Progeria is a very rare disease. The family of 12-year-old Miles shares how they deal with their son's death sentence. Miles himself is an ordinary teenager, even if he looks unusual. He refuses to think about death and prefers to live in the present. Looking at him, others learned to stop postponing everything for later, because even a healthy person does not know when he will die.
Miles is in sixth grade, but he already has the body of an old man. Despite all the difficulties and an uncertain future, his parents feel that they are lucky.
“It’s very easy to close in our cocoon and start feeling sorry for ourselves, but in fact, we are incredibly lucky,” says Miles's father Jakob Wernerman.
Miles Wernerman sits cross-legged on the couch googling his name.
"Nyhetsmorgon, 318 thousand. Malou, over a million."
He smiles in satisfaction from under the visor of his cap, his video has even more views than it did the last time he watched.
The first time I met Miles was in 2012 when he rode a tricycle at an amusement park in Stockholm's Södermalm. He just moved to Sweden and started first grade. He climbed to the very top of all the children's climbing frames and refused to take off the Spiderman cap. His new homeroom teacher agreed to make an exception for him and to deviate from the headwear rules because Miles hates being the only one in class who has no hair.
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That was six years ago. Since then, Miles's body has aged 48 years.
There are about a hundred known cases of Progeria worldwide, and Miles is one of those children. This means that he is aging eight times faster than an ordinary person. The average life expectancy of such people is 13.5 years.
It's time for an afternoon snack. Miles and his younger brother and sister sit down in the kitchen for tea and honey and begin to argue which artists and YouTube stars are cool and which are boring.
“I can't drink tea anymore,” Miles says and pauses theatrically as he looks around the table. "Because otherwise I will overheat!"
The brother and sister laugh. Miles kicks, in sheepskin slippers, on the high chair he's sitting on. He has absolutely no fat on his body, including in his feet, so it hurts him to walk without soft soles. Other consequences of the disease: he has early typical age-related ailments, no hair, and he is very small - with 110 centimeters in height, weighs 14 kilograms. According to him, this is the worst. They don't make cool clothes and sneakers for his size. He is 12 years old and does not want to wear clothes from the children's department, with trucks and dinosaurs on his chest.
I ask if it is strange to him that all his friends are growing so fast now.
Well no. I am with them almost every day. So I don’t notice that they are growing and all that stuff.”
He will officially become a teenager next year.
“Sometimes we forget how old he is,” says his mom, Leah Richardson. - He's so small. But then his friends come to visit, and they are so big! Just gigantic, almost adult men!"
"The worst period in my life." This is how she describes the time when they learned the diagnosis. Miles seemed healthy when he was born in Italy, where Lea and Jacob lived and worked at the UN. Three months later, doctors tested him for this incredibly rare disease. All results were negative and the family was able to breathe a sigh of relief. But when Miles was two, he was examined again. The family had already lived in New York, and now the result was different: Progeria.
“It was a death sentence. What about when your child is sentenced to death?"
Leia tells how the world collapsed for her over and over again every morning. In the early years, Miles talked about Jacob's illness, and she tried her best to make every second of her child's life as fulfilling as possible, and suffered a conscience when she was angry or when Miles was bored or sad.
“It’s very easy to hide in your hole and grieve there when you find yourself in a situation like ours,” says Jacob, and Lea adds that this is what she did for many years.
But over time, they somehow learned to accept it. And today they are even grateful for the realization that Miles gave them: life is finite.
"Will Miles have time to go to university?"
The whole family got into the car, and Miles' seven-year-old brother asked this question. And he clarified:
"Will Miles have time to go to university before he dies?"
“Probably,” Lea replied.
Miles considered. Then he remembered his senior progeria comrade: "Sam is 20, and he goes to university."
No more talk about it.
Once a year there is a meeting of European "families with progeria", this October it was held in Portugal. Miles has a photo of the entire party on his desk and shows his best friends - two older boys with Progeria from Belgium and England. They love to meet, because then they find themselves in an environment where their condition is the norm. But it's always sad to say goodbye. It is not known which of them will still be alive next time.
The fact that the average life expectancy of children with progeria is 13.5 years, Miles usually does not think or talk, even with his parents. Like many other twelve-year-olds, he is fully occupied with what is happening here and now. For example, playing football and night gatherings with friends. Or skirmishes with brother and sister. Or homework. Or watching funny videos on YouTube.
But every day you have to take medications: slowing down the course of the disease, lowering cholesterol, thinning the blood, as well as heart drugs. At Astrid Lindgren Children's Hospital, Miles goes to see doctors who treat his skin, eyes, joints and heart. For the most part, it is about preventing the typical age-related ailments like stroke and heart attack, from which children with Progeria usually die.
Svenska Dagbladet: What would you most like to do?
Miles lay down on a sheep's skin on the floor, he just told us that the stuffed toys that are there too are old.
“Probably … a computer! My own stationary computer with everything I need, which will stand in my room. It would be cool.
Now he mainly plays with the console. He says that he is allowed to "gamble" on Tuesdays, Fridays, Saturdays and Sundays.
"It's best on Saturday, then I can play in the morning and in the evening."
A puppy is also on the list of Christmas wishes. But for now, he has to be content with a stick insect. She and her younger sister Clementine each have a stick insect: one is called George W. Bush, the other is Annie Lööf.
Hanging on the wall are two blue T-shirts: a light blue one signed by Messi and the other from his favorite team, Djurgården. When asked if she also has autographs, he replies:
"No, well, maybe a couple."
Smiles and turns it over, showing autographs from the entire team.
When Miles was filming Nyhetsmoron this fall, host Tilde de Paula gave him a birthday present: a ticket to the Djurgorden home mast against Gothenburg, and he was able to go out onto the VIP salon and celebrate the 2-0 victory with the players. In the locker room.
