One year ago today

Where to begin. We had been surviving on next to no sleep. Layla's whole life thus far (9 months) she had only slept for an hour or so at a time through the night. Then in the day she would only ever cat nap in someones arms. She would cry so much of the day and night that it really got into my head. I would do anything just to keep her content. The only ways were to stand and rock her, breastfeed her and sometimes go on walks.. at a brisk pace and on a bumpy road. All were physical activities for me. There was no rest. For any of us. Aaron was doing a fast track program which left him with many hours of homework, while trying to help with Layla. I honestly don't know how he did so well in school with such lack of time and sleep. Though in my eye, at least he got a break from the craziness that was our home life. It's insane for me to look back and think so many things were normal parenthood for too long. You know when they say mothers should trust their instincts this what one of those scenarios. And I wish I trusted my instincts sooner.
Exactly one year ago today was our whits end. Layla had gotten progressively worse. Which we were told would happen for kids with SMA. We'd been able to (somewhat) deal with the crying, lack of sleep etc. but the one thing that scared the living daylights out of me was her breathing. As she became weaker, it became harder for her to breath. Her whole chest would concave, you could see her neck straining and a high pitch wheezing sound would come out as she tried to breath. Her doctor said because of her hypotonia (low muscle tone) she didn't have the muscle strength to open her airway wide, which made those dreadful scary sounds.
Aaron came home from doing five-twelve hour shifts in a row at his work placement for his schooling and I was standing there in the kitchen, crying and rocking Layla who was also crying. This was nothing out of the norm. I said "I can't do this anymore." (Which I'm sure I said multiple times but this time was different.) Aaron said without question "We're going to the hospital." We took a few minutes to pack up some clothes for us all, because I had a feeling that we would at least stay a few days. While on the road we called two people in our lives who work/worked in the medical field to ask their opinions on going to the local hospital where Layla's pediatrician would meet us or to keep on driving to get to the children's hospital. In the end we decided to drive to London Children's because we figured we would get likely sent there from the local one anyways. They are just more set up to deal with kids. As we drove, she fell asleep. Then I started doubting why we were going. I asked Aaron "What if they send us home, and think i'm just an over paranoid mom?" or worse "What if they can't help her?" I had this deep, dark pit in my stomach that was making me think we wouldn't get to come home with her again.

We were so fortunate to have a great friend attending med school at the time. He met us at the hospital and even parked our car in the staff lot to save us some money. He came in and stayed with us until the wee hours of the morning. He kept us calm and distracted us. He brought us food. He gave us ideas of what was going on in the background while we waited. I am so thankful for him in that time and especially the days to come.
I wrote once before about the nurse who admitted Layla and asked me to sit her on the scale. We had a funny little spat in triage.
"Please sit her on the scale."
"She can't."
"Why?"
"She doesn't know how to sit."
"But why?"
"I don't know.. that's why we're here!... The doctor thinks she has SMA."
"Okay, then lay her on the scale."
It was actually awful. It was one of those moments that I had a realization that there was something different about my child. (I have had a lot of these moments along the way). The number on the scale was wrong. It had to be. It was so much lower than what she weighed in at only a couple weeks before at her doctors office. I thought the nurse did it wrong.. but it was right. Layla had lost a lot of weight and fast. It made no sense to me because I was feeding her so often. We were admitted fast but just sent through to a private room where we waited longer. I put Layla out on the bed and she started to smile at the crinkle paper under her. "Sweet, now you smile. I haven't seen that smile in weeks and you choose now to smile!?"
Waiting for the doctor I decided to attempt to feed Layla. What I didn't know was that would be the last time I would breastfeed her. We say getting the gtube put in is the best thing (in hindsight) we could've done for her, and it was, but I never got that moment to kind of prepare myself for it to be done. Not that it was a great experience anyways. She fed every hour or so, never latched well, and it was always very wet and messy because she wasn't strong enough to swallow all the milk down. I pumped in the hospital for 3 weeks, giving it to Layla by an NG tube (through her nose) and then decided it was the end of the road for me, or rather for my boobs, right around the time we decided on going forward with the gtube surgery. That's a whole other story. Stopping pumping actually lifted a small weight off my shoulders. One less thing to worry about in all of this. In the stress of everything I wasn't producing enough milk for her daily intake anyways.
They decided right away that Layla was very dehydrated and needed fluids. Easier said than done. It's hard to get veins from low tone children. It's next to impossible to get a vein from a child who is very low tone and super dehydrated. They tried each arm and leg before asking if they could attempt in her head. They could see one through her pale head that they were sure they could get. We agreed. She needed fluids. They shaved a spot and gave us a little baggy from her first haircut.. is that even in the realm of thought when one thinks of their child's first haircut?
Quite quickly we were admitted to the pediatric critical care unit (PCCU). By quite quickly I mean by 5am after arriving at the ER at 10pm. They sent us to go lay down for a bit while they got the IV, NG, oxygen and sats tubes and monitors all set up. We lasted about an hour before coming back. One of the nurses overheard me and Aaron making fun of how I was uncertain the day before that we should've come to emerge. He said "If you end up in here, you really were supposed to come to the hospital." On the regular pediatric floor the nurses to patient ratio is 1:4 sometimes 1:6. In the PCCU the nurses to patient ratio is 1:1 sometimes 1:2. And they are set up right beside the patients 24 hours a day.
Prior to this hospital stay we had been waiting for June 4th to come around when we would meet a neurologist. He was going to confirm Layla's stage with SMA, be our foot in the door to the children's hospital and advise us on where to go from there. We obviously didn't make it to that day. But on our first full day in the hospital we met him at Layla's bedside. He first told us "Do not tell any other people that this is how you get to see me sooner than their appointments." Thank God for some humour. When we told him Layla's story he asked if blood work had actually confirmed SMA. We told him no. But our pediatrician said he was 90% sure that she had type 1 SMA. He understood why that diagnosis was thought of but was appalled that someone would give a life limiting diagnosis like that without having proof. And right there, right off the bat said "Looking at Layla, hearing her story, I do not think she has SMA. I can't guarantee any much better outcome but we're going to try to find out what she does have."
There you have it. Day 1 of 30 in London. Our first encounter with 1 of 13 amazing specialists. It seems like yesterday. Even though we didn't come out with a diagnosis. We came out so much more knowledgable about our daughter. She came out healthier. And here we are one year later.. she's still undiagnosed. Still working with amazing, but different specialists, in a new (but old) province. And most importantly, Layla is still here.

