Wednesday, July 20, 2016

It's Soooo Hot

So we are staying inside or in the pool (which all 3 girls LOVE) for sure.  It's ridiculously hot.

Unfortunately there's nothing really positive to report on Elle's condition.  She is still losing skills and losing words (she couldn't remember the word 'up' yesterday).  It's so hard to watch these things disappear.  I know it goes without saying, but it's just  really hard.

Also, we are seeing more disease progression with Milla. She seems to be losing her vision...not sure how much she has lost but I think a good bit.  And I think her dementia progressing.  She is having large emotional swings, including sobbing episodes that just break your heart.  This is a stage we have dreaded so's hard to see her confused and emotional with the darkness (visually) closing in.  Our prayer is that she will be able to be comforted, that she will still feel joy and happiness and that she will continue to know us.  (So far, all these things are still happening.)

Praise God for a good trip to St. Louis!  Thank y'all for praying for us and this trip!  It was a miracle...the girls all traveled well and slept well!  Ann Carlyle had a BLAST - she made some good friends and got to go to the City Museum and Grant's Farm and a Cardinals baseball game.  

Frazer and I also had a good time.  It was not as stressful for me as last year - amazingly even with the girls with us.  There were still times I felt overwhelmed and I came home exhausted but we had wonderful time with other Batten families and we came away with updates on the latest research (it's amazing what they have done in the last 2 years - HUGE strides).  And a big, big thank you to those that made this conference happen, from the BDSRA staff to the doctors to the researchers to the volunteers that cared for all the children...what amazing hearts you all have!  We are grateful to be a part of such an amazing community.

In other news, Frazer started a new job last week.  How about that for crazy?!  He is excited for the opportunity and we are blessed that his old place of employment was so, so wonderful to our family and we feel that the new place will be as well.  Please pray for his continued success and satisfaction with his job.

Things continue to ramp up as we are starting to do more therapy with Elle (speech, occupational and physical) and as we have school starting before we know it and some other changes here and there.

Oh and thankfully, I think we have settled into a new crew of CNA's to help us at night.  There will be 3 regular people each week.  There is a bit of adjustment and learning curve for everyone but they are doing well so far.

I feel so scattered writing this today - interruptions by a couple little girls do not make for good writing. But let's be honest - really, there aren't many moments that I don't feel scattered.  :)

God is good and His love is ever-present.  Thank you for being a part of that as He continues to use you to encourage us, hold us up and love us.  We praise Him in good times and in bad times.  For He holds us close to Himself during the storms.  What a Savior we have.

Much love,

Ms. Melba came for another visit!

Elle with Colson - cousin love :)

Ann Carlyle at The Children's Museum - Mommy date!

Ann Carlyle helping out at Milla's PT and OT with our awesome therapists

Elle with Granddad

Tuesday, July 5, 2016

Happy July!

Hey guys!  Hope everyone had a good 4th!  Gotta be quick today...

We are still not at a good place with Elle.  She is having such a hard time walking and doing things.  We are having to keep a hand on her much of the time because she is so unsteady.  And Milla's muscles continue being hypertonic (or flexed) more and more.  It is really affecting her sleep again...daytime naps and at night.  

In the last month, we have had to get rid of both of our CNA's (our nighttime nursing assistants) because of them both sleeping on the job.  It has been a tough month interviewing and training new people...and the girls are having to get used to new faces as well.  We have 1 CNA that will be a regular and we are waiting for another one to interview this week that will hopefully be another regular.  We have had a couple of 'filler' or substitute people that we have trained who can fill in when our regulars have to cancel, but we are still trying to nail down a new permanent team.

So we've been under some extra stress lately.  Please pray that we can get back to a 'normal' with our nights (sleep!) and early morning routines.

We are driving to St. Louis next weekend with all 3 girls (eek) for the annual Batten Conference.  It will be great to gain the knowledge and support that the conference offers and it will be great for Ann Carlyle to meet other kids that are like her.  They focus a lot on the 'healthy' siblings at these conferences - with activities and fun and support all weekend long.  They also provide care for the kids with the disease so the parents can go to seminars. 

But it will also be hard and stressful traveling with the girls and staying in a hotel.  There was a lot of emotional stress for me last year at the conference (we went without the kids) that I assume I will have again this year...and there will be the physical stress this time around.  Please pray that it is an easy(ish) weekend caring for the girls, that it will be an uplifting time and that Ann Carlyle will have fun and find a bunch of support as well.  (Also important to note: We haven't taken a road trip anywhere since last summer so please pray for safety and sanity!)

Ann Carlyle had Mud Camp last week and is spending a few days in Birmingham this week with her grandparents and aunt, uncle and cousins.  And she is having a ball!  We are missing her but I'm so glad and thankful she is getting to be there.  :)  She is having a rockin' summer so far!

Thank you, always, for everything.  Much love,