By Josh Saunders
A skeletal teen with a rare condition is unable to eat without agonising pain – while a hug could tear her skin to the muscle.
Madisyn Yuhas, 18, from Elizabethtown in Pennsylvania, USA, weighs a slight 6st (84lb) because chewing causes painful blisters in her mouth.
She has Recessive Dystrophic Epidermolysis Bullosa (RDEB) – a rare genetic condition that means her skin is as fragile as a butterfly’s wing and can tear off with the lightest friction.
Affectionate actions, like a hug or a cuddle, could shred the teen’s skin down to the muscle, which means she must be covered in bandages from the neck down.
People with the condition are recommended to consume more calories due to their body’s working tirelessly to repair skin damage and fight off deadly infection including MRSA and cellulitis.
To tackle this deficit, Madisyn is fed through a g-tube in her stomach day and night, only orally consuming foods that do not requiring chewing as they cause blisters inside her mouth.
Madisyn has a body mass index (BMI) score of 14, which according to the World Health Organisation puts her in the lowest underweight category – a healthy weight is between 18 and 25.
Mum Mary Yuhas, 46, a heart specialist, said: “We’re trying to up her calorie intake to help her gain weight, but she can’t chew otherwise it will cause blisters in her mouth.
“She only weighs 6st (84lb), we have to feed her through a g-tube and so she just eats pleasure foods through her mouth.
“The pain means she eats very little and requires nightly liquid feedings through the tube, if we can’t get fluids or meds into her she has to be admitted to hospital.
“The calories are vital to helping her fight off infection and keeping her body from shutting down.
“There is no cure for Madisyn’s condition so every day is a struggle for her.
“She needs the calories because any time she brushes up against something, if she opened a door, slipped on her shoes or even moved in bed she could tear her skin.
“Once at school when she was standing online, someone stepped on her foot and it ripped the hole top layer of her skin off, right down to the muscle.
“To lessen the effects, we wrap her up entirely in bandages from head to toe for protection.
“If Madisyn didn’t have the dressings on her skin, it would be horrifically painful and her skin would constantly tear right down to the muscle.”
Doctors realised Madisyn had a rare form of epidermolysis bullosa (EB) when she was born without skin from her knee down.
The condition that affects one in 20,000 people – means that her skin must be covered in bandages to shield her from any potential damage as well as helping her to heal.
Mary said: “We bandage her from the tailbone to the back of her neck, her feet, occasionally we can leave her arms and hands unwrapped, but she’s pretty much always covered up.
“The protective bandages minimise skin tears, which puts her at a greater risk of infection, even despite this she’s had cellulitis several times and currently she has MRSA.
“The bandages also help to absorb the fluid from wounds, heals them and keeps them dry.”
The size of her wounds can range from a small tear to blisters the size of a baseball and often leaves her whole back raw.
Mary added: “She has a lot of smaller wounds, they differ in size sometimes covering her entire back to blisters the size of baseballs.
“If we don’t pierce them and remove the fluid they can swell even bigger.
“She has these painful blisters and skin tears all over her body and blisters internally as well.”
To combat the effects of the condition, she has a daily bath that can last for up to four hours and has blisters on her skin lanced to prevent infection.
Mary said: “Every other day we give her a bath with bleach in the water to keep the infection down, after that we do the typical limb dressings and treat anything new that’s came up.
“We lance the wounds to make sure they don’t grow, after taking out the fluid we give her antibiotics, and wrap her up.”
Over the years, Madisyn’s condition has progressively worsened.
Up until her early teens she was a dancer but has recently had to quit due to the extreme damage to her feet and is now predominantly confined to a wheelchair.
Madisyn said: “I have people who look after me, I have nurses and then a home care teacher that comes three days a week, they are very understanding.
“It’s really hard because there are days when I just want to sleep, but want to get up and go to school.
“I do really enjoy dancing and school though.”
Her mum added: “She used to do tap dancing, ballet and jazz, she had incredible rhythm and caught on really quickly but now her feet are too bad for it.
“She has to use a wheelchair as she can’t walk long distances, she can only manage just over a street before needing to stop.
“Because of the issues with her feet and hands she can’t do a lot for herself, there’s no independence, she even has to have help going to the bathroom.
“You wouldn’t wish this disease on anybody.”
Madisyn’s family are fundraising to help meet the cost of necessities such as bandages, medication and lotions, all of which keep her alive.
To donate visit: www.gofundme.com/d2jdanng?ssid=840488768&pos=25
Recessive Dystrophic Epidermolysis Bullosa is a fatal skin blistering condition that means sufferers struggle with skin loss from the gentlest friction or graze.
The condition is caused by a lack of collagen protein in the skin.
Brett Kopelan, Executive Director of debra of America, said: “Wound healing takes up a lot of calories.
“There is actually a formula for how many calories one should receive, it takes into account how much body surface is wounded.
“In general though, an EB kid should take in 150% of the calories of someone of similar age – so if a 12 year old needs 1,000 calories, and EB child of the same ago would need 1,500 at minimum.
“Nutrition is the basis of everything – a person could suffer with growth retardation, malnutrition, the inability to heal wounds and all of these things from EB
Some epidermolysis bullosa sufferers face the added difficulty that skin can tear in the mouths and throats from consuming solid foods.
Brett added: “With certain types of EB, food may get stuck to the bottom of the mouth or the scarring within the mouth can prevent them from being able to manipulate food side to side.
“Dental problems are a big problem as well as erosion of the cheeks and gums, which means they can’t chew as easily and causes difficulty swallowing.
“A lot of people with EB go for blended food diets, a g-tube or formula feedings with specialized amino acids.
“All of this makes meal times incredibly difficult, it’s a slow and long process, we tease my daughter that she eats like a 105-year-old.”
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