Histiocytosis victim confronts ailment with will to be normal

ROYAL CITY - If you saw Jackson Noftle walking down the street, you likely wouldn't say he's ill. He looks like a normal 15-year-old.

But Jackson is ill. Very ill.

Twice Jackson has been diagnosed with Histiocytosis, a rare disease that afflicts one in 200,000 children in the United States. The first time he was four years old. The second time was two years ago.

Jackson's challenges have been an ordeal for the entire family, especially his parents, Mike and Becky Noftle. They've had to make adjustments most parents don't. There have been endless doctor visits and numerous trips to Seattle and Texas.

The Noftles' son's life tears at their hearts. On the other hand, they marvel at how he handles it. He battles to live as normally as possible.

"It amazing. If it were me, I'd be mad at the world," Mike said.

"It's been heartbreaking and heartwarming at the same time," Becky said with her voice breaking. "It's heartbreaking to see your son struggle with life and lose some of his talents. It's heartwarming to see all of the people who care. There are many people who are willing to help Jackson and our family.

Jackson recently completed his Eagle Scout Project with the help of family and friends. It is a new "Welcome" sign at the main entrance to Royal City.

Jackson has become an ambassador in the research battle against Histiocytosis. He speaks on the subject, gives PowerPoint presentations and heads up fund-raising efforts. Actually he's been in demand.

"He's been a good sport," Becky said. "It's hard to find a kid old enough and big enough to do these things."

An important event that Jackson spearheaded was the first annual Histio Heroes Bike-A-Thon/Walk-A-Thon at Royal High School on June 11. The purpose was to raise funds and awareness for the Histio Heroes Research Fund, which supports the research work of Dr. Kenneth McClain and Dr. Carl Allen of the Histiocytosis Program at the Texas Children's Cancer Center.

According to Becky, research is starting to pay off. The chemotherapy drug Ara-C is doing positive things in patients. Hope has risen for children and parents.

"In Jackson's case, his speech has improved, and his hand tremors have improved," Becky said. "He'll never get back to where he was, but he is improving."

Histiocytosis is a rare blood disease caused by an excess of white blood cells called histiocytes. It can be life-threatening.

Histiocytosis is so rare that there has been little research into its cause or treatment. It's considered an orphan disease, which means it strikes too few people to generate government-supported research.

When Jackson was diagnosed the first time, the disease was attacking his skull and orbital bones, pelvic bone and pituitary gland, which regulates growth hormone. He underwent six months of chemotherapy to stop progression of the disease and was left with a damaged pituitary gland. He's been taking growth hormone injections since the age of eight.

Two years ago, Jackson exhibited new symptoms that raised fears of a Histiocytosis return. A trip to Houston, Texas to see Dr. McClain confirmed the worst. It was a secondary and more debilitating form of the disease.

"For Jackson, lightning did, indeed, strike twice," Mike said.

This form of Histiocytosis is called Neurodegenerative Central Nervous System Histiocytosis. It attacks the brain stem and can leave victims with tremors and difficulty with walking, speaking, swallowing and learning.

"The disease affects his fine and gross motor skills, has slowed his speech and has made school and sports much more difficult," Mike said.

According to Mike, Jackson underwent a second round of chemotherapy under a treatment protocol established by Dr. McClain in 2010 and is dealing daily with the effects of his disease.

Dr. McClain, who works at the largest pediatric hospital in the country, sees more than 100 new patients each year. He has more than 400 active clients. Some come from as far away as Ecuador, Spain and the Philippines.