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Transplant gives parents, child hope

By ANDREW SKERRITT
Published September 10, 2006

When I met Elizabeth "Elly" Hulett, she was a handful - more than I expected.

She jumped around the living room with her grandmother Sandra Hulett and danced on a counter top. Mrs. Hulett held Elly's hands like a dance partner, beaming the way only adoring grandmothers do.

The 3-year-old is two months removed from a bone marrow transplant to combat the life threatening Hurler syndrome. But she's lighting up the Hulett household again. For the first time in a while, her parents, Brian, 34, and Heather Hulett, 31, can hope for Elly's future beyond fourth grade.

"Maybe she'll outlive us," her dad said.

A few weeks ago, I wrote a column about the songs in my head that take me back to my childhood. One song mentioned, Three Little Birds by the legendary Bob Marley, resonated with Brian Hulett. He wrote me an e-mail, saying that's the song he sings for Elly. In telling me about the song, he also told me about the battle his family has faced since Elly was diagnosed with Hurler syndrome last December. Several days ago, I visited the family at their home in Spring Hill to hear the rest of the story.

The couple, both respiratory therapists, came from St. Petersburg. They met in school. Elly was born less than a year after they were married.

She seemed normal, but as she got older she couldn't seem to shake the recurring ear and sinus infections. Inserting a tube into her ear didn't solve the problem and neither did increasingly strong doses of antibiotics.

So last fall, out of frustration, they visited All Pediatric Care in Spring Hill, where Dr. Ahmad Zuhdi examined Elly.

Just looking at her medical history and at her distinct features for the first time, Dr. Zuhdi suspected Hurler syndrome and he mentioned the diagnosis to his senior partner, Dr. Imad Jandali.

They called Brian to a room and told him what they feared.

Hurler syndrome results when a child's body doesn't produce a certain enzyme, resulting in deteriorating mental function, persistent infections, larger than normal head and distinct features.

In the old days, a child with Hurler would succumb to heart failure before his 10th birthday.

These days, Hurler children are usually diagnosed before their first birthday. Doctors start them on enzyme therapy and perform a bone marrow transplant. Without either treatment, by 21/2, a Hurler patient will show signs of mental retardation. Miraculously, Elly didn't.

Although she had most of the symptoms of Hurler, she was a smart, lively child. Her IQ was solid. Had she tested poorly, suggesting advanced mental deterioration, Elly wouldn't have been eligible for a bone marrow transplant.

"She is the most fascinating case I've seen in 18 years as a pediatrician," said Dr. Jandali, who has treated Elizabeth's since her second visit to his office.

With Elly's parents' permission, Dr. Jandali invited me to his office in Spring Hill to talk about his special patient. He showed me two thick manila folders containing her medical history. None of that information explained why Elly is functioning so well mentally.

Jandali credits Brian and Heather for acting when they did. Parents whose children are diagnosed with a life-threatening illness usually go into denial and try to seek a second or third opinion. But as soon as Brian heard Hurler syndrome, he went home and researched the disease on the Internet. When Dr. Janaldi confirmed the bad news, Brian was prepared. That meant their doctors could proceed without delay to prepare Elly for enzyme therapy and later the bone marrow transplant.

And there's Elly, home a month early from All Children's Hospital instead of the scheduled two.

She's a fighter.

To follow Elly's progress, visit www.caringbridge.org/visit/elly.

Andrew Skerritt can be reached at 813 909-4602 or toll-free at 1-800-333-7505, ext. 4602. His e-mail address is askerritt@sptimes.com.

[Last modified September 9, 2006, 21:06:09]