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

September 18th Update - Re-Entry

We.are.exhausted.  Mercy.

The adrenaline that has carried Frazer and I through the last few weeks has been depleted.  Utterly depleted and we are feeling it on every level.  It's hard just to keep our eyes open during the day.

Elle is absolutely non-stop.  She is constantly on the move and her attention span is about 1-2 minutes.  On a good day.  This has not made life easy at all as someone has to hold her hand everywhere she goes so she won't fall.  Here's the thing...her walking is better, y'all.  Like really, really better.  This is really good and really exhausting.  Her confidence is high but she is still super unbalanced and doesn't have the strength and coordination to keep from (eventually) falling whenever she walks.

One thing we have seen (praise God) is that Elle is finally hitting a plateau with her disease progression.  This could last another day or another month.  God only knows but we are thankful to see this happen as she has been in a disease free-fall all summer long.  And we are very hopeful as she is now getting those enzymes that could possibly stop the progression all together.

She is still having a very difficult time with her speech and her behavior has been trying, to say the least.  And her seizures are still frequent...hundreds and hundreds and hundreds every day.

So - lots to pray for with our Elle-belle.  :)

Her next infusion will be Sept 29th.  We most likely will be taking the whole family this time.  The neurologist at Nationwide Children's wants to see Milla and we are thinking it would be a good time to take Ann Carlyle too.  Ann Carlyle has never been to Columbus with us and we really want her to have that experience, to see the hospital, etc.  It will be crazy-town but just for 4 days this time.  

It's funny how fast the 29th will be here.  I was looking at my calendar yesterday and realized that we will be flying back up to Columbus in a mere 10 days. Eek.

Milla is doing a little better with her seizures this past week but not great.  We've adjusted one of her meds a little and adjusted a couple of other things that could help with medication absorption, but she's still having a tough time every day.  I don't feel like she's in a free-fall anymore but we are still seeing definite disease progression every week.

And then our Ann Carlyle - poor thing is always at the bottom of the totem pole.  She has been pretty clingy and needy this weekend.  I can tell the past few weeks have had an impact on her.  But she is still upbeat and loving on her sisters and taking it all in stride.  She really is amazing.  I think I would be a total pill if this happened to me when I was her age.  Her positivity and laughter makes us smile and laugh along with her and challenges us to see the every day joy in life.

Pray for health for all of us.  Ann Carlyle has had the sniffles this weekend.  Frazer and I are run-down and I fear our immune systems aren't 100%.  An infection for either Elle or Milla is a very scary thing right now.  Partly, for different reasons - Elle because of her every-other-week infusions and needing to be healthy for that; Milla because of her very high risk for pneumonia and other complications.  Anyway, we do NOT need sickness in our house and we are heading headlong into cold and flu season.  Eek again.

Please pray for me and Frazer.  We are just drained and sad and hopeful and scared and not hopeful and thankful and a million other things.

My brain can't think of anything else at the moment even though I know there is tons more to say.  

Thank you all.  

"But for you who fear My name, the sun of righteousness will rise with healing in its wings.  You shall go out leaping like calves from the stalls." Malachi 4:2

My heart leaps at the thought.

Oh how He is our hope.  Our hope does not lie in this world, but in Jesus.  

Much love from the Gieselmanns - we thank God for you every.day.

Dana

(Oh and ps - we are still having lots of issues with out aide-help at night.  This is extremely stressful and tiring.  Please pray for this to be resolved...and quickly.  That God will bring us the perfect people to love our girls well and that are able to take care of them well as we sleep and that we are able to trust.)

September 15th Update - Almost Finished!

Hello from Ohio!  Still here...but going home tomorrow!  We are just a little ready to all be back together again.  Just a little.  :)

It's me and Elle here this week.  She had her first infusion Tuesday and it went very well.  She even stayed still and calm the whole FOUR hours (with a little help from Zyrtec, crazy but true).  Miracles still happen, people.  Her incision from the surgery had some edema so there was a little difficulty getting the needle in the reservoir initially, but then everything else went perfectly.

We had to stay in the pediatric ICU (PICU) for 24 hours just for observation and we had the most amazing nurses the whole time.  Seriously y'all, God-given nurses.  They cared so much and loved my girl (and me) so well.

We were supposed to be discharged around 9am yesterday but they needed a urine sample as part of the routine work up and somehow she managed to pee everywhere but in the urine bag.  All Tuesday.  All Tuesday night.  And all Wednesday morning until they gave her a huge IV bolus of fluid and finally we got a 5mL bit...juuuuust enough for their needs (and of course, there was a whopping 93mL in her diaper that missed the urine bag - how, I'll never know).  So we got back to our Ohio 'home' around 1:30 just in time to take a little nap and then to go out and enjoy the weather in the downtown area by the river.

By the by, Columbus has a beautiful downtown area.  The weather has been wonderful and we walk and walk and walk and walk...it's a super old city with cool old homes everywhere that were built in the 1800's and brick streets and yummy food and great local shops and restaurants everywhere.  And the people are so friendly!  (If you are a non-southerner, please don't take offense by my comments but us southerners generally don't expect an ultra friendliness to strangers when we leave the south, to be honest). But people here are so warm and kind and welcoming.

