That in itself was a miracle. 

 

Daniel, the son of Joanna and Marcus Kerner of Orange County, Calif., suffers from a form of Batten disease called late infantile NCL (LINCL). Like most children, Daniel initially developed as any healthy child would. Then, when he was 24 months old, his language began to regress. The Kerners searched for answers. One year later, they got one: atypical autism. However, Daniel’s special education teachers were convinced the toddler was not autistic. Their intuition proved to be right: when Daniel was four, Joanna and Marcus finally got the devastating news that their youngest child in fact had LINCL. 

 

We were devastated beyond belief,” says Joanna. Suddenly, their perfect little boy, the youngest of their three children, had been given a death sentence. Even today, Joanna describes her son as someone who thrives on adventure. She says Daniel loves to be outside – to experience the ocean and mountains and feel the warmth of the sun and the wind in his hair. Daniel is a lover of life. Before he made it to kindergarten, though, this vivacious child was discovered to have a disease that, if history held true, would take his life away from him at a tragically young age. 

 

Not too long after Daniel’s diagnosis, Joanna and Marcus learned of a neural stem cell transplant phase one safety trial that was under review. The procedure? It had never been done anywhere in the world, at any time in history. The chosen subjects? Children with infantile and late infantile Batten disease. Just six would be invited to take a leap of faith in an operating room in the Pacific Northwest. 

 

The day the trial was accepted by Oregon Health and Science University (OHSU) in Portland was a day of celebration for the Kerners. “There was no question that we wanted to participate,” says Joanna. “We wanted Daniel to be the first one to receive the transplant.”  The trial was extremely risky, and as a phase one safety trial, it offered no guarantee of a cure or even that it would not kill the children who underwent the harrowing brain surgery it involved. Such are the decisions that families like Daniel’s have to make: the Kerners knew the transplant could be Daniel’s only hope.

 

Daniel was indeed selected as the first participant for the trial, making him the first human to ever undergo a neural stem cell transplant. The historic surgery was scheduled for November 14, 2006, two months before Daniel’s seventh birthday. “It was like coming out of profound darkness and seeing the brilliant sunlight of hope for the first time,” says Joanna. “When he went into the hospital on that dark and cold day, he had an incurable, terminal disease. By that night, after nearly nine hours of surgery, Daniel had the best chance of all.” No one knew if Daniel, already fragile from the disease, would even survive the surgery. Today, it is still not known if the procedure he and five other children underwent can one day be a cure for Batten disease. The children in the trial and their families, though, have put the disease on notice. They stand as a testament to the will of all children fighting this disease: the will to fight back.

 

In the years that have passed since the surgery, the Kerners have taken advantage of every opportunity to improve Daniel’s quality of life. Physical, occupational, massage and vision therapists work with him to maximize his potential by keeping his cognitive functioning, muscles and skeletal system as strong and intact as possible. His family uses memory books and reads familiar stories to him to keep him connected to his surroundings. They find ways to adapt activities Daniel enjoys to prevent the disease from taking all that it wants from him. Beginning with his sixth birthday, the Kerners established a tradition of celebrating the occasion by skiing together with friends and family. A company called Disabled Sports Eastern Sierra took Daniel all over Mammoth Mountain in an adaptive Bi-ski. One year, Joanna, says, the high temperature was eight degrees above zero, but Daniel was the warmest person on the mountain. “Tucked into his Bi-ski, warming packs, goose down, a tarp, helmet and face mask, he looked like he could take on the world, and he did.” 

 

Despite his family’s efforts, Batten disease is unforgiving. Since the day Daniel became the child with the best chance to survive Batten disease, still it has marched on. Though his spirit remains strong, Daniel is in the late stages of the disease. No longer able to attend school, he instead meets with his teachers at home. Perhaps because he received stem cells in the upper regions of his brain, he continues to function at a high cognitive level and has maintained his ability to recognize and understand things around him. He loves to smile and laughs often. He loves to get hugs and looks forward to frequent cuddle sessions with his family members. However, Daniel’s brain stem is under constant attack and is withering away. All life functions pass through the brain stem; Daniel’s heart rate, breathing and body temperature are erratic, and he has multiple seizures each day. His intellect and his soul are resilient, but his body is dying. And on his birthday this year – his 10th birthday – the Kerners were not able to continue their tradition of skiing with Daniel on Mammoth Mountain. 

 

His family has already taken their leap of faith; Daniel has already become a pioneer. Now, more than three years later, they can only hold him and each other close and make happy memories. “We pray for strength and that Daniel will not suffer,” says Joanna. “When we hit a tough time, we hold each other closer, our friends rally around us and we ask God for help. We know that it is okay to look death in the face – to feel the pain of watching our brave Daniel confront this disease.  There are times when our despair seems so overwhelming that it suffocates our lives…but then we regroup. We put one foot in front of the other, and we push on.”