Leah asks him to tell him what football star Kevin Walker has told him, and Miles lowers his head to hide a smile.
"No, mom, you tell me."
“He thanked Miles for winning because he was their mascot,” she says, and pokes him in the side.
Not for the first time, Miles had the opportunity to do what other children only dream of. Jacob and Leia believe that it is difficult to maintain a balance: they do not want him to be spoiled, but at the same time it seems to them that "you need to take everything from life."
We ate sushi, and it's already dark outside. The children, as always, objected a little and went to do their homework, each in his own room. We sat on the couch and I asked Jacob and Leia how their attitude towards life had changed since that day in New York ten years ago. When doctors with their analyzes turned their lives upside down.
“None of us knows how long we will live, whether you have a fatal diagnosis or not. A bus might run over you one morning. So the main thing is to make the right use of the time that we can spend together, and not to postpone too much for later,”says Jacob.
He points out that Miles really lives in the here and now.
“He thinks very little of what will happen next, he is focused on living as fun as possible and doing what he likes, sometimes almost to the point of absurdity. And this kind of encourages us."
Leia chooses her words carefully. Emphasizes how difficult it really is for her to accept this way of thinking, no matter how many years she spent on it.
“Of course, everyone understands how good it is to live in the present. But it's a completely different matter when you know that there is a certain date, that you have only five or ten years."
For a long time, the main thing for her had become to make Miles' short life as complete as possible. But, besides this, she and Jacob themselves strive to live as fully as possible in order to become more cheerful people and good parents.
“This does not mean that we always shout 'yes' no matter what the kids ask, but rather we just try not to procrastinate and dream in vain. For example:' Oh, we always wanted to go to Australia, but it's so far away, so expensive, and we have three children … "And we say: 'Let's do it!""
According to Jacob, he came to the conclusion that in fact they were lucky. It could have been worse. Both he and Leia have worked extensively in developing countries, she is in disaster medicine, and he is in urgent international emergency assistance. They saw in what terrible conditions many people live.
“It may sound awful, but I say we were lucky. Of course, I myself would never wish that. But everyone has their own problems in life. Not all of them relate to life expectancy. Apart from the fact that Miles is Progeria, we are a completely ordinary, happy family. For this I am very grateful."
One day, baby, we will grow old, Oh baby we'll be old
And just think what stories
We can then tell."
Asaf Avidan's song pours from loudspeakers at a climbing wall in southern Stockholm. Miles came here straight from his algebra test, wearing a red sweatshirt and a black cap. The blue and yellow climbing shoes are several sizes too big for him, and he refuses mom's help by putting them on. This is his third session with physical rehabilitation specialist Åsa.
“This is a great way to prevent joint problems. You really figured out what the trick is, Miles, crawl like a little spider up the wall - aloud! It's great that you are physically active, otherwise my job would be much more difficult,”she says and smiles.
Miles is insured and has a bottle of water in his hand. He confidently walks to the wall, toes are slightly turned to the sides, hands on hips.
A few days later, I received a letter from Leia. She wrote that she would have to reschedule the planned photography on the football field. Miles' thighbone popped out of the joint while he was playing football at school. This is the first clear sign that his body has aged, and it's time to start a completely new course of rehabilitation. “The whole world seemed to stop. Although we knew that someday it would happen, but you are never ready for that,”Leia wrote.
Miles becomes famous, and his family sees only good in it. They want people to see him and say, "look, it's Miles with that disease," not "look how strange this man looks." He himself was used to being looked at. I think it's okay, he says, and shrugs.
“Sometimes someone comes up, most often children, and says that they saw me on TV or in the newspaper. That's cool.
"Svenska Dagbladet": Do you want to convey something to everyone who will read it?
He smiles very broadly and it is clear that he is missing one front tooth.
“Everyone should come to me and take a selfie. All! It'll be cool.
I ask the same question to his parents.
“Everyone should come and take a selfie with us,” Leia replies, and they roll with laughter.
Then she gets serious. Talking about Swedish politeness, which is sometimes inappropriate. The family goes to the amusement park, and sometimes they hear the children ask their parents "what's wrong with him?", And in response they receive boos and stern glances.
“They don't want to talk about it and explain it for the child to understand. They probably think that it would be unpleasant for us."
Jacob nods and explains that the opposite is actually true.
“This is not unpleasant, it is encouraging! It is much more annoying to hear whispering. Come, ask, take a picture - whatever!"
Before we say goodbye, I ask Miles, what would he do if he could conjure for a while? What big wishes would he fulfill? He pauses, but only for a couple of seconds. Then it shines.
"I would do so … to always conjure!"
Progeria
Progeria or Hutchinson-Guildford syndrome is an extremely rare fatal disease that is caused by premature aging and cell death. The body ages about eight times faster than healthy people. Progeria is caused by a genetic mutation that causes the prelamin A protein to malfunction. Prelamine A is needed to keep the cell membrane stable.
Symptoms begin in the first two years of life, and at about five, the baby stops growing. He loses hair and subcutaneous fat, the joints become ossified, and the skeleton becomes fragile. None of this affects intelligence.
The average life expectancy of such patients is 13.5 years, but some survive to more than 20. The most common causes of death are stroke and heart attack. Today, approximately 100 children in the world are living with this diagnosis. Miles is the only one in Sweden. The disease is not hereditary. Now there are no cures for her, her development can only be slowed down.
Werner's syndrome is a lesser known form of progeria. Because of this disease, a person's signs of aging appear earlier than would be adequate for his age.
Werner's syndrome, unlike Hutchinson-Guildford syndrome, is most often detected no earlier than 20 years of age.
Matilda Bjerlöw, Yvonne Åsell
