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Una publicación sobre discapacidades visuales, y sordera y ceguera, para familias y profesionales.

Summer 2000 Table of Contents
Versión Español de este artículo (Spanish Version)

By Brenda Placette, Grandmother, Houston, TX

My three-year-old grandson, Ben Rickerl, died last Tuesday night. For the last fourteen months of his short life, his only nutrition was Neocate, a special formula available by prescription only, which his parents administered to him through a bottle. Because of Ben's severe allergic reactions to food, it was the only nourishment he could tolerate. In early 1999, an attempt to feed him goat's milk, recommended by one of Ben's doctors, resulted in anaphylactic shock and an extended stay at Texas Children's Hospital in intensive care.

Ben was born with Joubert Syndrome, a very rare genetic disorder. His 7-year-old sister, Meglyn, is unaffected, but Madison, age 5, also suffers from the syndrome and is blind. Her speech and gait are affected by hypotonia, low muscle tone, but she is a happy child who is attending kindergarten. Joubert's is characterized by multiple sensory and physical disabilities as well as health impairments.

In Ben, Joubert's manifested itself in particularly insidious ways. Although classified as deafblind, he had some hearing and loved reggae music and the sound of his Daddy's voice. He had many health problems resulting in numerous hospital stays. He had to have round-the-clock care to access the medications, nebulizers, and interventions to keep him alive.

Ben had started school two months ago, and loved the wheelchair lift on his special education bus. His teacher, Mrs. Cockrell, and his two aides reported progress, and his mom and dad noted excitedly that he was making new sounds.

By the time Ben was born, my daughter Alison was already immersed in the special world of the disability community. The Rickerl home was full of people constantly: orientation and mobility and vision teachers; occupational, physical, and speech therapist; people delivering and setting up durable medical equipment. Alison sought out all resources for Madison, joined many disability groups, and became a leader in some.

In March of 1997, the Texas Association of Education and Rehabilitation of the Blind and Visually Impaired named Alison, Exceptional Parent of the Year. Their annual conference was held in El Paso, and I attended to help with Ben, since Alison was nursing. As I sat in the audience of 200 educators holding my precious grandson, Alison accepted the award and without notes thanked two-dozen people in the audience by name and talked about the importance of teamwork between parents and teachers to optimize educational outcomes. It was a bittersweet moment: I thought what an incredible person my daughter is becoming through the gifts of these special children.

When Ben died, Alison and Ted's first thoughts were how to help other parents who have children with Joubert's, and they donated Ben's brain, kidneys, and retinas - the organs most affected - to the Joubert Foundation.

One hundred and twenty-five people attended Ben's visitation and five-hundred attended his funeral mass. Shortly before the graveside service began, my 90-year-old great-great Aunt Peachie walked unsteadily down the long gravel road to the gravesite, aided by a walker. Her daughter, who is waging her own battle with pancreatic cancer, accompanied her. At the service, my daughter Alison eulogized her only son. She said she felt that Ben had served his purpose in life — something that takes many of us years to identify and fulfill. He had saved some souls and touched so many lives.

The disability community is a big, extended family. They share their pain, but they also share their joy. Grounded in realities that are sometimes incredibly harsh, they are very connected to each other emotionally. I am so grateful that I live in this world.

Growing older is always about loss. We all lose our parents. Our joints, muscles, and organs react differently. Our spouses and friends die; occasionally we lose a child or grandchild. It hurts; for a while we think we may not be able to endure the pain.

On the three-hour trip to Union Cemetery in Teague, Texas bright splashes of bluebonnets and Indian paintbrushes adorned the countryside. I thought, "Right now babies are being born, people are falling in love and getting married, people are celebrating their 50th anniversaries, and people are being buried." Life is glorious and it is also very painful. In the past week, I've witnessed what people refer to as the "triumph of the human spirit over adversity" many times. As the small white coffin was being closed for the last time, five-year-old Madison said, "Bye-bye Little Ben." All of us who knew him and loved him are better for that experience. Isn't that the ultimate measure of a life?

For more information about Joubert Syndrome, visit www.joubertfoundation.com.