Family, friends, faith come together for teen with rare bone disease
Lincolnville — There will be a benefit for Noah Botley, who needs surgery for a rare bone disease, starting at 7 p.m. Saturday, Oct. 26, at Lincolnville Central School.
Admission will be by donation, and there will be desserts and music by several local performers. On the bill will be John Stephens, Michelle Chadwick, Jason Moody, the Webber family and the Phil Clement Combo. Donations may also be mailed to Becky Stephens, 141 Tucker Brook Road, Lincolnville, ME 04849.
Noah, who turned 15 Oct. 22, talks about the disease he has had since birth with articulate matter-of-factness.
"It hasn't changed my life at all," he said of the fibrous dysplasia that affects his left maxilla, the facial bone under the eye.
Many people would think that was an understatement. His mother, Jean Botley, home-schools Noah and his brother, R.J., because Noah's disease requires him to travel to Pennsylvania every three or four months for checkups, which would require a school absence of several days. Surgeries require him to be away from home for two and a half to three weeks.
A few months after he was first diagnosed, back in 2008, he had daily headaches, blurred vision and was very frightened by what was happened to him, his mother said. He is mostly symptom-free now, except for occasional headaches or difficulty breathing.
It is clear from talking to Noah and Jean that the family is deeply faithful.
"We trust God with every single detail of our lives," Jean said.
She said the evening of the day Noah was diagnosed at age 9, she went to his room to tuck him into bed and found him reading his Bible.
"He was thanking God for the disease, because he knew he would be able to help some other kid."
The disease, which Noah explained is caused by an extra gene, causes affected bones to grow without stopping, rather than the normal pattern of growth alternating with periods of no growth. Noah said there are three forms of the illness: the form he has, which primarily affects one bone; a form that affects multiple bones; and McCune-Albright Syndrome, which includes endocrine diseases and skin pigmentation along with fibrous dysplasia. He said he feels "blessed" to have the most benign form of the disease.
He said the disease has caused bone to grow into his sinuses and to partially obstruct his left nostril. He "can definitely notice the difference" in his breathing, he said, but it does not hurt, and it has not changed his appearance.
"If you didn't know I had it, you wouldn't know I have it."
Noah has had the disease his whole life, but it was diagnosed about five years ago during a visit to Dr. Edward Zanca, his orthodontist. Observing some unusual bone growth, Zanca ordered some CAT scans, Noah said, which showed he had fibrous dysplasia. His mother credits Zanca with saving her son's life.
"If Dr. Zanca had not been thorough … Noah would be blind today," said Jean.
Noah has had two surgeries already at Geisinger Medical Center in Danville, Pa. The first, in 2008, removed excess bone around his eye and on his cheek to preserve his sight; the second was in 2011 to reduce the blockage from bone growing into his nostril, and remove growth on the maxilla. He sees Dr. John Frodel, an otolaryngologist.
The surgery Noah will undergo in early November will remove excess bone growth around his teeth on the left side, from his nose and from his cheek that could encroach on his optic nerve, potentially blinding him. His left eye will also be rebuilt.
The procedure includes lifting the skin slightly off his face so that microsurgery can be performed underneath, which avoids scarring.
Unfortunately, because Noah is getting his treatment out of state and his doctor is not a MaineCare provider, the family will not have secondary insurance coverage for it, Jean said.
The hospital trips are time-consuming, said the youth. He must arrive three days before the surgery to meet with his doctor and have tests done, and stay for 10 days afterwards for follow-up care. Noah and his family will stay at the Ronald McDonald House in Danville close to the hospital, as they have previously, he said.
Eventually, he said, the affected bones should stop growing, but it is uncertain when that will be. He explained that cessation usually occurs at the end of adolescence, but sometimes it continues into adulthood. In some cases, the bones never stop growing.
There is another complication to Noah's illness: bone tissue has grown partway around his carotid arteries. Because of the risks of surgery, he said, his doctor is just monitoring the growth for now; if it should become life-threatening, Noah might need an operation to correct the problem.
Noah stressed that his life is not much different from those of his peers. He plays linebacker and offensive line for the Camden Hills Regional High School football team, and enjoys skateboarding, hiking and hunting with his dad and brother.
He and his family attend Abundant Grace Community Church in Searsmont, where Noah runs the sound system for worship services, including making CDs of the services to be given to shut-ins.
"It's pretty cool," he said.
In addition, Noah volunteers the first Tuesday of the month at the pet pantry in Rockport with his brother and stacks wood for neighbors.
"We firmly believe that our children need to give back to their community," his mother said. "No matter what you don't have, you always have something to give."
Jean said she was overwhelmed by all the people who have helped her family since Noah's diagnosis.
"I just want to make sure the community knows how much we love them, and how grateful we are," she said.
Noah said his faith enables him not to worry about his disease.
"It hasn't changed my life at all. I don't let it, because God's got it covered."
Sarah E. Reynolds is a reporter for the Camden Herald.
Sarah E. Reynolds has been a reporter and writer for more than 20 years, winning awards from the Maine Press Association and other professional organizations. She loves to read, hike and play word games.
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