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.

Pictures!

Elle before her MRI

Milla and Frazer on one of her really great days!!!

Elle and Baby Bear at her swallow test

Elle is fearless - hanging out with Granddad and Tigger!

Ann Carlyle with Cousin Lucy, Tigger and Granddad

Love Our Village - New and Old!!!

I know y'all don't believe me but I really do have every intention to update the blog at least every week!  (Seriously!)

So Frazer and I just returned from the Annual Batten Disease Conference in Chicago.  We had not intended to go but about a week ago decided to do it.  Thank you to Kathy Mize for her herculean effort to organize (last minute!) all the people who helped care for the girls while we were gone for 3 days!!!  And thank you to those that did the caring too (last minute!)!!!!!  And thank you to those that got me on the plane Thursday afternoon after a little bout of a stomach bug!!!!  Y'all are amazing and the girls had a wonderful time without us.  :)

The conference was amazing and completely overwhelming and it's hard to put into words all we felt while we were there.  But bottomline, we have added to our village in these people...families that have been affected by this disease, specialized doctors and the Batten Disease Foundation staff that live and breathe this disease and are dedicated to helping Batten families, and researchers that are committed to finding treatments and/or cures for this terrible disease.  We came away with a wealth of information and things to process.  

A BIG update is that our insurance-covered help has kicked in!!!!  We have a nursing-aide every single night from 10pm-10am!!!  There are 2 women that come each week - one comes 4 nights each week and the other does 3 nights.  Everyone is still relatively new at all this, but it is going well and we are thankful for the help...and for the sleep!!!

As for the girls...

Milla is doing really well.  She is talking more, moving around more, laughing more.  She still fatigues relatively quickly and has times of lethargy but she is so determined to try to walk and stand and crawl and even jump on the trampoline!  And one of my favorite things to do is to get her giggling - her shoulders will bob up and down and if she gets super tickled, no sound comes out, just a huge smile and the shoulders bobbing.  Good for the soul.  :)  We are tapering one of her seizure meds down a bit and that seems to have brought Milla out of the fog she was in.  But we have to be careful as we are trying to find that ‘sweet spot’ between controlling seizures and over-medicating…both of which can cause lethargy and fogginess.

Elle is doing well too.  Her seizures have been mostly under control.  She still has some coordination issues at times but all in all, she’s doing fine.  She continues her speech therapy and we are seeing her make some gains there.  She had a swallow test done a few weeks ago and mostly did okay, but there was a little bit that concerned them enough to tell us to go ahead and thicken her liquids.  (This was a little discouraging to us that she is needing this so much earlier than Milla did but it has helped decrease her coughing/choking by leaps and bounds!)  And she had a baseline MRI done yesterday - it was a long day at LeBonheur due to an emergency that came in - but we got through it.  

There are still days like today, that both girls seem to be ‘off’ - more seizure activity, sleepiness and fussiness.  But hopefully we will see a rebound tomorrow.

Ann Carlyle has had a summer full of playdates and swimming and fun.  :)  She has had some pretty heavy questions about her sisters but she seems to be processing things in a very healthy way even as she is grasping the gravity of the situation and for this we are thankful.  She remains a happy and relatively carefree 6 year old!

We all 5 actually were able to travel to Birmingham to visit my family (and go to my 20-year high school reunion - yikes!).  The girls all traveled really well and we had a fun time playing with cousins, swimming, riding Nana and Granddad's horses and seeing old friends.

I'm keeping this somewhat short today (maybe?) so that I can go ahead and get this update out...but as always, we treasure your prayers...

Please continue to pray for safety for the girls, for mercy, for all of them to know the deep, deep love of Jesus.

Please pray for Frazer and me also to know and feel the deep, deep love of Jesus and pray for our marriage.  Pray that God would grant us clarity and wisdom as we consider some possible new ways of treating the girls' seizures and sleep issues and as we continually make medical decisions for Milla and Elle.  I am feeling a lot of anxiety of late and my body is suffering because of it.  Please pray that we both are able to rest in God's promises and faithfulness that He will never leave us or forsake us and that He will continue to provide us, love us and give us strength to walk this long road.  Again, pray for clarity and wisdom and for God to go before us and pave the way.

