I was home Monday afternoon, when I got a call from the NICU resident at Primary Children's Hospital that Dr. Swoboda, the top neonatal neurologist, would in an hour be doing at EMG test on Scarlett to determine whether or not she had a neuromuscular disease. I packed up the kids really quick and called Jon to meet me at the hospital after he got off work.
When we got there, we decided that Jon would stay with Scarlett while Dr. Swoboda did the testing, and I would sit in the waiting room with the kids, hoping Scarlett didn't have anything seriously wrong with her, but praying that if she did, we could handle it and maybe she'd improve with time.
About an hour later, Scarlett's nurse came into the waiting room and told me that Dr. Swoboda wanted to talk to me. I just figured she was going to tell me they didn't find anything with the test and she'd be on her way, but as I walked through the NICU doors and towards Scarlett's bedside next to Jon, I immediately knew something was wrong. Jon was quiet and looked at me and put his arm tightly around me in a way that told me the news I was about to hear was not good.
Dr. Swoboda proceeded to tell me she found something with the test and that Scarlett had a degenerative neuromuscular disease. Upon hearing this news, I was surprised because I didn't feel hysterical like you see in the movies. Instead, my body went completely numb. All I could hear was the word "degenerative" and everything else she said from that point on was a blur.
I do remember, though, her telling me the name of the disease, I first thought 'I have never heard of that in my entire life' and then I thought, 'I will never remember that when people ask what Scarlett has', but somehow I've been able to memorize it. It's called Pontocerebellar Hypoplasia Type 1. It is similar to SMA, but includes problems with the brain as well.. If you want to read more about it, you can go here. Dr. Swoboda told us that in her 14 years and thousands of neuromuscular patients seen at Primary Children's, she has only seen 5 cases of this disease. It is so rare that they don't even test for the gene in the USA- it is only done in Germany and would cost thousands of dollars to do. Jon and I tried to do research on this disease to know what to expect with Scarlett and how fast it will progress, but most babies that have this, the parents decide to withdraw support and let there babies pass.
We had a care conference with the doctors and everyone else working with Scarlett yesterday and they gave us as much information as they know about this disease. They know that the growth and function of her brainstem and cerebellum were affected which causes the nerves to not respond to her muscles. That explains why she hardly moves, why her mouth can't close, why she has no facial expressions and why she can't swallow and that it is something she will never regain. The movements she does have now with her hands and head, she won't be able to do down the road. They told us the muscles around her lungs will weaken to the point where she won't be able to cough to clear out her secretions. Her muscles in her intestines will progressively get weaker to the point where she won't be able to tolerate her feeds anymore. The little winces she does when she's in pain, she won't be able to do and we'll only be able to tell by checking her heart rate and by tears that well up in her eyes if she is hurting or uncomfortable. And her contractures that cause her limbs to be so stiff will only stiffen more with time and cause pain.
Just writing all the things that are expected to happen with Scarlett, makes me cry. Right now she seems to be breathing comfortably and although she is having problems with her feedings, she still can somewhat tolerate them, and it seems like this past week she learned to blink her right eye, so hearing that she has a neurodegenerative disorder- we almost have to see her get worse than she is now, to believe it. I guess I am still clinging onto that little hope that maybe she will not progress in the disease for awhile, at least so we could bring her home and enjoy her before it does. But that brings in another set of problems- All the surgeries she would have to have to come home and the possibilities of getting a cold, and she would require 24 hour constant care, etc.
But with all of this, when I take a look into this trial we are going through, I feel like I should be angry at Heavenly Father. I should be angry that Scarlett was born this way and that she is not ever going to get better. And I should be angry that I will probably never get to bring her home and watch her grow and laugh and play. And I should be angry that we have to watch her get worse.
But I'm not.
And that's not to say that this heartache and sorrow I feel will go away. I know things will get harder as the weeks go by, but I pray that Heavenly Father will replace these feelings one day with pure hope and understanding and complete peace and gratitude.
But I'm not.
And that's not to say that this heartache and sorrow I feel will go away. I know things will get harder as the weeks go by, but I pray that Heavenly Father will replace these feelings one day with pure hope and understanding and complete peace and gratitude.
My sister in law, Jessie emailed us yesterday and described Scarlett in the best way. She said she is our "beautiful little blessing". And that is how I like to think of her. There is no way I could have grown as much as I have in my faith and in my love for the Savior and in my trust in Heavenly Father's plan without Scarlett. She is able to touch peoples' hearts in ways that I never could and most people couldn't- and she can't even talk. When I look into her deep dark eyes, I see love, happiness and understanding. When the other NICU babies are crying around her, she is at peace. And when I look at her sweet face, I don't even notice the tubes and tape that cover half of it, but I see a child that I am eternally grateful to my Heavenly Father for sending her to us.
I don't know what is going to happen these next few weeks, but right now I just want to enjoy my baby just as I have my other babies. I want to hold her and sing to her and read her stories of the things she'll never know of. I want to change her diaper and smell her wonderful baby smell and stroke her soft dark hair.
I love you Scarlett and so does Daddy and Hunter and Jaina and Grey and the many many other people you have touched. You are our "beautiful little blessing".
