Morphine is now our friend

Ever make a decision on faith one day and then question it the next and then be so grateful you just went on in faith?  That's where we're at.  Cicily went to sleep happy last night.  But at midnight she woke up crying and grasping for anything to help her.  Her eyes wouldn't focus, she wouldn't respond to us talking to her, and she was obviously not "with it".
So Chris went to get her usual lately, tylenol pm and ibuprofen.  Then I realized, that hospice gave us morphine and lorazepam (for anxiety/seizures) to give under her tongue whenever she needed.  This behavior was new and seemed perfect for immediate morphine and lorazepam.

 It worked and she slept well until 4 am when she woke up the same way.  Morphine/lorazepam to the rescue again.  Then she slept until 12:30 in the afternoon.

I love this picture even though it's a little sad.  Cis usually only sleeps flat on her back with her arms to her sides, or on her tummy.  She's been sleeping in our bed since Tuesday night as we all sleep better that way (thank goodness for King beds!) and I love how she sleeps on her side and reaches out her arms for her Mommy.  This morning she literally had her arm wrapped all around my neck.

How does Cicily feel about it all?

We've always been very open with our girls that Cicily wouldn't live a long life.  Sophie used to say things when she was very small (3) like, "when I have kids, they'll have an aunt on earth and an aunt in heaven". Cicily has NEVER liked talking about her dying.
Every once in a while when she'd get really sick over the years, I'd have conversations with her about the spirit world and what it would be like when she lived there.  Every single time she'd give me her grumpy "no" face.  She hated talking about it.  So I stopped and haven't mentioned it for a couple years.  But of course, we're discussing it a lot more recently and she still HATES it.  Even when Grandma and Grandpa talk to her about her great grandparents waiting for her, she still gives them her "no" face.
Cicily just LOVES her life!  She is not interested in being able to move around by her own free will or talk.  She just wants to stay here with her family and friends and soak up every tiny bit of this earth life that she can.  It's so inspiring to me.  She really can't do much anymore, but she still loves life and gets so much joy from being around people and interacting with them.
So Chris and I feel Cicily will really try to stay in her body until everything really shuts down and she has no other option.  She is ridiculously stubborn (she's even out-stubborned me over the years, which is impressive), so she may be here a while.  As long as Cicily is happy and finding joy in her life, we are so happy to have her here.

Our girl on Hospice at school

This morning the hospice nurse and social worker came out to meet us and talk about all the great services they can provide if we'd like.  Then Cicily went to school!  She had another great happy, healthy day so why not!  While Cicily went to school, Chris and I went to the funeral home to start the process.  We picked out her casket.  We've talked about a few details of her funeral over the past 6 years, but to truly plan it was very surreal.  I actually held it together very well until we started talking about closing the casket.  I can't handle the finality of that too well.  The thought of never seeing her body again in my life feels unnaturally empty.  So we'll probably have them do it after the family leaves the room.

 After school we went to cousin's house for a water balloon fight and swimming.  We're just living up all the time we have.

We've been so loved by so many already.  Dinners brought, prayers offered, love extended.  It has already made such a difference for us.  Thank you all.

Party day

Wednesday, the day after Cicily scared us, she was back to her happy, healthy self.  She got rid of a lot of that gas by farting it up in her sleep! (Sophie was proud.)  

Since we had let our friends and family know how horrible Cicily was doing, Cicily had a revolving door of visitors and love all day.  She was LOVING it!!!

 We had our first cousins come and Cicily got a foot massage (I can't believe I missed photographing it.  You'll have to do it again soon Brooklyn!) Then our other cousins came and Cicily got to do one of her favorite things and hold baby cousin Rosette.  Few things make her happier.

At night our cousins came with cards for Cis!

 And our dear friends Savannah and Shanell came to sing and read with Cicily.  She truly loved all the time and attention and love shared.  It was a great day for her.

How long will it be before...

So the logical question after hearing someone is on hospice would be, how long will it be before they die?

