surrounds us and the things that God is up to...the reason we have hope...the hope, not in things seen, but in things unseen, the hope in things to come.

The book is called Voyage to the Star Kingdom, by Anne Riley, illustrated by Amy Grimes.  And it's amazing.  It has been in the works for awhile now - many of you have already heard about it.  You can get it on Amazon tomorrow!!!  Here's the link...

http://www.amazon.com/Voyage-Star-Kingdom-Anne-Riley/dp/1519421532/ref=asap_bc?ie=UTF8

See more about Anne on her website http://annerileybooks.com and Amy at http://www.storypaintings.net

As for us...how are we doing?  I've been getting that question a whole lot lately, especially since it's been almost 2 months of radio-silence.  

2 things:

#1 - I am going to try (really really really hard) to update the blog more frequently.  My goal is weekly.  I'm shooting myself in the foot not writing about our comings and goings since the whole reason for this blog was to keep everyone in the know about how we are doing.  (That sentence had way too many 'sayings' in it.  I apologize.)  Anyway, my goal is to update weekly...a short blurb about how the week has been, new prayer requests, etc.

#2 - So how are we doing?  Well, it's complicated, right?  Standard answer, we are hanging in there.  

Milla is trending downward slowly.  Mostly, we see she is losing strength and is having a hard time getting her eyes to find what she's looking for (for example, if I say something to her, she takes awhile to find where I am and then has trouble finding my eyes).  She is still eating well and was actually drinking really well until a couple of days ago.  We tried to wean one of her seizure meds a few of weeks ago, but immediately, her tremors went through the roof, so we had to go right back up to the original dose.  But she remains a happy kid and sweet as can be and for this, we are so very thankful.  

Elle is also trending downward slowly.  (We are thankful, though, that it's not quickly right now.)  Her seizures are mostly small but are, along with her general brain degeneration, really affecting her balance.  She also is still not wanting to eat much.  Too, I worry a lot about her not wanting to take her meds.  But our go-to lately has been getting Ann Carlyle to give Elle her meds.  It's a win-win!  Ann Carlyle loves that Elle wants her to do it...Ann Carlyle may sigh and roll her eyes at times, pretending it's such a hassle, but she loves being given such an awesome job.  :)  (She is 6 ½ yrs old going on 13 for sure.)

And speaking of, Ann Carlyle is doing good.  She asks some tough questions some times and she has been connecting more of the dots, even realizing that when Milla and Elle die, she won't have any sisters anymore.  I can't tell you how much my heart is utterly broken, hearing her come to this realization, but God is truly providing comfort to her little heart as we sit in sadness with each other, giving lots of hugs and reminding myself as much as I remind her how much God loves us.  But all in all, she is still super silly and a little feisty and is a fashionista and loves school and loves being with friends and loves legos.

Christmas was really good - Frazer and I really felt like we had a wonderful balance of seeing most of the family (on both sides) but also having some great time with just the 5 of us.  My heart was trained on some simple but staggering truths of the birth of Jesus - young Mary and all that was required of her...the shepherds in the dead of night, all of the sudden seeing and hearing a bazillion angels singing, heralding Jesus' birth.  Just putting myself in their shoes and imagining the craziness and absolute glory of that moment.

One of the best parts of it all was that Frazer and I got to go to an 11:00 Christmas Eve service.  We haven't done that in at least 6 years and honestly I wasn't sure I would be able to make it that late!  But I did and it was amazing to be there, worshipping and setting our hearts on things above as Christmas day began...a truly wonderful time for us both.

I promised myself to keep this relatively short so I'm going to sign off.  As always, thank you for your prayers (and everything else!) and we so appreciate your perseverance with us down this long road.  

Oh - and because so many of asked - My sister and her family are not moving to Memphis at this time.  They thought that is what God wanted them to do last summer but then God closed those doors, so they remain in Birmingham.  A dear friend told me recently, sometimes God just wants us to walk in obedience to Him, even if, in the end, it is not the ending that we had foreseen.  So for now, although we don't understand it and although it hurts our hearts, they are staying put.

Real quick, I have spoken of this before, but I want to say it again because it has stayed on my heart for weeks now and because it is such good truth...about Christmas and light and darkness…that Christmas means nothing if it doesn’t start with darkness.  Jesus was born in the dark.  He was resurrected in the dark.  God perhaps does His greatest work in the dark.  When the world couldn’t see its hand in front of its face, He showed up. 

(Sorry I'm still writing!  I get going and can't seem to stop!)