Thank you for praying for us, with us.  Thank you for supporting us in every way possible.  Thank you for being a reminder that God has not left us - for being our awesome, amazing and incredible village.  We bragged a lot on y'all this weekend at the conference.  :)

Much love!!!

Dana

(My laptop's start-up disc is full and I can't figure out how to get some pictures on here but I'm working on it.  I'm not real bright when it comes to computers and start-up discs and storage.  Grrrr.)

Oh the deep, deep love of Jesus

Vast, unmeasured, boundless, free!

Rolling as a mighty ocean, in its fullness over me!

Underneath me, all around me, is the current of Thy love Leading onward, leading homeward to Thy glorious rest above!

Quick Note

A quick post to share a story and ask you to give $1 to support this.  Every little bit may help someone else not experience what we have.

http://deadline.com/2015/06/gordon-gray-daughters-batten-disease-fundraiser-pledge-1201439934/

Thank you!

Frazer and Dana

Some Changes

Hey y’all - so we’ve had a few changes happen over the last week or so since Frazer’s update that I felt was important to let you know about.

MIlla is continuing to get weaker with her swallowing and breathing and she probably has more than 100 little seizures per hour (sometimes many more).  The amazing thing is that she has been super strong in other areas…she has had times of wanting to bear weight in her legs and even taking some steps (assisted, of course, but this is something we haven’t seen in a very long time).  And she has been more alert and responsive.  She is quick to smile and laugh and still takes such joy in making others laugh as well.  We are so thankful her mind is still ‘with us’ most of the time.  Her disease causes dementia along with everything else, so everyday we have with her where she knows us and knows our love for her, is a blessing.  Please continue to pray for this specifically…and praise Him for His provision in this way!

Some of the most dramatic changes, however, have been in Elle.  She is seizing more and she is walking around like she is in slow-motion.  It is like the wind has been sucked out of her.  She even talks in slow-motion.  We have seen changes in her balance and coordination as well and 1 eyelid is occasionally drooping a bit (as we see with Milla).  We aren’t sure at this time what is due to seizures/meds and what is due to the degeneration in her brain.  Today, actually, has been the first day in a week that I’ve seen some kind of plateau in her condition.

Ann Carlyle is doing okay but she is definitely seeing all these changes in her sisters - we are having little conversations about hard things frequently.  We’ve seen a few behavioral things here and there - clinging, not feeling good, emotional.  She threw a fit yesterday about not being able to sit beside Milla in the car.  Please pray for her little heart and mind and pray for us as we strive to protect her when she needs it, to gently show her the reality of what is happening, to have patience with her when it’s hard to have patience.

On a positive note, everyone has slept through the night the past two nights!!!  Praise God and please pray that this continues!

Please continue to pray for the help provided by insurance.  We are still in this waiting place to see what they will pay for and what we need…and then a bunch of other steps have to be taken before we physically get that help in our home.

Please continue to pray for mercy for all of us.  God is love and He is merciful.  His character never changes.  As you can imagine, this has left us reeling and we aren’t even really able to process it all.  I think we both feel a bit like we are just getting through the days, doing what needs to get done, and the processing will get processed later.  In other words, survive.

And so we press on with the hope of Christ in our hearts.

Thank you all for standing with us - and for us when we can’t.  Much love-

Dana

Long Overdue Update...

It’s been a while and in some ways our world is the same, but in many ways it is ever changing, so an update seemed appropriate.

 Elle – her seizures are under control.  We see small ones, typically at night and a longer one will pop up from time to time (it’s been over a week since the last bigger one).  As it stands today, the med combo she is on seems to be pretty effective, but that can change tomorrow as the disease progresses.  As for the rest of Elle, she seems to be doing well, playing and doing typical 3 year old stuff.  We see very small development things with her, but Elle is very smart and overall doing well right now.   Elle eats less than a bird and she is still light as a feather, but she seems pretty healthy.  But, her sleep is not good.  Typically she is up 3-4 times during the night and while she generally goes back to sleep with relative ease, she is starting to get up earlier in the day.

