For I Know the Plans I Have For You...

First of all, a continued THANK YOU for all those praying for us (near and far), loving us, serving us, helping us and living all this crazy (our ‘normal’) out with us.

Unfortunately, the phrase, ‘no news is good news’ has not really been the case for us the last month or so.  But I’ve just been too busy and covered up to sit and write out my thoughts.

I’ll start with the week leading up to my birthday in August…Frazer got pneumonia.  The real-deal pneumonia.  He was flat on his back for a solid week and then in the middle of that, we realized that all of us girls had lice.  (My head still starts itching every time I think of it.)  Not the best of times in the Gieselmann household.  But the Lord’s graciousness prevailed as our village, once again, showed up and carried us through the darkness that almost consumed me.  And thankfully, no one else got really sick…we all had a bit of a cough for awhile, but nothing that put any of us in the bed.

Soon thereafter, Ann Carlyle started first grade!  She is at a new school this year.  We were completely happy with the school where she attended kindergarten but God gave us an incredible opportunity to put her in a small, Christian school where my sister-in-law actually works as a counselor and where she can be nurtured in ways her old school would not be able to.  And she LOVES it there.  Really, really loves it.  Thank you to all that made this possible and for those that are driving her to school and back home again every day!!!  She is happy and being challenged and is thriving and is well-cared for!  This has been a ray of light for us in an otherwise pretty dark month.

Elle and Milla also started back at PDO, where Milla’s one-on-one teacher also returned this year to help with her.  A HUGE thank you to everyone at PDO that love us and our girls so well and who have made it possible for Milla to go there.  I have no doubt that being there has improved Milla’s quality of life…she is happy to go and the socialization she gets to experience is such a gift.

So all in all, it was a good week when the schedule and routine made their ways back into our lives!  I actually got to go to the store for the first time in 2+ weeks for more than just 3 or 4 things to get us through the next day.  Everyone was relatively healthy and completely lice-free and while there was still plenty to be anxious about, I let out a little sigh of relief that we had made it through sickness and critters and no schedule of any kind.  (Only by the grace of God and the people He continually puts in our lives.)

But we were about to be hit again - knocked clear off our feet.

So back in July, we went to the Batten Conference in Chicago primarily to meet with certain doctors and researchers that are involved in a clinical trial of a drug that could possibly save the lives of children with the exact mutation of this disease that our girls have - possibly even stop the progression of the disease.  (When the study was open last year, Milla was too far along in her disease process to qualify and Elle was not old enough.)  But now Elle is old enough and we learned there was a possibility that the study would reopen sometime this fall and that Elle could be a good candidate.

This would be an amazing and life-changing possibility for Elle and all of us but it would also require us to move to Columbus, OH.  And move soon.  And the thought of leaving our vital support and life-giving team here in Memphis and leaving our families to move so far away was excruciating and scary and completely overwhelming, especially in light of Milla’s condition.  Of course, we were willing to do whatever it takes if it meant saving Elle’s life, but the logistics and details and Frazer’s job situation and on and on have been hard to comprehend and we had to keep telling ourselves that God would provide for us as He always has done.

But I’m going to be honest…there’s been a whole, whole lot of anxiety.  And too, I struggled with fear.  Did we dare to hope that Elle’s life could be spared?

However, we found out about a week ago that Elle would not be getting into the study.  As you can imagine, we were crushed.  It felt like we had found out about her diagnosis all over again.

As I have said before, I have no business questioning why God.  He is God.  I am not.  But I know His love.  I know He has not abandoned us.  I know He loves all 5 of us more than we could ever hope or dream.  But sometimes (most of the time) it is hard to not understand…to not be able to see.

And literally, the day after we got the news, Milla started to decline again.  Her lungs have filled with fluid that she has not been able to clear completely.  She has been struggling to breathe and had a day last week that we had to give her a bit of oxygen through a mask on her face to keep her sat greater than 90%.  (‘Sat' is short for the oxygen saturation in her blood.  Normally, a person sats around 98-100%.  We want the sat to be greater than 92%.  If it falls below 92%, we need to give her oxygen.)  So we started her on an antibiotic just in case of an infection and also started a drug that should help dry up some of the fluid in her lungs.  It seemed to work a bit but then she would get worse again.  Her condition seemed to oscillate back and forth for a few days.  But yesterday, she had a great day, relatively speaking - her lungs sounded better than they had in a long time and her sat was staying in the upper 90’s most of the day.  And today, even a tad better!