Ok.  We love y'all.  And goodnight.  And may the Lord keep us close to Himself no matter what 2016 holds...may we have joy and hope in abundance.

Dana

PS - Our Christmas card that didn't happen again this year :)

Fall is Here!

Just a (hopefully) quick word on how we are doing these days.  :)

Milla is doing GREAT.  I mean, really, really, really great!  We have been slowly weaning her down on some of her seizure meds.  (The reason isn't so great - it's because her brain has degenerated to the point that it can't generate seizures like it used to be able to.)  BUT, the amazing thing is that she is tons more alert and her strength has increased exponentially.  She is holding her head up more and doing sit-ups and pulling herself up to a standing position.  She can also hug me tight enough to hold herself up without me doing a thing.  She is swallowing better and breathing better.  We've also seen more of her personality coming back.  She is silly and giggles and belly-laughs and also fusses and cries some (which I like seeing as well because it's a normal thing to have all those emotions).  She still has some seizures but they are definitely mild and manageable and not worth medicating.  She is loving school and is so proud of herself with her new-found strength.  These changes have made life more exhausting and we have to keep a pretty close eye on her (because she is sometimes over-confident in her abilities) but we are super thankful for this time.

Milla turned 5 on November 2nd and had an amazing birthday on the afternoon of the 31st.  Some sweet people offered their barn and land for us to have her birthday extravaganza...and with the help of many, many people, we were able to celebrate our sweet girl's life.  It was a super rainy day, but God stopped the rain literally during the hours of the party, 2:30-5:00.  I know many people were praying that it would clear up that day, and all praise be to God, it did!!  The party was more special than Frazer and I had imagined it to be and Milla had a blast!  We will never forget it.

Elle is a bit of a different story.  For much of this year, we were able to stay ahead of her seizures because we knew what meds worked for Milla and we didn't have to go through so much trial and error as we did with Milla.  But we are now in the uncontrolled stage with Elle's seizures.  We have been increasing doses on 2 seizure meds and are considering a 3rd one...although I think we will be able to hold off on that one for a bit longer.  A little over a week ago, we spent 3 days in the EMU at LeBonheur to have EEG and video monitoring around the clock.  We were able to figure out a few things...whether or not she is having a new type of seizure called 'atonic' or 'drop' seizures.  We are thankful she is not having those yet.  However, we are concerned that a longer seizure she had while there was not a seizure except for the first few seconds (the episode lasted 5-6 minutes - she was limp and unresponsive and her eyes were fixed - very typical of a seizure).  We were told that it is just the disease progressing but we don't have any more details than that.  This was not the case with Milla so we are very confused and scared about what this means for Elle.  Please pray that we will get a little more of an answer with this as she has had 2 more of these episodes in the last week.  

Also, we are still having a hard time with Elle's behavior - although she has calmed down a good bit from a few weeks ago.  She has screaming fits but also has aggressive/violent outbursts as well.  Thankfully she is no longer having these outbursts unprovoked but still has them 'provoked' (meaning she isn't getting her way, etc).  She is 3 years old and I've been through the terrible 3's twice before with my other 2...but there is a different level of neurotic behavior when you throw in Batten Disease.  And it's hard.  Because you know it's not your child but this awful disease doing things unseen in your child's brain.  Milla went through this as well but she never had the aggression that Elle displays.  Elle grabs her head and covers her eyes a lot when she is seizing and she does this during these aggressive outbursts as well...so I think clusters of seizures are happening much of the time before, during and/or after these outbursts.

To make matters worse, Elle is not wanting to eat much these days.  Every meal is a battle to get anything in her tummy.  (And low blood sugar is just what we need.)

So throw all of the above and mix it around and it's just a hard and utterly exhausting time in the Gieselmann household right now.

On the upside, Ann Carlyle seems to be doing well...she is still loving school and having a great time with friends.  There have been a few things that are probably some acting-out because of what is going on with her sisters, but in general, she is taking it all in stride.

Frazer and I just celebrated our 13th anniversary last week and got to spend a night out at the Peabody Hotel Friday night.  It was wonderful and we had a truly good time with each other, laughing a lot and getting some quiet R&R.  The hotel staff made our stay special with little kindnesses throughout our stay.  And we had my mom here helping with the girls and a whole team of friends and family to help her while we were gone!  So thank you to each and every one of you!!!

