Quick Update

Hey everyone - I just wanted to send a quick update on the girls.

Elle is headed to Ohio for infusion #6 Monday with Aunt Dawny (my sister) and will be home on Wednesday.  Infusion day changed due to Thanksgiving, back on Thursdays on 12/8.  We are so thankful Dawn is able to help out.  Frazer can't make the trip and I need to stay with Milla right now.

As for Milla, she is not doing well.  Since going into LeBonheur last Wednesday, we have yet to find a good balance with her meds that keeps her happy and comfortable.  This past week, we have had her on an IV antibiotic to fight a UTI and we have changed her med schedule to getting meds every two hours and we have not seen great improvements.  She is either sleeping/passed out, upset or dealing with spasticity (involuntary tightening of muscles) and she gets upset (crying/sobbing) often.  In addition to all of this, she is not eating much, at all.  We are working hard to get things calmed down for Milla (more medication) and to find a balance so she can get back into her groove, but we have not been able to get there yet.  

Please pray for us and our girls.  Pray, pray, pray that we can keep Milla comfortable - we are having a hard time with that right now.

Thank you as always and much love,

Dana

Commercial Appeal Story

Wow - just got this link.  I think it's going to be in the Sunday paper...

http://www.commercialappeal.com/story/news/health/2016/11/12/memphis-girls-battle-rare-and-incurable-batten-disease/93416132/

So thankful for a reporter that told our story well.  :)

Milla Update

Hey guys - so Milla's ultrasound was fine (thank God!) but her UTI bug is pretty yucky so we are having to do an IV antibiotic for a week.  Thankfully, we are still going home today!!!  We will just have to manage an IV at home with her...which is fine by me as long as we can be at home. Now, if it was Elle having to have an IV at home for a week...that would be a different story!

Elle and Frazer will get home around 2:00 today.  Ann Carlyle has been staying with family here in Memphis and we'll grab her sometime this afternoon to come on home.  We have 2 LPN interviews this evening (for night help) - they will start this week if all goes well.  Please pray that the interviews go well!!!

Milla is doing okay.  She still had a couple of times over-night that she wasn't doing so well and needed extra medication...seizure/spasticity episodes.  But hopefully this will continue to get better as our new medication regimen gets settled in her body.

The doctors will be by in about an hour and then maybe we will get kicked out and home by lunch.  :)

Much love!!

Dana

What a Week

Hello, hello!

I am currently at LeBonheur, writing to you from the 7th floor while Milla sleeps.  Frazer is currently in Columbus, OH with Elle - they just got discharged about 30 minutes ago from the hospital there after a successful 5th infusion.  This is how we celebrated our 14th wedding anniversary Wednesday.  :)  One for the books for sure.  But it's all part of it right??  Sickness and health, good times and bad times.  God is good through all the times and we are thankful for our marriage, our friendship, our girls and our life together.  

AAAAAAND Milla is 6!!!!!!  There has been much to celebrate and much to grieve over the last couple of weeks.  We roll with it...1 day at a time...sometimes ½ a day at a time.

I'm excited to say that we have a couple of new people in our lives to help us out in the afternoons, and hopefully we will have a couple of new LPNs to help us out at night starting next week.  But the night situation is still iffy and difficult and frustrating, so please, please pray for this.  The LPNs are through a staffing agency, so we only have so much control there.  It's been a heavvvvvy burden for awhile now - and we are pleading with God to ease this burden.  Milla will start getting regular medication every 2 hours, for awhile at least, so we really need to have help, more so than before.

Elle's infusion went fine and she and Frazer will be flying home tomorrow. Yay!  She has not been doing good - walking, talking, seizing all going downhill.  But Frazer said this morning that she was walking (still with assistance) better than she has in 3 weeks.

Milla and I will be here in the hospital another night.  She has a fierce UTI and she will have a kidney ultrasound in a bit to rule out any kidney stones and to see if she has had chronic UTI's.  This may or may not be the cause of her downward spiral with spasticity and seizures and just not feeling good.  We are also starting her on more frequent doses of some of her current meds (so she gets a higher dose total for the day) and starting her on a new med today to see if that helps.  It could make her blood pressure bottom out though, so we are monitoring that closely while here.

Milla has not been doing well since my last update and Frazer ended up going to Columbus last minute with Elle a couple weeks ago for her last infusion because of Milla's condition.  The same thing happened this week.  I was scheduled to go with Elle and we switched at the last minute so I could stay with Milla.  So she's been down for about 3 weeks now.  Please pray that we can figure out all this mess out with her little body and figure out how to make her comfortable and happy so that she will be able to do her life pain/discomfort-free.  We know her road is only going to get more difficult but we long to make her as comfortable as possible and want her to be able to do-life as much as she can until our sweet Jesus takes her to be with Him.