For Cicily we have no clue.  Apparently it's very common for pediatric hospice patients to have horrible days and great days and inbetween days.  Knowing Cicily, she'll likely surprise everyone and do very much her own thing.  

Cicily could be here another few days or another 6 months or more.  All hospice means for her is that she's not doing great and it could be anytime.  But she also may rally and have some great time ahead.  We're just focusing on taking all the photos and making all the memories we can while we can. 

Hospice or Healing?

Not a decision anyone wants to make in life, but one that many of us are faced with (hopefully with a parent not a child) is  - when is a person too sick to continue trying and failing to "fix" their body?
So as you know, Cicily has had significant loss of function over her lifetime (crawling-not, sign language of 75 signs- 5, hand control-tight fists, etc...) Along with these outward muscles and function deteriorating, her inner muscles have also been losing some function (more lung issues, less bowel movement).
On Tuesday Cicily went to school like normal and ended up falling asleep at school (NEVER happens for her, she hates sleep).  When I went to pick her up, her 1:1 nurse Tamara (who has been a miracle for 8 years of Cicily's life) let me know her pulse was weak.  I brought her home and we snuggled in the chair.  Around 4 I texted Chris and let him know her tummy was very huge (presumably with gas, but worse this time) and her breathing was quite labored, but that I thought he was fine to stay at work.  (When you have a child with a shortened life span, you do weird things like staying at work when they might be dying, because you have to keep living!)  Chris came home at 5:30 and agreed she looked bad so we called her grandparents over to see her and help Chris give her a priethood blessing (a special blessing/prayer of healing or comfort).
Chris and I felt this blessing would guide us in our decision on what was best for Cicily.  We both felt like her body was wearing out enough that an ER visit and subsequent surgery would be more harmful than helpful, but we wanted the clear guidance from Heavenly Father that that was Cicily's path in life at this time.  I'm sure Chris was still hopeful to bless her with healing and a strong body as he's always felt he could.  But at this blessing, Chris felt very impressed that he was to bless Cicily to be able to meet her angels (great-grandparents, family, friends) waiting for her in the spirit world (where we believe our spirit selves live after they leave their earthly bodies).
We honestly thought Cicily was doing badly enough that Tuesday night could have been her last night on earth.
Then Wednesday she woke up (after farting up a storm in her sleep!!)  Since we felt that surgery and other major medical interventions were not going to help Cicily at this time and it is her path to move on, Chris called hospice (usually I do this work, but I couldn't make that call).  We are now officially set up with hospice and meds like morphine if she needs them for pain.

Pneumatosis intestinalis - muscles wearing out

In February we started to notice Cicily having loud gassy bowel sounds and pain when we moved her legs around.  Her GI (digestive system dr) ordered an x-ray and they found she had free air outside of her colon.  This means she had pneumatosis intestinalis (small wholes and inflammation in her intestines).  We tried giving her straight pedialyte for a week to give her colon rest time to heal and repeated the x-ray and it was still not looking good.  So on March 7, Cicily was admitted to the hospital so she could be on IV nutrition (TPN/lipids).  She was there 6 days and the x-ray showed improvement so they tried pedialyte again and after 10 hours the small holes were back.  So we decided we wanted to bring her home and continue to give her TPN at home to allow more bowel rest for healing.  Chris and I were skeptical from the very beginning that her colon would heal.  In speaking with the palliative care (comfort/child's not going to live long) doctor, we found out that most kids with degenerative brain conditions end up needing TPN because their intestinal muscles wear out.  We've seen signs of Cicily's intestines losing function so this made sense.  Typically colons heal in 1-2 weeks.  We're in week 4.
So now our only option to heal Cicily's intestines would be surgery, where they would cut her intestines and piece the healthy ones left back together.  In Cicily there aren't many healthy parts to piece back together so this option would really create a whole world of new problems.  If Cicily were healthy we'd of course do this option and she'd heal and be ok.  But, as Cicily's intestines are wearing out, so also are her other internal muscles.  Unfortunately there aren't any tests on internal muscles to check for function (I asked).  So Cicily will stay on TPN for the rest of her life (we think, of course we never say never for Cicily).