Not long ago cystinosis was most definitely a terminal disease. Even up until the early 90’s parents whose children were just diagnosed were told their child would likely not live past 10 years old. But with the introduction of Cysteamine treatment (now a days Cystagon and Procysbi) people with cystinosis are living longer than ever! At the time of Elsie’s diagnosis we were told we could (hopefully) expect her to live well into her 60’s. So the first time I heard cystinosis referred to as a terminal disease I was upset. To me, my child is not terminally ill.
I never really thought of myself as a person to get hung up on words and phrasing. To me, words are just words, it’s your own experience and prejudice towards that word that gives it power. For instance we tell Elsie she has to take her medicine, rather than saying “vitamins” or “supplements” because to her, medicine isn’t a dirty word that refers to sick people, it’s just a word for what she has to take to stay healthy. She doesn’t know any different. But terminal? Terminal brings images of frail people lying miserably in hospital beds to my minds eye.
I had to know if cystinosis was indeed terminal because I just could not merge what I thought the word meant with my happy, playful little girl. Turns out that technically, yes it is terminal, but terminal may not mean what you think.
: causing death eventually : leading finally to death
: having an illness that cannot be cured and that will soon lead to death
More often than not people with cystinosis pass away due to complications caused by their disease, which fits in with the first definition. However the second definition does not fit as cystinosis won’t soon lead to death. In fact with medication compliance and generally taking care of themselves (exercise, healthy diet, ect.) many adults are being told they can expect to live a normal life span.
When I looked up the term “terminal illness” I found several sites that defined it as a disease/condition that will likely lead to death within a very short period of time, most common was 6 months. This is the definition that I believed terminal meant, but clearly cystinosis does not fall into this category. I think the need to define how long a terminally ill patient could live was done for legal reasons such as applying for access to special programs like social insurance and extended care. While it’s nice to know that cystinosis is not one of these illnesses it is a shame that once something is labelled as terminal, this short lifespan is the first thing most people think of.
However there’s a truth commonly heard that life is itself a terminal condition. All of us, healthy or not, will eventually die. So why even bother getting hung up on a word that some use to describe cystinosis? We should be focusing on the things that make us happy, living fully and in the present and doing all that we can to ensure Elsie lives her life being true to herself. I don’t want her to be scared or worried about what her future might bring. I want her to take chances and not let cystinosis hold her back. She deserves to have the world at her feet and the courage to live a truly happy life.
So does Linden, Dave, myself and everyone else in this world. It’s sad that sometimes it takes something like a life altering diagnosis to teach us how to let go and focus on things that support us and make us happy but at least now it’s not too late. While I still prefer to use terms like chronic, progressive and life long when describing cystinosis, it no longer bothers me when someone uses terminal. 1) because by definition it is and 2) because who cares anyway, I know my daughter is happy and living each day joyfully and that’s what really matters to me.