The Whitehouse

Met with Layla's specialist in Vancouver yesterday. She is new to BC children's from Washington DC. She is another one of those scientists who I love watching. As she studies Layla I can see the wheels turning in her head. She often takes breaks after I've answered a question to just be in her thoughts. I've found that these doctors tend to not be as good with the social aspect of their job but I have no less respect for them.. possibly more. I wish I was that smart. 
For the first time she told me of the undiagnosed disease program at the National Institute of Health (NIH) in Washington. This is where she used to work. She is going to refer and help us apply to have Layla accepted into the program. If accepted we will be invited to come stay at the NIH for about a week where they basically would just go hard in looking for a diagnosis. Instead of continuing slowly as appointments come available and we book them. For example, Layla's currently on the waitlist to get another MRI to see where her brain development is at. This is something that is super important for us to see how far she has come from a year ago when we were in the children's hospital in London. However, the waitlist is up to a year long, sadly. At the NIH, they would look at everything that has already been tested, of course, but likely retest some of these. They would do bloodwork, an MRI, EEG, EMGs.. anything they see fit, that might help with the diagnosis.. and we'd get the answers from these tests right away! No guarantees of diagnosis but it's got to be fairly high.. right? I mean, these are top of the line specialists with top of the line equipment.  
I'm so excited. We could finally get the answers we've been looking for, for so long. An idea of the future. An idea of how to best help my baby girl. So often I feel so helpless with her. She can't tell or point to where her pain is. And sometimes it's just neurological where there really is nothing I can do.. and that is so incredibly hard as a mom. All you want to do is take the pain away. If we can get more of an idea of what's going on in her brain, body, chemistry etc. I just feel like we'll only get positive results. Of course there is the chance we will get a very negative diagnosis... but we've been there. We've processed SMA, and in this medically fragile, unknown world we have to constantly process the thought of Layla possibly not being with us long. Or with us a long time while being 100% dependant on us. The uncertainty is worse than any diagnosis they might give. 

Literally moving forward

I wrote last time about the phone call from our physio therapist inviting Layla to Power Mobility Day. Both Aaron and I thought there was no way Layla would be able to move a power chair on her own. At the weekly physio appointments I continued to discuss what it would be like. My hopes weren't high for anything exciting. The only thing that started to intrigue me was this really old sensory toy we were loaned. It has a string with a wooden ball at the end. When you pull it there is a fan that starts blowing at you (cause and effect). Again, this was a toy that I didn't believe we would use very often, if at all. But once I wrapped Layla's hands around the string I quickly saw she was able to pull on it to get the fan going. This sprung a big smile as the wind blew on her face. Then she would release a bit, and do it again! Pretty amazing. Not after long her hand would slip off but we would still cheer her on as she's completed a little milestone. 

Fast forward to this past week. To my surprise we got to experience the most incredible thing with her development so far. Layla moving herself, on her own. Well with the help of the cutest power wheelchairs you've ever seen. The first chair we were able to fit in her custom seat from her stroller so she was completely comfortable and supported. We placed her hand on the joystick and pushed it forward so she could get the idea. Almost right away she was able to pull it to the side and start moving, ON HER OWN. *Que the waterworks* She started by mostly doing circles. Then there were quite a few jerks but changes in direction and speed. To me that shows it wasn't just by chance. We didn't just help her start going and then the weight of her hand just sat there, continuing to move her in that direction. No, she was working hard. You could see it in her face and jaw. (She often clenches her jaw when she is trying hard.) She got a chance to try three different chairs. 
Everyone was in awe. I don't think anyone in the room thought Layla would be able to do what she did. It was little but oh so big. Now we feel like we have to get one. She needs this! Unfortunately these wonderful contraptions are only available in the UK and are unable to even be shipped to Canada. Boo. But either way this is such a positive thing to know that she is capable! It makes me realize how important it is for Layla to have the opportunity to have some independence to help with her development. There are very few things she can be independent in but a power wheelchair could be one of them.
I don't really know where to go from here but I'm starting the research. Is the government able to fund some or all of a power wheelchair for Layla? What are the options in the area? Can we possibly find a used chair? Should we fundraise ourselves? Many questions to be answered. One thing is forsure, Layla is going places. Literally.