Richard Engel, senior international correspondent for NBC News, revealed the death of his six-year-old son, Henry, due to Rett syndrome on Thursday, August 18. Engel announced the news of his son’s death on Twitter. Engel said his child had Rett syndrome, a rare genetic disease, in his obituary on the Jan and Dan Duncan Institute for Neurological Research website. Richard Engel, senior international correspondent for NBC News, revealed the death of his six-year-old son, Henry, from Rett syndrome on Thursday, August 18.
Engel announced the news of his son’s death on Twitter. Engel said his child had Rett syndrome, a rare genetic disease, in his obituary on the Jan and Dan Duncan Institute for Neurological Research website. The 6-year-old died Aug. 9, according to an obituary posted on the Jan and Dan Duncan Institute for Neurological Research website.
According to the obituary shown above:
“Henry has a mutation in the MECP2 gene.” MECP2 mutations cause Rett syndrome, a condition that mainly affects girls after the first year of life, robbing them of acquired abilities and leaving them with cognitive deficits, loss of speech and a range of physical problems.”
In 2018, Richard Engel and his son Henry visited the facility, and Dr. Huda Zoghbi took care of the condition of the young child. According to Dr. Zoghbi, Henry’s treatment and diagnosis spurred research into the disease. The doctor went on to say that Henry will be an inspiration to her and the team that is actively researching and developing therapy for the MECP2 gene abnormality.
Rett syndrome causes, life expectancy, drug development and other information
The syndrome is a rare inherited neurological and developmental condition that damages brain function and reduces motor and verbal abilities. Although this condition mostly affects female offspring, it is also seen in male infants. The disease occurs in newborns at a relatively young age, often during the first six months. This condition is caused by a mutation in the MECP2 gene, which also causes the MECP2 protein to malfunction. This protein is necessary for proper brain development. According to Boston Children’s Hospital, the disease can strike at any time and in any child. However, as previously stated, female children are more susceptible to the disease.
Although the condition can cause a variety of symptoms, the most common include degeneration of motor skills, cognitive decline, communication disorders, heart problems, scoliosis, kyphosis, seizures, and many others.
According to Boston Children’s Hospital:
“There is no cure for Rett syndrome.” However, research is underway on new drugs that may improve symptom management.”
Average life expectancy
Due to the rarity of the syndrome, it is currently difficult to determine an accurate approximation of life expectancy. According to Rett Syndrome News, some of the affected people are now in their 40s and 50s. However, data outside that age range is difficult to obtain. Meanwhile, according to the International Rett Syndrome Foundation:
“According to the Natural History Study, a girl with RTT has a 100% chance of living to age 10, a 90% chance of living to age 20, a greater than 75% chance of living to age 30, a greater than 65% chance of living to age 40, and more than a 50% chance of living to 50.” These odds are predicted to increase as nutrition and general care improve.”
It is likely that a drug to reduce the mutation or to ensure its prevention may be available within the next few years. However, the rarity of the disease is one of the reasons why research is taking so long.
Categories: Entertaintment
Source: HIS Education