Lehi girl continues to beat the odds against Immune Deficiency Disease

Lehi girl continues to beat the odds against Immune Deficiency Disease

Heidi Toth – Daily Herald | Posted: Thursday, February 4, 2010 12:30 am

Second grade is a mecca for germs.

That should make it the last place for a little girl with a disease that has wiped out all of her natural immunity.

Yet, 8-year-old Emily Heaps fits into second grade easily, playing with her classmates at recess, drawing and asking questions. One might never know she was different just from watching.

That’s just fine with her. The hope is that maybe one day, she won’t be different.

Emily has severe combined immunodeficiency syndrome, which is a genetic defect that destroys the body’s natural immune system. It’s nicknamed “bubble boy disease,” after a 12-year-old boy who lived in a plastic bubble to keep him away from all germs. It’s more common in boys than girls, and Emily’s mutation is the least common, and hardest to treat, of all. In August 2008 she had her second bone marrow transplant; the first happened when she was just two months old. In the months after that second surgery, Emily’s doctors have looked at her low white blood cell counts and worried the surgery didn’t work.

Emily’s parents look at their happy, energetic, healthy daughter who is thriving in a public school and able to play sports and play with her friends at recess and have no problem believing the treatment was successful.

“She does have an immune system, finally, and it’s small, but it’s growing,” Jill Heaps said.

A year ago, as Emily sat out first grade because being around other people, especially children, was too dangerous for her fragile body, her family and doctors were waiting to see if the second transplant had been successful. They were following news of gene therapy treatment, an experimental procedure that showed good results in early tests but had sometimes resulted in leukemia and other complications. They also were keeping their fingers crossed that they wouldn’t need this radical treatment but said a year ago that they were open to it.

So far, that hasn’t been necessary. Emily is back in school, and while she has her own pencils and own scissors and her teacher is daily wiping down desks and chairs, she’s basically a normal 8-year-old. Jill has scaled back her deep housecleaning from daily to twice a week, and while the house and the children and even the dog still are hygienic and probably more sterile than many a doctor’s office, the family doesn’t obsess about cleanliness the way they used to. Not all the time, anyway.

“We went to the dentist last week, and every time they’d get a new tool out she’d say, ‘Is that clean?’ ” Jill Heaps said.

Her teacher is losing the obsessive-Lysol-spraying itch as well. Kinna Harris, a second-grade teacher at Sego Lily Elementary, didn’t know how this year would turn out, since Emily was not used to being in a classroom and had to be so careful. Her worries didn’t last long either; Emily knows what she needs to do, they have a special sanitizer on hand and Harris wipes down the desk and chair almost daily.

“I like being in school because we go to recess and do fun stuff,” Emily said.

More than that, however, is the transformation into a more independent, capable second-grader who has acquired problem-solving and critical-thinking skills instead of going straight to her teacher for help.

“Now she will figure it out,” Harris said. “She’s more willing to trust herself.”

Trust has figured heavily into the Heaps family as they dealt with Emily’s illness. Jacqueline, her 14-year-old sister, was 7 years old when Emily had her first bone marrow transplant. Doctors took the donated marrow from her closet match — her sister.

“After I realized it would save her life, I was all right,” she said.

The sisters have a unique connection because of that piece of Jacqueline that’s in Emily, rebuilding her immune system and producing the white blood cells that they hope will keep her alive longer than the average bubble child.

It has been difficult, of course. The Heaps children learned that Emily’s needs almost always came first, which really hurt last year when 11-year-old Thomas came down with H1N1 influenza. Because of Emily’s vulnerability, he was quarantined in his room for 11 days, and anytime Jill or Matt went in to see him, they wore masks and gloves and kept contact to a minimum. Eleven days without a hug from his mother was difficult for them both, Jill admitted. But they also had to keep Emily safe.

For now, they meet monthly with an immunologist to keep tabs on her white blood cell count, grateful that Emily is as healthy as she is, as healthy as she’s ever been, her mother said, but knowing the future still is full of uncertainty. They don’t know how the disease, the chemotherapy before her second transplant or the medications will affect her development. They don’t know her life expectancy. Jill Heaps said the oldest SCIDS patient they are aware of is 33 and doing well, so they’re hopeful. They do not know if she’ll hit puberty or if she could simply grow out of the disease as the transplanted bone marrow continues to produce healthy white blood cells.

But for now, they’re a normal family with a dog and chores and soccer practice and family dinner.

Heidi Toth can be reached at (801) 344-2556 or htoth@heraldextra.com.

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