“I just want to get a tan on my legs,” said Lower Kootenay Band resident Jenni Jacobs last week, in response to her mother’s admonition to her about going out into the sunlight. “My legs are too white.”
Tanning, though, or any direct exposure to the sun or even fluorescent lights, is a deadly activity for Jenni, who has a rare condition in which her body is unable to repair damage caused by ultraviolet light. Xeroderma pigmentosum, or XP, is a rare genetic disorder that affects one in a million Americans and one in 250,000 people on the planet.
“We don’t know what Jenni’s life expectancy is,” said her mom, Shelley. Only 40 per cent of XP suffers survive past the age of 20. Jenni is 16, about to enter Grade 12. Like any young women, she has dreams.
“She had wanted to be a pilot, but the condition also affects her eyes, so she might not be able to,” her grandmother, Anne Jimmie, said.
Heavily covered with dark freckles, Jenni’s face shows the scars left from “many surgeries” to remove skin cancers. That same face, though, also shows a bright smile and twinkling eyes. When asked what she would like to do for a career she said she had eliminated the legal profession because she doesn’t want to continue going to school for such a long period.
“Maybe — what’s that called? — spelunking!” she laughed. Exploring caves is a perfect vocation for someone for whom the sun is a deadly foe.
Jenni was diagnosed with XP at the age of two-and-a-half years. The condition is so rare that it required a skin sample be sent to a lab in Washington, D.C., to confirm she had one of the eight different forms of the genetic disorder.
Shelley began to read about XP and soon learned that Jenni would never be able experience life the way the children around her would. At the age of five, Jenni told someone, “Thanks be to God, the sun hates me.”
Exposure to the sun affects more than the skin.
“Her vision is going — she may end up legally blind,” Shelley explained. “And she has added risks of getting other cancers.”
Throughout her childhood, Jenni’s family worked to keep her exposure to sunlight at a minimum, driving her to the school bus and then to school, ensuring she was well covered and wearing sunglasses. But she began to push the boundaries when she entered high school.
“We found out she was going outside at recess to be with her friends,” Shelley said.
“But I did cover up,” Jenni countered, holding up her floppy black hat and big, dark sunglasses.”
“We went through a few years of butting heads,” Shelley laughed.
Like most families, the Jimmies and Jacobs want their kids to experience all the world has to offer.
“We had an opportunity to go to Disney World,” Shelley said. “I know there’s a risk, but it’s a once in a lifetime thing.”
That followed up a trip several years earlier to Disneyland.
“Disney World was awesome. Disneyland sucked,” Jenni said, forgetting to add that she was too small at the time to go on the most popular rides.
Perhaps more important was a trip to New York, where mom and daughter visited Camp Sundown. What began as a family retreat in Pough-keepsie, N.Y., in 1996 turned into a full-fledged camp in Craryville in 2003. Founders Dan and Caren Mahar, parents of an XP daughter, fulfilled their dream of creating a “night camp” to offer sun sensitive children and their families a way to meet with others with similar problems, search for solutions and experience camp like their peers, but on a different time clock.
That experience, Shelley said, was invaluable. It allowed the family to interact with others and to understand they weren’t alone in what they had faced since Jenni was a child.
“Jenni wants to be a volunteer at Camp Sundown now,” Shelley said. “Maybe next year we can take the train to New York so that she could see the country. That would be better than just flying.”
Unlike her mom, Jenni loves to fly. On Aug. 18, if things work out, she will get her first helicopter ride. Weather permitting, she will land in Centennial Park, where friends and family are gathering to celebrate Jenni’s Night Out. The Rotary splash park will remain in operation after dark so that Jenni and others can enjoy the water. A barbecue is planned, too.
“This has been planned by Chief Jason Louie and Mayor Ron Toyota,” Shelley said. “They thought it would be nice to have a party outside when Jenni didn’t have to worry about covering up from the sun.”