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 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, and unfortunately may experience all
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 had 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 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,
such as 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, 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.
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.
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.
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 ways.
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 where 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.
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 were 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, took part in the trial
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 Stone Man Syndrome?.
Thanks for watching, and as always, don’t forget to like, share and subscribe.
See you next time.