Please continue to pray for Ann Carlyle as she has to continually deal with changes in her schedule and mom and dad being gone and sisters in and out of hospitals and general life up-heaval.  Bless her, it's a lot.  I know I always say this, but, truly, she amazes me.  She's such a great kid and is strong as an ox.  I don't like to throw toys and treats and gifts at her to 'make-up' for the craziness of her life (she has to learn to cope in other ways, of course), but I've got a lego set with her name all over it when we get home (hopefully) tomorrow.  :)  That kid loves legos and I love that about her.  She is AWESOMELY creative.  And silly and funny and beautiful inside and out. 

Frazer and I are doing okay...as always, it will be good to get everyone under the same roof soon.  I think we are running on adrenaline and manna.  God is sustaining, as only He can do.  Thankful for all the ways He is showing up.

Oh - side note - many people have asked how many times we will be going to Ohio with Elle.  She has to get this infusion every other week forever...it is a treatment, not a cure.  In a way, it is like a diabetic needing insulin.  She doesn't make the enzyme she is supposed to have in her brain cells (like a person with diabetes doesn't make his/her own insulin) so she has to continually get the enzyme as her body uses it up and needs more (like getting insulin).  We are praying and hoping that soon, the enzyme will become FDA approved/commercialized so that she can get it infused in Memphis or somewhere close to Memphis.  It is, of course, a complicated process.  But until then, she will always have to go up to Columbus every other week.  

I will try to send another update when we know more about Milla's kidneys. 

Thank you for every, every, everything!

Much love,

Dana

Happy Birthday, Milla!!

Milla is 6 years old today, y'all.

Happiness and thankfulness :)
Dana

Happy Birthday, Frazer!!

Hey there!  I can't believe it's been so long since I last updated everyone.  Time flies, right?

Elle has had her 3rd enzyme infusion - Frazer took her up to Ohio a couple of weeks ago.  All went well with no complications.  Unfortunately, she herself hasn't been doing great...her drop seizures have been nearly constant most days, she is super agitated and is struggling with her walking and talking.  But we have had a couple of better days thrown in there - she has seized less and has not been as agitated.  

Frazer and Elle had a good time in Columbus together and stayed for a couple of extra days so that they could cheer on the Nationwide Children's Marathon, where one of the precious little girls with Batten Disease was celebrated at mile 22.  They hung out with a bunch of other Batten families and friends of those families and the professionals that help them (doctors, nurses, therapists, teachers, principals, etc) - it was very good for their souls.

And here we are already about to head back up to Columbus for infusion #4!  Elle and I are flying up tomorrow afternoon.  I'm so thankful for this opportunity to help our baby girl.  It's been pretty hard - all this travel - but we all daily give our lives for our children, right?  We do it with a happy and thankful heart.

We've seen some behaviors with Ann Carlyle that we think show that she is struggling a bit with the travel as well.  But, she is a very open kid and we have lots of conversations with her about everything and like always, she is a champ and doing well overall.

Please pray for Columbus and Ann Carlyle and Elle and especially for Milla today. 

Milla is having a very rough time and we are having a tough time making her comfortable/figuring out what to do for her.  The last 2 days, her little body has been in a tight ball - every muscle flexed and trembling.  She has been anxious most of the time.  Sometimes 'just' looking worried, sometimes whimpering, sometimes sobbing.  We've seen this before - lots of similar episodes but we've never seen her body do this 99% of the time for 2+ straight days, despite all kinds of medication for seizures and spasticity.  

I do feel like for a few hours this morning she was a little looser and perkier but she is all drawn up/locked up again now.  I just want to help her, to make her comfortable and happy and we just can't figure out this mess.  She can't tell us what is wrong or what she needs, so we are left guessing.  I hate it.  I'm so thankful though for the times in the last couple of days that we have been able to get her smiling and even giggling, despite what her body is doing.  My heart is happy seeing our Milla-willa shining through it all.

On a good, great, awesome note, today is Frazer's 39th birthday!  Holler at him if you can today - he deserves to be celebrated.  :)  He's the best and we love him so, so, so much.  

And whenever Frazer's birthday comes around, we have Milla's birthday peeking around the corner.  Next Wednesday, November 2nd, our middle baby is going to be SIX years old!!!  

