- Associated Press - Wednesday, September 14, 2011

JOHANNESBURG The elfin child with the big personality and bright smile calls herself “the first lady” and dreams of the future.

But doctors say 12-year-old Ontlametse Phalatse has only, perhaps, another couple of years to live.

“I call myself a first lady because I’m the first black child with this disease. … Which other black child do you know with this disease?” she challenged.

Ontlametse is the only black child living with progeria, a rare and fatal genetic condition that accelerates the aging process, the Progeria Research Foundation said, adding that it was aware of two other black children who died from the disease.

Nobody knows how many children have the illness. In a two-year campaign to identify them, the Progeria Research Foundation says the number of diagnoses around the world has risen from 54 to 80 on five continents.

The foundation’s executive director, Audrey Gordon, said Ontlametse and a 5-year-old white girl, also in South Africa, are the only Africans identified as living with the disease.

On her office wall, Ms. Gordon keeps a world map studded with colored tacks where the disease has been diagnosed. But the map has “big, empty spaces” where the foundation suspects children may be suffering from progeria but have not had it diagnosed, she said.

“We know that there are children [with progeria] in Africa, in China and Russia, but we just can’t seem to get to them,” she said from the foundation’s office in Peabody, Mass.

Ontlametse’s mother, Bellon Phalatse, said her baby was born looking normal but she realized early on that something was wrong. The baby suffered constant rashes.

Before Ontlametse celebrated her first birthday “her hair was falling, her nails weren’t normal, the skin problems, we were going up and down to the doctors,” Mrs. Phalatse said.

As the child aged prematurely, her father abandoned the family when Ontlametse was 3.

Despite her frequent illnesses, Ontlametse enrolled in school at 6 and proved a bright pupil, but she was often scorned by classmates, teachers and others who thought she she had AIDS. South Africa has the highest number of people living with AIDS of any country, but the disease still carries a terrible social stigma.

“It was horrible, I don’t know how to explain” what we went through, Mrs. Phalatse said.

Two years ago, a doctor friend suggested she have Ontlametse tested for progeria and brought her a book about the disease with pictures of children afflicted by the disorder.

Children with progeria look remarkably similar, despite different ethnic backgrounds. They are small and bald with oversized heads, bulging eyes and gnarled hands. Thinning skin exposes a network of blue veins on the heads of white children.

A doctor confirmed Mrs. Phalatse’s fears that her daughter had the disease.

The diagnosis came with the news that most children with the disease die at 13. However, Mrs. Phalatse gained a better understanding of what to do to try to prolong Ontlametse’s life. The diagnosis also has brought Ontlametse the specialized care she needs.

The progeria foundation pays for Ontlametse and her mother to travel to Boston for periodic treatment at Children’s Hospital, where she receives cutting-edge drugs in a research project.

Back home, Mrs. Phalatse, who is unemployed, struggles to feed her daughter the required healthy diet while living on a government disability allowance.

Ontlametse is unfazed.

“Sometimes when my mommy has money, she buys lettuce and cucumbers, and I help her do salad,” she said.

At school, Ontlametse keeps her hat on her head. One of the things on her wish list is a specially designed wig.

She has two friends in her class, but she said not all her classmates are kind.

“I don’t care what people say about me,” she says, making a throwaway motion with a hand misshapen with arthritis, knobby fingers and discolored nails.

In her run-down brick home in the small town of Hebron about 50 miles north of Johannesburg, Ontlametse ditches the hat. She does her homework and daily chores such as washing her socks and cleaning her shoes.

She reads books and watches television but cannot play sports or even a game of hopscotch because physical exercise tires her out.

Asked what she would like to be, she breaks into a big grin that shows irregular teeth.

“I would like to be a psychologist, so that I can work on the problems of other people and so that they can accept the way that they are because they can see that I accept the way I am,” she said.

Children with progeria die almost exclusively from heart disease between the ages of 8 and 21, commonly suffering high blood pressure, strokes, angina, enlarged heart and heart failure.

Ms. Gordon said research into progeria has had remarkable success since her family founded the foundation in 1999 after the disease was diagnosed in her nephew.

In 2003, the foundation was instrumental in the discovery of the progeria gene. Now they hope it can help provide answers about the ordinary aging process and cardiovascular disease.

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