The chief foreign correspondent of NBC News, Richard Engel, shared the news that his young son Henry died this week. Henry Engel was only 6-years old at the time of his death.
"Our beloved son Henry passed away," Engel wrote on Twitter. "He had the softest blue eyes, an easy smile and a contagious giggle. We always surrounded him with love and he returned it, and so much more."
Our beloved son Henry passed away. He had the softest blue eyes, an easy smile and a contagious giggle. We always surrounded him with love and he returned it, and so much more. Mary and Richard. https://t.co/M8LV8SHv6r pic.twitter.com/21Ja6TOtjH
After missing some developmental milestones, Henry was diagnosed with Rett syndrome in 2018. He was treated at Texas Children's Hospital's Duncan Neurological Research Institute and the hospital described him as "special in so many ways" in a heartfelt tribute, declaring emphatically that they will "honour his life" by developing treatments.
The illness is a rare genetic disorder that affects the brain resulting in serious disability. It is rarely seen in boys, making the death of Engel's son more tragic. It affects around 1 in 12,000 young girls.
Symptoms are not always obvious, but the common theme is a slow in developmental growth. The British National Health Service (NHS) lists four stages of the disease. These are listed as: early signs, regression, plateau, and deterioration in movement.
The initial stages are hard to spot but begin with a stagnation of the early developmental features of children. Whatever has been learned is quickly lost in a two-year span between the ages of 1 and 4. There are problems with communication, co-ordination, memory, and mobility. These symptoms may gradually improve for sometime before the loss of the ability to walk, combined with muscle weakness and scoliosis.
Researchers are making amazing progress using Henry's cells to help cure RETT Syndrome so others don't have to endure this terrible disease. To support the research: https://t.co/M8LV8SHv6r pic.twitter.com/UNnDONMtR1
There is no cure for the syndrome and treatment is focused on managing the disease. Most people will be dependent on 24-hour care throughout their lives.
Almost all cases are caused by a mutation of a gene from the X chromosome. This gene, MECP2, contains instructions for a protein needed for brain development. The lack of this protein causes the symptoms listed above.