Thank you to all who came to pray for us at the service last week and for those that prayed absentia and for that matter, all of you that are praying so often for us!!!  We can't believe all the wonderful people that God has put in our lives in all different ways.  Amazing, just amazing.

And we are so, so thankful for God continuing to make connections for us in Columbus - it is truly becoming our home away from home.  There are big, amazing things going on there.  People are giving so, so generously of themselves to specifically help us and the other 4 families that are in this extended/open-access enzyme program.  I'll have to fill you all in another time about this, but I'll just say that needs that we didn't even know we had are being met left and right!!

I'm glad to end on a happy note.  I hate having bad news every time I send an update out.  But God is always present, always showing up - sometimes in such unexpected ways, sometimes in big ways, sometimes in small ways.  As tough as things continue to be, I am always reminded of how much He loves me and my little family.  The chronic nature of this disease and the grief that comes along with it is exhausting and can be debilitating at times.  Praise God for giving us hope - Jesus.  I pray that you all experience His love and presence as well this week.  

Good reading awhile ago...can't remember where I read it...but it's good...

"...if He can give us peace that passes all understanding, our lives and struggles might also pass our understanding on this side."

Stay the course everyone. 

Much love,

Dana

Ohio - Part 2

Hey there, everyone!  I wanted to send a quick update to tell you what we have been up to the last few days.

We flew up to Columbus Wednesday evening so that Elle could get her 2nd enzyme infusion Thursday morning.  She did great - no complications at all!!  Again, we have seen her walking improve a tid bit and her seizures are down a tid bit.  This is super encouraging, but we are still a long way from knowing how this will all play out.  However, we are so thankful and cautiously optimistic!!

As most of you know, we took the whole family to Columbus this time.  Milla was seen by our neurologist up there to just check in and to see if there needs to be any changes to her meds.  Because Milla has had these severe seizure-like episodes lately, her doctor wanted to admit her for a 24 hour EEG while we were there.  So Friday, just before lunch, Elle got discharged and we got the call that Milla was being admitted for the night.  Crazy-town.  So we trekked down to the cafeteria and enjoyed a few minutes of freedom and then went back up to be admitted.

Of course, Milla didn't 'perform' like we needed her to do (so that her doctor could distinguish if she was having seizures or if she was just tightening up, etc).  But she did have a super mild episode that was similar to her severe ones.  The episode that was captured was not a seizure, but rather involuntary movement.  This is basically another indicator of her brain degeneration...that her brain has been damaged.  We are not sure yet how we are going to treat this but we have a couple of plans to try out.  

We just want her to be comfortable and happy without making her too lethargic.  And she has been very uncomfortable and much more troubled lately, so we have to figure out something.

Because of Milla's admission, we had to stay an extra night in Columbus and got back home today around noon.  We were glad to have everything done while we were up there, but for the love, we are glad to be back home.  Frazer and I haven't really slept well lately even at home but especially not for the last 4 nights.  Argh.  And everything is just so constant when we travel, especially with all 3 girls. Constant, exhausting, draining.

It was good for Ann Carlyle to get up there and see the process, the hospital, etc. even though much of the time, she was bored to tears.  She had her moments but mostly, she was super sweet with her sisters and super helpful.  

We got together with another Batten family last night in Columbus and a few other families in their neighborhood they are friends with.  It was really, really wonderful.  A welcoming and warm bunch of people that were super helpful and loved on us all a good bit.

We were also able to visit with a couple of other Batten families that were in the hospital the same time we were.  Precious families, precious children, precious time with them.  Please, please pray for the Bowman family as they lost their little boy, Titus, last Sunday and then had to fly out to Columbus (from California) so that their other little boy, Ely, could have his brain shunt surgery Friday (and will have his first enzyme infusion on the 11th).  Oh how the brokenness of this world weighs so heavily, even as we rejoice that Titus is whole and with Jesus now.

I've had a sinus infection/cold/something since Thursday that is just getting worse - feeling pretty crummy (ps I hope this update is making sense as my head is kind of fuzzy right now) - so please pray that sleep will be the best medicine tonight and I will wake up refreshed and healthy (or at least healthier) tomorrow morning.  And please pray that no one else gets this!  So far, so good!

Ann Carlyle has fall break this week but the other 2 will have their regular schedule of schools and appointments.  I'm not sure I'm quiiiite ready to face the week, but we put one foot in front of the other and we will get through it.  Thank God for His sustenance.  

I'm so humbled and thankful for all you people that think of us and pray for us and love us so much and so well.  Y'all are the best.  :)

Much love,

Dana