Emily Rapp, the author of “Poster Child: A Memoir”, writes an editorial in the New York Times about her son, Ronan, who was diagnosed with a rare genetic disorder called Tay-Sachs. Her moving text has touched the hearts of thousands of readers and has managed to raise awareness regarding the disorder in a delicate and personal manner.
“MY son, Ronan, looks at me and raises one eyebrow”, the editorial begins. Ronan is one year and a half old and his mother will probably have to say goodbye to him before he reaches the age of three. Ronan, which means “little seal” in Irish, has been diagnosed with a rare genetic disorder, named Tay-Sachs.
Tay-Sachs disease appears in a person at a very small age (3 to 6 months old), through the genes inherited by the parents. If both parents carry the disease gene, and pass it to the child, then he has 25% chances of developing it. However, if the child inherits the gene from only one parent, he will not develop the disease, but will be a carrier. Tay-Sachs represents the defective state of a gene on chromosome 15. It is the lack of a protein called hexosaminidase A, that triggers the disorder. Its lack causes the gangliosides (chemicals found in the nervous system) to add up in the nerve cells, causing the symptoms to appear.
Tay-Sachs makes the body turn to a vegetative state in a matter of months to years. The symptoms include: loss of sight, hear, motor skills, decrease in mental and social skills. The brain cells die, causing dementia and the body develops in a much slower rate than in the case of a normal healthy child. In the end, the child does not react to anything, entering a coma state which ends with his death. The maximum age these children normally reach is somewhere between 3 and 5 years.
To this day, there is no cure for the disease, nor a treatment. The doctors can only try and make the patient as comfortable as possible throughout the struggle with the disease.
Emily Rapp reveals the extremely different perspective that parents of children with terminal diseases have when it comes to the future. “We will not launch our children into a bright and promising future, but see them into early graves”, her editorial says. “We will prepare to lose them and then, impossibly, to live on after that gutting loss.” Even though it is depressing to read or hear about such cases, it is parents like her that can teach us the best life lessons. “Parenting, I’ve come to understand, is about loving my child today. Now. In fact, for any parent, anywhere, that’s all there is.”