So this morning we have some speech therapy and some physical therapy and then after lunch and nap, we will go out on the town again for awhile.  :)  Yesterday, Elle's seizures were pretty bad and she was pretty (a lot) grumpy but so far, this morning, she's been a little more cheerful and seizures haven't been quite as bad.  (She slept better last night, so that always helps.)

We fly home tomorrow!  Tomorrow!!  And while, yes, we will be back up for 2 or 3 nights every other week for the next foreseeable future, our big almost-3 week Ohio adventure is finally coming to an end.  And we've made it!! The finish line is in sight.

Thank you for praying.  As the enzymes were being infused into Elle's brain Tuesday, I prayed and prayed that those enzymes would bathe Elle's brain cells and give her life.  It will most likely be a few months before we see any kind of changes in her, but we will pray for immediate changes!!

Thank you for praying for our family - everyone has been doing really well relatively speaking!  I'm sure the re-entry into our norm is going to be a bit of a bumpy ride - please pray that all the girls will be patient with Frazer and me as we will have to divide our attention between the 3 girls.  And pray that Frazer and I will have patience as well!  It's going to be a circus for a few days.  But a good circus because we will all be together again.  Finally.

Also, please pray for the other Batten kiddos that have been getting this medication here in Columbus for the last year and a half.  And pray for the 5 kiddos (Elle is one of the 5) that will be starting it this fall.  It is a huge stress on all the families.  Some are moving, some are commuting.  And please pray for the around 19 kids (I think) that didn't qualify for this round for one reason or another as there were only 5 spots available.  These people are all amazing and wonderful and my heart is broken.  Pain and joy always go together, don't they?

So Elle's next infusion is September 29th.  We will come to Ohio for 3 nights.  Please pray for that trip.

I feel like I could go on and on about details and the comings and goings of things and about certain prayers but Elle's getting restless and mischievous.  :)

God is good.  All the time.  ALL the time.  In joy, I will praise Him.  In pain, I will praise Him.  My heart is raw and open and broken and full and I feel the absolute love and beauty of Jesus.  He is here.  He is truth.  He is life.

Much love!!!
Dana

September 6th Update - Ohio

Hey y'all!  I flew back to Memphis Sunday while Frazer stayed with Elle in Columbus.  I will be here until Saturday, when we will switch out (I will go to Columbus and Frazer will come home).  

Elle is doing great - really great!  She is actually talking and walking better than she has in a month.  Not sure why.  She hasn't received any new medication yet, but we are excited to see her doing so well!  She is just hanging out in Columbus this week, with a few appointments for therapies and an eye exam; but mainly just letting her surgical site heal.  She hasn't seemed to have any pain at the site and has had no complications at all.  Praise God and thank you for praying for her!

Elle will get her first infusion of the experimental medication a week from today, Tuesday, the 13th.  She will have to stay in the hospital for 1 night and then we will be in Columbus 1 more day after that (hopefully) and then home.

It was super hard to leave Elle and Frazer but super good to get home and to see Milla and Ann Carlyle!!  They have done wonderful without me, but I think it was good for all of our souls to be together again.  :)

Milla is still having a hard time somedays with seizures, but a little less than before.

So not much news this week so far - pray that we stay boring!  Pray we will all stay strong and encouraged even though we are split up and things are all crazy for awhile.  Pray that Elle stays healthy so she can get the infusion next Tuesday.

Much love to everyone!  God is good!

Dana

September 2nd Update - Surgery Day

Hey guys - quick update from Ohio.

Elle and I have been here since Tuesday (Frazer was here for the day Tuesday but flew back home that evening).  She has had a test or 2 everyday and had an MRI at 11 (eastern time zone) and is currently in surgery.  She is having a reservoir put in just under her scalp that has a small catheter that runs into the middle of the brain - her right frontal ventricle.  It's a pretty 'routine' procedure neurosurgeons do all the time, but of course, surgeries always come with risks and nervous waiting.  Elle will be in the OR for about 3-4 hours total time (including MRI time), so we still have 1-2 hours to go.  (Oh and Frazer got here this morning before Elle's surgery.)

She will stay in the PICU for a night I think and then maybe a regular room in the neuro unit until Sunday.  We are praying there are no complications!

Frazer and I will be here with Elle together until Sunday when I fly back home for the week.  I am missing my other 2 babies like crazy but I already feel heartsick thinking about leaving Elle.  My heart won't be whole again until we are all back under 1 roof.  Frazer will be here in Ohio all week and then we will switch next Saturday.  (The logistics of all this are ridiculous but are also being made soooo much more manageable through the help of awesome people helping with the girls and with flying us to and from Columbus.  THANK YOU to those people!!!!!)

We are staying at a house my sister and brother-in-law found through Airbnb and it's just a couple blocks from the hospital.  It's so great to be able to have a house to stay in with a full kitchen and space to move around in since we are going to be here for so long.  2 nights in a tiny hotel room with Elle was long enough!!

