Monday, August 26, 2013

End of Summer




Photo by Jen Pinkerton

Photo by Jen Pinkerton
 I know as each year passes it will become more and more cliche to say, "Wow, summer really flew by".  I'm acutely aware of the countdown of summers we have with the kids before they go off into the world as adults.  Each new milestone is exciting and makes me so aware that we won't share them with all of our kids.  As Aria and Miles excitedly got ready for their first day of school this year I focused on them, and their joy, and how much bigger they had gotten and their excitement at seeing friends again.  In the back of my mind was the reality that this would have been the year that Liviana stood next to them in her uniform, ready to start kindergarten.  I really can picture her long, dark curly hair in her St. C's uniform smiling, arm and arm with Aria and Miles.  Two days into school the same realization hit Miles and Aria as Miles said, "Liviana would be at school all day with us this year".  They then went on to say how they would have seen each other during the day and waved, how Miles would go get Liviana at the end of the day and then they both would go run and find Aria.   Miles stood next to Livi's bed during the conversation talking about school.  Livi was already ready to be like Aria and Miles with her little backpack and lunch bag in hand after I would take them to school back before her diagnosis.  I have a feeling she still has those images in her mind as they get dressed the mornings and leave for the day.

After my last post in March things were just a roller coaster of oxygen and respiratory issues for Liviana.  I could not get a good handle on getting her well until one day it all hit me.  I made her a batch of food for her g-tube and it wasn't running through the pump (even with my usual modifications to the bag), so I ended up setting it up so I was slowly hand pushing her 80ml an hour food through a large syringe, directly into her g-tube.  I would watch the clock and go push a little through over the course of the hour.  As had been her pattern for a few weeks, she started needing oxygen support, breathing heavier and getting very junky sounding.  Because I was sitting right there pushing the food through, rather than her pump running throughout the day on its own, I suddenly realized the issue was that she was aspirating her food...her rate and volume was too great for her.  We have had to make modifications to Livi's allowable food volume since she lost her ability to swallow and started on a g-tube. I think she was on 240ml an hour, 3x a day when we first started.  That slowly trickled down to less volume and then switched to continues feeds instead of bolus. All signs of the disease progression.  She had been on 80-90ml an hour for about a year.  I started reducing the rate....70ml, still aspirating, 60ml, still aspirating, 50ml, 40ml, 30ml...until I settled at 20ml an hour.  She still had some issues with aspiration at 20ml an hour but was able to stay off oxygen with that rate.  20 tiny ml an hour was all her little body was able to take any more.  That is less than an ounce an hour.  I knew what this meant and I worried non-stop that her body won't be able to last much longer against this darn disease. I suddenly felt extreme anxiety, helplessness and fear.  I felt like I paced all day and almost took on "nesting" type behaviors, obsessively cleaning the same places over and over in a failed attempt to control something, anything, for Livi's sake.   Since I had been making her food I also realized that without a lab in my home, I could not reduce down what I was making for her into such a small volume and have it contain the benefits that she needed.  I immediately contacted her doctor to discuss our options.  Something I never thought I would have to do, since I made sure to always provide Livi with "real" food...I asked if there was a formula that was broken down enough, that she could be put on instead.  This was really hard for me to do but at the same time, easy...because I want my baby still here.  We decided on Vivonex Plus which is completely broken down to aid in getting in out of her stomach quickly to help prevent aspiration.  In that small volume I would be able to give her what she needed throughout the day.   I still checked with the surgeons office to see if a g-j tube would help her, if different medications to help with gastric emptying would help her.  I know I probably seemed like a desperate, slightly irrational person to some of the doctors offices and nurses.  It was as if I thought I could outsmart the MLD...."Ha, take this MLD, I will do xyz so you can't further take my baby from me".  The day she started the formula I felt so relieved but also like I had failed her too...suddenly helpless like I couldn't even provide her food for her anymore and keep her alive.
Photo by Jen Pinkerton


She continues on 20ml an hour.  When I have tried to add fat to her formula with avocados or other options she aspirates....it is just too much for her and I think it sits for a long time and slows her gastric emptying.  So, Livi is tiny...really tiny and it breaks my heart.  Every time some well meaning, person says, "she is SO thin".  I want to scream, "I know, I know, it rips my heart out every single day and there is nothing I can do about it...she is here and that is what matters".   I know people are just reacting and it is shocking to see.  It is such the base of mothering, keep them healthy, fatten them up, give them nutrition...I feel so helpless.

