When a rare condition ‘chooses’ you
THIS article reflects on the life of Ontlametse Phalatse, who was known among her family and friends as “first lady” as she was reportedly the only known living black female child with Progeria disorder in South Africa, on the continent and in the world.
Progeria is a rare genetic disorder which causes fast ageing at a very early age.
“Chosen” by it, Ontlametse experienced complications in breathing on Tuesday, 11 April, 2017. She was admitted at Dr George Mukhari Academic Hospital in Ga-Rankuwa outside Pretoria, South Africa, and she later passed away.
President Jacob Zuma said, “A brave young fighter has fallen. May her spirit inspire all others living with disabilities or facing any difficulty, to soldier on as she did and live life to the fullest? We are really proud of her.”
Her family wrote, “We will miss you sorely, Tsontso, our first lady. May you find eternal peace among the angels. Rest in Peace.”
Although doctors had said she would not live beyond her 14th birthday, last month Ontlametse celebrated her 18th birthday on 25 March, 2017.
Her life The mother of Ontlametse, Bellon Phalatse, narrated that her baby was “normal” at birth but she developed rashes which at the age of three months made her appear like she had a skin disease. By the time she turned one, Ontlametse’s hair had started falling, and at such an early age, she began to show signs of ageing. By the time she turned three, her father abandoned the family.
Ontlametse’s mother enrolled her to start school at the age of six and she proved to be an intelligent pupil, albeit the stigmatisation she endured from some teachers, classmates and others who said she was small and skinny because she had AIDS.
Wrongly labelled, with only two friends in class and most of her classmates unfriendly, Ontlametse was unfazed and she told Faul in 2011, “I don’t care what people say about me. 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?”
The diagnosis of Progeria was confirmed by a doctor when Ontlametse was approximately eight-years-old. Ontlametse and her mother would travel to the United States each school holiday, where she participated in research funded by the Progeria Research Foundation, thereby gaining access to emerging and advanced medicine which at the time may not yet have been available commercially in South Africa.
Back home, in their dilapidated brick home in the small town of Hebron, about 80km north of Johannesburg, maintaining a healthy diet which was demanded by the nature of Ontlametse’s disorder was not easy.
She was raised by her formally unemployed mother who was struggling to make ends meet, she had quit work so that she could devote time to taking care of her daughter.
Again unfazed, Ontlametse was quoted by Faul as saying, “Sometimes when my mommy has money, she buys lettuce and cucumbers and I help her do salad.”
Because of her bald head, Ontlametse would always keep her hat on her head whilst she was in school, hoping that one day she would acquire a custom-made wig. At home she would remove the hat, undertake her homework and watch TV and carry out tasks such as cleaning her shoes and washing her socks. Physical activity would make her feel very tired, hence she did not participate in sporting activities.
Asked by Faul in 2011, about what she would like to be, Ontlametse said, “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.”
What is Progeria disorder? Progeria disorder is a genetic condition which shows through dramatic and very fast ageing. The baby is “normal” when he or she is born and then starts to age very quickly within the first twenty-four months at a rate which has been estimated as six times faster than normal. If we go by that rating it means that when Ontlametse passed away, she was an 18-yearold who was in the body of a 108-year-old.
Progeria is described by WebMD in simple terms as: a “mistake” in a particular gene which causes that gene to make an abnormal protein and when cells use that protein they break down very easily, causing affected children to start growing old very quickly.
Progeria is not inherited or passed down in families, and it cannot be cured, but certain medicines as well as occupational and physical therapy may help to manage it.
So what can parents do? Consult your family doctor or paediatrician for a formal diagnosis, if you see your child developing symptoms of Progeria. Such symptoms include developing a bigger head, large eyes, a small lower jaw, thin nose with a “beaked” tip, ears that stick out and veins that you can see, a high pitched voice, slow and abnormal growth of teeth, loss of body fat and muscle, losing hair, including eye lashes and eye brows. What are the lessons? Life has a tendency of throwing anything at anyone at anytime and being “chosen” by a rare condition or circumstance does not mean the end of the world. With doctors having predicted that she would not live beyond the age of 14, Ontlametse and her mother did not sit at home and wallow in misery, wishing if Ontlametse had been born in a different way or waiting for her death day.
Her mother enrolled her in school and in spite of the stigma that surrounded her disorder and her awareness that she would not live long, Ontlametse took her school work seriously until she completed matric, hoping that one day she would go to university, work and buy a car and a house for her mother.
Ontlametse has been widely described as a person who loved life, lived it to the fullest and never wished she was born in any other way, other than the way she was. After a documentary about her life was produced a few years ago, Ontlametse used her newly found fame to motivate people to get on with their lives, thereby touching the hearts and lives of many.
Should we stigmatise those who have bodies that are different from ours or who find themselves in challenging circumstances as if we know what tomorrow will bring to us, to our children, to our grandchildren, great-grandchildren and other members of our family lineage?
No! If we go by the Shona proverb “seka hurema wafa” (laugh at disability when you are dead), we realise that as long as we live, anything is possible, we should not shun those who have bodies that are different from ours but we should embrace, love, understand and support them.