Richard Engel is an American journalist and author who is the chief foreign correspondent for NBC News. He was assigned to that position on April 18, 2008, after serving as the network’s Middle East correspondent and Beirut bureau chief.
What disease does Richard Engels child Henry have?
Henry Engels is well-known to be the son of an American journalist and author named Richard Engels. Henry happens to be suffering from Rett syndrome.
Let’s look at how one can get the disease Rett syndrome.
Rett Syndrome is a rare genetic neurological disorder. It is usually seen in girls — about one in 10,000 are diagnosed with the condition — because the mutated gene responsible for symptoms resides on the X-chromosome. Girls have two copies of the X, which means they have a functional copy of the gene to somewhat mitigate the effects of the mutated one. That means about 50 per cent of their cells will make normal copies of the protein MeCP2, which is present in cells throughout the body, especially brain cells.
Boys, who have an X and a Y, only have the mutated gene, which generally results in their death either in utero or shortly after they are born. There is currently no treatment or cure.
Engel’s son, Henry, is alive because his mutation isn’t severe enough to kill him, although it has affected the toddler’s cognitive and motor skills. The protein produced by his cells is somewhat functional, albeit at lower levels than optimal.
“In Henry’s case, it doesn’t inactivate the protein,” said Dr Huda Zoghbi, one of Henry’s doctors and the discoverer of the gene for Rett. “But it’s not functioning fully and not at 100 per cent at a normal level.”
Engel and his wife, Mary Forrest, knew early on that something wasn’t right with their son, Henry. Even as the boy approached the second year of his life, he couldn’t talk or clap his hands. He also couldn’t walk or even sit up straight.
“It was a mystery and we thought, ‘Okay, well, there are late bloomers,'” Engel said.
The MeCP2 protein, it turns out, is very important for building connections in the brain, said Dr Jeffrey Neul, a Rett specialist and a professor of paediatrics and director of the Vanderbilt Kennedy Center at the Vanderbilt University Medical Center. “This gene works inside the nucleus — the control centre — of the cell,” Neul said. “It’s controlling what other genes and proteins are made within the cell in response to stimuli.”
Without enough functional protein, the brain can’t cement connections that are built when a person acquires new abilities, such as walking and talking.
Source: Ghanafuo.com