Our days are always changing and yet, in some ways, the days are all the same, with the same hurts and same grief and the same prayers over and over again.  Sometimes I feel like there will never be a break and that I have absolutely nothing left to give.  Sometimes it feels like my heart is broken in so many pieces and is beyond repair.  Sometimes it feels like the darkness will truly overtake me.  But the morning always comes and somehow I am able to make it through the day and somehow I have moments of joy and laughter and I have true thankfulness for however this life looks.  I have more love for Jesus than I ever have before.  He is the.only.reason that I can physically and spiritually stand.  If you don't know Jesus, oh how I pray for you to know Him.  

Thank you always and always for your prayers and words of encouragement (written and spoken) - thank you for hugs and help and all of it.  We just had our 1 year anniversary of moving into this house and finding out the diagnosis for both Milla (in October) and Elle (November 1st...the day we moved).  My memories of this time last year are foggy at best.  I remember that there was always a slew of people in my house unpacking boxes and hanging blinds and helping take care of the girls (and me).  I remember sadness, bewilderment, shock and thankfulness all at the same time.  At times, I cringe at the way we have been so needy with meals and help with the day-to-day of our life (it's been a year and a half now with 3-4 meals a week provided for us and over a year of childcare/household help - all volunteers).  It's so hard to accept help...why is that??  Pride?  Independence (false, of course)?  A gnawing sense of not ever, ever, ever, ever being able to repay all of you who have prayed and helped and generously given in such self-sacrificing ways?  All of the above?

Well praise Jesus, it's okay.  All of it's okay.  And all of that sacrificing is done because of Him and by Him and through Him to sustain us in this (too-long) season of life.  Whether you know it or not, you are His servant - serving a great and mighty King, Who has never left me nor forsaken me.  Who has engraved my name on the palm of His hand.

Side note: I took a break writing this update this morning to feed Milla.  And I was asking her, "Who loves Milla-willa?!" It's a game we play (based on a book we read forever ago) where I raise my hand and proclaim, "Me! I do!"  It always brings big smiles and now everyone in the family does it.  But today when I was saying, "Me! I do!" Milla smiled big and then gasped and clamped her hands together (which means she wants to pray - she does this a lot).  It was like she was saying "Jesus does!! And let's pray and tell Him we love Him too!"  I know I'm putting words in her mouth, but I know one thing for sure, she knows Jesus loves her.

He will wash away my sins, let the little child come in.  Yes, Jesus loves me.

Well, this is getting long as it always does!  But real quick, I have to say that I've been meditating of late on how chronic and long this season of life has been and will continue to be for awhile.  And how amazed and humbled and so very grateful I am for you who have continued to stand with us and for us even though the road is long.  You have persevered with us.  We will always remember you and the light you bring into the darkness.  And may God bless you richly, for He has truly blessed us.

Much love,

Dana

Oh - and prayer requests - many are the same...our marriage, Ann Carlyle dealing with this not-so-normal life, strength, perseverance, for us never to lose sight of the cross.  Specifically, pray for Elle - behavior and seizures.  Pray we can get some answers...and if no answer, that we will rest in God, knowing that He knows every nook and cranny of Elle's brain and knows how many hairs are on her head and loves her more than we can imagine.  Pray that we will be able to keep weaning Milla's meds and seeing her come out of this fog she has been in.

Milla and her infectious smile

Elle in the EMU

Our night at the Peabody Hotel

Milla's birthday party - She LOVES riding horses!

Ann Carlyle and Milla

Elle - Tada!

Sweet big sister - Ann Carlyle and Milla

Elle

Elle flying high!

Milla with Mommy

Ann Carlyle with Daddy

Milla is 5!!!

Milla-Willa turned 5 today!!!!

Blessed be the name of the Lord.

That is all. :)

Milla's party this past Saturday

Races and Planes and Boats (and a couple of pics)!

Hello all!  Can you believe I'm already sending out another update?!  (Frazer might've written much of this...and that maaay be why y'all are getting an update so soon after the last one...)  

