Tenley’s story: Our experience with ZOLGENSMA

LACRETIA: My daughter, Tenley, is 4½ years old. She has SMA Type 1.

When Tenley was born, she was 4 weeks early, and she didn’t move a lot, but she didn’t show, like, any signs of serious illness. Then, at about a month old, she developed a wrist drop. And that’s what really prompted us to go forward with seeing if there was anything else going on with her as far as her muscles or things like that.

So then they started more testing and they did blood work. I got the call that the blood work came back and it was showing something genetic. He said, “It’s SMA Type 1.” He said he’s seen it before and started explaining it to me. He also said, “You know, you just need to take her home and love her. Call in hospice. You know, you have maybe ’til she’s 3.”

I just remember I was alone that day at her appointment because we didn’t think she would be like diagnosed that day and especially with something terminal. I just felt very alone. And just absolutely heartbroke. Like I just felt helpless, really, cause I mean, this neurologist just told me that there was nothing I could do to save my child’s life.

For probably the first 3 days we cried. And just kind of were in mourning for the life that she was losing. But then something just came over me one day and I was like, “No! I don’t accept it, and I’m gonna find a way to help her.”

And so I went online, and I actually started on Facebook. And on there I met someone who was in their upper 20s with SMA. She pointed me towards a hospital that offered the gene therapy clinical trial. I immediately got on the phone and tried to get Tenley an appointment to see if she qualified.

The actual treatment was around 60 minutes and went through an IV. Diagnosis day was very disheartening. And treatment day was a chance—a chance that Tenley could get stronger. That she could live longer. So that day did and does mean a lot to us. I’ll always remember the day that Tenley received the one-time-only dose for SMA.

At the time of treatment, SMA had taken Tenley’s ability to swallow thin liquids on her own. She was not able to move her legs very much at all. She was never able to hold her head up on her own.

We didn’t start noticing results right away. It came over time. But I would say within the first month, we could tell a difference in her strength and abilities. She started moving her legs more and started being able to hold her head up for short periods of time.

When we found out Tenley was gaining strength, just as soon as we saw signs of that, we were just so grateful. Every little thing meant the world to us.

Stopping the progression of SMA meant everything to us. Because the further it progresses, the more muscle tone Tenley loses—and abilities. And especially with breathing, eating on her own. Those are things that would eventually become fatal.

Managing Tenley’s SMA today is not as extensive as it could be. But we still take extra precautions. She has to have a feeding tube for extra nutritional support and to gain and maintain weight. She is on a BiPAP machine at night. We also do a cough assist machine and suction.

This treatment has given Tenley opportunities. She is getting ready to celebrate her fifth birthday, and, you know, that’s a really big milestone for someone with SMA Type 1. Recently she got her own wheelchair that she can move with her own hands, and so she just loves to play hide-and-seek and chase her brother. She’s able to really like see her world in a different view than she has and she’s like opening cabinets and, you know, just exploring like a normal 4-year-old.

Even though we were facing a very difficult diagnosis, Tenley was always raring to go. And she’s been through so much. But she continues to smile. And she is truly the epitome of never give up and keep pressing forward. Tenley’s never-give-up attitude definitely helps me to stay strong and when I’m feeling weak, to keep pushing for her because she has that willpower and that fight to live. And she deserves the best care.