Teen, family prove success with Prader-Willi is possible

Kristina Ho’s appetite is out of control, forcing her family to padlock the fridge and lock the pantry door.

But the answer to her compulsive eating isn’t simply a matter of willpower. Ho, 19, suffers from Prader-Willi syndrome, a rare genetic disorder that causes poor muscle tone, low levels of sex hormones, mild intellectual and learning disabilities, and a constant feeling of hunger.

The part of the brain that controls feelings of satiety and hunger doesn’t work properly, causing people with the disorder to overeat. If not controlled, the result can be morbid obesity and premature death.

Kristina has a continuous urge to eat but needs less food than others her age because her body has less muscle tone and burns fewer calories. Her meal portions are kept at least a quarter of a normal serving size, sometimes smaller.

"If someone left a bag of food, she would pick it up and start eating or hide it for later," said her mother, Tami Ho.

Those diagnosed with Prader-Willi need lifelong supervision of their dietary habits. The disorder also affects Kristina’s family members — parents Tami and Steve; sister Erika, 22; and brother Brandon, 15 — who live under strict meal and snack rules. Meals are eaten around the dinner table, there’s no snacking in front of the TV, and desserts are allowed only a couple of times a week.

Despite her condition, Kristina keeps busy with normal everyday activities, takes gymnastics classes, participates in soccer and track with Special Olympics and volunteers at Kailua Public Library. She loves Hello Kitty and enjoys doing phonics homework.

May is Prader-Willi Awareness Month, and Tami Ho hopes other parents will see the successes of children dealing with the disorder instead of viewing it as a likely death sentence. Her family’s active involvement in managing her condition has helped get Kristina through high school, and she will receive a graduation certificate this month from Kala­heo High School. She’s also a part of a work-study program at Hardware Hawaii.

Although there is no cure for Prader-Willi syndrome, an early diagnosis can help parents prepare for the dietary and behavioral needs of a child with the disorder. Families can be referred to appropriate agencies to deal with developmental delays and other issues.

Kristina was diagnosed with Prader-Willi syndrome at 5 weeks old, through a blood test, after doctors were concerned about her recovery from heart surgery when she was just 2 weeks old. Later, as a youngster, she experienced high fevers and seizures. Her low muscle tone delayed her physical development, and Kristina was also diagnosed with juvenile scoliosis and wore a back brace from age 9 to 18.

Pediatrician Lynn Yana­gi­hara, Kristina’s doctor, said genetic testing and early intervention are critical in children with Prader-Willi syndrome. Infants with the disorder generally have trouble sucking, appear "floppy" and have a weak cry, she said.

"Sucking can be a problem, so these babies sometimes need special nipples to ensure adequate growth," Yana­gi­hara said.

"Kristina’s success can be attributed to her thoughtful, committed and supportive family. Tami and Steve, Erika and Brandon have a solid understanding of Kristina’s challenges. Together they have addressed her appetite by setting limits and giving her choices," she said. "They have encouraged her to be physically and socially active."

For more information on Prader-Willi syndrome, visit www.pwsa­usa.org.

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“Be Well” features inspiring stories of people dealing with health challenges. Reach Nancy Arcayna at narcayna@staradvertiser.com or call 529-4808.