Well - it's been a bit since I've written - but kind of by design. I decided I would check out for awhile during December. Life has felt pretty heavy as lots of birthdays and holidays and anniversaries are all clustered together this time of year. Milla's birthday was November 2nd, the 1-year anniversary of her death was November 26th, right after Thanksgiving and then Christmas, New Years, etc. Even though technically this was our 2nd Christmas without Milla, it kind of felt like the first. I remember almost nothing from December 2016. Pretty much after Milla's funeral, my memory is as foggy as can be. So, much of this December felt new and empty-ish and hard and dark with splashes of numbness thrown in there.
I think Ann Carlyle was in over-drive trying to keep us all happy and excited and full of the Christmas spirit in December which is sweet and awesome but trying to keep up with her almost did me in. The long break from school and routine does not bode well for our family. And here we are with an extra long weekend due to the snow in Memphis closing the city down and running into the MLK Jr holiday. And I just heard that Monday night might bring 1-3 more inches of snow. Heaven help me if we are homebound for another day Tuesday. Heaven help us all. I'm barely keeping myself on the right side of sanity today.
My teetering mental status is mostly due to the events of this past week (aka mental, emotional and physical exhaustion and lack of sleep). Frazer and I took Elle to Ohio Sunday for a checkup with our doctors and therapists up there. We spent the entire day Monday at the hospital as Elle was getting checked out and tested all morning and then she had an EEG that afternoon. Tuesday, we saw an eye doctor all morning and then flew back home after lunch. It was good that we went - we got lots of good information - but it was exhausting (along with very little sleep) and it was like a fire-hydrant of people and information about Elle's current condition. There were some things we expected and some things that we did not necessarily expect.
In general, she is doing very well for her age (especially cognitively and with her gross motor strength) but her disease is still progressing. The enzyme therapy has definitely slowed down her progression but it has not stopped the disease. Also, Elle's seizures were very well controlled last summer but the frequency and severity of her seizures have been on the rise all during the fall. Over the last few months, we have increased the dosage of her 2 seizure meds but that hasn't seemed to make much of a difference. So we are now starting a 3rd seizure med. It will most likely take a few weeks to know if this 3rd drug will help and we have to watch Elle closely to make sure this drug doesn't impede her other activities/quality of life, which it could.
Besides the seizures, our biggest concern has been her frequent coughing/choking with food and water but most often with her saliva. We already thicken her water to make it easier for her to swallow and she's done great with any kind of food and her own secretions until about mid-December. There is a possibility that this is happening because of her seizures. But there is also a possibility that her swallowing abilities are weakening. Aspiration (of anything: saliva, food or water) is a big concern as that can lead to pneumonia. We will have to wait and see if we can get her seizures better controlled, if that also helps this choking. We will be doing a swallow study at some point as well to see if she is actually aspirating even small little bits into her lungs. And hopefully, the doctors can give us recommendations/medication to help with that.
As I said, Elle's gross motor strength (the big muscles doing the big movements) is so much better than it would be without the enzyme therapy, but she is still weakening and her legs/feet continue to turn in and lose flexibility. We have a few things in the arsenal that we will try out to help this issue as much as we can. Right now, we are going to try night splints. These splints are basically hard braces that go over her foot and all the way up to her knee. I'm worried this will affect her sleep (which is already awful) and also, please pray that the splints work so that we won't have to do things like injections or muscle relaxers that can exacerbate other conditions and/or have to do serial casting again. Serial casting is where both legs are in casts up to her knees for at least a month - we did this last May and it.was.awful. We really, really do not want to have to do that again.
