Monday, March 12, 2018

Spring Break Update

Hi there to all of you!

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

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

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

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

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

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

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

We are truly fearfully and wonderfully made.  

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

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

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

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

Saturday, January 13, 2018

2018

Hello!

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

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

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

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

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

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

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

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

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

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

Whew.  It's a lot.

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

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

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

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

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

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

Much love as always,
Dana

Tuesday, November 21, 2017

Happy Thanksgiving Week

Quick update from Frazer:

As our family is plugging through November with many mixed emotions, we wanted to share a few things briefly with all of you:

1. If any of you have 40 minutes and would love to hear one of our favorite interviews, please take the time to listen to this piece. It is very well done and tells a larger part of our family’s story and history than just Battens.



2.  As you will remember, Dana and I were able to go to California for a few nights in August. The trip was amazing on many levels. The main reason for the trip was to see the premiere of The Race for Brineura which BioMarin put together. It is amazing and was released to the public last week!  It’s about 30 minutes long and amazingly well done!  Elle is in it also and the movie really captures the essence and heart of BioMarin’s passion for their work.




3.  We are working to get a head start on planning for the 2nd annual Miles 4 Milla’s House which will be on May 19, 2018 at Wiseacre Brewing Company.  Last year was a huge success and we got it together in less than 60 days!  Since we have more time, we are working to gather sponsors. If you or someone you know would be interested in sponsoring the 2018 event in May, please email Frazer at millashouse1@gmail.com.



We hope you all have a wonderful Thanksgiving and we greatly appreciate your prayers and support that you continue to pour out on us, especially during this difficult holiday season without our Milla-Willa.

Fraze and Dana

Thursday, November 9, 2017

Infusion Day and 15th Anniversary!

Infusion 31 and our 15th wedding anniversary today! Last year on this day I was actually at LeBonheur with Milla and Frazer was in Ohio with Elle and Ann Carlyle was staying with family. It was a hard day. A hard week, a hard month, a hard season, a hard year, I could go on and on. It’s still really, really hard. But I’m finding myself thankful too that we are able to be in Memphis together this year and that Milla is with Jesus. My heart feels swollen with love and it feels achy and heavy and broken. Where would I be without my God? He loves us so much. Y’all - bind your heart to His - sit in His love. Praise God He has already bound you to Himself. 

Monday, October 30, 2017

October Update

Hey there!  I'm going to dive right in!

Frazer turned 40 last week!!  He had a great day and we were able to celebrate him well with a a bunch of friends that night.  I'm so thankful for him.  He is truly my best friend, he is my rock, he is my heart.  I love him so.  :)  Our 15th wedding anniversary in a couple weeks on November 9th...so, so grateful God brought us together.  We've been through a lot during those 15 years and I can't imagine not having Frazer at my side living this life with me.

Elle's med situation is going GREAT with her gtube.  She tolerates it well and she (and we) are so, so grateful to have Bob the (gtube) Button taking the medicine instead of Elle.  It has been life-changing for sure.  And shockingly enough, she doesn't try to pull it out - she only is aware of it occasionally when her hand brushes over it and she will absentmindedly fiddle with it, but other than that, she leaves it alone!  

After Elle's gtube placement, we noticed a decrease in her seizure activity as her drug levels stabilized and her abilities kind of leveled back out to her 'norm', appetite came back, etc.  But then she got a sinus infection and was down for the count for a good 10 days.  That was not helpful.  She is healthy now but the frequency and duration her seizures still seem to be on the rise...even more so this weekend and today even though she just had an infusion last Thursday.  Not sure yet what we are going to do about that.

The good news is that cognitively, she is doing really, really well.  We see it at home and her teachers see it as well - an understanding of things that we haven't seen before (or in a really long time).  But just like with everything with this disease, it's complicated.  With her increased awareness of herself and the world and what's going on, it seems like she is struggling - like she gets frustrated because maybe she can't do what she wants to do or what she used to be able to do or what she sees other kids doing.  Her awareness of these things makes her sad or mad and/or belligerent.  And that's hard.  

One of the other moms who's son is on this enzyme-replacement therapy said it well earlier today - there's forward movement and backwards movement all at the same time.  And it's so very hard to process and deal with that and we struggle so much with trying to figure out what's going on with our kids and trying to fix the problems the best we can.  It's heavy and sad and hard and exhausting and it just beats you down.

As I write this, my heart feels raw and broken.  I struggle.  But I also know my Jesus lives.  I know He loves me so much that I can't ever even fathom the depth.  I know that He is carrying me and holds my raw, broken heart in His hands and grieves with me over the brokenness of Elle's body, over the loss of Milla.  I have nothing if I don't have Jesus.  

We are entering into the Christmas season in all it's crazy glory.  Be still and know that He is God.  Remember and celebrate and be in awe with me that He came to earth so that we might have true life.  

Milla's birthday is in 3 days - Thursday, November 2nd.  She would've been 7 years old.  She was doing great last year on her birthday, but just 3 days later she began to decline.  As this week passes and as the next 3 weeks come and go leading up to the 1-year anniversary of her death on November 26th, I feel (and remember) fear and sadness and anxiety and such loss.  Our grief over Milla flows into our grief over Elle.  It all runs so deep and is all so complicated.  Please pray for us as we enter this season.  

Also, we would love it if you celebrated Milla's birthday with us Thursday by enjoying doughnuts from your favorite doughnut shop.  Milla LOVED doughnuts and that's what she always wanted for her birthday treat.  :)

There is so much to grieve over but yet much to be thankful for as well.  As Milla used to say all.the.time, 'Pray!'  She would pray without ceasing, especially during meals, and she would point to all the things she was thankful for...the lights, the table, the food, the ceiling, the chairs, the people at the table...things we take for granted every minute of the day, yet she would remember to be grateful.  God used her, and continues to use her, in mighty ways.  Don't ever underestimate a life - no matter how small - God's precious image-bearers that we are.

