What I Wish People Understood About SMA

Posted by on Jul 25, 2016 in Blog | 0 comments

If there’s anything I wish people understood about SMA it is the idea that I am not dying from it.

Lately there’s been a lot of controversy in the SMA world with the story of Jerika Bolen, who at just 14-years-old has decided to end her life with SMA Type 2. While a lot of the arguments stem around the right to die, another significant source of controversy is stemming from the media making it sound like the disease is a death sentence.

Even I say in my presentations that SMA can be life-ending for many. BUT, that doesn’t mean I am saying that the disease itself is what kills you. It’s secondary things that unfortunately sometimes SMA bodies can’t endure. Oftentimes it’s complications from the flu or pneumonia that results in the death of someone with SMA.

I know, I know, families who have had members die with SMA might disagree – especially when it’s young children. I can only imagine how terrible it is to go through that and suffer that loss; I, myself, have known people who have died with SMA. But it’s a misunderstanding that it is SMA that kills you.

SMA is not something like cancer that obviously causes death. SMA is not terminal in that sense. A terminal illness is something that is likely to result in death in a short period of time. The disease itself does not attack your organs and eat away at your body. SMA causes muscle weakness, in very simple terms due to a poor connection between the nerves and muscles, which causes the muscles to atrophy over time from not being used to their full potential. Typically it’s extremities and the torso that see the most weakness. Respiratory weakness is also very common. Weakness with chewing and swallowing is not unusual. Note, even though I say “common” and “not unusual,” it’s very, very important to understand that is not the case for everyone with SMA; each one of us are quite different from one another and we all do not experience the exact same weakness and issues.

Because our bodies are weak, we have a more difficult time fighting off things like colds, the flu and pneumonia. Depending on how strong we are and the severity of these illnesses, it can take us weeks to recover and get back to our normal. Unfortunately sometimes the severity of these illnesses is just too much and our bodies aren’t able to fight it off, and people die from these illnesses. It’s the cold, flu or pneumonia that is the cause of death.

It’s really no different than a skinny, frail person getting sick. If you don’t have enough muscle and weight on you, your body is going to have a much harder time fighting off colds, the flu or pneumonia even if you don’t have any medical conditions. Any weak body – disabled or not – is going to have a hard time fighting off illnesses.

SMA also is not like a heart attack where you suddenly die. While I cannot say for sure, I think that is incredibly rare and I would guess there was probably an underlying cardiac issue that may or may not have been known. SMA in general has not been known to cause cardiac issues. It’s only in recent years that doctors have started seeing signs of heart failure in older adults (because as we are outliving our expectancies from the old days, our hearts are simply working harder against weakness, longer). In this case, the cause of death would be heart failure, but again, technically, not SMA.

Generally speaking, SMA also does not cause pain. It’s not like arthritis that causes inflammation and again it’s not like cancer attacking your body. Yes, some people with SMA do experience pain, but this pain is secondary. Oftentimes it’s from positioning or medical procedures (that maybe weren’t necessary or that don’t go right). Sure, I’m a normal human with pretty normal occasional pain like headaches (oftentimes stress or positioning), back (from spinal fusion and positioning) and knee (from “wear-and-tear” arthritis and positioning). These are normal and certainly far from unbearable and being constant. As with any pain, proper management and preventive measures, can keep it away or under control. But the key is managing it before it escalades.

I don’t know how doctors come up with their estimated life expectancies, especially for diseases like SMA, but when I was diagnosed my parents were told I wouldn’t make it past the age of two. Boy were they wrong! My childhood neurologist also told them that people with SMA tend to go through a rough phase where we teeter on the edge of making and not making it, but if we can pull through those years, we typically grow up to live healthy, full and successful lives. Somehow he thought I would be one of those people and he was absolutely right!

I’ve outgrown my frequent respiratory illnesses and in fact, my lungs are stronger than ever. I’ve learned how to manage any chewing and swallowing weakness, and am now at my best weight ever and am maintaining it. My heart gets thoroughly checked out and it’s in tip-top shape. In general, my body looks and feels the best it ever has, and has been showing signs of physically getting stronger and having more endurance. I am healthy, strong, energetic, happy, working hard for a living, full of life and absolutely living with, not dying from, SMA! I am not in pain, I am not miserable, I am not suffering and I do not want to end my life (and if I ever say that I want to, please get me mental health help because obviously I am not of sound mind if I say that). And when the day comes that it’s time for me to go, it will not be from SMA. I can promise you that.

Parents out there (both those who have lost a child with SMA and those with a child living with SMA), I realize this is hard for you. I really do. And I know doctors say a lot of horrible things about the disease that are probably scary to hear. But it’s important to maintain a positive and optimistic attitude. You simply can’t hate SMA and treat it as a death sentence. You can’t live like your child is going to die. I can somewhat see how you might feel that way, but you just can’t have that attitude. It is or has been a part of your life, like it or not. Embrace it and the life you have been blessed with, because far too many people lose their life from truly deadly things. With the proper medical education, medical supports, social supports, and support from family and friends, your child can grow up and live a long and happy life. Your days may not always be great and you will face some challenges, but any kind of life is a life worth living and you were blessed with it for a reason!

For the media out there, please understand you have a significant amount of influence over people. As a journalism major, I know you have a code of ethics and a duty to not be one-sided. And you have an obligation to fact check, and then fact check again, and verify your sources. Not doing this, not being thorough and not being clear in your reporting can contribute to or cause inaccurate stereotypes in the community about a large segment of the community. One of my major college projects (and Ms. Wheelchair Minnesota-America 2003 platform) was the inaccurate stereotypes of disabilities in the media and how these inaccurate portrayals negatively affect people living with disabilities. Using inaccurate facts and wishy-washy, pity-me tactics in your telling of Jerika Bolen’s story has already caused negative effects on the SMA community. And, if she wasn’t a teenager with a disability, you probably would not even be telling her story.

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