Little girl covered in painful SCALES defies docs to live a normal life after mum was told she ‘may not survive’
Maddie's skin condition causes her skin to grow ten times faster than normal and become so dry she cannot move
A LITTLE girl with a painful condition that causes her skin to crack and shed has defied doctors who said she “may not survive”.
Maddie Hoffman, nine, was born with a rare scaly skin condition that causes it to grow ten times faster than normal.
She has to be bathed for two hours a day to remove dead skin cells from her body to reduce the chances of her developing life-threatening infections.
Maddie, from Texas, USA, has epidermolytic ichthyosis, which causes her skin to overgrow and peel.
It can become so dry she is unable to move.
Mum-of-two Emily, 42, was told her daughter may not survive after she was born due to her high risk of infection but since then the little girl has beaten the odds.
Thanks to the daily scrubbing off of Maddie’s scales, moisturising and treating wounds she’s defied expectations and lives a normal life.
Emily, full-time career, said: “It was visible at birth that she had ichthyosis and shortly after that all the layers of skin fell off in sheets, she was bright red and looked like a burn victim.
“The ER doctor told us, ‘You realise your baby may not survive?’ it was very scary she was touch and go for the first two weeks.
“Ichthyosis can be deadly when a baby is first born, Maddie needed to remain hydrated, stay infection free and quarantined, we even had to put on gloves to touch her.
“She has one of the most severe forms of the condition, requiring aggressive and painful treatment administered for up to three-hours every day.
“The build-up of scaling is her worst enemy, the more it layers up the more likely she is to have skin infections, can’t bend or move.
“Each hour that goes by, her skin gets harder for her to move, she gets drier and starts to flake-off.”
Maddie’s skin can become so red she looks like a burns victim.
“There are days where she can barely move, the most affected part is her feet, any friction causes blistering, even socks and shoes affect her,” Emily added.
“We have a daily battle with her blisters and her not being able to walk, these are open wounds so we have to work tirelessly to stop them from getting infected.
“If she has an infection on her feet, time can be critical to get it curated, she has been hospitalised for severe infections and the most dangerous type of MRSA [bacteria that is resistant to antibiotics].
“If it’s not dealt with immediately with antibiotics, the infection could get into her bloodstream and she could lose limbs or die from this.
“But in spite of everything Maddie’s going through she is an extremely happy and positive girl who loves to help others and is determined to raise awareness.”
A PAINFUL CONDITION THAT CAUSES THE SKIN TO CRACK AND PEEL
Epidermolytic ichthyosis is a rare genetic condition that causes the skin to overgrow.
The skin cells are produced at a normal rate, but they do not separate normally at the surface of the outermost layer of skin and are not shed as quickly as they should be.
The result of this retention is the formation of scales.
Symptoms:
It is characterised by thick, blistering, warty hardening of the skin over most of the body.
Scales tend to form in parallel rows of spines or ridges.
The skin may be fragile and may blister easily following injury.
Babies are usually born with red, blistering, and denuded skin with visible areas of skin thickening.
Overtime there is usually a decrease in blistering but the scales will become thicker and more severe.
The exact cause of the condition is not known.
Treatment:
Treatment for the condition can be tricky.
It can be treated topically with skin barrier repair formulas containing ceramides or cholesterol, moisturizers with petrolatum or lanolin.
It can also be treated systemically with oral synthetic retinoids.
Retinoids are only used in severe cases of lamellar ichthyosis due to their known bone toxicity and other complications.
There is no known cure, but several clinical studies are active around the world.
Maddie was born early at 33 weeks.
She was rushed to intensive care where she was put into isolation to avoid her contracting a deadly infection.
Emily said: “She had a collodion membrane, which is a very thick white skin, you could tell it was something very different from a normal birth.
“Immediately after that, all of her skin in its entirety started falling off in sheets, she looked like a burn victim.
“I wasn’t allowed to hold her until she was seven days old in an isolation room, no one was allowed in there but me and immediate family using gloves and masks.”
After a month, she was released from hospital and her mum had to adapt quickly to her daughter’s extensive skin routine.
Despite Emily having ichthyosis herself, Maddie’s is a much more severe form and requires a rigorous two-to-three-hour bathing and moisturising ritual.
Emily said: “Before her routine the skin is white, very scaly, extremely dry and itches a lot.
“Without the routine, she wouldn’t be able to walk, the skin build-up would be very thick, the odour would be bad and she could die from infection.
“One day in the bath we use diluted bleach and the next baking soda, it helps soften and soothe her skin, it’s very calming, helps with itching and reduces the build-up of scaling.
“We have to get as much of her scaling off through scrubbing every morning so that she isn’t restricted as much, every hour her skin gets drier and begins to crack.”
The daily build-up of skin not only risks infection and causes a lot of pain, but also affects her ability to move which has required hours of therapy to help with.
“Everything we take for granted cannot be done by Maddie without a lot of practice or determination,” Emily said.
“Before she couldn’t open a bottle of water or barely grip a pencil because of the skin build up, so she needs weekly occupational therapy.
“Despite this she puts a brave face on in her videos because she wants to raise awareness and is a very happy child.
“Bullying is a big deal for her, she goes through a lot of stares and teasing, kids get nervous seeing her skin shedding and bully her about her odour caused by the skin build-up.
“It’s hard to watch as her mother, seeing people stare at her and look with disgust, it’s very disheartening which is why we try to speak out.”
Maddie, who is a Make-A-Wish kid, dreams of dancing with Ellen DeGeneres to raise awareness of her condition.
Single-mum Emily is fundraising for her daughter’s care as they have been rejected disability allowance.
“Maddie’s condition is very obvious when you meet her you can see she has unhealthy skin, it’s been very challenging,” she said.
You can donate to Emily and Maddie at their
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