We are praying that she stays on this course and keeps improving.  We are not sure, however, if she is temporarily responding to these drugs and will continue to do so, or if we are just seeing a couple of good days.  But what we ARE sure of is that Milla is a fighter.  She is stubborn and determined and absolutely amazing.  She still has her silly sense of humor and still tries her best to stand and walk and crawl and talk and be a part of the action.  She laughs and bosses Elle around and loves to be with people.

We are not sure yet if we will be able to send her back to PDO or if her needs are simply too great at this time.  But please pray that she can return there.  It is so good for her to be there and out of the house and to be with people and kids her age.  The days that Elle went to school without her, she was so sad.  She kept pointing towards the driveway and saying ‘car.’  I don’t want to keep her cooped up…a happy heart strengthens the body.

And please pray for all of us as we watch Milla's disease progress.  It is incredibly difficult watching her struggle to breathe and wonder if she will be able to fight through it.  Ann Carlyle is seeing it all too and trying to process it.  (She is doing okay for now, but pray that she continues to process in a healthy way.)

Elle has had some hard weeks as well.  We have had to increase one of her meds 5 times over the course of 6 weeks or so.  We are seeing more seizures and more neurologic irritability and physical instability.  In other words, she is screaming a lot and bumping into things a lot.  It’s been about 4 days since our last increase and I think we are starting to see a little decrease in her screaming fits.  But she’s been really hard to deal with lately.  We remember Milla going through this as well - the difference is that now we know why Elle is acting this way.  Knowledge is a very good thing but it doesn’t make it much easier emotionally, especially when we have a front-row seat to all that Elle has in front of her.

I’m sorry this is so, so long but there is just a lot to explain and update.  Thank you for hanging with me through it!!

So we continue to ask for your prayers - please plead to God for His mercy.  We feel like we are getting hit with one thing after the other lately and we are weak and tired and constantly fighting the fear of drowning in all this mess.

Pray for perseverance and stamina and unwavering trust in God.  Pray that we will not succumb to doubt and fear and sadness.  Pray that we would be able to live with joy and thankfulness for the time we have with our girls.  Pray that we would have energy to keep up with all of them and parent them and love them.

I pray that the light of Jesus would shine ever bright - in us, by us, through us - that no one would ever doubt His goodness and love and saving grace.  I can’t tell you why 2 of our little girls have this disease and will die from it, but I can tell you that Jesus is all we need.  I can tell you that saves us from our brokenness.  And I can tell you that when our girls die, they will be with Jesus and they will be whole.  He created them and loves them beyond measure.

Oh my Strength, I give praise to You.

In joy or pain, I give praise to You.

Night and day, I give praise to You.

Always, much love and thankfulness for you all.  I cannot express all my heart feels for you.

Dana

PS - On September 13th, Frazer and I are taking Milla and Elle up to Columbus, OH to meet with the doctors at the Batten Center at Nationwide Children's Hospital.  Please pray that Milla will be strong enough to go and that we will be able to travel safely and keep our sanity, as we will be driving. :)  We are hoping to learn more about the disease and how to possibly better medicate them for seizures, sleep and other disease symptoms.

Pictures!

Elle before her MRI

Milla and Frazer on one of her really great days!!!

Elle and Baby Bear at her swallow test

Elle is fearless - hanging out with Granddad and Tigger!

Ann Carlyle with Cousin Lucy, Tigger and Granddad

Love Our Village - New and Old!!!

I know y'all don't believe me but I really do have every intention to update the blog at least every week!  (Seriously!)

So Frazer and I just returned from the Annual Batten Disease Conference in Chicago.  We had not intended to go but about a week ago decided to do it.  Thank you to Kathy Mize for her herculean effort to organize (last minute!) all the people who helped care for the girls while we were gone for 3 days!!!  And thank you to those that did the caring too (last minute!)!!!!!  And thank you to those that got me on the plane Thursday afternoon after a little bout of a stomach bug!!!!  Y'all are amazing and the girls had a wonderful time without us.  :)

The conference was amazing and completely overwhelming and it's hard to put into words all we felt while we were there.  But bottomline, we have added to our village in these people...families that have been affected by this disease, specialized doctors and the Batten Disease Foundation staff that live and breathe this disease and are dedicated to helping Batten families, and researchers that are committed to finding treatments and/or cures for this terrible disease.  We came away with a wealth of information and things to process.  

