Long winter days

I usually try to keep my posts upbeat and positive and focus on the good but that is just hard to do all the time. I also told myself I wanted to be more honest and real in my blog, sharing with you all what life with SMA is truly like. And this week, frankly, it has sucked. One of the words that I would use to best describe SMA is limiting. Believe me, I am more than thankful that Lily is here with us and I would chose her being here and having SMA any day versus the alternative. However, SMA has taken so much away from Lily. Sometimes I just want to scream and I imagine that is how she feels too. Wanting to move her little arms and legs and run and play and kick and not be able to. Sigh. It's so frustrating. And its even harder in the winter because we are basically on lockdown. 

We don't get out much because we can't expose her to germs. I literally have cabin fever. (For those who don't know, we are living at my in-laws cabin). It has been great, but it is smaller than our apartment that we had and we have limited space. Anyways, the days get long. Lily and I are home most days (unless we have Dr. appointments or venture out to grandparents.) It is hard to keep her entertained and happy, especially with her having to lay flat the majority of the time and to keep her 18-month old mind stimulated. She is smart and she knows what is going on and I think she is starting to realize even more what she can and can't do, which might be contributing to her increased fussiness or discomfort.

It has been a long week. She has started to have episodes where she gets fussy/upset which then leads to more secretions, dropping her oxygen sats, and ending up on bipap to get her calmed down/sats back to normal. She was on the medication albuterol which a lot of people use for breathing issues, such as asthma. But kids with SMA often use it to help motor strength as well. I feel like we had seen an improvement in her leg movement at times with the use of albuterol. However, as we increased her to the maximum dose, her face got beat red, her heart rate would spike, sats (oxygen) would lower, and she would need to be put on bipap to get her back to baseline and calmed down. After I was able to talk with Dr. Schroth's (Lily's pulmonologist) we decided it was best at this time to take her off the albuterol. We could always resume it later, but if it was causing her so much distress, it wasn't worth it. So I thought that was going to take care of the fussiness, high heart rate, low sats, etc. Not so much. She has continued to get upset which leads to crying, which leads to all of the above symptoms. Frustrating for both her and I. 

Yesterday I had washed all of her tubing for her bipap machine and cough assist, which I do not do as often as I should but I was motivated and was going to get it done! So of course, as all her tubing was air drying (which takes some time) she got upset and had increased secretions and decided to desat on me. I was scrambling to get her bipap set back up and dry her tubing with a hair dryer (which I'm not even sure you're supposed to do) but I was desperate, while at the same time trying to calm her down and get her sats back up. Usually when her sats drop, we just put her bipap on and she is fine. So I ended up having to use our emergency 02 (plus trying to hold her to comfort her and play her musical snow globe) to get her happy again. It stressed me a little-just not feeling prepared. So next time I will know better and I will have extra sets of tubing for each (hindsight is 20/20 right?) Such a learning game this journey is!! Mom needed a nap after that all happened. I wouldn't have even been as stressed about her sats dropping a little bit, but kiddoes with SMA can get mad, fill up with secretions, and just pass out (as my daughter as done before.) Not fun and I would rather avoid that! So stressful episodes like those make me so angry and frustrated with SMA, especially when I'm already worn out. 

Thankfully, Lily had a pretty good night tonight and didn't require additional bipap time after her nap, which is great. That being said, it's just hard to know if these little "episodes" are anything to be concerned about or if its simply just toddlerhood. That is another hard thing about SMA-whenever anything is abnormal or a little off with Lily, we always wonder is it something SMA-related or is this just a normal thing that occurs. We have to be so cautious with Lily because a little cold or change in respiratory status could be detrimental to her health. As if I wasn't already psycho enough being a first-time mom :-) I'm sure all you moms can relate! But after those intense, frustrating moments pass and things are well in Lily land, I thank God that she is here and that He helps me get through those times. And I have the best little girl to treasure and enjoy-even if she does wear me out. It is and will always be worth it. 

There have been many, many times I didn't think I was going to get through obstacles or stressful experiences in my life. Since our journey with SMA began, I have often thought, "I can't do this anymore!" but He has never failed me yet! His mercies are new every morning-whew! 

#neverlosehope #curesma #loveforlilygrace

A few pictures of my girl:

                          

Lily received this awesome doll, cat Mustard, as well as the Olaf hats from an amazing crafter with Feel Better Friends, an organization where volunteers donate handmade dolls (and accessories) to children with illnesses or terminal diseases. So cool----Lily loves her Lily doll and pet Mustard! Her doll also has an angel arm-wow! Check them out on facebook- https://www.facebook.com/FeelBetterFriends