Imagine if you aged incredibly fast,
say for every year you aged 10 years.
It sounds like something from a fairytale,
a magic spell put on someone by a wicked witch.
But in the real world something does indeed at least resemble this.
It’s known as Progeria syndrome,
which is just one of what are called Progeroid syndromes.
These are basically genetic disorders
that seem to fast-forward the aging process.
No doubt you’ve heard of two fairly common diseases
that fall into this category that affect older people,
and those are Alzheimer’s disease and Parkinson’s disease.
Progeria is different
as sadly it starts manifesting at a very young age,
and so by the time children are aged just a few years
they already resemble a much, much older person
Let’s now look deeper into this.
The Progeria Research Foundation tells us
that the full name given to this disease is
Hutchinson-Gilford Progeria Syndrome,
named after the two people that discovered it
and wrote about it in the 19th century.
The first part, Progeria, actually comes from Greek
第一个词 早衰 实际源自希腊语
and means premature ageing.
We are told that the basis of this disorder is a faulty gene,
and scientists explain that
absence of the Lamin A/C protein makes the nucleus of cells unstable
and has the effect of very speeded-up ageing.
This doesn’t mean of course
that the young people have the wisdom of the aged
or that they are old in any way in their minds.
What does happen is the body ages,
meaning these people as early as two years old
may start to have a wrinkled appearance;
they lose their hair, have stiff joints,
have a high-pitched voice, a larger head, a thin nose,
嗓门尖 头部更大 鼻子干瘪
may experience kinds of problems associated with older age
including atherosclerosis and heart disease.
They might also suffer many other diseases
that people don’t usually get until their autumn years.
These differences in physicality don’t exactly mean
the children age and look as if they would have if they aged normally.
People with progeria often look quite alike.
According to various sources,
it can affect any race and occurs in both males and females
across the globe at about the same frequency.
You can usually tell if someone has Progeria at around 18 to 24 months.
The first signs might be loss of all body and head hair,
as well as a small pinched nose,
the appearance of many veins visible through the skin,
the absence of growth – including
what looks like a very small face but large head.
This won’t happen to many young people,
with Progeria it’s said to affect one in about four million people
There is no cure as yet,
and most of the sufferers can only expect to make it into their teens
or in very few cases perhaps the early twenties.
We are told the reason for their premature death
is almost always related to the heart having a stroke or having a heart attack.
These young kids may have some kind of heart surgery to extend their life,
but no amount of medical procedures so far
have managed to prevent serious heart-associated problems for very long.
But what about the people suffering from it,
how do they get on?
One of the people who survived the longest
with this disorder became something of a celebrity.
His name was Leon Botha
and he lived a full life until his death aged 26.
This South African man was a DJ, a Hip-hop artist,
他来自南非 是一位DJ 嘻哈音乐艺术家
writer, photographer and a painter.
You can see him spinning on YouTube,
or look at some of his impressive drawings.
The disease certainly didn’t hold him back in an artistic way.
We should stress again that
progeria affects the body, not the mental faculties.
In the British media in 2015 it was announced
that a progeria sufferer who had captured the hearts and minds of the public
had died at age just 17.
Her name was Hayley Okines and the press said
at the time of her death she had the body of a 104-year old.
She had been told she might not make it past the age of 13,
but she fought on, only succumbing to pneumonia in the end.
During her life she did some amazing things,
including writing her own biography called “Old Before My Time.”
She was also featured in two documentary films,
one called “ The Girl Who Is Older Than Her Mother” and “World’s Oldest Teenager.”
Her father told the press that he was besotted with his little girl.
As she got older, though, he noticed that she wasn’t growing.
他注意到 随着女儿年纪的增长 身体却停止发育
At first he thought it might not be anything as he is a very short man.
He then said that
at age 13 months she was still wearing clothes for a 3-month old and
he and his wife knew something was wrong.
She was then diagnosed with this unusual illness.
It didn’t hold the girl back, though,
and she fulfilled a few dreams by travelling around the world
swimming in the ocean with dolphins…
and maybe the best of all, she got to meet her idol, Justin Bieber.
最棒的或许是 她见到了自己的偶像 贾斯汀·比伯
She also had some words with Prince Charles and Kylie Minogue.
Another well-known sufferer was called Sam Berns.
You may have seen him when he did one of the TEDx Talks.
You can see quite clearly that this disease has no effect on how articulate he is.
He said if he wanted anyone to know anything about him,
it was this, “ I have a very happy life.”
