Happy Birthday to Elle!!

As crazy and fast and busy as life always seems to be, there's always room for it to get even crazier and faster and busier, right?!

I'll jump right in...

We celebrated Elle's 4th birthday last week!!!  We are so thankful for that spunky firecracker/tornado!  :)  We had a big (huge) princess bouncy house party for her Saturday and the weather was perfect and she LOVED it.

The crazier part of life comes in with how the girls are doing.  Unfortunately, it's not good news.

Elle's balance and falling and seizures just keeps ramping up.  It's super hard for all.  I'm not sure if we are going to try to combat it with meds yet at this point or not.

Milla's bounce-back has pretty much leveled out and she is regressing a bit.  I'm pretty convinced though, that part of it, at least, is due to her getting sick.  She started getting a chest/head cold Friday afternoon and it's still going strong.  Her lungs sound terrible but she is coughing productively, which means she is moving all that junk up (and totally out hopefully).  She's been tired and clingy and weaker.  But she's hanging in there.  She is truly Mighty Milla!!

Also, I have the same cold and I am feeling pretty yucky myself.  Please pray that this infection gets outta this house asap!  

This is the second time in a row I have written under the influence of drugs...eek.  Hopefully, this time I didn't misspell something like 'route canal' instead of 'root canal'.  (Eye roll)  

So I'm keeping it super short again - just wanted to keep everyone in the loop!  Please pray for good health!

Much, much love!!

Dana

PS - I'll try to post some pictures soon

April!

Hello friends!

So sorry for the lapse in updating - Frazer had written one but we decided not to send it because things are a bit different for now.

Milla is doing much, much better!  She has had a huge surge of energy, alertness and an overall decrease in tremors and seizures!  We increased her depakote just a little bit a couple weeks ago and it is making a big difference - one that we didn't expect at all.  We are so thankful for this time with Milla!  

Elle is about the same - falling a lot and seizing a good bit.  Her rages seem to be on the rise again as well.  It's been pretty hard to watch.  We are having her fitted for some spring-loaded ankle braces that will hopefully help her bring her toes back up while she is walking/running and ideally, this will help her not trip so much.  We did increase one of her meds - an effort to better her sleep.  We will give it a few more days to see if it works.

Monday, we went up to Columbus, OH for an appointment at the Center for Batten Disease and met with a myriad of specialists.  No big changes - mostly, confirmation of what we are already doing. Thank you to those that helped us get up there and back home so quickly and kept Ann Carlyle while we were gone!!

Last week was Ann Carlyle's 7th birthday!!!  She and her friends went to the planetarium for a show and then we moved the party to our house for doughnuts.  She is so interested in stars and space and all right now.  Space and legos are her obsessions.  :)

Next Wednesday is Elle's 4th birthday!!  I can't believe our baby is turning 4.  Until last month, she still looked so much like a 3yr old to me but then she had a growth spurt and her legs are all of a sudden longer and she has transformed into (almost) a 4 year old!

So, I had a root canal this morning (not cool) and I'm still somewhat woozy and the pain is starting to kick in as the numbing wears off so I'm going to wrap this up but I wanted to keep everyone in the loop! (This is the choppiest update - I'm not very with-it!)

Much, much love to all!!

Dana

Thursday Update

Hey everyone - so I really didn't feel like writing this week because it's just so hard to write the yuk and to actually see the yuk written out.  But on the other hand, I want to keep everyone updated and if I wait too long, I'll get too overwhelmed.  And then 2 months will pass.  :)  

So this will be relatively quick.

It's been a rough week.  Easter weekend was really rough.  Bottomline, we are seeing more and more progression with both Milla and Elle.

It makes my stomach hurt.  It makes my heart hurt.

Funny thing is that I still know and feel God's love and I have joy in Him.  Truly.  And that is supernatural, my friends.  I mean, how real is my relationship to Jesus if I don't believe Him when it hurts?

I read One Thousand Gifts this past week.  Found a lot of truth in there.  Here's one of those truths that struck me...

"All fear is but the notion that God's love ends."

I am choosing (most of the time) to not be fearful - but also I know that it's okay to be sad and grieve over my babies.  2 different things.  Joy is not an emotion that is 'instead of' but it is an abiding thing.  Found in Jesus alone.  Without Him, my faith is gone in a moment.