Since the big food switch Liviana has stayed off of oxygen except for a few hours here and there after an outing (which seems to stir up the respiratory junk for her).  Nobody has been sick and she has stayed well.  I absolutely dread going into winter again.  Her body is weaker and I honestly do not know if she will be able withstand an illness like she had last December/January.  I think even a minor respiratory bug may be too much for her little body.  Aria is sick right now (one week into school) with a fever-headache combo and is on isolation upstairs in the playroom.  She has a couch and TV to keep her cozy.  I spray Livi's area with germ killing essential oils and douse any other area of the house I can think of to keep the germs at bay.
April 2013 Photos


It was around this time that Brad and I decided we needed to do something we had been putting off, and that was to make Livi's pre-funeral arrangements.  Her Hospice team had been reminding us of the importance of doing this so we are prepared when the time comes and given her sudden decline we knew it was now the time.  I can't even describe the knots in my stomach leading into that appointment.  It isn't something a parent should ever have to do and not something I ever thought I would have to do when we had children.  The man at the funeral home was very kind and soft spoken and put our minds at ease that things will go smoothly when the time comes.

As with her food intake, there have been other signs of disease progression.  Liviana has become much more rigid.  It has been as slow process but is now to the point that we can't get her to straighten her right leg anymore.  In late May we had to start being selective of where we took her because she just wasn't comfortable for that long if we were out and about.  We pretty much only took her to events that were at the school so we could walk and I would usually hold her once we got there so she was more comfortable.  We always had to plan ahead and give her a little lorazepam so she would be more comfortable.  I hate leaving her at home.  I can't stand going to soccer games or other events without her.  I feel so incomplete and anxious the whole time.  The kids always seem more excited when we all are together.  At the end of July there was a car show at Joslyn Castle and since it is just a short little walk and it was when we had the unseasonable fall-ish weather, we thought it would be the perfect outing for us all.  I was SO HAPPY leaving the house with all of us together.  It felt complete, at ease, smiling, happy, normal.  I didn't feel like I had left a child by mistake or was missing some huge part of me while we were out.  I really could have stayed there all day just so we would all be together.  I am not sure how the upcoming soccer season will go.  I think we will try to bring Liviana to the first games at the school but we will have to see if she can handle the walk up there.  Her body just has gotten so weak and fragile and her rigidity makes it harder for her to be comfortable no matter how much we pad her seat.

My daily routines of changing her and cleaning her up always leaves me in tears.  I really can't even describe how emotionally difficult it is to have your fragile child in your hands.  It just isn't fair for Livi.  Saturday as I was getting her changed I picked out a favorite polka dot dress.  I still always show her what cute outfit I am going to put on her.  I told Brad I didn't think I could get it on her without making her really uncomfortable.  It has gotten increasingly difficult to dress her and change her diaper.  Brad said we could cut her dress.  I knew that was the next option for her clothes but it left me crying, to myself, when I left to run errands shortly after.  It just absolutely broke my heart for Livi...she loves her pretty dresses and she deserves to wear them normally darnit.  Probably a silly thing to get upset over but you never know when your emotions will catch you off guard in these situations.

Despite these challenges Livi faces she still smiles....we are so, so blessed to still see smiles and happiness.   The whole world could learn a lesson from her spirit.  More than once I have been sitting with her and she will hear her brothers and sisters laughing or playing behind her and she will get the biggest smile.  She can't see them, she can't join them, she can't do what they do but she takes such joy in their joy.  What a powerful lesson.  Livi has pain, Livi has physical struggles but Livi is not a suffering person.  If only people who have oodles and oodles more than she has could stop and be happy with the simplest things and not feel sorry for themselves.  Gosh she is a powerful spirit of joy in my life.

