On Thursday we met with a pulmonary specialist in Portland. I had really built up this visit in my mind as one where we would be overwhelmed with information, learn about using a variety of different machines and walk away with ways to help our son. Let’s just say that wasn’t quite our experience. The doctor seemed nice enough and made it clear he was not there to judge any decisions we make- which I appreciated. He has had 10-12 patients with this disease and was very direct about the fact that his role was not to “save” our son, but rather to monitor the progress and help us make choices as we reached times when choices needed to be made.

Having read almost everything we can get our hands on about this disease, we didn’t really learn any new information. We did discuss the importance of getting a feeding tube put in for little Ezra. I have been in contact with a few different families who have had or have children with SMA Type I. Each mother has shared how the feeding tube helped their baby conserve energy and took away the stress of eating. It seems to make sense to have one put in before Ezra loses the ability to nurse and swallow- both of which will happen. So, we will be scheduling surgery to have a port put into Ezra’s little belly. In terms of machines, for now the doctor has started paper work for us to access a cough assist machine. We will use this occasionally to help clear some of the congestion from Ezra’s chest and hopefully help him breathe easier. At some point we will probably use a bipap machine and maybe oxygen therapy.

I asked our doctor how it is that some children live to be 4 or 5 or older with SMA Type I. He explained that there is a range of severity of the disease and some children have a “milder” form of it. Because of the symptoms Ezra is already presenting with, the doctor said he believes Ezra has a “more severe” type. Ezra is very gurgly and congested through his chest. His cough is very weak and for the past week, he has been waking up every half hour at night. But some good news we learned from a chest x-ray taken at this last visit is that his lungs are clear and there is no infection in his body. It will definitely be helpful to meet with the doctors at Boston Children’s Hospital this week as they have seen hundreds of kids with SMA. I am curious to see if their assessments are the same as this doctor we saw.

If you haven’t picked up on it yet- I can quite easily relate the above information because it is way too hard to believe that I am sharing this information about my own child. I think we have entered the self preservation stage of this experience. We found ourselves sitting in the doctor’s office discussing all of this really intense stuff and for the first time, I wasn’t a sobbing mess. Ethan shared that he almost felt like an actor playing a role of a parent who’s son is dying. I wouldn’t say we are disconnected, but it’s almost like we have to protect our hearts and in certain situations, we have to rely solely on our brains. We don’t allow room for the emotions to take over. The emotions sneak in other times- like when Daisy asks me, “when will baby brother feel  much better?” or when she started singing to Ezra one night, “I’ll love you forever, I’ll like you forever, as long as you’re living my baby you’ll be”. Not sure how many of you are familiar with this touching (if not slightly disturbing) book about a mother who rocks her son through all stages of life- even as a grown man. In this context it just broke my heart. There were many tears that night.

Oh Daisy… our spunky little Daisy  is the epitome of someone who lives life to the fullest. This child is a bundle of energy, imagination and joy. She is curious, hilarious and smart as a whip. Daisy experiences everything with a full range of big emotions and is not shy about letting us know exactly how she feels about something. We are trying to be intentional about the way we talk about Ezra with Daisy. We have started reading a few books to her that discuss death and let me tell you… it’s insanely uncomfortable to do. Obviously a 3 year old processes things much differently than a grown up does and we are trying to only share these books from time to time. I’m sure the questions will change as she gets older and we will see her grief manifest itself in different ways as she grows. It is so painful to think about Daisy growing up without her little buddy. Her innocence will come to a screeching halt the day her brother dies. It will change her life story and forever she will have to say that she had a brother who died when he was little. I don’t know how she will handle it when he dies, but I know that it will be something she will have to process and work through her whole life. It is such a saving grace that all Daisy really knows how to do is to live in the present. She does not know that her brother has a terminal illness- and of course she doesn’t need to know this. She is living each day, soaking it all in- as we all should be doing. We have no idea how long we will have Ezra for… but the truth is in life we never know how long we will have people for. So I wish I could bottle up Daisy’s ability just be in the here and now and not worry about how and when sad things will happen.

Over the past several weeks, Ethan and I have heard from a few parents who have had children die or are frantically trying to learn more about a condition their child is struggling with. It struck me that there seems to be an awful secret club of parents who truly understand how helpless and desperate it can feel to try to help a child without the knowledge, tools or ability to do so. Somehow each of these parents finds a way to get up every morning. We go to work and doctor appointments and the grocery store. Several people have kindly commented on a strength they sense through my writing. While I appreciate hearing this, the truth is that anyone who finds themselves suddenly  a member of this awful club would find a way to gather the strength to get through the challenge. I know that Ethan and I have to be strong… we have a precious little baby that is counting on us to be determined and vigilant. We have a beautiful little girl who relies on us for a stable foundation so she can go on being a silly, carefree 3 year old. Life continues to go on and so we must find a way to do the same.

Over the past few weeks, we have had some amazing visitors come to see us. They brought with them food, recipes and lots of toilet paper from Costco… most importantly they brought fresh energy to share. They read with Daisy, make food for Ethan and me and shower Ezra with love. My sister Karen was here when we first were told to go to the neurologist. Her presence was so amazing as we were trying to hold on to shreds of sanity through this crazy news.  Soon after my Uncle Rich and Aunt Vicki came armed with Costco goodies and a willingness to do anything that needed to be done around the house. As I type this, my sister in law Kara (from Houston) is in my kitchen cooking up some delicious soups and breads. She is also organizing my closets and making my children crack up.  Amazing. My in-laws (aka Mimi and Papa) are willing to drop everything in their busy lives to help us in any way they can. They have watched Daisy countless times as we run back forth between doctor’s visits and their support is priceless. We really cannot express how much this love- and the love we feel from phone calls, emails etc…- really helps. It makes all the difference.

We visit the SMA clinic at Boston Children’s on Thursday… thank you for keeping us in your thoughts.

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Some books I recommend for bringing up the topic of death with children:

-Lifetimes by, Bryan Mellonie and Robert Ingpen

-I Miss You by, Pat Thomas

-The Fall of Freddie the Leaf by,Leo Buscaglio

-I’ll Always Love You by, Hans Wilhelm