Monday, June 6, 2016

Little boy covered in scales must have them scraped off his body twice a day to save his life

When Evan was born his skin was so thick he couldn't open his eyes for the first two weeks. He spent two months in newborn ICU
A five-year-old boy is battling an agonising and potentially lethal condition that causes his skin to grow at ten times the normal rate.
Evan Fasciano, from Goshen, Connecticut, was born with Harlequin Ichthyosis, a genetic disorder that leaves him with scales across his entire body.
Suffering from the severest form of the disease, Evan is at constant risk of infection - requiring him to have two baths a day so his 33-year-old mother De De can scrub off the scales.
She said: 'We're very strict with Evan's skin management, so we try to keep him scale-free, and because of that his scales tend to be very thin, so they're easier to get off.
'If we didn't bathe him twice a day then the scales would get much thicker, much faster and it would be harder to get off.
Evan Fasciano, now five, was born with Harlequin Ichthyosis, a genetic disorder that leaves him with scales across his entire body. His parents try and keep him scale free to minimise his discomfort 
Evan Fasciano, now five, was born with Harlequin Ichthyosis, a genetic disorder that leaves him with scales across his entire body. His parents try and keep him scale free to minimise his discomfort 
Suffering from the severest form of the disease, Evan is at constant risk of infection - requiring him to have two baths a day so his 33-year-old mother De De can scrub off the scales
Suffering from the severest form of the disease, Evan is at constant risk of infection - requiring him to have two baths a day so his 33-year-old mother De De can scrub off the scales
'I can use my hands to remove most of it and then we'll use a wash cloth or exfoliating cloth - his hands and his scalp are the thickest so that's where it takes a little bit more effort.'
And once his bath is done, Mrs Fasciano has to use a net to scoop out the large volume of excess skin because of concerns that the skin and Aquaphor ointment residue could cause issues to the house plumbing.
After bath time, either she or her husband Joe covers Evan in Aquaphor to moisturise and protect his skin from getting too dry, with a second prescription emollient used for areas of particular concern.
Mrs Fasciano said: 'He needs to be coated with an emollient to keep his skin moist and protect it from cracking or ripping. 
'He's usually very giggly and happy when he gets Aquaphor.'
Because his skin dries out so quickly, Evan is also constantly at risk of dehydration, which can potentially cause seizures and sunstroke.
His mother said: 'Overheating is a very scary health complication. He can't sweat so he can overheat very easily.
'We're constantly pushing fluids and food as much as we can just to keep him up on the calorie intake.'
When Evan was born his skin was so thick he couldn't open his eyes for the first two weeks. He spent two months in newborn ICU
When Evan was born his skin was so thick he couldn't open his eyes for the first two weeks. He spent two months in newborn ICU
'During bath time, I use my hands to remove most of the scales on Evan's skin- and then we'll use a wash cloth or exfoliating cloth,' explained his mother, De De
'During bath time, I use my hands to remove most of the scales on Evan's skin- and then we'll use a wash cloth or exfoliating cloth,' explained his mother, De De
The skin's exceptionally fast growth rate means Evan's body burns calories at a much quicker rate than normal.
The skin around Evan's eyes is so tight, it can be difficult to close his eyes without careful attention.
Mrs Fasciano said: 'He rarely complains about anything, I don't know if I could say the same if I was in his condition.'

WHAT IS HARLEQUIN ICHTHYOSIS? 

Harlequin Ichthyosis is extremely rare, and comes out of the blue.
The parents of an affected baby are carriers and will have a one in four risk of any baby being affected.
The condition causes the skin to grow seven times faster than normal.
The skin appears tight, with thick plates of hard scales resembling armour plating or the harlequin suit of a jester.
The skin splits at several areas causing deep cracks. And a sufferer's face looks stretched with turned out lips and eyelids, and the ears, hands and feet may be hidden by scales.
The extra skin needs to be removed constantly and ointment is also applied four or five times-a-day.
Children who survive will need ongoing intensive skin treatment with creams and courses of retinoid medicine and many require physiotherapy and counselling.
Source: The Ichthyosis Support Group 
Evan also has issues with his motor skills, meaning he has to be strapped into a special walking frame to move around.
However, his younger brother, 19-month-old Vincenzo, doesn't suffer from the same condition and recently learnt to walk.
'When his little brother started walking he said 'good job'. He was very proud of him and never seemed to be jealous,' Mrs Fasciano said.
Delivered nine weeks early by emergency Caesarean, because of the severity of his condition Evan wasn't able to leave hospital for a couple of months. 
Mr and Mrs Fasciano said they first learnt of Evan's diagnosis just two days before he was born.
Joe said: 'When Evan was first born he didn't look like a baby you would picture. 
'Obviously, his skin was very thick and he didn't actually open his eyes for the first couple of weeks. He spent two months in newborn ICU.'
Doctors warned the Fascianos that many babies born with Harlequin Ichthyosis may not survive if they contract an infection.
His mother said: 'I remember looking at him and it was just a relief to see his tummy going up and down.'
The name Evan means 'young warrior' and Evan's parents believe it's the perfect fit for their brave son.
Mr Fasciano said: 'We both knew he was going to be a strong, little guy and that he would make it through anything.'
Currently there is no cure for Harlequin Ichthyosis, but in spite of everything Evan endures, he remains a lively, happy child, who is incredibly popular at school.
 After bath time, either she or her husband Joe covers Evan in Aquaphor to moisturise and protect his skin from getting too dry, with a second prescription emollient used for areas of particular concern.
 After bath time, either she or her husband Joe covers Evan in Aquaphor to moisturise and protect his skin from getting too dry, with a second prescription emollient used for areas of particular concern.
Evan with his younger brother, Vincenzo. Their father said: 'Some people stare at Evan - and people who don't know him will do a double take, so we're encouraging him to make sure he says hello to everyone who does'
Evan with his younger brother, Vincenzo. Their father said: 'Some people stare at Evan - and people who don't know him will do a double take, so we're encouraging him to make sure he says hello to everyone who does'
His mother has gone into Evan's class to teach the other children about his condition and Evan is always at the front of the class to help mum out.
She said: 'He is pretty much known as the rock star in his school. Everybody waves to him.
He literally doesn't put his hand down when he is walking in the hallway because he just has to wave and say hello to everybody, so we've been very fortunate.'
I want Evan to be somebody that people can look up to; somebody that they can say "wow he's got a lot of challenges" his appearance isn't the same but he can teach people a lot of things
Although looks and comments from strangers can hurt the family, they refuse to let it defeat them or and take away from Evan's upbeat nature.
Mr Fasciano said : 'There have been incidents when people have made bad comments and you do your best to stay positive.'
On one occasion, while out shopping, a stranger stopped  and accused her of letting her child get sunburnt. 
On another they mocked Evan, calling him a lobster.
Mr Fasciano: 'Sure people stare at Evan. People that don't know him will do a double take so we're encouraging him to make sure that he says hello to everyone who does.
'I want Evan to be somebody that people can look up to; somebody that they can say 'wow he's got a lot of challenges, his appearance isn't the same but he can teach people a lot of things.' 

No comments:

Post a Comment

DONATE