Richard Engel’s son Henry, age 4, has been diagnosed with Rett syndrome, a thus far incurable genetic neurological disease.
Engel is chief foreign war correspondent at NBC and has agreed to share his story in a recent article by Express Digest.
Just this month, while dealing with the continuous care that Henry’s condition demands, 45-year-old Engel and wife Mary welcomed their healthy newborn son, Theo.
Among other tasks, Engle’s wife spends one solid hour each day helping Henry exercise in a standing frame while holding him.
About Rett Syndrome
The disease causes physical impairment and cognitive deficits as well as premature death. One child in 12,000 is born with the disease which affects mostly females.
Symptoms include breathing issues, problems with language skills, hand movements, muscles and coordination as well as slowed growth. Until a cure is found patients are dependent upon speech, behavioral and physical therapy.
The disease is caused by mutations (changes) in the MeCP2 gene that issues instructions to make the MeCP2 protein, which is required for normal brain and nervous system function.
Engel explained that scientists at the Texas Children’s Hospital are attempting to produce the MeCP2 protein artificially which would alleviate some of Henry’s symptoms.
Rett syndrome as four stages:
- The first stage occurs between the age of 6 to 18 months with slow development and difficulty walking
- The second stage begins between one to four years old. This stage involves deteriorating brain functions causing difficulty in communicating and learning.
- The third stage occurs between age three and ten causing floppy limbs, weight loss and possibly epilepsy.
- The final stage may last for decades and includes curvature of the spine (scoliosis) and the inability to walk.
About Henry’s Condition
Henry was diagnosed in 2017. Only now has he begun to experience seizures and compulsive repetitive movements. One such movement, continuously “scratching” his eyes, requires his parents to be on constant watch. Engel said that they are now using arm braces as a temporary solution.
Henry has also undergone several surgeries. His parents are bracing for another surgery to correct problems with his hips that are not developing properly.
A Race Against The Clock
Henry was diagnosed by Dr. Huda Zoghbi of the Texas Children’s Hospital who is credited with discovering the genetic cause of the disease. Dr. Zoghbi is working with Henry exclusively as his case is somewhat removed from the usual variation of Rett syndrome.
While they wait for treatment that is in the developmental stage, Henry’s condition is getting worse. Engel is hoping that the treatment under investigation at the Texas Children’s Hospital will be available to Henry in the very near future.
A Very Emotional Moment for Engel
Last March, while Engel was on an assignment in Syria, his wife would tell Henry that his Dad would be coming home soon.
When Engel returned home as he held Henry and sang to him, Henry looked at his dad and for the first time, he said the word “Dada”.
As Engel explained, those words are a thrill to most parents, but in this case, it was such an emotional triumph that Engel said it lit up his entire world.
Engel spoke about that moment on the Today show. He said it was an acknowledgment that his son knows him. He said it showed that Henry knows that he and his wife are there for him and that Henry loves his parents.
Engel said that when you have a special needs boy or girl it makes you appreciate the little pieces of sunshine you see on the lonely road to a cure.
