My entire life I have known that I might have Huntington's disease. This is a fatal neurodegenerative condition that can lead to death. It was a fatal disease that my grandad, mum, and grandmother had. I'm yet to get the test to see if it is mine. There is a 50/50 chance that you will inherit the disease. At the moment, I don't know if I have the mutation. I could have two lives if I toss a coin. There is no turning back once I have the results. Everyone in my family who has been tested has so far tested positive. The odds seem to be against me. I am 27 years old and starting to think about my future. I have to wonder if this disease could overshadow the big decisions I'm making right now. This tension is explored in Fifty Percent, a new documentary.
People who are at high risk of inheriting the gene were given the opportunity to have a test to determine their fate. This was in 1993. This option was available to me as a child, having been born in 1994. There are many reasons why people don't choose to. One reason is that they have the freedom to live their lives as they would without knowing what day it might be. There is no one right way or the best answer. My mum knows that her diagnosis has changed her life. But can we let the devastating information overwhelm us all? How powerful is knowledge?
Huntington's disease is caused when the huntingtin gene mutation that we all have causes us to need a protein that helps us live. If the mutation is passed on to their child, there is a 50% chance that they will inherit the disease. You will usually live between 10 to 20 years after the first signs start to appear. Then, your brain cells can begin to deteriorate and become deadly. Although the symptoms can vary from one person to another, and even between siblings, they all affect individuals physically, psychologically, and mentally. It is possible to lose control over your movements and lose control over your mind. You risk losing your sense of self.
Lillian Hanly and Tamsin Hanly, her mother in Fifty Percent. Photograph by Lillian Hanly
I have always been fascinated by the decision of whether to take the test. When I was 18, I was old enough for the test. I wasn't worried too much about myself, but I was curious about how others would approach it. I can recall talking with my friends about it and asking them what they would do in my situation. Every person had a different answer. It makes sense for a journalist to weigh all options. For years, I wanted to explore the discussion that touches upon the philosophy behind how people choose to live their lives.
As I grew up, my mum spoke to me about her experience with Huntingtons. Since I was a child, I understood what Huntingtons meant. I also knew how it could affect me. However, I initially cared only for my mum and not for myself. My mum's bravery in facing the disease and accepting it has inspired me. I am inspired by her determination to continue living my life to its fullest. Like her father, my mum finds great joy in the wonders of the universe. My grandad was an artist and marveled at all that created life. They have always encouraged me to do the exact same in life and death. This has meant I have never felt the need to be tested. But, things are changing.
My grandad was in a unique position. He was diagnosed later in his life. Even my mother was tested after having children. I come from a completely different generation. Generation that has grown up with internet access and instant information. A generation that has witnessed scientific advances that allow for the possibility of treating different diseases. However, Huntingtons is a case in point. Some people refuse to participate in these trials and others choose to not get tested. What is the best choice? How do we Huntingtons children live our lives? We are the first generation to make decisions about the disease in front of us. It is important to think about whether the gene may be passed on to other generations. The first person to be able to participate in IVF, which would allow the selection of an embryo with the mutation.
Huntingtons disease has been a topic of significant interest since 1993 when the gene was isolated. Research is ongoing to find new technologies that could be used to treat the disease and eventually lower the level of huntingtin protein production. Pharmaceutical companies and biotechnology are exploring gene-therapy methods to reduce the amount of huntingtin protein. It could allow Huntingtons-positive people to have more time before their symptoms start and slow down the progression of their symptoms. Gene therapy is not the same as gene editing, but that technology is on the horizon. CRISPR, which is a gene-editing technique, would alter your DNA, rather than limit the things it tells your body. How badly would you like to open the Pandora's Box of gene editing and selection?
These developments and opportunities have raised more questions for me. I hope those who see the film will be able to see us grappling with these issues. The first step in opening up to a variety of discussions is taking the test. It also acts as a conduit for us to ask ourselves moral and ethical questions. My mum taught me to see beauty and goodness in everything. After my grandad's diagnosis, she was the first person in my family to be tested. Now it is my turn to place the next step. She has already taken one step. My molecular makeup and the bonds of love, energy and support that surround me are what give me the strength to accomplish this feat.
