URSA, Ill. -- When Darlene and Randy Royalty lost all three of their daughters to a rare disease, they could have put the experience behind them and never again have had anything to do with Batten disease.
But the Ursa couple did just the opposite. After learning everything they could about the disorder in more than a decade of watching their girls -- Amber and twins Sandy and Sarah -- fight the disease, the Royaltys decided to be there for other families whose paths were taken down the same road, a road that ends with no cure for a disease that primarily strikes children.
Every year, the couple uses vacation time to attend the Batten Disease Support and Research Association's annual meeting. Darlene Royalty is a member of the association's board, and the two vow to do all they can to help families who are dealing with the disease.
"If it wasn't for BDSRA, there was no way we'd be here today," Darlene Royalty said. "That program and support service has been there for us, and we've been there for other families. Our focus is family support, making sure the new families coming in find the support that they need and find the doctors they need."
Twenty years ago, the Royaltys had no idea what Batten disease was. It was not until their oldest daughter Amber began to lose her sight when she was 14 that the family was introduced to the world of Batten. Margie Frazier, executive director of the Batten Disease Support and Research Association, said the disease is extremely rare with somewhere between 1,200 and 1,500 known cases.
Frazier said the disease is autosomal recessive neurodegenerative disorder. Both parents have to be carriers for the disease in order for someone to be affected by it. Even if both parents are carriers of the recessive gene, as the Royaltys are, there is still a chance that the child will not develop the disease. In the Royaltys' case, all three of their girls wound up with it.
Amber, Sandy and Sarah each had times in their lives when they showed no sign of the disease. By the time the twins, Sandy and Sarah, reached first grade, they began to lose their sight and quickly fell behind in their school work. In addition to blindness, Batten patients can experience seizures, personality and behavioral changes, dementia and the loss of motor skills, including the ability to walk, talk and communicate.
The Royalty girls each lived into their 20s, which is a typical life span for a Batten patient. Sandy died Sept. 1, 2007, at 21 years old. The family was hit hard around Christmas of 2009 when 28-year-old Amber and 24-year-old Sarah died within 36 hours of one other.
"Sometimes it feels like it was a lifetime ago, and there are times when it seems like it was just yesterday," Darlene Royalty said. "You just miss them. I still cry and have some bad nights."
But the couple's work with the association helps keep them going and keeps the daughters' memories alive.
The Royaltys went to their 20th BDSRA convention earlier this summer in St. Louis. They recall being tepid heading into their first group experience at the convention in 1997.
"It was not a good experience at first, but by the end we met people who were having the same problems. We just found they knew us," Darlene Royalty said. "They knew what we were going through, and we knew what they were going through. The support you got was unbelievable. We just kept going back year after year because it was like a big family reunion every year."
Randy Royalty said the couple has been able to add to their family as a result of their work with the organization.
"A lot of those families, we're closer than our own family because they understood what we were going through," he said.
Frazier is happy to have the Royaltys to lean on to help other families.
"They are exceptional people," Frazier said. "They have taken this devastating tragedy and been really beacons to other families who are going through this."
The Royaltys help with a program at each conference called "The Life Goes On." Parents who have lost children to Batten gather and take a trip at the conference to bond over their losses.
"It's a way to let them know that life does go on," Darlene Royalty said. "It's OK to laugh. It's OK to cry. It's OK to move on after you lose your children."