What is Rett Syndrome? Life expectancy explored as journalist Richard Engel's 6-year-old son passes away

On Thursday, August 18, NBC News’ chief foreign correspondent Richard Engel announced the passing away of his six-year-old son, Henry, due to Rett Syndrome.

Engel announced the news of his son's demise via Twitter. In an obituary on Jan and Dan Duncan Neurological Research Institute’s website, Engel mentioned that his son suffered from Rett syndrome, a rare genetic disorder.

The obituary post mentioned:

“Henry made the best of every single day and worked tirelessly in his many physical and developmental therapies. He continues to be an inspiration for Dr. Zoghbi and her team as they work to find effective treatments for Rett syndrome, and they already are making significant progress with Henry’s own cells.”

As per the obituary post on Jan and Dan Duncan Neurological Research Institute’s Website, the six-year-old passed away on August 9.

According to the aforementioned obituary post:

“Henry had a mutation in his MECP2 gene. MECP2 mutations cause Rett syndrome, a disorder that typically affects girls after their first birthday, robbing them of learned skills and leaving them with cognitive deficits, loss of speech, and a variety of motor difficulties.”

Richard Engel and his son, Henry, came to the facility in 2018 and Dr. Huda Zoghbi looked after the toddler’s case. As per Dr. Zoghbi, Henry’s treatment and diagnosis further advanced the research on the syndrome.

The doctor further insinuated that Henry will serve as an inspiration for her and the team, who are currently researching and developing the treatment for the MECP2 gene’s mutation.

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Rett syndrome - Causes, Life expectancy, cure development, and more

The syndrome is a rare genetic neurological and developmental disorder that hinders the brain’s functionality and affects motor as well as linguistic skills. While the disorder mostly affects female children, it has been also seen in male babies. The disorder manifests itself in babies at a very early age, usually by the first six months.

The cause of this syndrome is the MECP2 gene’s mutation, which also causes the MECP2 protein to malfunction. This protein is essential for brain development. According to Boston Children's Hospital, the disease can occur in any child at random. However, as mentioned before, female children are at a higher risk from the disease.

While the syndrome may pose various symptoms, the primary ones are the deterioration of motor skills, decrease in cognitive capabilities, communicational impairment, heart disorders, scoliosis, kyphosis, seizures, and many more.

As per Boston Children’s Hospital:

“There’s no cure for Rett syndrome. However, research is underway on new drugs that may improve management of symptoms.”

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Life expectancy

Because of the rarity of the syndrome, an accurate representation of life expectancy is impossible to figure out as of now. As per Rett Syndrome News, some of the affected individuals have even reached their 40s and 50s.

However, any data beyond that age group is difficult to come by. Meanwhile, International Rett Syndrome Foundation states:

“Data from the Natural History Study have determined that a girl with RTT has a 100% chance of reaching age 10, a 90% chance of reaching age 20, a greater than 75% chance of reaching age 30, a greater than 65% chance of reaching age 40, and a greater than 50% chance of reaching age 50. As improved nutrition and overall care are provided, these probabilities are expected to improve.”

It is possible that in the next few years, some sort of cure to mitigate the mutation or to ensure its prevention will be available. However, the rarity of the disease is also one reason why the research takes so long.