We wanted to update you on the girls since our last post and to provide an update on our recent trip to Ohio.  Before we dive into that, we want to pass along some info.  We would love for any and all to join us Sunday, October 4th at 2pm for a 5k and/or 1 mile fun run benefitting the Center for Good Grief.  It is a wonderful organization and has had (and continues to have) a huge positive impact on our family since we started going last December.  Here is a link for more info about the race and feel free to contact us about it (we have created a G Team so make sure you sign up with our team!)...

https://campgoodgrief5k.racesonline.com/ 

Generally speaking, Milla’s congested lungs and difficulty in breathing has been better the past few weeks, with a scare or two here and there.  For the most part, the fluid seems to have left the lungs and we are thankful for that but her swallowing is becoming increasingly more difficult.  However, she has been back at PDO and that has been so good for her (I can't begin tell you how good this has been for her spirits!), she LOVES her PDO teachers and friends and they take such great care of her!  She does continue to weaken, but her spirits have been good and boy, is she is a fighter.  When we were naming Milla, we chose Louise Mildred because they were both family names, but specifically Louise had a ring that Frazer loved, especially after he searched the meaning of the name.  “Louise” means renowned or famous warrior.  "Mildred" means gentle strength or gentle advisor.  What a combination - gentle warrior - one that is amazingly fitting of Milla, even as she struggles with Battens.  She is fighting it each day, and it’s been amazing, not only to witness her character ourselves, but also to see how her beauty affects others.

Elle is doing okay and while we are seeing more seizures and less “spacial awareness” (bumping into doors, walls, etc.), she is still learning things and running around.  However, she also continues to have much harder days, temperamentally speaking.  Those days are hard for everyone and will run us all down pretty quick, as most screaming kids do.  

Many of you know that we went to the Batten Disease Center of Excellence in Columbus, OH to meet with Dr. Emily de los Reyes and her team at Nationwide Children's Hospital last Sunday through Tuesday.  Dr. Emily and Dr. LeAnne are two of the doctors we met in Chicago in July at the Batten’s conference.  While not necessarily “ground breaking,” our meeting was very, very good.  We met with both neurologists as well as physical, occupational and a speech therapists.  We were there for about 5 ½ hours Monday and Milla went back the next morning before our flight to work with the speech therapist on her drinking and eating, so it was pretty informationally intense for all of us.

Overall, we learned a lot about the girls, their meds and Battens and the doctors were able to shed some light on our path moving forward.  The therapists gave us some exercises and recommendations to help both girls with their strength, eating, drinking, motor skills, etc.  We have had both girls in all three therapies for a while and meeting with this team provided us confirmation with what we are already doing but also some new things to try.  

Dr. LeAnn's specialty is sleep and she was especially helpful with the girls' sleep - we are trying a new drug at bedtime for Elle - and so far it's been working!  However, Elle's been a bit more sleepy in the daytime hours - although this could also be due to a cold she's been fighting this week.  Time will tell.  Dr. Emily spent the most time with us and the girls gave us good insight about where Milla is now and how Elle is doing, both of which were encouraging and helpful.  We decided to tweak one of Milla's meds and we have a few more ideas on some tweaks down the road (we don’t want to make too many changes at one time we will know what is working and what is not).  Milla seems to be doing pretty well, relatively speaking, with the large exception being the lack of muscle tone in her neck.  Dr. Emily has not seen it deteriorate to this point this early.  However, she was encouraged by her interaction and awareness, as well as her desire to want to be active and involved with those near her.  As for Elle, she also seems to be doing well - again, relatively speaking - and we are going to keep her in all her therapies and continue to work with her to maintain as much strength, coordination and speech as we can, as these things can be beneficial, even long-term.  

You might have noticed that we said 'flight' a few paragraphs ago and not 'drive'!!  A generous soul offered us a private plane to get us to Ohio Sunday afternoon and home again Tuesday morning.  This not only saved us 2 or 3 days of travel time, it really removed a lot of the stress of making this trip happen.  It. Was. Wonderful.  We want to express how immensely grateful for those that made this happen!!!  We continue to be blessed with people helping us in every way possible.  God is good!!!

We also wanted to share one final piece of news.  Our Palliative Care doctor is Dr. Melody Cunningham and she has been so good to our family as we go through this journey.  She and her team have come along beside us and have helped us every step of the way.  She has been named a finalist as Caregiver of the Year award (http://www.lebonheur.org/news-events/news/press-release-archive/2015-press-release/schwartz-award-finalists.dot) and we are so excited for her to receive this honor!!  (We are pretty confident she has this award in the bag!)

Oh and one more bit of good news...we have gotten approval for an LPN (nurse) to help us 18 hours per week(!!!).  This is an amazing blessing especially as Milla progresses - we are hoping to use this LPN to accompany Milla to PDO for as long as possible - as we continue to see such benefit from her going.  As I've said before, going to PDO gives Milla strength and joy.  We will also have time to use the LPN here at home during the week.  So please pray for this transition and for this person - that it will go smoothly and that she will be a perfect match for our family.  (Also, we have recently had a new weekend CNA to help us at night.  She started this past weekend and all is going well!)  Pray that all of these relationships will continue to grow and will be a true help and blessing for all.

