Disneyland has always been a magical place for us, especially experiencing it with our dear friends, Jennifer and Chris Calafiore, and their daughter Hayden (who also has SMA Type 1). She and Gwendolyn adore each other. They love all things princess and sparkly, have tons of fun being imaginative, and also have a wild streak and crave adventure. We love being together and Disneyland meet-ups have been our special thing for years. In fact, this was our sixth visit. (Read about other Disney trips here: 1st, 2nd, 3rd.) We always get rooms next to each other at the Grand Californian so even when we aren’t at the parks the girls can have slumber parties and we can all be together. They love to have slumber parties, wear matching outfits, and, of course, boss us all around. Though filled with people and chaos, the Disney staff always go out of their way for families like ours and never make us feel we are asking too much of them to accommodate our very special needs. We have always had very positive experiences at Disneyland.
What started as a typical fun day this week turned very scary, very quickly. We nearly lost Gwendolyn this time. And we are all bruised and scarred.
But this is SMA. Especially Type 1 where the margin for error is hairline slim. The most subtle things cause distress and can literally be life ending:
- Head position
- Turning her head without clearing her throat
- Bumpy terrain that jostles her body
- Sitting up
- The bipap mask shifting or not properly fitting
- Hot weather
- Cold weather
- A few seconds delay in responding to the subtle nonverbal signs of distress
- And in combination, these are especially dangerous.
What happened exactly, we aren’t sure. But we think it was some combination of the above. The 95 degree heat made her lethargic and vulnerable. She was wearing a new bipap mask. She had been on this ride many times before with no problem, but this year she is bigger which meant she had to sit up more so her wheelchair would fit in the ride’s accessible car. This ride is especially bumpy and we think she had some anxiety because perhaps her new bipap mask jostled more than usual making her feel like she couldn’t breathe. And with her already weaker from the heat, the anxiety hit her hard. She panicked, hyper-ventilated, and stopped breathing completely.
Bill had been standing up to check her face throughout the entire ride. She literally just told him she was okay. They went through a long dark area as the ride made its way toward the end and then her pulse-oximeter alarm went off. He stood to look at her face and she was blue and completely unresponsive. He immediately started trying to get her off the ride in her chair so he could get her flat, suctioned, and start trying to revive her. This is where things got bad because the workers didn’t realize what was going on and, per their safety regulations, wouldn’t open or move the car and kept telling him to sit down. Jennifer saw Bill standing up in the car frantically working on Gwendolyn and understood right away and started yelling at them that it was an emergency. They pulled the cars up a bit and then opened the car door and Bill picked Gwendolyn and the entire chair up, off the ride, over the tracks, and out into the first light he could find. Jennifer ran out of the ride to get me and Chris; I had taken Eleanora on a walk to help her take a nap. When I saw Jennifer’s panic I started running and she took Eleanora who was, thankfully, fast asleep. I couldn’t find them right away because they were still partially inside the ride. When I got to them Gwendolyn’s oxygen was at 6%. 100% is great, less than 90% is not. 6% is the lowest we have ever seen. Her heart rate was at 30. She was stone white and completely lifeless. Bill had immediately declined her wheelchair, suctioned her, and switched her bipap mask to the one we usually use. He was also giving her chest presses to simulate the cough assist machine and try and keep her breathing. With Chris and I now there we had extra hands to start other things. I held her bipap mask over her nose, stroking her face and calmly talking to her and encouraging her while Bill got the Ambu bag out and ready to use. Chris started reading her numbers and then, music to our ears, they were slowly, slowly rising. 6% became 10%. 10% became 15%. It felt like an eternity but her color starting coming back. We kept talking to her telling her to take deep breaths, stay calm, that we loved her, that we were all there with her. She was still very pale but slowly her numbers kept climbing. Two Disney RNs arrived with a bag of medical supplies — none of which could help us. But they did have oxygen and we knew that would give her an added boost while she recovered. We didn’t have our attachment though and taking her off her bipap would kill her. Thankfully Chris and Jennifer had an attachment on their bipap, which they quickly ripped off, so we could use the oxygen with our bipap. She was really weak so we rested in the shade and talked her through what was going on. The ride staff had called an ambulance but with her now coming back we cancelled that knowing they couldn’t do anything to help her — in fact, moving her out of her wheelchair into an ambulance would most likely cause another episode. We just wanted to get back to our hotel so she could rest.