Praise God, from Whom all blessings flow!  Praise Him all creatures here below!  Praise Him above, ye heavenly hosts!  Praise Father, Son and Holy Ghost!

Much love and thankfulness for you!
Dana

Wednesday, September 27, 2017

Elle's Surgery Update

Rough night. Elle had lots of breakthrough pain, a little fever, heart rate up, o2 sat fell for just a bit so she had to have some binasal oxygen for a few hours. She's off it now and her heart rate is still a bit high (130s). Fever is down. She just ate a little and drank a little. 👍🏼So she's doing ok but jury is out on discharge plans.

(Lots of rational and 'ok' reasons for all these things but keeping a close eye on her.)

Sunday, September 24, 2017

Hello Again :)

Hey there, everyone!  It's been awhile.

We have had a busy couple of months - some of which has been no big news but busy all the same.

On August 10th, I turned 40.  And my precious family and friends planned a month full of celebrations for me that included a weekend trip to the lake with my amazing sister and a bunch of my utterly wonderful and hilarious Memphis girlfriends, a trip to Birmingham for another girls' weekend with 2 of my oldest and dearest friends (also hilarious, by the by) and then, Frazer and I were able to take a trip out to California together to visit Biomarin (drug company) and participate in their official launch party for Elle's enzyme drug, Brineura.

All of it was AMAZING.  I've had a ton of health issues the last 6 months (one of which is my thyroid, which of course, affects every.thing) but the month was awesome and it fed my soul, which gave me energy and emotionally, it gave me a little boost too.  Most likely, my body said 'I'm over it' because of the chronic stress of our lives for the past 4 years but trying to piece it back together has been difficult.  So I wasn't sure if I would be up for all the traveling and celebrations but it seemed to be exactly what I needed.  THANK YOU to all of you that gave me the best 40 year celebration month a girl could ask for.  It was super fun and super special and super soul-filling.  We cried together and we laughed even more.  I can't thank you enough for sticking with me, especially the past 4 years.  You have given so much of yourselves and sacrificed greatly - all with joy and with no expectations.  

Our trip to Northern California was just ridiculous.  It was so good for both of our souls to be out in God's beautiful creation, to be away from everything, to be able to have 5 days of being on an awesome vacation with my best friend.  Our marriage thanks you, California.  It's no secret that the majority of people in our situation or in a similar situation get divorced.  Marriage is hard work in 'normal' circumstances (whatever normal is).  And we have to work so stinking hard to keep our relationship strong - we have had to (and continue to) learn to lean into each other when we least feel like it.  We have had to figure out ways to connect when we feel like ships passing in the night.  Thank you for continuing to pray for our marriage.  From the bottom of our hearts, we thank you.

Also exciting news...this weekend, we celebrated that Ann Carlyle has a brand new heart in Jesus.  :)  Thank you, Jesus, for making her Yours!  There is no other celebration in this life greater than this!  (Heart exploding with gratitude.)

Much of this email is written in machine-gun style - just trying to hit the highlights quickly because Elle's kicking the keyboard and wants to be IN my lap with my arms around her (sitting beside me nestled in isn't good enough), the washer just beeped that it's finished, I have a to-do list 2 miles long, etc.  (Another reason it's been nearly 2 months since my last update.)

Speaking of Elle and how she is doing - well, it's complicated.  She has been having some hard times.  But there are also times of victory in her cognitive ability (she was able to understand and actually accomplish the command to 'go around'!! Things like this are amaaaaazing!), and there are times of victory in her balance and core strength.  The complicatedness is that none of it is consistent.  Her speech and her ability to take assisted steps have both declined a good bit the last couple of months.  And the last couple of weeks, we have seen a weakness in her core that we haven't seen before.

The biggest hurdle we have had with her in August and September has been her meds.  She was choking a lot on the meds and then that seemed to get better for a couple of weeks but then she stopped complying with taking them.  She has been spitting them out and we have tried every trick and maneuver in the book but we are still lucky to get a third (if that) of her medicine in her on any given day.  It has been absolutely awful, utterly stressful and heart-breaking.  And because she isn't getting all of her meds, her seizures are getting worse and worse and we are risking an infection (from aspirating) or a really bad seizure that could do permanent harm.  We are seeing a consistent decline of every ability now that she's having more seizures.  Also, her appetite has decreased and she's been fussier (no surprise there).

So, we have made the decision for her to get a G-Tube (feeding tube) placed.  She will only need to use it for her meds as she is still able to eat just fine.  And we are hoping that once we are able to get her drug levels back up to par and leveled out, her abilities will come back a bit, seizures will decrease, appetite come back, etc.  It's been a particularly emotional few days as we are processing this place we've gotten to with Elle.

Her surgery will be this Tuesday and we will be there for 24-48 hours depending on how she recovers and then she will have her regularly-scheduled infusion Thursday morning.  Please pray for her this week.  The surgery plus infusion back-to-back will be very taxing to her body but we just don't have any other choice.  Please pray that we make it to Tuesday without her getting sick and with no harm coming to her.

Thank you for all your support and prayers!!!!  Saying it a million times doesn't do it justice.  Broken record and I don't care.  What a village you are!!!  You help us get through these long, hard days.  Whether I know you or don't know you matters not.  God has provided for us richly through YOU.  

Much, much, much love!!
Dana
(PS - Any typos and lack of writing ability I attribute to Elle's right foot)