A BIG update is that our insurance-covered help has kicked in!!!!  We have a nursing-aide every single night from 10pm-10am!!!  There are 2 women that come each week - one comes 4 nights each week and the other does 3 nights.  Everyone is still relatively new at all this, but it is going well and we are thankful for the help...and for the sleep!!!

As for the girls...

Milla is doing really well.  She is talking more, moving around more, laughing more.  She still fatigues relatively quickly and has times of lethargy but she is so determined to try to walk and stand and crawl and even jump on the trampoline!  And one of my favorite things to do is to get her giggling - her shoulders will bob up and down and if she gets super tickled, no sound comes out, just a huge smile and the shoulders bobbing.  Good for the soul.  :)  We are tapering one of her seizure meds down a bit and that seems to have brought Milla out of the fog she was in.  But we have to be careful as we are trying to find that ‘sweet spot’ between controlling seizures and over-medicating…both of which can cause lethargy and fogginess.

Elle is doing well too.  Her seizures have been mostly under control.  She still has some coordination issues at times but all in all, she’s doing fine.  She continues her speech therapy and we are seeing her make some gains there.  She had a swallow test done a few weeks ago and mostly did okay, but there was a little bit that concerned them enough to tell us to go ahead and thicken her liquids.  (This was a little discouraging to us that she is needing this so much earlier than Milla did but it has helped decrease her coughing/choking by leaps and bounds!)  And she had a baseline MRI done yesterday - it was a long day at LeBonheur due to an emergency that came in - but we got through it.  

There are still days like today, that both girls seem to be ‘off’ - more seizure activity, sleepiness and fussiness.  But hopefully we will see a rebound tomorrow.

Ann Carlyle has had a summer full of playdates and swimming and fun.  :)  She has had some pretty heavy questions about her sisters but she seems to be processing things in a very healthy way even as she is grasping the gravity of the situation and for this we are thankful.  She remains a happy and relatively carefree 6 year old!

We all 5 actually were able to travel to Birmingham to visit my family (and go to my 20-year high school reunion - yikes!).  The girls all traveled really well and we had a fun time playing with cousins, swimming, riding Nana and Granddad's horses and seeing old friends.

I'm keeping this somewhat short today (maybe?) so that I can go ahead and get this update out...but as always, we treasure your prayers...

Please continue to pray for safety for the girls, for mercy, for all of them to know the deep, deep love of Jesus.

Please pray for Frazer and me also to know and feel the deep, deep love of Jesus and pray for our marriage.  Pray that God would grant us clarity and wisdom as we consider some possible new ways of treating the girls' seizures and sleep issues and as we continually make medical decisions for Milla and Elle.  I am feeling a lot of anxiety of late and my body is suffering because of it.  Please pray that we both are able to rest in God's promises and faithfulness that He will never leave us or forsake us and that He will continue to provide us, love us and give us strength to walk this long road.  Again, pray for clarity and wisdom and for God to go before us and pave the way.

Thank you for praying for us, with us.  Thank you for supporting us in every way possible.  Thank you for being a reminder that God has not left us - for being our awesome, amazing and incredible village.  We bragged a lot on y'all this weekend at the conference.  :)

Much love!!!

Dana

(My laptop's start-up disc is full and I can't figure out how to get some pictures on here but I'm working on it.  I'm not real bright when it comes to computers and start-up discs and storage.  Grrrr.)

Oh the deep, deep love of Jesus

Vast, unmeasured, boundless, free!

Rolling as a mighty ocean, in its fullness over me!

Underneath me, all around me, is the current of Thy love Leading onward, leading homeward to Thy glorious rest above!

Quick Note

A quick post to share a story and ask you to give $1 to support this.  Every little bit may help someone else not experience what we have.

http://deadline.com/2015/06/gordon-gray-daughters-batten-disease-fundraiser-pledge-1201439934/

Thank you!

