A nine-year-old girl could die at any moment battling the deadly scaling condition that causes her to shed skin all over her body.
Maddie Hoffman, from Burleson, Texas, has been confused for a burns survivor by cruel strangers due to the reddish appearance of her skin. She is also bullied at school for her unusual appearance and odour – caused by the disorder.
She is plagued by epidemolytic ichthyosis – meaning her skin can grow up to ten times faster than normal, peels and becomes so dry she can’t move.
Each day she undergoes a two-hour routine to remove dead skin cells and protect her from catching a life-threatening infection. As a child her skin was very flaky and raw, but it is believed the treatment has improved her condition.
Her mother, Emily, 42, was told her daughter may not survive after she was born – but the daily scrubbing has helped to keep her alive.
She wasn’t allowed to hold her until she was seven days old in an isolation room – and she had to wear gloves and a mask.
Maddie Hoffman, from Burleson, Texas, is often confused for a burns survivor by cruel strangers due to the reddish appearance of her skin (pictured as a baby)
Ms Hoffman, a full-time carer, 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.’
Her mother’s concerns
She added: ‘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.
‘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.
‘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.
‘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.
She is also bullied at school for her unusual appearance and odour – caused by epidemolytic ichthyosis – meaning her skin can grow up to ten times faster than normal
After being born prematurely at 33-weeks, Maddie was diagnosed with epidemolytic ichthyosis – a condition that affects one in 200,000 people.
She was immediately taken to intensive care where doctors put her into isolation to avoid her contracting a deadly infection.
Maddie’s skin in its entirety started falling off in the hospital bed sheets. ‘She looked like a burns victim,’ Ms Hoffman added.
Tiresome daily routines
After slightly less than a month, she was released from hospital and Ms Hoffman had to adapt quickly to her daughter’s extensive skin routine.
Despite Ms Hoffman having ichthyosis herself, Maddie’s is a much more severe form and requires a rigorous two-to-three-hour bathing and moisturising ritual.
Ms Hoffman 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.
Each day she undergoes a two-hour routine to remove dead skin cells and protect her from catching a life-threatening infection
Her mother, Emily, 42, was told her daughter may not survive after she was born – but the daily scrubbing has helped to keep her alive (pictured together recently)
‘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 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
Emily Hoffman, 42
At risk of infections
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.
Ms Hoffman added: ‘Everything we take for granted cannot be done by Maddie without a lot of practice or determination.
‘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.’
After being born prematurely at 33-weeks, Maddie was diagnosed with epidemolytic ichthyosis – a condition that affects one in 200,000 people
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
Ms Hoffman is determined to inform others about ichthyosis in the hope of reducing the stigma surrounding the condition and to hope someday a cure is found.
Bullied at school
She said: ‘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 wants to help children in general to stay motivated and positive, we try to teach others that it’s okay to be different.
‘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.’
Dreams for the future
Maddie, who is a Make-A-Wish kid, has dreams of dancing with Ellen DeGeneres to raise awareness of her condition.
Ms Hoffman, a single mother, is fundraising for her daughter’s care, they have been rejected disability allowance.
She said: ‘As a single mom without a lot of support for her condition and other children to take care of too, it’s very hard for us, ichthyosis is very financially draining and a strain.
‘But we try to stay as positive as we can and take each day as it comes, in the hope one day there will be a cure.’
To donate visit: www.youcaring.com/emilyprestonmaddieandkinlee-881990
Ms Hoffman wasn’t allowed to hold her until she was seven days old in an isolation room – and she had to wear gloves and a mask
Ms Hoffman, who is single, said: ‘She has one of the most severe forms of the condition’ (pictured with her sister Kinlee, four)
Maddie, who is a Make-A-Wish kid, has dreams of dancing with Ellen DeGeneres to raise awareness of her condition