Ultimately, it is her brain that controls all of everything so as long as the brain is degenerating, her body is not going to work like it is supposed to. We can only do so much to combat all the issues without fixing the root of things - her brain. The enzymes are not fixing her brain, but they are slowing down the rate at which her brain cells are dying. I'm going to be honest, it is haaaard to live in the limbo of hope and keeping Elle here with us longer and quality of life and experimenting with her brain and this disease. For example: we have seen amazing cognitive preservation and even tiny improvements the last few months. Typically, Elle would have greater dementia at this age (without the enzymes). So while it is awesome to see her understand things and do things that defy her disease, it is not awesome to see her so frustrated and sad and angry at the things she sees other kids doing that she can't do...or at the things that she remembers doing, but can't do now. She tries to get up and walk on her own (we haven't seen her do that in a long time) but then can't and she's sad and frustrated. So while dementia is not something I wish for, I see a certain mercy in some of that dementia. Does that make sense? It's murky stuff sometimes.
We have also been struggling with her sleep disturbances. She's always had issues with sleep (as do most Batten kids) but those issues reared their ugly heads in a new way this fall. Elle was waking up multiple times every night with screaming fits that we could not sooth. It was absolutely terrible. We don't know if she was seizing or hallucinating or something else but she was clearly having some kind of neurological problem. We increased her one of her meds a couple of times (it helps with sleeping and also seizures and neuro issues) and that seemed to do the trick with the screaming fits. She is still waking up a lot throughout the night and/or super early for the day (like 3am-early) but we are thankful the fits have subsided.
We do have regular night help (night nurses) but for the last couple of months, we have not had help on Saturday nights and for the last 3 weeks or so, we have had many nights that our nurses have been sick and unable to work (or we have been in Ohio with no help). Frazer has taken the brunt of this, staying up with Elle most of these sleepless nights but we are both so exhausted. This carries over into our days, of course, and no sleep = everything else becoming harder. (Hence, the question of my mental health this weekend and my grinchiness over the snow.)
Another issue Elle is dealing with is her vision. We know her vision will continue to deteriorate until she becomes completely blind and it seems that her vision has taken a bigger hit over the last few months and even more so the last week or so. So far she has compensated pretty well but this is another reason for her frustrations. She's been screaming/fussy much more these days, and I know the lack of vision does not help. (Her eye doctor in Ohio confirmed this Tuesday as well.)
Whew. It's a lot.
I was reading today that sometimes 'our emotions aren't in sync with our beliefs about God, and it's okay to tell Him.' I've felt that much of the time lately. God is the source of my hope, courage and delight but in these moments, I feel fear and hopelessness (in this world) and sadness and sometimes I feel nothing at all. It's just hard.
On another note, Ann Carlyle is doing really well. She soaked up every last drop of the Christmas season and is enjoying all her many lego sets she got for Christmas. We talk about Milla a lot and Ann Carlyle misses her greatly and sees/has questions about what's going on with Elle - but overall, she continues to 'work her grief' very well and we are thankful that she is so open about all her thoughts and feelings. Ann Carlyle is a big ball of energy and joy and light. Unless it's time for homework. Then she is a black hole of pouty-ness and unhappiness. Like ya do.
Frazer started a new job in December and that has been a better environment for him and therefore better for all of us. He was able to take some time off between jobs which was also great for him. That time-off correlated well with all the difficult things in November so he had a little more time to process things than he would have had had he been going to work everyday.
We are slowly getting back into our routine with school and therapy appointments and doctor appointments and enzyme infusions (if we could just get through these snow days!). Our infusion days are changing to Tuesdays instead of Thursdays which I think will help Elle tremendously in relation to all her other activities. Thank you to our infusion doctors and nurses at LeBonheur, who are so gracious to accommodate us in this way!
Thank you all for your steadfastness in praying for us and helping us and loving us, for we need your prayers and your help and your love so much. Our hearts overflow with gratitude for you all the time.
Throughout my days, I am telling my eyes to look to the hills and remember from where does my help come? My help comes from the Lord who made heaven and earth. He will not let my foot be moved; He who keeps me will not slumber. The Lord will keep my going out and my coming in from this time forth and forevermore. I will continue to preach the truth to my heart in the midst of these hard days.
Much love as always,
Dana
