At-home treatment providing hope for those with spinal muscular atrophy

A local teacher has a new sense of hope after a treatment was finally developed for her rare neuromuscular disease. The timing was perfect, because she has been confined to her home during the pandemic with less help than usual.   

Everyone who knows Angela Wrigglesworth says she handles her diagnosis of spinal muscular atrophy, or SMA, with style and grace, even once crowned Ms. Wheelchair Texas! Her condition makes her body too weak to walk.

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"I was diagnosed with spinal muscular atrophy type two when I was 16 months old. It was way back in the late 70's, and it has just physically affected me. It affects all of the muscles in my body and it slowly makes them weaker. I was able to crawl and pull up on furniture when I was younger, but slowly the muscles got weaker and I use a motorized wheelchair for my daily mobility now, and I have pretty limited range of motion with my arms, so I require a lot of help," states Angela.

Angela started relying on a wheelchair when she was only five years old. Through all of these years, there has not been one treatment available to either stop the progression of her disease or help alleviate the symptoms.

"I basically went 40 years with no treatment, just kind of do the best you can and live the best life that you can, and just continue to get worse. And so it's a very slow progression, but it certainly is something that is obviously noticeable from when I was younger, so this new treatment is a big deal and it's an incredible thing that's happened in our community," says Angela.

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SMA is usually diagnosed during childhood. It can be fatal in-utero or early in life, so it was a big relief when the first chance at treatment came along three years ago. However, it involved an invasive procedure.

"It took a lot - it was a lumbar puncture and I would have to go in every four months to the hospital and it's a pretty significant procedure that just took a lot of time. I'm a third grade teacher and so missing a day of school is kind of a big deal," explains Angela.

Angela is teaching from home during the pandemic and misses her students, but she no longer has to go in for those procedures, now that the FDA has approved a medication called Evrysdi for SMA. It's a liquid medicine that Angela can take in the comforts of her home. She still can't believe it. The treatment is expected to give her a better quality of life and longevity.

"The most important piece of it is it's stopping the progression of my disease. So where I'm at, is where I'm going to stay. My last name is Wrigglesworth and I joke with my students that I'm going to be Ms Wrinklesworth, as I get older because I'm going to hopefully get to grow to be an old an old lady, who happens to have SMA and has done really well on this drug, and so stopping the progression is a big deal. Additionally, I have some stronger muscle tone, my diaphragm muscle is significantly improved my sentences and I'm able to just breathe a lot easier. I've been able to fight off a lot of colds and things and then most importantly, it literally feels like I'm taking a shot of espresso every morning because my energy level is just off the chart. It's really remarkable," says a smiling Angela.

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SMA can be a lot to deal with, as it attacks the muscles. It helps Angela to reach out to a network of others who are dealing with her condition. She says many of her friends are also taking the new medicine.

"Most of us have lived into our adulthood, just kind of living and not really expecting a treatment to be in existence and so just the conversation of, can you believe this is happening in our lifetime. What a tremendous blessing that we have lived to see this. In addition to the fact that so many people didn't live to see it and so for me, I know those conversations are so important, just that gratitude," reflects Angela.

The treatment is for anyone with SMA who is over the age of two months old and comes with the possibility of side effects, like other medicine.

For more information, https://www.evrysdi.com