Please continue to pray for us as we feel the strain and exhaustion of such a long and chronic situation.  There are days when it feels like we are in the middle of the ocean...in a storm...with nothing more than a half-deflated life vest to keep our heads above water.  But then God's hand moves and we are given a nice, sturdy boat to rest in, even as the storm rages on.  You people are the boat.  He is ever-faithful even when our faith fails.  His love is everlasting even as our love wavers and is lukewarm.  He is our Rock to stand on so we are not consumed by anything.

But we are tired.

When I said, 'My foot is slipping,' Your love, O Lord, supported me.

When anxiety was great within me, Your consolation brought joy to my soul.

Psalm 94:18-19

Thank you for meals and prayers and planes and boats (real and figuratively speaking) and for loving on our kids and for oil changes and your written words and your spoken words and your time and flowers and help with our dog and drapes and your smiles and immense generosity and hugs and tears...there are approximately 1,984,329 more things we could list...

All in all, we love you and never fail to thank God for you!!!

Dana and Frazer

Milla on the plane - she LOVES to fly!!!

Elle - always dressed in something with tulle

The beautiful sunset in Columbus, OH one of the nights we were there.

For I Know the Plans I Have For You...

First of all, a continued THANK YOU for all those praying for us (near and far), loving us, serving us, helping us and living all this crazy (our ‘normal’) out with us.

Unfortunately, the phrase, ‘no news is good news’ has not really been the case for us the last month or so.  But I’ve just been too busy and covered up to sit and write out my thoughts.

I’ll start with the week leading up to my birthday in August…Frazer got pneumonia.  The real-deal pneumonia.  He was flat on his back for a solid week and then in the middle of that, we realized that all of us girls had lice.  (My head still starts itching every time I think of it.)  Not the best of times in the Gieselmann household.  But the Lord’s graciousness prevailed as our village, once again, showed up and carried us through the darkness that almost consumed me.  And thankfully, no one else got really sick…we all had a bit of a cough for awhile, but nothing that put any of us in the bed.

Soon thereafter, Ann Carlyle started first grade!  She is at a new school this year.  We were completely happy with the school where she attended kindergarten but God gave us an incredible opportunity to put her in a small, Christian school where my sister-in-law actually works as a counselor and where she can be nurtured in ways her old school would not be able to.  And she LOVES it there.  Really, really loves it.  Thank you to all that made this possible and for those that are driving her to school and back home again every day!!!  She is happy and being challenged and is thriving and is well-cared for!  This has been a ray of light for us in an otherwise pretty dark month.

Elle and Milla also started back at PDO, where Milla’s one-on-one teacher also returned this year to help with her.  A HUGE thank you to everyone at PDO that love us and our girls so well and who have made it possible for Milla to go there.  I have no doubt that being there has improved Milla’s quality of life…she is happy to go and the socialization she gets to experience is such a gift.

So all in all, it was a good week when the schedule and routine made their ways back into our lives!  I actually got to go to the store for the first time in 2+ weeks for more than just 3 or 4 things to get us through the next day.  Everyone was relatively healthy and completely lice-free and while there was still plenty to be anxious about, I let out a little sigh of relief that we had made it through sickness and critters and no schedule of any kind.  (Only by the grace of God and the people He continually puts in our lives.)

But we were about to be hit again - knocked clear off our feet.

So back in July, we went to the Batten Conference in Chicago primarily to meet with certain doctors and researchers that are involved in a clinical trial of a drug that could possibly save the lives of children with the exact mutation of this disease that our girls have - possibly even stop the progression of the disease.  (When the study was open last year, Milla was too far along in her disease process to qualify and Elle was not old enough.)  But now Elle is old enough and we learned there was a possibility that the study would reopen sometime this fall and that Elle could be a good candidate.

This would be an amazing and life-changing possibility for Elle and all of us but it would also require us to move to Columbus, OH.  And move soon.  And the thought of leaving our vital support and life-giving team here in Memphis and leaving our families to move so far away was excruciating and scary and completely overwhelming, especially in light of Milla’s condition.  Of course, we were willing to do whatever it takes if it meant saving Elle’s life, but the logistics and details and Frazer’s job situation and on and on have been hard to comprehend and we had to keep telling ourselves that God would provide for us as He always has done.

