Local Parents Lose Baby to Rare Genetic Disease 

Joy Hollowell

Anita and Troy Hood were overjoyed when their little girl was born last January.But a few months after her birth, Ella began having health problems.What happened next turned out to be a roller coaster ride for the Hood family.By all accounts, Ella Hood was a perfect baby. Her mother, Anita had no complications during pregnancy or delivery. But when Ella was four months old, the Hoods noticed something strange.”She had been tensing her arms and balling her fists up and kind of arching her back,” says Anita Hood.Ella’s pediatrician recommended physical therapy. But the tiny girl was also having severe reflux problems as well as trouble breathing. Ella was eventually admitted to the hospital.”They finally diagnosed her heart defect,” says Troy Hood.A surgery date was scheduled, but first Ella would need to gain some weight. Doctors inserted a feeding tube to help her. But after ella stopped breathing one night, she was rushed to a portland hospital, where they performed the operation.”And then, we thought things were going to get better,” says Anita Hood.But they didn’t. In fact, Ella’s reflux got worse. After numerous treatments and trips to the hospital, Ella was diagnosed with a perforated bowel, and again another risky surgery.”We didn’t know if we were going to see her again, but she did make it through the surgery,” says Troy Hood.A few days later, Ella stopped breathing again. Troy says after some heavy persuasion on his part, Boston Children’s hospital agreed to admit their daughter. Multiple departments conducted tests on the young patient, including a bone marrow biopsy.”That’s actually when they found the Gaucher cells in her bone marrow.”Gaucher disease, pronounced Go-shay, is a genetic disorder. Those with it lack a type of enzyme that the body uses to remove old cells. Both parents have to carry the defective gene, and it is extremely rare. Most children who are diagnosed with Gaucher disease don’t live past the age of 5.The Hoods did learn that Ella’s heart defect was completely separate of the disease.By November, she could only breathe with the help of a ventilator. Doctors allowed Anita and Troy to take her home. The night before Thanksgiving, severe pneumonia was found in both of Ella’s lungs.”Ella passed away the next day,” said Troy. “It would have been nice to be able to see what she could have done with the determination she had and the toughness and the beauty. As a father, you wonder what could have been.”Because so little is known about Gaucher disease, the Hoods have agreed to work with the National Institutes of Health. They’ve already provided DNA samples.There are a number of websites with information on Gaucher