High Doses of Testosterone Halt the Extraordinary Growth of US Boy

US doctors seem to have successfully halted the runaway growth of 12-year-old Brenden Adams by pumping in testosterones. The harmones have accelerated the onset of puberty, closing the growth plates sitting on either of the bone. Doctors say he is the only known case of his kind in the world.

At birth, there seemed nothing out of the ordinary about Brenden, who weighed in at 7lb 3oz and measured a regular 19.5 inches.

"But at his two month check-up," said his mother, Debbie Ezell, 40, "the doctor kept re-measuring and said 'Something's just not right, he's way too big for his age, too long.' At four months, he got all his teeth at once and they were like, 'OK, something's going on here.'"

By the age of eight, his height was that of an average 15-year-old and his head circumference the equivalent of a grown man's. "Sometimes he would grow several inches a month," his mother recalls.

"We were concerned that he would continue growing and there'd just be no stopping it," said Dr Melissa Parisi, a geneticist at Seattle Children's Hospital, who has been seeing Brenden since he was four years old.

"He really is a remarkable young boy. He's unique."

With the help of Brenden’s haematologists and oncologists, Parisi found out that Brenden’s chromosomes had been quite unusually rearranged. The condition called the inversion of chromosome – 12, had actually been the one affecting every single cell of the boy’s body since the beginning.

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In our bodies, our genetic material, that is, the chromosomes are present in pairs. However, in Brenden’s body this was not the case. Not all his chromosomes were present in pairs. The 12th chromosomes in his body did not match.

Quite strangely, inexplicably and uniquely does a thing like this happen, where a piece of a chromosomal strand breaks off in the middle, flips around and then reattaches itself, quite innocently running havoc with some critical gene in the body – the growth gene in Brenden’s case.

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“This gene is functioning despite the regulation that it shouldn’t be,” said Dr. Gad Kletter, Brenden’s endocrinologist at Swedish Hospital in Seattle. “It’s over-functioning and there seemed to be no stopping it.”

Kletter finally figured out how to stop Brenden Adam’s hyperactive growth gene - by inducing puberty.

“We induced puberty,” explained Kletter about the weekly shots of testosterone, “to fuse the bones and stop the growth.”

"Before treatment, he was on a trajectory that was still taking him up and up, rather than reaching a plateau. Plotting his growth curve, we think he would have been over eight feet now," said Dr Parisi.

But Brenden has not grown any more for six months.

Still he requires a team of doctors simply to stay on top of his ever-growing frame. He has oversized joints that restrict his movements, fatty tumors, and extra teeth - twelve of which were recently removed. He has a heart condition too. These mean his long-term outlook is uncertain.

But Brenden carries on unfazed, making the best of a bad situation. He has two families in Ellensburg, Washington and stays with each a week at a time: his mother, Debbie Ezell, and stepfather Sam Ezell, and his father, Willie Adams, and stepmother Julie Adams. Each couple has children, too, who love and accept Brenden.

He’s also accepted by his sixth-grade classmates at Morgan Middle School. Teacher Gretchen Holmstrom said in December last year, “He’s a delight to me and all the kids in class,” Holmstrom said. “He’s got a lovely spirit that just flows. He’s accepted who he is.”

Holmstrom said she’s impressed with how his classmates and all those in the school accept and respect Brenden.

“They see him for who he is and treat him just like any other kid,” Holmstrom said.

Holmstrom said she’s 5-feet-9 inches tall and is not used to looking up to the sixth graders under her care. She said she has to remind herself, at times, that Brenden is a 12-year-old.

“Sometimes he puts on that impish grin and is playful and teasing in a gentle way,” Holmstrom said. “It’s that sweet spirit that impresses me.”

Debbie said Brenden’s ability to be at peace with the unknowns of his future health, and yet keep a good attitude about his problems and the coming need for surgeries makes him special, unique and not just like any kid.

He has a quiet determination within himself to keep going, Debbie said, when it would be easy to be discouraged and depressed.

“Brenden has a gift, that way,” Debbie said.

Brenden said not many things get him down, although he’d like to not have any more surgeries. Yet, knowing they’re coming, Brenden said he doesn’t want them on his birthday or in summer when he wants to ride his bike.

“I’m sort of, well, I’m always mentally and physically happy all the time, I guess,” Brenden said.

Indeed the local community has embraced the good-hearted youngster as one of their own.

Their fundraising helped Brenden’s family build a new home with extra high ceilings and doorways, but the gangly preteen still has a hard time folding himself into mom’s SUV.

When a charity offered to grant him a wish, he asked for a bicycle big enough for him to ride without his knees cramping in pain. Doors and ceilings have been built higher at his home, friends and neighbours raised funds to help pay for a custom-made bed, and his mother has spent hours trawling the internet for affordable, over-sized school clothes.

"The hardest part is just not knowing what the life expectancy is," said Mrs Ezell. "But it's truly Brenden that keeps everyone on the positive side. If he's not here in a couple of years, we'll look back and think 'Yes, but when he was here, wasn't it good?'"

Source-Medindia
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