Here is Nicholas in his new
EZ-On Vest which is a
specialized adapted car seat for kids with
SMA. The straps fit
snugly around him and attach to the seat belt to keep him securely in place when traveling. It also allows him to lie flat which helps keep his mucus at bay for long car rides. We
received the vest from
Families of SMA at no cost to us, which was fantastic considering they cost a few hundred dollars. We will return it to them when Nicholas out-grows it so another child can use it.
He was practicing his wave this afternoon and feeling pretty proud of himself!
Ella & Nicholas were both so happy to get out of our house! Because it is cold & flu season and because the flu hit our area fairly recently, I haven't been taking the kids out much at all. I think it has helped to keep them healthy, but it was also so nice to finally get out, even if it was just for Nicholas's pulmonology appointment at OHSU.
Nicholas has a new pulmonologist that we like very much! His name is Dr. Hsia (pronounced Shaw) and he has a good bedside manner and took his time in answering all of my questions during our visit. He suggested we schedule a sleep study for Nicholas to see if he is getting rid of his carbon dioxide gases while sleeping. I was hesitant to do this because of the logistics of it all...I am nursing Ella, Jeff is working swing shift, I would have to lug all of Nicholas's equipment with us (pulse/ox, cough assist, bi-pap, suction, feeding supplies, etc), and I just didn't see how it could be done. After a conversation explaining all of this, Dr. Hsia explained that we wouldn't need to bring any equipment with us since Nicholas could do the sleep study at Doernbecher's instead of a hotel (which was where we went last time) and my mom volunteered to watch Ella overnight, so it sounds much more appealing to me now. I am sure Nicholas will absolutely hate being in the hospital again, but it's really only for 12 hours, so I think he will manage okay.
We also discussed getting Nicholas a new ventilator, the Trilogy, because his current one is so darn loud and not very portable. Hopefully we can transition to the new ventilator very soon. We also discussed getting an ambu bag for Nicholas, as well as a nebulizer and portable oxygen. Dr. Hsia also discussed that we create a form which expresses our wishes for Nicholas health care and how it is handled in case of an emergency. He suggested that Jeff and I write it all out while Nicholas is stable so that we can be level-headed regarding all our our decisions about Nicholas's care and so that we can decide what will be in his best interest. This kind of discussion would have brought me to tears a year, even six-months ago. But I was proud of myself for keeping my wits about me and I totally agree with Dr. Hsia's advice. It will be so beneficial to Nicholas's care team to have a written document to follow when the unexpected happens. It's also nice to know that we can change or modify it at any time.
The last thing we discussed was whether to have Nicholas's trached (given a tracheotomy) when the time comes. I explained to Dr. Hsia that we would like to go the non-invasive route, which means Nicholas would not be trached and would be on a bi-pap most of the time when it becomes too difficult for his little body to breathe on its own. Dr. Hsia believes (as I am sure most pulminologists do) that a trach is the way to go. He said that it is easier for kids to communicate with the world and it is a better quality of life. I listened to his advice, but at this point, Jeff and I feel that a trach would be our last resort. This is a big debate in the SMA community and many kids have trachs and many do not. We continue to seek guidance from families we trust and our feelings may or may not change as we watch this terrible disease take over our son's body. However, we will always keep Nicholas's best interests at heart when we make all of our decisions in care for him. We only want to do what is best for him and what will give him the best quality of life. We hope that he will be able to experience life with as much love, laughter, and joy as possible and it helps to remember that as we make these difficult decisions for him.
Ella with my co-worker, Lisa 
I am so lucky that I work in an environment where I can bring my children into the office with me! I brought Nicholas to work with me for several months when he was an infant and have been able to do the same with Ella. I will say that it has become more and more difficult to actually get any work accomplished as Ella gets older, so I think she will be able to come in with me for maybe another month or so and then she will stay at home with her brother and Gramme. She loves to interact with all of my co-workers while she is there and enjoys playing in her Jenny Jump-Up as well. Her Jenny Jump-Up doesn't actually allow her to do a whole lot of jumping, but she doesn't mind just spinning around and taking in the office scenery. I am sure I have said this before, but it's worth saying again: I am truly so blessed to have such a wonderful part-time job that values the importance of family! 
Our six-month old girl is becoming a busy, busy bee! She is rolling everywhere and starting to get into things and, boy, does she love to play! She has also started squealing constantly which is very loud and hurts my ears if I am holding her. I try to ask her to "be quiet" when her brother is sleeping, but there is no reasoning with a six month-old. I am pretty sure she will have a tooth making an appearance soon, as she has been biting lately and chewing on absolutely everything. She has also been a grumpy pants the last couple days, which is not typical for her, so I assume her teeth are bothering her. The other day I let her chew on a bitter biscuit and she was as happy as can be sitting up in her high chair chewing away. Her personality is starting to come through and she is still our sweet little girl, but can 
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Here is Nicholas wearing his bi-pap (ventilator) mask before bedtime tonight. He just got a new mask because his old one was hurting his nose. Believe it or not, he really doesn't mind wearing it and is quite used to it by now. He sleeps really well with it on and rarely wakes up during the night. It's hard to tell, but he's actually smiling for his photo!
So, our sweet, nearly three-year-old boy has a lot of medical 
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