Defining quality of life

Defining the Quality of Life
By Jen Onsum

People living with disabilities strive for acceptance and inclusion in this world that wasn’t necessarily designed for us. But, above all, we strive to have people see us as being no different than someone who lives without a mobility device and without being hooked up to medical equipment that saves lives. So when CNN Senior Medical Correspondent Elizabeth Cohen deemed a little girl’s life with an “incurable disease” and potential life dependent on a ventilator and other medical equipment to be a “terrible quality of life,” (Cohen, Elizabeth, “Heaven over hospital: 5-year-old changes the conversation about death,” 11/04/15) it struck a chord with me.

As an advocate for people living with disabilities I feel compelled to respond to Ms. Cohen’s assumptions and stereotyping when reporting on little Julianna Snow, whose parents say that at the age of four (now five) has chosen to go to Heaven instead of the hospital the next time she gets sick (Cohen, Elizabeth, “Heaven over hospital: Parents honor dying child’s request,” 11/03/15). Julianna has a genetic neuromuscular disease called Charcot-Marie-Tooth (CMT). I, myself, have a genetic neuromuscular disease called Spinal Muscular Atrophy (SMA). While our diagnoses are not scientifically the same, they are in the same family and Julianna is living a life similar to one I once knew.

When I was diagnosed at the age of 18-months my parents were told I wouldn’t make it past the age of two. Then the prognosis turned into my toddler years. Eventually it was maybe 20. At the age of 20, I successfully navigating college and today, just a couple of months away from turning 35, I’ve accomplished more than anyone expected and am happily living my version of the good life.

I spent much of my childhood in the hospital, oftentimes with respiratory issues. Sometimes I’d be in the hospital every couple of weeks and sometimes every couple of months. I’d be there anywhere from a few days to a couple of or three weeks. My longest hospitalization was 52 days. I spent many days with a tube down my throat with a ventilator breathing for me. And when I wasn’t in the hospital, at home I had to do vigorous respiratory treatments every four hours, or more, and I had to wear BiPap to help me breathe at night, and many times during the day where I could barely go a few minutes without it. Was this fun for a child? No, not really. But it’s what was necessary to get me where I am today. Some might not think of a quality life when a child lives with having their day disrupted with medical treatments and being hooked up to a machine for even a couple hours, but my parents believed otherwise.

My parents were told many times that I wasn’t going to make it. They were told that I was going to keep going through respiratory infection after respiratory infection and that my body wasn’t going to handle it. Oftentimes they were advised to stop fighting it, to stop giving me a chance to live because the experts believed it wasn’t a life worth living. Sometimes it was suggested that my parents agree to life-sustaining medical interventions such as having me receive a feeding tube and a tracheotomy, but even then, that wouldn’t be a quality life, in some opinions.

My parents could have given in. They could have listened to what the doctors, who are supposed to know everything, said about what my future would be like. They could have given up on me and let me go, or they could have given in and changed my life forever by agreeing to a tracheotomy and a feeding tube, and a life spent hooked up to a ventilator and with a nurse by my side. And, you know what? That would have been OK. If that’s what was going to allow me to keep living this precious life that I was given, then that would have been fine.

As a small child I tended to think otherwise. I thought no way did I want a hole in my throat with an ugly tube coming out of it. No way was I going to go to school with a tube coming out of me. I was under the belief that I wouldn’t be able to go swimming with a tracheotomy and being the fish that I was, there was no way I was going to be kept out of the pool. As a small child I believed that having to be hooked up to a machine by a tube coming out of my throat wasn’t any kind of life to live. But that’s because I didn’t really have any real-life role models living that life to look up to and to show me that life would go on and be great. Fortunately my parents never left this decision up to me, because as a small child I could not have logically and rationally made that decision.

As I grew into my teen years I started to see both girls and boys living with these life-sustaining measures. Truly living. They were going to school, playing sports, having fun with friends, going on dates and most importantly to me, still able to at least go into a pool and being fashionable. My eyes were opened and my opinion was changed. I was seeing that life was still good and still worth living for these individuals. These people were actually happy. If my lungs deteriorated and I needed these life-sustaining measures, that would be ok.