Almost every single day we save Gwendolyn’s life in some form. We only have seconds to react and rectify the cause. Usually we are on it so fast that things don’t get too bad. In fact, we have often been in the midst of crisis around a whole group of friends and they have no idea. We have grown so used to this that most days it no longer feels like we are saving her; it is just our life. But sometimes things are worse. And sometimes, like this experience at Disneyland, things get so close to the edge that a return seems unlikely.
These events feel out of body. Though they usually occur in a span of minutes, everything slows down. While on one hand everything is a blur, we can remember what we were thinking: “Not now. This can’t be it. This isn’t our day. Not here. Not like this.” We can remember sequence of events in detail. Pushing people to get through, screaming “Where is she?” Seeing Bill working on her and certain she was already gone. Being fully hands on and present but feeling like we are watching from above. Seeing the triage we created completely from SMA instincts and knowing the four of us — just parents — could help her more right then than an ambulance or truthfully any medical professional.
Every SMA Type 1 family walks this tenuous line between life and death, saving their child and then carrying on. Because we have to. It’s a crazy life. We know that. But it’s ours so we have to live it the best we can. Some days are harder than others.
We were all traumatized and cautious the next day, but seeing our child happy is the most healing thing we can do — for all of us. In fact, if we hesitate to get back out there for too long, it makes finding the courage to do so even harder. We took things slowly but spent the day having fun. We saw a few shows, met every single princess and had extra special time with them, and even went on the Ferris wheel. Seeing Gwendolyn light up that day is everything. We know her spirit is bruised but her sparkle is still strong. And that gives us courage.
That night the girls got into new matching pajamas, snuggled close to read princess stories, and Gwendolyn played with Hayden’s hair before falling asleep in her arms. Their bond is beautiful and we know they understand each other on a level that we cannot. That night, I’m sure they both needed the extra hugs.
Some things we learned from this — because we know other SMA families will read this and we hope this may help them:
- Stay calm. Talk to your child. I honestly think hearing our loving voices brought Gwendolyn back.
- Do something to stimulate a breath. If you have Cough Assist, use it. If you have an Ambu bag, use it. And if not, use your hands to press on the chest.
- Ambu bag — carry one with you. Practice using it now when you are not in a crisis.
- Always carry an in-line oxygen port with you. We used to have an oxygen tank on her wheelchair but we never used it and the tank made narrow doorways hard to get through. Emergency crews usually have oxygen but you must have a way to use it with the bipap. A cannula will not help an SMA child in crisis. They need the bipap to maintain chest rise.
- In a crisis be very clear to others so they know how to help. Say “She stopped breathing.” Not just “It is an emergency.”
- When trying something new, like a bipap mask, always bring the go-to option with you as a back up. Thank goodness we had that with us.
- Always have your child on pulse-ox for rides. It’s dark, bumpy, and adds so many variables.
We really want to commend Disneyland for how they handled everything. While getting off the ride quickly was definitely not ideal, the way they managed to shut down and evacuate an entire ride filled with people and the crowd control in the process that they somehow navigated around our triage area was remarkable. They never rushed us. They never pushed us to move or even interrupted what we were doing. Bill went back the next day to thank everyone and they were all relieved to hear Gwendolyn was okay. We know that even when we left things did not look so good.
We also want to thank those of you who reached out. When things like this happen we feel very alone and very shaken and we often retreat and hunker down to our own little world. But reading your messages of support, knowing Gwendolyn matters to you, is encouraging and helps us find strength.
We know the reality is we will lose our daughter one day. Every single day we live with this. Every single day we get up in the morning we know this is a possibility. Every single day we feel the weight of that reality. And it never gets easier to know this truth. But we must push forward — even when we are petrified. Even when we get to the edge of the cliff like we did this week at Disneyland. What choice do we have? We know her time is short but we are determined to make every single day meaningful — for her and for us. One day SMA will decide that it is her time and we hope we can find peace in knowing we gave Gwendolyn the life she wanted. We are beyond grateful that day is not here just yet. And we will hold on to her as tightly as we can until then.
NEVER GIVE UP.