 Milla – not much good to report with “Milla Willa.”  Since we last updated, she took a significant downturn at the end of April around the time she had the cough that was in our last update.  At that time, she had generally weakened, could not chew the same foods and her chest cold/cough all caught us off guard.  Most recently, the past weekend was another significant downturn for Milla.  A noticeable weakening and unresponsiveness, increasing seizure activity and excessive drooling.  After visiting with the doctor yesterday, the seizures seem to be less concerning, if they are even seizures.  It’s hard to distinguish a seizure from her just “checking out” briefly (due to disease progression).  Thankfully, they are not violent convulsive seizures.  She is weak to the point that she cannot sit up on her own, the majority of the past few days, she struggles to get even a word out and she can barely lift cereal to her mouth.  In the midst of this, she is still with us and playing hide and seek under the blanket, showing everyone her newly painted fingernails and toenails and asking to go outside to see the bubbles every chance she can. 

 In February, we were not thinking in terms of time frames for Milla’s life.  But by early April, we were talking with a doctor on our team and, while no one will give dates or predictions, we were thinking at the time, “I don’t know if Milla will live past the end of the 2015 or maybe early 2016.”  By late April and after the downturn I mentioned above, we were shockingly asking if Milla would even live to the end of summer/late September.  The doctors agreed that that was not an unreasonable conclusion based on her state.  And this week, we are facing the reality that Milla is dying now.  I mean, we know it’s a terminal illness, but the term is short.  Technically, Elle is dying too, but her term doesn’t seem short right now.  The largest concern now is the Milla's drooling, which indicates the likelihood that fluid is or very soon will be getting into her lungs, leading to pneumonia.  Recovery from pneumonia is not likely for her.  All this to say, we are on the home stretch with Milla and the home stretch gets shorter and shorter than we expect.  It is obvious but, at the same time, surprising that she has an aggressive case of Batten’s.

 Ann Carlyle – She is plugging along.  She asks questions and understands ultimately what is happening, but I don’t think she grasps timeframes.  It is interesting to watch her because she will ask and talk and then in mid-sentence change the subject or walk away.  It is her way of saying I have had enough, let’s move on.  I am so grateful for the Center for Good Grief and Lauren, her counselor.  Lauren and Angela (our counselor) have been awesome at helping her understand her feelings and to talk about how she feels and they have been awesome at helping me and Dana work with Ann Carlyle.  It is a process and it takes a lot of work.  Please pray for Ann Carlyle, especially as it is likely we will be having some tough conversations with her in the coming days.

 

I have a few observations.

- When I put each of the girls to bed, I sing a song and pray and each time, I ask for healing for our girls and then ask for God’s mercy and strength to make it through each day.  Coming up on the end of May, He has provided us seven months of making it through each day.  It is very hard to ignore that God is answering our request and providing mercy for Milla by giving her an aggressive case.  I figure that I will not be thankful for this mercy at the moment she is not with us.  But He has answered us through all this so far.  He has carried us through the storm.  He will provide for us in the future.

- God is preparing Milla’s room and it seems as though He just about has everything ready.  I try to find peace in knowing that she will be healed and with Him, but it is not very comforting right now.

- All of this only reminds me of Elle’s journey.  This does not take me to a good place.

- I recently read a person’s situation of suffering and here is the comment:
  “We’re surviving. Because we have no other choice. We aren’t going to 'keep fighting.' Because that implies we have a choice to stop fighting. This is our reality.” Hay Farris 9/8/14

 I love this quote and it accurately describe us.  We take small bites, rarely more than one day at a time, and most often half or 1/3 day at a time.

- I had been thinking that as we approached the girls’ death and given the slow nature of the disease, I would have plenty of time to process and prepare for their dying.  Our parents are still living, but I remember as my grandparents neared the end of their lives, many of us around them had time to prepare for their deaths. I  have heard many people speak of death in this way or I have heard this: “at least they are no longer suffering.”  These comments are made mostly about the elderly.  I have learned and I am learning that this is not even close to the case when thinking about your children.  Things don’t work that way when it’s your children, it is very, very different.