Because my parents never gave in and never gave up on me, I was also taught to never give up on myself. I fought hard and toughed out every single respiratory treatment, even when I needed them every hour, and reluctantly wore my BiPap all night and all day if I had to, to get through every pneumonia and every respiratory infection I went head-to-head in battle with. I never gave up on myself (not that my parents would have let me) because I knew there was so much life yet to experience. So much I wanted to do and see. There was no way I was going to miss out on any of what life has to offer, even if it meant frequent respiratory treatments, oh so much BiPap, going to the hospital, having a tube down my throat and maybe one day having to have a tracheotomy and feeding tube. I saw this really was a quality life.

If Ms. Cohen actually read the letters pouring into www.dearjulianna.com, or read them without bias and with an open mind, she would see that so many people are living a quality of life with a neuromuscular disease, instead of shrugging them off and deeming these letters as a negative response (Cohen, Elizabeth, “Heaven over hospital: 5-year-old changes the conversation about death,” 11/04/15). If anything, it’s a positive response because these individuals are the role models to Julianna that I didn’t have as a small child. They are telling Julianna their stories – many who know exactly what she is going through and will continue to face – of a positive life living with a neuromuscular disease and growing up against the odds and being told they were going to die, to becoming happy and successful adults who have seen and done so much in life. These individuals do have the same family of disease as Julianna, contrary to Dr. Michael Shy saying in Cohen’s story that they don’t. Remember, CMT is a neuromuscular disease. These real-life stories encourage Julianna that even though she might need respiratory treatments, to use BiPap, to go in-and-out of the hospital, and potentially be dependent on life-sustaining equipment, she can still live a happy, fun and full life. That she can have a good quality of life.

Because we didn’t give up, I proved the doctors wrong. The fragile little girl who wasn’t going to make it, ended up outgrowing many of her respiratory challenges and today at the age of 34 is healthy (no, I’m not cured), strong and absolutely thriving. Yes, I still get an occasional cold and the idea of coming down with the Flu is scary, and I still require some respiratory treatments and BiPap, but I’m living. I’m living the good life. It may not be what the popular notion of what the good life is, but it’s my good life. It’s a quality life I’m happy to be given. And while I know I could one day become weaker and get sicker again, and might have to make the decision to live with life-sustaining measures, I can easily make that decision and say ‘yes’ because I know it’s still going to be a quality life full of exciting activities and memorable moments in life surrounded by friends and family that is a life very worth living. It will still be the good life.

Experts are wrong every day. Doctors are wrong every day. They were with me. They have been with many people I know. They have been with others. Life is too precious to just give up because what a textbook tells a doctor. No two patients will ever be the same and it’s not fair to make assumptions on one because of another. Giving up and quitting is one of the easiest things you can do in life, but does that mean you should? Should you give up because someone thinks a life isn’t a quality life? No. Absolutely not. Giving up won’t get you anywhere. It won’t help you live the good life.

Everyone dreams of living the good life and they have this idea in their head that it’s being rich and famous, having a big house and a fancy car, and having a vacation home in the Caribbean. Maybe for some people this is true because this is what makes them happy and what is a quality life for them. But the reality is, for many people this isn’t true. The good life is so much more and so much more simple. It’s whatever makes them personally happy versus what society stereotypes and assumes makes people happy. The good life is a quality life that is something different for each person.

Here’s the thing about quality of life. It’s a very personal belief. What is considered to be good, valuable and of quality to one person, may very well not be for another. What makes someone happy and fulfilled can be absolutely meaningless for someone else. Some people think being poor, without a consistent source of food or money and just barely getting by is no kind of life to live, while those living like this truly are just happy to be breathing and to be alive. We should never pass judgment on or make assumptions about what makes people happy. We should never pass judgment on or make assumptions about what makes someone’s life good or bad. We should never pass judgment on or make assumptions about what makes someone’s life a life worth living. No one knows what someone’s life really is like, what someone’s life feels like, what makes someone happy, except for the person living it. Just because it may not be your idea of a good life or a quality of life, that doesn’t mean it’s going to be (or is) a terrible quality of life for someone else.