Special Abu Dhabi unit to treat 'butterfly child' syndrome

Epidermolysis bullosa causes terrible suffering in young children

Saleh who passed away a few months ago from a rare genetic skin disease.

Imagine suffering excruciating pain for every second of your life, where every part of your body constantly itches and tears into open wounds that never heal.

This is the reality for the "butterfly children", who have skin as fragile as a butterfly wing. Even the slightest scratch can create a blister.

The disorder is caused by epidermolysis bullosa (EB), a rare, genetic skin disorder for which there is no cure.

Those with the most severe form of the disease die before they reach the age of five, and those ith the disorder who live beyond that struggle with constant pain and irritation.

Every bathing day was a nightmare for the whole house. Imagine a small body that is mostly skinless. Yet after every bath, Saleh would try to smile and tell me, “I am done baba. It is all done now. Don’t be sad"

Jaafar Al Ameri

At present there is no cure, but doctors in the UAE are striving to improve the quality of life of children born with the condition, with one of the first comprehensive clinics in the region for EB.

Dr Shaden Abdelhadi is an Emirati paediatric dermatology consultant who established the new clinic at Sheikh Khalifa Medical City in Abu Dhabi.

“This disease is tragic and frustrating for the children and their parents. Sadly, it is not rewarding or appealing for physicians," said Dr Abdelhadi, who specialises in human genetic skin disorders.

"It requires a lot of attention, knowledge, an open heart, and a passion for dealing with it.

“Nobody in the world can cure this disease at present, but we have many examples where proper care and management can give the children hope, and support their quality of life."

There are no happy endings for children with EB, so pain management is a priority, because the dressings on their wounds have to be changed every few hours to prevent infection.

Saleh Al Ameri died this year after complications resulting from the disease

Ghaith, 11, has not let his condition get in the way of him living his life like any other child.

The young boy, whose body is covered in open sores, goes to a government school in Abu Dhabi, plays with friends and enjoys playing computer games.

His father, Adel Hassan, said: “I’ve tried my best to make him live as normal a life as possible.”

Gaith’s mother tries to hide her tears every time she changes the gauze covering his wounds – an exercise that causes him to scream in agony.

“We know that he is in pain, but there is nothing we can do about it," she said. "If the dressings are not regularly changed and cleaned then the infection will get worse."

“I know that children with EB don’t live long, but I have faith in God,” the father of three said.

The severity of the disease varies – some patients live for many years – and those with an acute case must be covered entirely in protective and antibacterial dressing, and may have their fingers and toes fused to protect them from injury.

The disorder is notoriously difficult to treat because patients with EB suffer from many comorbidities, including dental and malnutrition problems, swallowing difficulties, eye problems, sleep disturbance, pain, and limited movement.

The new clinic is staffed by nine paediatric specialists to meet all the children's clinical needs in one convenient location, Dr Abdelhadi said.

"All that I want for these children is to bring them adequate attention, reduce their pain and discomfort, and make their lives as comfortable as possible.

"We work in aseptic conditions together with a team of highly qualified wound nurses. We also provide training to homecare nurses, other caregivers, and family members.

"We stand hand in hand with affected patients and their families throughout their EB journey."

EB is an inherited disorder and cases commonly occur in the same family. This can be prevented, Dr Abdelhadi said.

"We provide parents with detailed genetic counselling and encourage them and other family members to take all the necessary measures to eliminate the re-occurrence of risk in future offspring,” she said.

A parent's pain

Five months after losing his seven-year-old son to EB, Jaafar Al Ameri can still hear his son Saleh’s screams.

“This was one of the most difficult things that I have experienced in my life. I would not wish it on anyone," he said.

"It is difficult to helplessly stand by and watch your son suffer knowing full well that there was nothing you could do about it.”

It is customary for fathers to be called after their first-born sons. Mr Al Ameri, 40, has five other sons and four daughters – Saleh was his middle child.

Nevertheless, he insists on being called Abu Saleh, or father of Saleh.

“I will always be called Abu Saleh. I have never loved anyone or been attached to anyone as much as I was attached to Saleh,” he said.

The gauze and wound dressings of patients with EB have to be changed at least twice a day and they have to be bathed at least every two to three days.

The baths and dressing changes take three hours and are incredibly painful.

This disease is tragic and frustrating for the children and their parents. Sadly, it is not rewarding or appealing for physicians. It requires a lot of attention, knowledge, an open heart, and a passion for dealing with it

Dr Shaden Abdelhadi,
Sheikh Khalifa Medical City

Every parent of a child with EB dreads those moments, but they are necessary to prevent infection.

There is nothing that can stop the pain and children with EB can take only over-the-counter pain medication.

“Every bathing day was a nightmare for the whole house. Imagine a small body that is mostly skinless. The baths were the true meaning of suffering for him and everyone around,” Mr Al Ameri said.

“Yet after every change and bath, Saleh would look at me and try to smile and tell me, 'I am done baba. It is all done now. Don’t be sad'.

"I have never seen anyone braver and with more courage than him,” the Emirati father said.

Saleh died in January from sepsis resulting from all his wounds.

“When I carried his small body to wash him there was nothing except bones and blood. He had no skin. I didn’t know how to wash him,” his father said.

"If I would like to see anything come out of this experience, I would suggest a specialised centre.

“This experience was very, very hard. You wouldn’t want to see your child suffering every second of the day like we did. Please get tested before deciding to have a child if you’ve had any cases of EB in the family."