So please continue to pray for us!  Pray for Elle's safety and recovery.  Milla and Ann Carlyle are doing okay but are missing momma - but they are also currently being spoiled by Nana (my mom) and Auntie Dawny (my sister) so I'm sure they are doing just fine!  :)

I am planning on sending out a quick word when Elle gets out of surgery to let y'all know how she is doing.  We actually won't see her for another hour after surgery until she gets out of the recovery room but we will be able to talk to the surgeon.

Thank y'all for praying!!!  We love you!!!
Dana

UPDATE:
Elle is out of surgery and in my arms. So thankful. She is doing well. Sleepy/groggy but good. We are resting in her room.

August 25th Update - Bad News, Good News

We got more bad news over the weekend.  Elle had her 24-hour EEG and did really well (behavior-wise) while we were there.  But the EEG confirmed what we thought was the case, that she is having hundreds and hundreds of little seizures every day.  She is also having ones all night long, which we did not know and she has not had before.  Too, the EEG confirmed that she is having atonic or drop seizures along with the absence and myoclonic ones.  

Also, Milla's doctor thinks that her 'attacks,' that I described in my last update, are seizures...possibly grand mal seizures...they just look different because of her condition.  I have had to give her rescue meds at least once a day for the last 12 out of 14 days or so.  For reference, we haven't given rescue meds to Milla in months and months, maybe even a year.  And when we last gave it, it was a super long time before that that she needed rescue meds.

So not much good there.  We are adjusting meds for both of them and hoping that will help.

Another rough part to our week is that we had to put our dog, Maddy, to sleep earlier this week.  She was 13 years old and had stopped eating last weekend.  I took her into the vet Monday and they found a huge cantaloupe-size mass in her abdomen.  We were pretty worried about how Ann Carlyle would handle the news, especially since she was at school when I took Maddy to the vet, but she's doing okay.  We miss having Maddy around!

The big news is good news though!  We will be taking Elle up to Columbus, OH next week to receive an experimental treatment.  She will have surgery to place a brain shunt which the doctors will use to give her an infusion of the experimental medication.  She will be up in Columbus for a couple of weeks to heal from the surgery and to get her first infusion.  (After the surgery, Frazer and I will alternate being home with Ann Carlyle and Milla and being in Columbus with Elle.)  Then she will come home, but we will have to take her back up there every other week to get another treatment.  For each treatment, she will be in the hospital for at least 24 hours - more if she has any complications.  This is a treatment, not a cure, so she will continue to get the treatment for the next foreseeable future.  The clinical trial that has been studying this medication has had some good results so far (slowing or stopping the progression of the disease, regaining some lost skills), though the trial is not even 2 years along yet.  Unfortunately, Milla is too far progressed in her disease to qualify for the medication.

We are beyond thankful that we are able to commute up to Columbus at this time and we don't have to move up there.  Also, if, at some point, the medication is approved by the FDA and commercialized, we are hoping to be able to get the infusions locally or somewhere much closer to home than Columbus.

So please, please be in prayer for all this - so much to pray for.  

Pray that Elle qualifies for this treatment.  We technically still have a couple of steps to go through when we get up to Columbus next week, which are mostly formalities, but until the papers are signed, nothing is set in stone.

Pray for Ann Carlyle as she continues to go to school and have homework, etc that she has to keep up with while her parents are in-and-out of town for almost 3 weeks.

Pray for Milla - that she will bounce back with these medication changes and that she won't be confused or scared because of us being in-and-out.  Pray that she will stay healthy.

Pray for all the family and friends that will be helping us care for Ann Carlyle and Milla while we are in Ohio.

Pray for my heart - I will be leaving one or more of my precious girls constantly for the next 3 weeks and that is really, really, really hard as it will be 6 days or so between the days Frazer and I switch places.

Pray for Frazer - for his heart as well and that he will be able to balance work with all this travel and caring for the girls.

Pray for us both because we will be apart from each other for so long...getting to see each other, at best, for a couple of hours when we switch cities.  Super not-excited about that.

Pray for Elle - for health and her safety/no complications with her surgery and the medication.  Pray for her time in Columbus, that it will be good and fun for her and helpful and that she will tolerate all the things that she will have to do/go through.  Pray that she will be compliant and that she will sleep!  Pray that she will be her happy, silly self!  And pray that her seizures decrease and that her motor skills maintain.

Pray for all the doctors, nurses and other medical folks in Columbus that will be taking care of Elle/us.  Pray that they will have wisdom and skill and understanding.

Pray for all the logistics to go smoothly.  There will be A LOT of moving parts!

I could go on and on but I gotta go help Ann Carlyle with homework, make some dinner for the girls, feed them and in between all that, interview a possible new aide for the weekend nights.  For the love.

I will try to update y'all as much as possible as we go through all this the next few weeks.

I can't tell you how much we appreciate (understatement) your love, prayers and help in all ways, big and small!!!!!

Much love!

Dana

Let us strive to know the Lord.  His appearance is as sure as the dawn.  He will come to us like the rain, like spring showers that water the land. (Hosea 6:3)