 Final comments:

- We have been having help overnight two days each week, which has been great!  I just found out ten minutes ago that we are now qualified for 12 hours of nursing help five days a week and it looks like it will be covered by insurance!  Again, God provides for us in the midst of a storm that does not seem to ease up.  God be praised.

- Thank you to those coming to help with the girls, especially the morning servants.  They show up at 5:15am and the past two or three weeks, there has been no routine.  Some days the girls are up at 4am, some days 6:30am.  I appreciate each of you coming whether the girls are up or not, it helps us get rest to make it through the day.  God be praised for His provisions.

- Thank you to First Evan for hosting a prayer meeting for us.

- Thank you to all who are praying.  This is the single most important thing that you as people can do to help our family through this.

 

One step at a time, even if it’s only an inch or two,


Frazer

Home!

We are home!  

No breaking news for Elle - she didn't have any seizures while at LeBonheur and we are hopeful that is because her medicine is at the right levels.

Milla's congestion is a bit better and she was much perkier today.

Thank you all so much for your prayers, for your encouraging words, for sending God's Word to speak peace into my soul. 

We are pretty exhausted tonight but thankful to be home with our girls and anxious to get back into our 'routine.'

Please continue to pray that Milla's congestion clears up with no complications.  

Much love!

Dana

Here's an attempt to capture Elle's awesome post-EEG hair.  :)

Day 2

Day 2 at LeBonheur...

Not much good news to report.  :(  No seizures, Elle's been pretty agitated and just put herself to bed for a nap.  Last night, she woke up once or twice every hour, but fortunately went right back to sleep.  Unfortunately, she was waking up because she was caught in her wires and uncomfortable, not due to seizure activity.

I spoke with her doctor a couple of hours ago and he was very vague about going home today or staying.  I'm assuming we will be here another night - and really, I want to stay at this point.  As much as I want to go home, I don't want this stay at LeBonheur to be all for naught.  So, please pray for seizures!  And pray this evening and night she will do what she typically does at home...the fits, the little seizures, etc.

Also, please pray for Milla.  She has a cold of some sort - right now it's just severe congestion.  Believe it or not, this is the first time she has gotten sick in about a year and a half.  In other words, she has never been sick along with uncontrolled seizures and/or this far into her disease progression.  This is very worrisome because she has an extremely weak cough and is highly susceptible to pneumonia.  Also, infections can make seizures worse.  

So, I'm worried.

Also, I spoke to our doctor today about how Milla is doing in general (despite her cold) and he told me that she is pretty much maxed out on her current seizure medications.  Along with the increase in seizures, there is much happening with her that is her disease and, therefore, not much we can do about it.

I'm fighting a lot of fear and anxiousness right now.  Lots of fear.  I feel like I'm caught in crashing waves - like I keep getting pushed back down under water, beaten up, gasping for air only to be pushed back down again.  Please pray that this crushing fear will be driven out.  I know it is not from the Lord.  I am only human, so I know fear and sorrow will be ever-present on this road, but I don't want to drown in it - I don't want it to own me.

Thank you, thank you, thank you for your prayers and help this week,

Dana

LeBonheur

Just wanted to send a quick update and ask for prayers for us - Elle and I are at LeBonheur in a room and she is all hooked up to her continuous EEG (also being continuously video monitored).  

She was a bucking bull getting her EEG leads placed but about halfway through, she calmed down.  She’s been curious about her ‘ponytail’ of wires and her turban but she’s really been generally okay with it all.  She does keep saying, “Go,” but she’s hanging in there with her toys and videos so far and right now she is napping.  (Yay!!)

Please pray for seizures while we are here!!  I know it feels weird to pray for seizures but that’s what we need her to do while we are here.

Also pray that she stays content with all those wires, with being confined, etc.  There is a playroom we can visit that is set-up to monitor her, but other than that, we are stuck in this room.  We can’t even wander around in the hallway.  I remember worrying about this with Milla’s first EMU stay - we were here for 6 days - and she was so busy and boisterous back then.  But she really did okay…praying that Elle is okay with all this for a few days.

Thank you, always, for praying!!! 

Dana

All ready to go!

Never seen her so focused on coloring...

Selfie!