How is sweet Giovanni doing?  He is keeping us laughing.  I swear, I have said it before but he needs his won TV show with a camera on his head to follow him around during the day.  Giovanni "played soccer" this past spring.  If we are being honest, he had a solid 45 seconds on the field and then decided that he was done and just wanted to retrieve his ball and come sit back down.  I think next year will be his break out year on the field :).  He loves to play with Liviana and bring his toys next to her and pretend they are having fun together.  I will find toys in Livi's hands throughout the day and he likes to give her money to hold sometimes too.  I think Livi holds a very special and large place in Giovanni's life and heart.  I have no doubt that those two are connected in ways we could never understand.  Every single time we leave for a soccer game or other function Giovanni asks, "E going" (that's what he calls Livi).  When I say, "No, she is going to stay here" he gives an "Ohhhhhhhh".  I know buddy, I know.



He continues to be a normal 3.5 year old boy.  He jumps probably 100 times a day for everything from Fireman Sam coming on, to snack time, to the mailman coming by.  Every time he jumps I love it!  Livi could never jump and Giovanni makes sure that we know he is an expert jumper.  He physically is doing awesome although Brad and I have both been aware of some transient balance issues and falling down more.  Now, Giovanni has HUGE feet.  Very large.  He is a very tall boy and maybe a tad awkward because of his height and long legs (and big feet).  I think some can be attributed to being a little clumsy but other things are definitely of another concern to us.  I am hoping this visit to Italy next week reveals no changes and possibly improvement in that nerve conduction test.  It stops me in my tracks each day what a miracle everything he does is...I know the alternative he would face and his life is amazing!

Giovanni and his hero buddies made the international news in July when the initial research from his trial was published and created quite a buzz in the medical world.  http://www.sciencenews.org/view/generic/id/351534/description/Gene_therapy_treats_children_with_rare_diseases

We think Giovanni is going to be an engineer of some sort.  He spends much of his day building things out of random things that aren't meant for building.  He creates ladders out of anything and everything, he constructs homes for his people and trucks, he builds buildings, garages and anything else he can think of.  One day, Brad went upstairs and he had built a pulley system to lift items onto one of the back of his trucks.  On the 4th of July he asked Brad for a few random items and this is what Brad found he had constructed when he went outside to investigate.  This was a cheap little hand held rocket launcher Brad got the kids.  I guess G decided it would be more useful if he modified it some.



Isn't he amazing?  He is just 3.5.  I love watching his mind work.  He is also so gentle and sweet and conscientious.  He listens so well.  He started speech in June along with Miles, at Stones Worth Stepping.  They are wonderful and within one week he finally started calling Miles by his name instead of just  the "M" sound.  I think he is a joy to his speech teacher (right Sarah :)) and she is a KU grad which is bonus points :). At the age that G is, Livi already could no longer walk, talk, sit unassisted, swallow or eat.  It leaves me in awe so often.
 
I finally broke down and took G for a haircut in early August.  I loved his long hair but it was time for a change and oh my goodness it made him look so much older.  What a handsome, handsome, amazing little boy! 

 
I was originally taking Giovanni to Italy on the 31st but we had a change of plans and Brad will be taking him and I think Mr Miles go the lucky draw to go along since there was already another ticket.  It will be very hard sending my 3 boys off to Italy and it will be very hard to not be there and hold Giovanni through these tests.  Please pray that they have a safe trip and that G's test results all come back with excellent results.  I need that peace of mind after the last visit's results.








Miss Adelina is up from nap now, with less than stellar blood glucose numbers, so it is time to run.  I will try to update after G gets back from Italy.  Please pray as we approach the fall and winter season that we can keep Livi well and with us.

Hug Your Babies!

3 comments:

Hannah @Supermommy!...Or Not. said...

Thank you for sharing your story. Your children are all absolutely beautiful!

Jennifer Stam said...

Thank you for the update. I am so happy to see G doing so well and so sad with tears for Livi )-: I pray this winter she stays healthy and keeps that smile and that you also get fantastic news in Italy!! Looking forward to the great news with that! I'm hugging my babies!

HSofia said...

Thank you for taking the time to put this down. I love hearing about your children.