Estate Gift Leads to Medical Breakthrough

Left: Dr. Cheryl Gregory Evans, Right: Sharon Stewart
Left: Dr. Cheryl Gregory Evans, Right: Sharon Stewart

As a lifelong advocate of research for a rare eye disease, Sharon Stewart’s belief that she could help others through an estate gift is becoming a reality. Recently, Sharon’s support has led UBC researchers to unlock what could be a major medical breakthrough.

The University of British Columbia was a special place for Sharon. Her father and mother, Jim and Helen Harmer, met during a physics class in 1943. Twenty-five years later, Sharon also donned the cap and gown and graduated with a Bachelor of Commerce degree.

Sharon was born with aniridia, a rare disease that caused her eyesight to deteriorate. When she graduated in 1968, only five percent of her vision remained. In 2005, Sharon began making significant gifts to support aniridia research at UBC, hoping for a cure. During her lifetime, Sharon contributed over $2.5 million toward this research and an additional $2.65 million through a trust established in her will.

Because of this generosity, there is new hope for aniridia sufferers. Sharon’s gift funded the Gregory-Evans
Developmental Neurobiology lab, which focuses on developing treatments for eye diseases. There, with a team
of scientists from the University of British Columbia, Dr. Cheryl Gregory-Evans developed Ataluren, a new drug that has shown promise for reversing aniridia when administered after birth.

Aniridia is caused by a mutation that interrupts the production of a protein crucial for eye development. Sufferers can be identified by the absence or partial absence of the iris, the coloured ring around the eye. Ataluren seems to have the ability to override the mutation, allowing the body to produce the missing protein.

Although researchers originally thought the drug would have to be administered as a preventive measure in utero, they discovered that when Ataluren was formulated as eye drops, it actually restored vision in young mice. These eye drops, named START, are notably the first time a drug has successfully been used to treat a birth defect.

A clinical trial led by Dr. Gregory-Evans is expected to begin sometime this year. If START proves safe and effective, children would likely have to use it for the rest of their lives. It might also benefit adults if their disease has not progressed too severely.

As a discovery Ataluren not only benefits aniridia sufferers, the findings could extend to other eye conditions caused by similar mutations. And if Ataluren reverses damage in the eye, it raises the possibility of using drugs for other congenital disorders.

Although Sharon passed away in 2008 and never saw her dream realized, her support paved the way for this
breakthrough. Ataluren represents a new hope for thousands of people suffering from aniridia and other congenital conditions. It’s a fitting legacy for a woman remembered by so many for her determination and optimism.