God's love never, ever, ever ends.  Therefore, what do I have to fear, really?  I have a lot of sadness about a lot of brokenness going on in our world and outside our world.  But I also know that God will make it all okay and one day, my girls will be made whole.  He has already won.  It is finished.

Thank you for everything.  Always.  Our gratitude for you is immeasurable.

Dana

Easter Sunday - extra hugs and snuggles needed

I had a lunch-date with Ann Carlyle and Milla on Monday - we had such fun :)  Her smile lights up the room.

Milla excited about seeing Ms. Melba - her one-on-one teacher at PDO - oh how they love each other!

Mostly Much of the Same...

So I may just start doing the date as the subject line.  Boring but coming up with a title is brutal.  :)  Not my thing.

Anyway, quick (I think) update...

Not much has changed.  Milla's tremors are still super bad.  We had our appointment with the girls' neurologist a week ago and also got an EEG last Wednesday to make sure she was not having a ton of seizures.  Sure enough, her tremors and muscle contractions are due mostly to her disease and not seizures.  As I've said before, this is pretty hard to process (not that seizures are good) because this confirms that her brain is further degenerating and her disease progressing.  We are entering a new phase of this with her.  Disheartening to say the least.

We are increasing Milla's valium, with hopes that it will calm some of the tremors, especially at night, so that she might sleep better.  Her whimpering at night has been a bit better, but she is still waking up a lot.  And some mornings, like this morning, she is up at 2 or 3am for the day.  Lack of sleep can exaggerate all of her symptoms, so, of course, we want to combat that. 

We are also adding another drug she has been on before to help decrease her oral secretions.  She has been choking, mostly between meals and at night because she can't manage her saliva...hopefully, this will help without causing pooping issues, dehydration, etc.

Another way we see progression is just her body not being able to handle stress.  Sunday night around 10pm, she woke up with a massive nosebleed.  It took a bit of time to stop it but all in all, it was fine (the nosebleed part).  However, it took about 2 hours and 2 extra doses of valium to get her body to relax enough to go back to sleep.  Her whole body was contracting and shaking.  It was awful.  

Also, Elle continues to check out and seize and fall and get bruises and bumps.  She's about the same as the last time I updated, I guess, but we have since increased her depakote (seizure med) a good amount.  Just like with Milla, we feel that we are moving into a new phase with Elle and we are trying to adjust to this new norm.

Let's see, what else...

Atlanta was great - exhausting but it was so good for my soul to be with my sister for a couple of days and we were glad Lucy and Ann Carlyle could be together too.  :)  By the by, if you ever have the opportunity to go to the aquarium there, you should go!!  It is wonderful!!

Oh and I have had 3 consecutive nights of good sleep!!!  I am feeling sooo much better.  Still processing and dealing with very hard things, but I feel more stable at least.  Oh how I love sleep!!!

Praying for a meaningful weekend for us all, as we reflect on and celebrate our Jesus, Who died on the cross for our sins and rose from the dead!  How amazing.

As usual, songs fill my soul...and these words struck me especially deep this past week from How Deep the Father's Love For Us...

"How great that pain of searing loss,

The Father turns His face away."

Oh how He knows our pain.

And this...

"I will not boast of anything, 

no gifts, no power, no wisdom,

But I will boast in Jesus Christ,

His death and resurrection.

Why should I gain from His reward?

I cannot give an answer.

But this I know with all my heart, 

His wounds have paid my ransom."

(The whole song is wonderful and beautiful.  I encourage you to look up the lyrics and/or listen to it.)

He is alive!!!  Much love to you all!

Dana

Cozied up with Milla for her EEG

Milla getting some hugs and love from Sissy (Ann Carlyle) - how I love this picture!!!!

Ann Carlyle, Auntie Dawn, Lucy and Dana at The Georgia Aquarium

Rain, Rain, Go Away!

So we are on day 2 of rain, rain and more rain.  And it's not stopping anytime soon.  But just like last time, we had a beautiful few days before the rain came and we soaked it up.

It's been a rough couple of days for Elle.  She has scrapes and bruises all over her arms and legs from the last week or so.  She had an accelerated drop last night which resulted in her face-planting into the hardwood floor and a huge goose egg on her head.  She was on her hands and knees (relatively close to the floor already) but the seizure had such force behind it, it slammed her head into the floor so hard.  Then about 15 minutes later, she had another accident on the same side of her face on her cheek which bruised up pretty fast.  But fortunately today, her face and head look much, much better with surprisingly little bruising.  

