At night I lay in bed with this little girl, holding her hand, simply amazed at how much I love her. How could you ever know before becoming a parent how your heart could grow, and grow ….and grow every day, consumed with so much emotion. As Sophia’s dad I have learned so much about life from this precious little package. There is not a single thing in this world that I would not do for my family. Today I have learned that sometimes you have to nudge your heart out of the way in order to make the best decision for your child. In the beginning of our journey with Spinal Muscular Atrophy, I was the parent who was prepared for dealing with the diagnosis and learning the best care options for Sophia. We were both extremely devastated and Catherine just needed a little more time than I did to grasp the realities of the disease. This time however, I was unprepared to face the fact that Sophia needed to be trached. I am so incredibly thankful to my beautiful wife for allowing me the time to come to terms with what needs to be done, and for allowing me to come to a place of acceptance.
Last year in May,we started noticing that Sophia was beginning to struggle to come off of her Bipap. She started to labor off of Bipap and would start to become extremely sweaty on her eyebrow and forehead. What seemed like over night, Sophia became dependent. We could no longer take Sophia off without her oxygen saturation levels plummeting and her heart rate would quickly follow. We tried to slowly wean Sophia off to no avail. In June I had called Dr Schroth and told her what we were seeing. She was surprised and upset since Sophia had looked so good at her visit in May. Over the next few months we began speaking to many other SMA families offering advice on different ways to wean Sophia. Nothing would work. We had multiple chest x-rays and Sophia has always been clear, I was hoping to see something that would point to a direct cause of Sophia’s dependency. In September Dr Schroth was in town and was nice enough to come by to see Sophia. While she was here I wanted her to see what happened when we took the Bipap off of Sophia, after witnessing Sophia immediately drop Dr Schroth informed us that for Sophia the safest option was for her to be trached. Catherine and I cried and Dr. Schroth consoled us telling us we had done all we could but unfortunately it was a part of the disease. After seeing Dr Schroth we wanted to get a second opinion on Sophia. Later that month we took Sophia to see Dr Bach and Brian Weaver. At that visit we tested Sophia’s vital lung capacity……..it was a .5 and Dr Bach informed us that unfortunately Sophia would probably never return to being able to come off of Bipap. Brian Weaver had suggested a Trache for Sophia’s safety and Dr. Bach informed us Sophia could possibly maintain just being on 24/7 Bipap. These were some of the most highly respected professionals in our disease, but I still wanted to believe in my heart that there could be a chance Sophia would somehow bounce back.
Living the past year with Sophia’s condition has been extremely challenging both emotionally and physically. Sophia’s dependency was every second of every day. To bathe Sophia and to wash her hair required both of us. I would carry Sophia to the bathroom, trying to support her in a flat position while Catherine would tailgate behind carrying the Trilogy. When I would gently place her on her bath chair Catherine would set the machine down and begin to wash Sophia’s body. When we were ready for Sophia’s face and hair we would remove Sophia’s headgear. I would hold the bubble mask on her nose while Catherine washed her hair. It was tricky to keep her nose in the mask while her head was moving from being shampooed. When bath time was done we would tell Sophia to take 3 breaths and to hold it…………..I would pick Sophia up and Catherine would follow. As soon as we would get her to the bedroom we would put the mask on Sophia’s face. I would hold the mask on Sophia while Catherine would blow dry her hair. Once we were done we were able to put a new dry headset back on……..and you could see the relief in Sophia’s face. The truth is for the last year we could not take the mask off for a minute, not for a family picture, and not for a kiss. It has been a long year since I have been able to rub my cheek against my daughter’s. A year since I have been able to rub my nose against hers. A year since I have been able to run my fingers through her hair without the headgear in the way. The fear that consumed us was Sophia’s health. When SMA kids are sick……they are generally on Bipap 24/7 till they are better. What scared me was ………………..what happens to an SMA child who is already on Bipap 24/7 who gets sick? What started to make me see that Sophia needed a secure airway was the look of absolute panic on her face every time her mask was removed.
Sophia had recently started to have really bad bloody noses. Every time we would cough her the cough mask would turn red. The suction lines and canister would turn bright red. Sophia would begin to choke on her blood and you are stuck as a parent. You know you need to keep coughing her to clear her, but the more you coughed the more she would bleed. Her dependency over the past year 24/7 was not allowing her nose to heal. It has now become every set that we have scary bleeding episodes. Sophia knew she would bleed and choke. Now every time the Cough machine would go on Sophia would start to cry with tears streaming down her face. On Saturday I knew in my heart that it was time. Sophia was suffering. I looked at the quality of her life, having a mask that could not come off her face, always in her line of view. The mask was always there. I would not want to live like that, the constant fear……….so why should she.
I know we are making the right choice for our daughter, but it comes with so much fear and trepidation. I love the cute sounds Sophia makes when she is calling me and I fear never hearing her voice again. I fear that Sophia may not like the trache, even though I know she has to like it better than the mask at this point. I fear that because Sophia has a tube in her throat rather than a mask on her face that my daughter will be disqualified from future potential trials. I know that I must make the right decisions for Sophia today in the here and now, and I know this is the right choice. I know that I am not ready to lose her, and she still wants to be here. On Thursday we will be taking Sophia to the hospital to be admitted and Sophia will be trached on Friday.
I know we are doing the right thing for our child……………and yet it still weighs so heavy on my heart.
Thank you to this wonderful community for all of your support. Thank you Sarah Turnbull and Jana Gundy for making and sending us videos to ease us through this process.
This was the last picture we had of Sophia without her mask……cannot wait to see this face again!!!