Just by looking at my brother you wouldn’t be able to tell anything is wrong. You might eventually notice his hearing aids, but discovering his degenerative eye disorder would prove difficult.
Jamie was born with a hearing impairment that is corrected with the use of hearing aids, but it was only later we learnt he had a rare disease called Usher’s Syndrome.
Contrary to popular belief it has little to do with R&B singer Usher [Raymond]. It does not liken to the hysteria surrounding Bieber Fever. It’s a tad more serious than that.
Usher’s Syndrome is a genetic disease caused by mutated genes that lead to both hearing and vision loss. There are three types of Usher’s Syndrome which include Retinitis Pigmentosa—the deterioration of the retina, varying degrees of deafness, and issues with balance. The symptoms of RP are night blindness and loss of peripheral vision, otherwise known as tunnel vision (try looking through two Scott towel rolls to get a better idea) which may result in complete vision loss.
On that note, Jamie is a brilliant pianist studying music theory at McGill University. What makes Jamie unique is his incredible zest for life and his admirably positive attitude and outlook on life. Had the roles been reversed, I don’t think I would have fared as well. He refuses to let anything stand in his way, and rightly so.
The future is looking less dim for Usher’s Syndrome sufferers. Genetic research is well underway in developing gene therapy and gene replacement to restore vision. Clinical trials are being done on humans in Europe for retinal implants. There seems to be light at the end of the tunnel.
Jamie is part of the reason I want to be a journalist. I want to share stories and issues, like Jamie’s with others. It seems fitting that as I mentioned this very story whilst writing my entrance exam into Concordia University’s Journalism program three years ago, and as I prepare to graduate, this story has finally come to fruition. I have come full circle.
Jamie may never be able to drive, but he has taught me and many others that life is what drives you.
To learn more about Usher’s Syndrome, check out the video I put together about Jamie and this disease: