AJ Brown (Canada)

I’m a visual artist painter. I prefer creating pet portraits, using watercolour and other media, on wood. I am an advocate against poverty for people with disabilities. I love riding my adult tricycle, and practicing my yoga therapy.

I had no idea I have deficiency of gene ECHS1! I found out in the summer of 2019. Before that, I assumed I was just easily fatigued.

I am deaf and I have what looks like cerebral palsy. These are what this disease can cause. My understanding of this disease, is that when I consume protein, my body will make too much of three amino acids and this will block or interrupt the fatty acid breakdown. So, the body has more difficulty to extract energy from food. Hence, exhaustion all the time.

I was very, very sick after I was born. I was born in April, but stayed at the hospital until almost Christmas. I’m not sure what sicknesses I had. Recovery times from any sickness is a lot longer than everyone else. Trips to the doctor were frequent during my younger days. During my elementary school days, I had colds and flu often: the recovery times is always longer with any sickness. I had severe whooping cough as well, during elementary school. This took me many months to get better.  During my university years I had scoliosis. This required surgery and after surgery I went into coma for 11 weeks. During that, I think I died but came back. But I was changed after that experience, so my Mom said. After that surgery, I used a power wheelchair to get around on campus, but was able to walk short distances independently. Each illness is always a challenge for me. It feels like I’m dying again. With ECHS1 as the underlying thing, in the background, lurking… ECHS1 causes fatigue. With each meal I consume, fatigue will always follow. That’s just the way it is with ECHS1. I’ve been relatively healthy for a couple of years now. But always, I’m exhausted.

Being exhausted affects my social life. I am never sure how much energy I will have for going out and being with friends. I see them on Facebook going to events and I always wonder if I could have gone. This makes me depressed. But I am able to see some friends for meaningful conversations and interactions.

ECHS1 is a genetic disease. I lucked out in the gene pool! I stopped with self pity and wondering why me, many years ago. It’s just not worth the negativity. I struggle daily with my energy level, but I do what I can with what I have.

I will soon be using a power wheelchair to get around, after walking with a walker since 2008. This disease, I now understand, will claim my body, gradually. All organs will be affected because of the ongoing fatigue. Everything in my body will starve for energy. It takes more and more energy to do things. I have increasing weakness in my legs as a result. This is very frightening and frustrating.

I’m 54. I am tenacious and I love life. I don’t know if this gene therapy can help me, because I’m on the older side. However, I’m hopeful and grateful.