But I’m going to be honest…there’s been a whole, whole lot of anxiety.  And too, I struggled with fear.  Did we dare to hope that Elle’s life could be spared?

However, we found out about a week ago that Elle would not be getting into the study.  As you can imagine, we were crushed.  It felt like we had found out about her diagnosis all over again.

As I have said before, I have no business questioning why God.  He is God.  I am not.  But I know His love.  I know He has not abandoned us.  I know He loves all 5 of us more than we could ever hope or dream.  But sometimes (most of the time) it is hard to not understand…to not be able to see.

And literally, the day after we got the news, Milla started to decline again.  Her lungs have filled with fluid that she has not been able to clear completely.  She has been struggling to breathe and had a day last week that we had to give her a bit of oxygen through a mask on her face to keep her sat greater than 90%.  (‘Sat' is short for the oxygen saturation in her blood.  Normally, a person sats around 98-100%.  We want the sat to be greater than 92%.  If it falls below 92%, we need to give her oxygen.)  So we started her on an antibiotic just in case of an infection and also started a drug that should help dry up some of the fluid in her lungs.  It seemed to work a bit but then she would get worse again.  Her condition seemed to oscillate back and forth for a few days.  But yesterday, she had a great day, relatively speaking - her lungs sounded better than they had in a long time and her sat was staying in the upper 90’s most of the day.  And today, even a tad better!

We are praying that she stays on this course and keeps improving.  We are not sure, however, if she is temporarily responding to these drugs and will continue to do so, or if we are just seeing a couple of good days.  But what we ARE sure of is that Milla is a fighter.  She is stubborn and determined and absolutely amazing.  She still has her silly sense of humor and still tries her best to stand and walk and crawl and talk and be a part of the action.  She laughs and bosses Elle around and loves to be with people.

We are not sure yet if we will be able to send her back to PDO or if her needs are simply too great at this time.  But please pray that she can return there.  It is so good for her to be there and out of the house and to be with people and kids her age.  The days that Elle went to school without her, she was so sad.  She kept pointing towards the driveway and saying ‘car.’  I don’t want to keep her cooped up…a happy heart strengthens the body.

And please pray for all of us as we watch Milla's disease progress.  It is incredibly difficult watching her struggle to breathe and wonder if she will be able to fight through it.  Ann Carlyle is seeing it all too and trying to process it.  (She is doing okay for now, but pray that she continues to process in a healthy way.)

Elle has had some hard weeks as well.  We have had to increase one of her meds 5 times over the course of 6 weeks or so.  We are seeing more seizures and more neurologic irritability and physical instability.  In other words, she is screaming a lot and bumping into things a lot.  It’s been about 4 days since our last increase and I think we are starting to see a little decrease in her screaming fits.  But she’s been really hard to deal with lately.  We remember Milla going through this as well - the difference is that now we know why Elle is acting this way.  Knowledge is a very good thing but it doesn’t make it much easier emotionally, especially when we have a front-row seat to all that Elle has in front of her.

I’m sorry this is so, so long but there is just a lot to explain and update.  Thank you for hanging with me through it!!

So we continue to ask for your prayers - please plead to God for His mercy.  We feel like we are getting hit with one thing after the other lately and we are weak and tired and constantly fighting the fear of drowning in all this mess.

Pray for perseverance and stamina and unwavering trust in God.  Pray that we will not succumb to doubt and fear and sadness.  Pray that we would be able to live with joy and thankfulness for the time we have with our girls.  Pray that we would have energy to keep up with all of them and parent them and love them.

I pray that the light of Jesus would shine ever bright - in us, by us, through us - that no one would ever doubt His goodness and love and saving grace.  I can’t tell you why 2 of our little girls have this disease and will die from it, but I can tell you that Jesus is all we need.  I can tell you that saves us from our brokenness.  And I can tell you that when our girls die, they will be with Jesus and they will be whole.  He created them and loves them beyond measure.

Oh my Strength, I give praise to You.

In joy or pain, I give praise to You.

Night and day, I give praise to You.

Always, much love and thankfulness for you all.  I cannot express all my heart feels for you.

Dana

PS - On September 13th, Frazer and I are taking Milla and Elle up to Columbus, OH to meet with the doctors at the Batten Center at Nationwide Children's Hospital.  Please pray that Milla will be strong enough to go and that we will be able to travel safely and keep our sanity, as we will be driving. :)  We are hoping to learn more about the disease and how to possibly better medicate them for seizures, sleep and other disease symptoms.