The progeria, which turns children into old men, fails to overthrow the stubborn American woman
Tiffany Wedkind gets up early, puts on her ballet thumbs after the morning outfit, and performs several graceful moving figures. Then he rides his bike and heads for the office. By noon, hurry to a yoga class. In the evening he goes out for a drink with friends. She doesn’t care about the mocking or surprised looks and the biting remarks about her appearance that untidy passers-by make to her.
The 43-year-old American from Columbus, Ohio, is a strange woman – she looks like a child in the back and an 80-year-old woman in the front.
It is 1.37 m high
and weighs 26 kg –
measures of
10 years old
little girl
She looks like an anorexic, wears a blonde wig and has dentures instead of teeth. But the smile does not fall from her face and there is energy for an entire football team.
Tiffany suffers from a rare genetic disease – progeria, or Hutchinson-Guilford syndrome. It ages 8 times faster than other people. And not only the skin, but also its internal organs. But she survives and doctors consider her a miracle of medicine. It reaches a life expectancy 3 times longer than that characteristic of this syndrome. At the same time, she is vital, flexible, leads a normal life and is an incredible optimist.
Currently, about 200 people in the world suffer from progeria and Tiffany is the oldest patient with this disease, affecting 1 in 50 million people. A victim of a defective gene, people with such a disease most often die by the age of 12-15. Some survive to 20 or more, but in rare cases.
With Tiffany, things are slower. She has been aware of some oddities of her body since she was a child – she does not grow in height, her hair falls out and her permanent teeth refuse to break through, so they take them out in their infancy. But she is energetic and overactive. At school she enrolled in all possible sports, became a gymnast. The little one even plays basketball and softball. She took a dance class and became a cheerleader. She
hears the terrible
diagnosis chuck
at 20 years old
Her brother Chad has similar symptoms and breaks his thigh twice – the strongest bone. They replace his heart valve, give him a triple bypass. When he decides to get married and have children, Chad undergoes a genetic test. The whole family gives DNA material and finds out about the disease, whose name they haven’t even heard of. It turns out that the mother passed the damaged gene to both children, but she only carries it, has no symptoms and is now over 70 years old.
“We hardly fully understood what awaited us until my brother died at the age of 39 in 2012,” says Tiffany. – His death opened my eyes and motivated me to live at full speed. I’m lucky I’m still here. I am blessed. ”
And he embarks on endeavors that are impossible for very healthy people. Opens an interior design desk, a cleaning company. He makes luxurious scented candles, paints decorative paintings, makes backpacks decorated with figures. In the meantime, he managed to get married and the marriage lasted 8 years – until 2017. Of course, without children. She insists on sports and claims he keeps her alive. He practices yoga every day, rides a bicycle, and climbs mountains on weekends. That’s why she has muscles and is flexible.
“I am just like that
cats – I have
9 lives and now
I drive the sixth
or the seventh, ”jokes Mrs. Wedekind. She believes that her great achievements are yet to come. He takes good care of his appearance, puts on make-up, changes his wigs – blonde, red, brown – with which he covers his naked head. She wears original jewelry made of leather and metal, she has made tattoos.
The fact that the heart and bone problems characteristic of progeria are less pronounced contributes to its tone. But her external scars are not spared –
wrinkled skin,
baldness,
low height,
lack of teeth
and fats,
narrow face and lower jaw, sharp nose, squeaky voice.
“I happened to be born with this disease, which somehow makes me different and special. But I will not allow her to define my life – that’s who I am “, says the determined woman, who relatives and friends call the Stubborn Typhoid.
Her mother Linda is not so tough, she is tormented by guilt that she passed on the defective gene. He says: “I am constantly worried. Not a day goes by without the thought of losing Tiffany. But she is stoic and doesn’t let anything stop her. ”
Unlike the movie “The Strange Case of Benjamin Button”, where Brad Pitt’s character is born old and rejuvenated over time, progeria disease irreversibly dooms the patient to early aging. It is incurable. Its name comes from the Greek language and means premature old age. Caused by
gene mutation,
which is transmitted
of the children from the mother
The mutation affects the LMNA gene, which produces lamin A, a protein that supports cell nuclei like a scaffold. Defecting the gene destabilizes the nuclei and leads to premature aging.
Such children are born seemingly healthy. The symptoms appear at 1-2 years – slow growth, hair loss, dental problems. Then the disease develops rapidly and patients most often
they are still dying
teens from
heart or stroke
Tiffany Wedekind is a happy exception. It has been declared the longest surviving patient with progeria in history – a case that doctors say is extremely rare. At the age of 43, only skin and bones, with organs endangered by the disease, she races like a mare, dances, climbs peaks, travels the world. He lives completely normally and independently and does better in business than healthy people. He recently took a new anti-aging drug, Lornafarnib.
“Because I’m so young, some people think they can take advantage of me, but I don’t give up,” she said.
Relatives are encouraged by fate: “Existence like ours is difficult, but do not lose your positive attitude. Maintain harmony between body, mind and spirit. She assures that the disease and the question of whether she will live until tomorrow make her appreciate and enjoy every day of life.
According to Tiffany, it is very important to have solid, rock-loving people around you to help you overcome difficulties. But the real power is in yourself, she says, adding, “The disease has taken away my teeth, but not my smile.”
– .