He went on to say,
“ Even though there are many obstacles in my life,
many of them created by Progeria
I don’t want people to feel bad for me.”
His philosophy for a happy life,
he said, is that he is ok with what he can’t do,
because there are so many things that he can do.
While he can’t run a long way,
he only focuses on all the things he is able to do
Which are many.
Like many of you,
he must understand his abilities and adapt his life to knowing his boundaries
and then becoming the best at what he can do.
He also said he surrounds himself with people that are good for him
and that is something he believes we all should do.
They provide him with a positive influence
and he returns that back to them.
His other main reason for his happiness he said
was that he was always moving forward,
always planning the days ahead and making the best of those days.
Keep focused, he said.
“ I try not to lose energy feeling bad for myself, he said
“ because when I do, I get stuck in a paradox where there is no room for
any happiness or any other emotion. ”
He died aged 17.
You might also have seen someone
who has been called an Internet superstar,
having garnered much attention on platforms such as YouTube.
在不同平台 比如油管 获得了非常多的关注
Her name is Adalia Rose
and as we speak just one clip on YouTube featuring
her has been watched almost seven million times.
At the age of eleven in 2018
she already had 170 million views on YouTube
while her Facebook account had 13 million likes.
This girl, from Austin, Texas, said in one interview,
“ I like to make videos for my YouTube fans, because, I love ‘em I guess.”
Her parents say they try not to talk too much with her about Progeria
only that they treat her like any other child.
She seems to live just like any other girl,
with only the ageing process making her look different.
As we write this, one of her last videos posted to Facebook
Opens with the words,
“ If you have a disease, or a sickness, don’t be ashamed of it.
Because look at my eye ( she shows an eye that Won’t open)
and I have no hair, and I just went outside.
I felt ashamed, but now I’m ok.”
She is perhaps the biggest viral star that talks openly
about a condition that has handicapped her in some way
This disease is very uncommon as we said,
with the media saying right now about 500 children in the world have it.
But will any of these kids be cured?
What hope does science have of treating this disorder?
You can actually go the Progeria Research Foundation website
and see the bios of many of the kids that live with this disease or have passed away.
Just looking at a map of where cases were found,
it seems that Progeria sufferers are spread all over the world quite evenly
You can find Meghan from the USA,
a teenager that is an athlete, an author, a poet and a musician.
他同时是运动员 作家 诗人和音乐家
You’ve got 23-year old Sammy from Italy,
a budding actor and painter who’s been on TV in Italy many times.
Over in Australia there is Enzo,
whose mother says enjoys anything with wheels,
from playing Mario Kart to riding motorcycles.
In Belgium a brother and sister both have progeria,
Michiel who is 20 and his sister Amber who is 12.
They have a multi-lingual website
which details their day-to-day lives and their ongoing treatment.
Yes we say treatment,
because some of the people we have mentioned are taking part in trials.
Doctors hope one day to reverse this fast-ageing process.
We are told that there have been some success stories
when sufferers took the drug lonafarnib,
what’s called a farnesyltransferase inhibitor, during medical trials.
The drug was in the past used to treat people suffering from various kinds of cancer,
But during trials Progeria sufferers experienced positive results.
these results improved bone structure
better hearing, improved weight
and best of all the participant’s blood vessels became more flexible.
The reason this is very important is that
stiffness of the arteries is one of the reasons for heart attack or stroke.
Twenty-eight young people from 16 countries, all with Progeria,
take part in the trail that lasted two and a half years.
One of the doctors leading the trial said,
“ When we started this clinical trial
we had no idea whether any aspect of Progeria would be reversible,
because no one had ever conducted a clinical treatment trial for Progeria before.”
His aim of course is to give people diagnosed
with the condition the chance to live as long as anyone else
This is not a cure, however,
and if you read journals featuring Progeria you can see that
scientists are still busy trying to stop this abnormal protein
that advances ageing from doing its damage.
We are told that there is just no way to tell
if anyone will have this irregular mutation of genes
as it doesn’t seem to occur in families that have had any history of the condition.
It just seems to happen, albeit as we have said, not often.
How do you think you’d deal with living with this condition?
Have you been watching the people we’ve featured, either online or on TV?
What do you think about them?
Tell us in the comments.
Also be sure to check out our other show what is Stoneman
Thanks for watching,
and as always,don’t forget to like, share and subscribe.
Imagine if you aged incredibly fast,