It all is just so terribly familiar from going through this phase with Milla.  That's been really hard on both Frazer and me lately.  We generally know how this is going to play out and it's not been easy distracting ourselves from dwelling on that.

Elle's behavior has been okay overall, but there are times of each day that it gets bad and she is miserable.  We see her neuro Monday...not sure if we will change up anything but it will be good to have her looked over.

Speaking of neurologists, we are going up to see the Batten specialist neuro in Columbus, OH, mid-April.  She had wanted to see the girls every 6 months or so to lay eyes on them and check them out.  We will meet with a whole team up there like we did last fall to brainstorm ways we can manage Milla's and Elle's symptoms.  Just wanted to put that on your radar to pray for that trip.  

What else...

Elle is still taking her meds well!  Praise God for this mercy!

Milla's tremors and myoclonic seizures are getting worse again, despite the scheduled valium.  But we have also weaned her totally off of one of her other seizure meds.  I was really hoping she could stay off of it, but I'm not sure she will be able to do that.  Today her tremors and seizures have been especially bad - constant and fairly large.  

In one way, Milla's nights have been a bit better with the crying...it's been a little easier to calm her down and get her back to sleep...but she still has a crying episode pretty much every night.  And her overall sleep is not great, although she'll have a good night a couple times a week.

Speaking of sleep, I've had a terrible time of it the past couple of weeks despite taking ambien.  My doctor put me on the slow release ambien as of today, so hopefully that will work.  My body has been physically hurting and emotionally I have been much more sad and anxious.  (And if the lack of sleep isn't causing that, it certainly isn't helping the situation!)  Please pray this ambien will work and sleep will return.  And pray for Frazer's sleep as well.  He generally never has an issue falling asleep or staying asleep - he is one of those people that falls asleep the second his head hits the pillow - so jealous of him.  But he's had a couple of nights of bad sleep lately.

All the girls are on spring break this week and we are surviving, despite not going anywhere fun and despite the rain.  (Much of the survival is due to the sweet people helping me this week!  But really, I could say that about every week!)  And tomorrow, Ann Carlyle and I are driving to Birmingham and on to Atlanta with my sister, Dawn, and her daughter, Lucy.  We will stay in a hotel one night (which is half the fun for the girls of course!) and go to the aquarium.  To say Ann Carlyle has been excited about this trip, is a vast understatement. :)  I'm hoping it will be a special time for us together - and of course, it will be super fun to be with Dawn and Lucy!  (Pray for Frazer's survival this weekend - and thank you to those helping him while I am gone!)

Oh - and will you please pray for us as we are trying to decide the best route for Elle with preschool and therapy?  I think she could really benefit from as much school and therapy as she can get; however, I don't want to over-do it and I also don't want to lose time with her.  But I worry that while caring for Milla, I cannot give Elle the attention and teaching she needs to maintain her abilities.  (And the reverse is true with Milla as well.)  They both need intentional interaction - not just me trying to make it through the day.  There are some potential big changes and decisions up ahead - just needing some wisdom and clarity with this.

Thank you for all the encouragement and help we continue to receive - the faithfulness to care for us in so many ways is always humbling and we are so, so, so grateful.  

I pray that we and you would remember and store away in our/your hearts the beauty of Jesus and His death and resurrection.  His beauty is so much brighter than the ugliness of this disease.  His love is profound.  Thank you, dear Jesus.

Much love to all of you!

Dana

The injuries :(

Ann Carlyle at the zoo with her friend this week

Elle-belle

Milla-willa and her untamable hair enjoying the great outdoors before the rain came :)

Chilly No More!! (For now at least...)

Hey there!  I'm so excited to report that we have had at least 3 days of BEAUTIFUL, sunny weather!  As my friend put it, I am solar-powered.  :)  Some sun on my face and being outside is just what the doctor ordered!  I know rain is on the way tomorrow, but I am hoping that spring has truly sprung.  But you never know with Memphis in March - March is when we have had ice storms and tons of snow and all that.  As Elle says, "GO-way wain!" (Go away, rain!)

So Milla has done pretty good with the scheduled valium - maybe a touch more tired but not much - and her tremors and/or seizures are better!  

