Nicola Danielson is deaf and has navigated the world in silence since birth. While Nicola thrived as a young child, when she was 12 years old she began bumping into furniture and struggling to hit tennis balls. An eye exam revealed Nicola had Usher syndrome, a genetic condition leading to vision and hearing loss.
Usher syndrome causes gradual vision loss from retinitis pigmentosa, which affects the layer of light-sensitive tissue at the back of the eye. Vision loss occurs progressively as the light-sensing cells of the retina gradually deteriorate. There is currently no cure.
Now aged 41, Nicola lives on the Gold Coast with her husband and three children. She has 8% vision in the centre of her visual field but nothing beyond that.
“It’s like looking through a toilet roll holder,” Nicola signs through her mother, Trish.
“I can see mum if I’m right in front of her, but I can’t see above, below or to the sides,” she says.
Queensland Eye Institute is part of a clinical trial testing the safety and efficacy of a new drug therapy for retinitis pigmentosa caused by Ushers. Nicola heard about the trial through a friend and contacted QEI to find out more. After extensive screening over two visits, the QEI clinical trials team confirmed she was a suitable candidate.
Now in her second year on the trial, Nicola says the regular testing can be draining.
“I didn’t think it would be this hard,” Nicola signs, “but Brett (Caldwell, QEI Clinical Trial Manager) and all the staff here who work with me make sure that I’m happy. They support me, and I’m happy working with them because they’re all one team.
“They know it’s hard for me sometimes, so they work at my pace. They make sure it’s all about me, not about them,” Nicola says.
Brett Caldwell says one of the greatest challenges in recruiting people into clinical trials is distance, particularly in a state the size of Queensland.
“Participants might have to travel considerable distance to get here,” Brett says.
“This is particularly true for Ushers patients.
“Often their income is restricted because of their vision and hearing impairment, which forces them to live further out to ease cost of living.”
Nicola was born and raised in England but moved to Australia in her late twenties. As a young woman in England, she did a lot to raise awareness of deaf blindness, skydiving and doing stunts standing on the wings of a biplane among other things. In recognition of her work, Nicola was presented with the keys to the city and twice invited to meet The Queen, Her Majesty Queen Elizabeth II.
These days Nicola’s life is quieter, although it still holds some adventure. She recently returned from a holiday with her sister and friends in Ibiza, Spain, to celebrate fortieth birthdays.
Nicola is creative, using a magnifying glass and a lamp to pursue her passion for craft, making shadow boxes and coiling paper (quilling) to brighten her world.
At Christmas she transformed her walking cane with tinsel and lights. “Strangers avoid people with a cane, but the decorations on Nicola’s make them smile and take an interest,” her mum says.
Nicola has more motivation than most people to help find treatments for retinitis pigmentosa.
“I don’t want to go blind,” Nicola signs. “It’s more important for the children. It’s hard enough having a deaf mother, let alone having a deafblind mother.”
Nicola is reassured her children have not inherited her genetic condition but knows it’s possible her grandchildren could.
“It seems crazy, but I could be saving my grandchildren’s sight,” she signs. “I’m doing this for the future.”
Click here for more information about clinical trials at the Queensland Eye Institute.