Frazer and Dana

Some Changes

Hey y’all - so we’ve had a few changes happen over the last week or so since Frazer’s update that I felt was important to let you know about.

MIlla is continuing to get weaker with her swallowing and breathing and she probably has more than 100 little seizures per hour (sometimes many more).  The amazing thing is that she has been super strong in other areas…she has had times of wanting to bear weight in her legs and even taking some steps (assisted, of course, but this is something we haven’t seen in a very long time).  And she has been more alert and responsive.  She is quick to smile and laugh and still takes such joy in making others laugh as well.  We are so thankful her mind is still ‘with us’ most of the time.  Her disease causes dementia along with everything else, so everyday we have with her where she knows us and knows our love for her, is a blessing.  Please continue to pray for this specifically…and praise Him for His provision in this way!

Some of the most dramatic changes, however, have been in Elle.  She is seizing more and she is walking around like she is in slow-motion.  It is like the wind has been sucked out of her.  She even talks in slow-motion.  We have seen changes in her balance and coordination as well and 1 eyelid is occasionally drooping a bit (as we see with Milla).  We aren’t sure at this time what is due to seizures/meds and what is due to the degeneration in her brain.  Today, actually, has been the first day in a week that I’ve seen some kind of plateau in her condition.

Ann Carlyle is doing okay but she is definitely seeing all these changes in her sisters - we are having little conversations about hard things frequently.  We’ve seen a few behavioral things here and there - clinging, not feeling good, emotional.  She threw a fit yesterday about not being able to sit beside Milla in the car.  Please pray for her little heart and mind and pray for us as we strive to protect her when she needs it, to gently show her the reality of what is happening, to have patience with her when it’s hard to have patience.

On a positive note, everyone has slept through the night the past two nights!!!  Praise God and please pray that this continues!

Please continue to pray for the help provided by insurance.  We are still in this waiting place to see what they will pay for and what we need…and then a bunch of other steps have to be taken before we physically get that help in our home.

Please continue to pray for mercy for all of us.  God is love and He is merciful.  His character never changes.  As you can imagine, this has left us reeling and we aren’t even really able to process it all.  I think we both feel a bit like we are just getting through the days, doing what needs to get done, and the processing will get processed later.  In other words, survive.

And so we press on with the hope of Christ in our hearts.

Thank you all for standing with us - and for us when we can’t.  Much love-

Dana

Long Overdue Update...

It’s been a while and in some ways our world is the same, but in many ways it is ever changing, so an update seemed appropriate.

 Elle – her seizures are under control.  We see small ones, typically at night and a longer one will pop up from time to time (it’s been over a week since the last bigger one).  As it stands today, the med combo she is on seems to be pretty effective, but that can change tomorrow as the disease progresses.  As for the rest of Elle, she seems to be doing well, playing and doing typical 3 year old stuff.  We see very small development things with her, but Elle is very smart and overall doing well right now.   Elle eats less than a bird and she is still light as a feather, but she seems pretty healthy.  But, her sleep is not good.  Typically she is up 3-4 times during the night and while she generally goes back to sleep with relative ease, she is starting to get up earlier in the day.

 Milla – not much good to report with “Milla Willa.”  Since we last updated, she took a significant downturn at the end of April around the time she had the cough that was in our last update.  At that time, she had generally weakened, could not chew the same foods and her chest cold/cough all caught us off guard.  Most recently, the past weekend was another significant downturn for Milla.  A noticeable weakening and unresponsiveness, increasing seizure activity and excessive drooling.  After visiting with the doctor yesterday, the seizures seem to be less concerning, if they are even seizures.  It’s hard to distinguish a seizure from her just “checking out” briefly (due to disease progression).  Thankfully, they are not violent convulsive seizures.  She is weak to the point that she cannot sit up on her own, the majority of the past few days, she struggles to get even a word out and she can barely lift cereal to her mouth.  In the midst of this, she is still with us and playing hide and seek under the blanket, showing everyone her newly painted fingernails and toenails and asking to go outside to see the bubbles every chance she can. 

 In February, we were not thinking in terms of time frames for Milla’s life.  But by early April, we were talking with a doctor on our team and, while no one will give dates or predictions, we were thinking at the time, “I don’t know if Milla will live past the end of the 2015 or maybe early 2016.”  By late April and after the downturn I mentioned above, we were shockingly asking if Milla would even live to the end of summer/late September.  The doctors agreed that that was not an unreasonable conclusion based on her state.  And this week, we are facing the reality that Milla is dying now.  I mean, we know it’s a terminal illness, but the term is short.  Technically, Elle is dying too, but her term doesn’t seem short right now.  The largest concern now is the Milla's drooling, which indicates the likelihood that fluid is or very soon will be getting into her lungs, leading to pneumonia.  Recovery from pneumonia is not likely for her.  All this to say, we are on the home stretch with Milla and the home stretch gets shorter and shorter than we expect.  It is obvious but, at the same time, surprising that she has an aggressive case of Batten’s.

 Ann Carlyle – She is plugging along.  She asks questions and understands ultimately what is happening, but I don’t think she grasps timeframes.  It is interesting to watch her because she will ask and talk and then in mid-sentence change the subject or walk away.  It is her way of saying I have had enough, let’s move on.  I am so grateful for the Center for Good Grief and Lauren, her counselor.  Lauren and Angela (our counselor) have been awesome at helping her understand her feelings and to talk about how she feels and they have been awesome at helping me and Dana work with Ann Carlyle.  It is a process and it takes a lot of work.  Please pray for Ann Carlyle, especially as it is likely we will be having some tough conversations with her in the coming days.

 

I have a few observations.

- When I put each of the girls to bed, I sing a song and pray and each time, I ask for healing for our girls and then ask for God’s mercy and strength to make it through each day.  Coming up on the end of May, He has provided us seven months of making it through each day.  It is very hard to ignore that God is answering our request and providing mercy for Milla by giving her an aggressive case.  I figure that I will not be thankful for this mercy at the moment she is not with us.  But He has answered us through all this so far.  He has carried us through the storm.  He will provide for us in the future.

- God is preparing Milla’s room and it seems as though He just about has everything ready.  I try to find peace in knowing that she will be healed and with Him, but it is not very comforting right now.

- All of this only reminds me of Elle’s journey.  This does not take me to a good place.

- I recently read a person’s situation of suffering and here is the comment:
  “We’re surviving. Because we have no other choice. We aren’t going to 'keep fighting.' Because that implies we have a choice to stop fighting. This is our reality.” Hay Farris 9/8/14

 I love this quote and it accurately describe us.  We take small bites, rarely more than one day at a time, and most often half or 1/3 day at a time.

- I had been thinking that as we approached the girls’ death and given the slow nature of the disease, I would have plenty of time to process and prepare for their dying.  Our parents are still living, but I remember as my grandparents neared the end of their lives, many of us around them had time to prepare for their deaths. I  have heard many people speak of death in this way or I have heard this: “at least they are no longer suffering.”  These comments are made mostly about the elderly.  I have learned and I am learning that this is not even close to the case when thinking about your children.  Things don’t work that way when it’s your children, it is very, very different.

 Final comments:

- We have been having help overnight two days each week, which has been great!  I just found out ten minutes ago that we are now qualified for 12 hours of nursing help five days a week and it looks like it will be covered by insurance!  Again, God provides for us in the midst of a storm that does not seem to ease up.  God be praised.

- Thank you to those coming to help with the girls, especially the morning servants.  They show up at 5:15am and the past two or three weeks, there has been no routine.  Some days the girls are up at 4am, some days 6:30am.  I appreciate each of you coming whether the girls are up or not, it helps us get rest to make it through the day.  God be praised for His provisions.

- Thank you to First Evan for hosting a prayer meeting for us.

- Thank you to all who are praying.  This is the single most important thing that you as people can do to help our family through this.

 

One step at a time, even if it’s only an inch or two,


Frazer

Home!

We are home!  

No breaking news for Elle - she didn't have any seizures while at LeBonheur and we are hopeful that is because her medicine is at the right levels.

Milla's congestion is a bit better and she was much perkier today.

Thank you all so much for your prayers, for your encouraging words, for sending God's Word to speak peace into my soul. 

We are pretty exhausted tonight but thankful to be home with our girls and anxious to get back into our 'routine.'

Please continue to pray that Milla's congestion clears up with no complications.  

Much love!

Dana

Here's an attempt to capture Elle's awesome post-EEG hair.  :)