Last night, she woke up crying around 10:30pm and cried for about an hour and at one point she got out the word, 'help.'  Totally heart-breaking not being able to fix it.  Her breathing was fast and labored for a bit but recovered after awhile.  I feel like these times of discomfort and sadness, although few and relatively far-between, are becoming more frequent.  And I so wish she could tell me what is wrong and what she needs.  Please pray that we are able to comfort her and that she will not feel fear or sadness or pain.

Elle had a not-so-great week but the sunshine and outside air did her good over the weekend.  During the week, her rages (crazy fits) were 24/7 (or close to it).  I felt like an abuse-victim of verbal and physical assaults at the hand of my pre-schooler.  It was so discouraging and stressful and it definitely had caught up with me by the end of the week.  I felt utterly exhausted, beat-up and so worn down emotionally.  But fortunately, she calmed down over the weekend, although yesterday, she had a TON of 'checking-out.'  Again, not sure if it is seizure activity or lack of brain connectivity, but they were split-second episodes that were literally every 5-10 seconds pretty much all day.  We hate seeing her like that - goes without saying.  Please pray that we will know how best to treat her and help her. (Oh and on a super-encouraging note, Elle is still taking her meds well!!!!!)

I truly do feel like we are all a bit refreshed from the sunshine and playing at the park this weekend.  Ready to face-down another week!

Thank you for EVERYthing, dear friends and family!!!

This has given me hope and comfort this week:

"Keep the broken-hearted sure,

Clinging to Thy cross, our cure."

We cling to Jesus.

Dana

Ann Carlyle and Elle running around at the park :)

Chilly Monday Update

Good Monday morning!  It's chilly and gloomy here today so I'm glad to be done with some of the morning's errands and can hole up with Milla until I have to go pick up Elle from her little school.  

Milla has homebound school right now, so I have a minute to send an update.  And speaking of, I just have to say what a blessing her teacher has been.  Homebound school is a program through the city school system to provide education for kids who have special needs and who can't attend a regular school.  Milla's teacher is phenomenal. She comes twice a week for an hour on Mondays and Thursdays.  She is so dedicated to the kids she teaches and she cares for Milla so much.  These sessions help maintain Milla's skills as much as possible - and she absolutely loves it - so it's a happy part of the week for me too.  :)

So it's been a tough week, honestly.  (Although yesterday both Milla and Elle had a great afternoon - probably because Nana and Granddad came to visit!)  We thought that Milla's tremors were getting worse, but it turns out much of that activity was actually seizures.  So we are changing up her meds again...playing with the dosages of her current meds and adding scheduled valium.  I've been pretty worried about the valium but so far she's done okay with it.  We are upping the dose (of valium) and weaning back another med today, so time will tell how her body will handle that.

Her seizures have pretty much been under control for awhile now, so it was a bit of a blow to hear that her meds weren't working as well anymore.  It just chips away a little bit more of my heart.  I know things are always progressing and that Milla's and Elle's brains are broken but some days it is harder to deal with than others.

And Elle continues to fall and trip and lose her balance and we are definitely seeing drop seizures now.  (The rather violent ones that throw her body around.)  The seizures are all of 1 second long and fortunately, she is only having around a handful a day, but that number will continue to go up and it's just ugh.  We feel that she isn't having enough to warrant more medicine yet - we are going to wait it out for now.

Oh but one awesome thing I've been excited to tell y'all about is that Elle has been doing GREAT at taking her meds the past week/week and a half!!!  Thank y'all for praying and praise the Lord!!!  I can't tell you how wonderful this is and how much this affects my day (as she takes meds 3x a day) and my state of mind.  It's sooooo great.  Pray that this continues!

Ann Carlyle is doing well - I've said this before, but I'll say it again - her school (Christ Methodist Day School) is just plain awesome.  Her teacher is amazing.  She had a President's Day Program Friday with a couple speaking parts and she nailed it.  :)  We are so, so thankful for the opportunity to have her there.  And her eating continues to improve so thank you again for the meals!

Elle, Ann Carlyle and I have been dealing with colds this past week but we have all turned a corner and are on the up and up.  Amazingly and thankfully, Milla never got it.  I saw on the news the other day that the flu is apparently alive and well, so please pray for everyone's health, but specifically Milla, as she is the most medically fragile.  (Although, that girl is probably the strongest one of all 5 of us!!)

As always, I am ever grateful for you, our village!

Much love and coziness today!

Dana

We had a super warm weekend and Milla had an incredible burst of energy yesterday - check out this picture from the park with Uncle Kent!