Thumbelina Kids: Tiny as Dolls, They Strive to Fit In

PHOTO: Russell Silver syndrome
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Ian Earley wore preemie clothes until he was 9 months old. During potty training and beyond, he was too small to get on the toilet. And he couldn't reach the sink to wash his hands until he was 6.

Ian has Russell-Silver syndrome (RSS), a form of primordial dwarfism that affects 1 in 100,000 babies, according to the National Institutes of Health.

Without growth-hormone treatment, boys will only reach an average height of about 5 feet 1 inch, and girls will only grow to about 4 feet 10 inches.

"Clothing is a huge challenge," mother Heather Earley of Libertyville, Ill. said, "This past summer, even at the age of 7, Ian could wear certain size 4T shorts. We have to buy him 6-slim jeans due to his height. But they still need to be cinched at the waist."

Ian was so tiny that he needed pillows and head insets in strollers and car seats. And well beyond infancy, Ian had to sit rear facing in the car seat because of the weight requirement.

In about 40 percent of the cases, Russell-Silver results from the abnormal regulation of certain genes that control growth. Severe long-standing placental insufficiency can also lead to severe intrauterine growth deficiency that resembles RSS.

Children such as Ian struggle not only with the mechanics of the physical world but with the social stigma that comes with being unusually small.

His mother, a divorced mother of two, fought to get the right diagnosis for her son, one that often eludes pediatricians. She writes about the challenges of living with the syndrome on her blog, Russell-Silver Advocate.

"This child has the most amazing charisma of anyone I have met in my life," Earley, 43, said. "He has friends all over the place. When he was 4 and looked like 2, all his friends wanted to pick him up like he was a baby. Even to this day, I want to pick him up, he's so cute and cuddly.

"But he knows he is different and knows he is small," she said. "He has some anger issues because his brother is only a year and a half older and three times his size. He is an adorable and loving child but he hates being different."

RSS, sometimes referred to as Thumbelina syndrome because of the fragile, doll-like stature of the children, is characterized by slow body growth, poor muscle tone and digestive problems, such as reflux and constipation.

Their heads, however, are unusually large, compared to the rest of the body.

"Try putting a 12-month shirt over a 3-year-old head," said Earley, half joking that she has considered approaching the Gap about clothing for special needs children like Ian.

Ian Earley, 3, with his older and bigger brother Alexander, who was 5.

Unlike dwarfism, where one segment of the body is smaller than the other, RSS is a different kind of short stature; it's perfectly proportionate.

Other than their size, "they pretty much look like other members of the family," said Dr. John Graham, director of clinical genetics and dymorphology at Cedars Sinai Medical Center in Los Angeles.

But the small children have diminished fat and glycogen storage and many cannot break down sugar.

"They are underweight, even as much as people try to over-feed them," he said. "They can go for long periods without eating."

RSS is a set of clinical features that results in severe prenatal onset growth deficiency that affects the body, but not the head.

In less than half of all cases, the condition begins shortly after conception.

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