What is it like caring for a child whose fragile skin might blister and tear with every touch? One mum shares her story with Eveline Gan.
What is it like caring for a child whose fragile skin might blister and tear with every touch? One mum shares her story with Eveline Gan.
Even before Muhammad Raed (pictured) received his first cuddle or tasted breast milk, morphine – a potent painkiller – was already coursing through his tiny 2.68kg body.
At birth, little Raed’s shrill cries not only announced his arrival into the world; they also told of the unimaginable, excruciating pain he suffered due to a rare genetic skin disorder known as epidermolysis bullosa (EB).
Now approaching two years old, Raed was born with no skin on his lower limbs, back and hands.
In the first few weeks of his life, he relied on strong medication around the clock to dull the pain from his raw, open sores. Often dubbed one of the cruellest diseases a person can be afflicted with, EB is an incurable genetic disorder that causes the skin to tear and blister at the slightest trauma.
Little ones like Raed are often known as “butterfly children” because of their extremely fragile skin, which tears as easily as butterfly wings do at the slightest friction. Raed’s condition is considered a severe type of EB, known as junctional EB.
Lifting him by the armpits can lead to blisters in the area, friction from a quick flip of his hand may tear off skin, while simple everyday items like clothing labels could lead to sores.
A living hell
“The pain children with severe EB endure every single day makes life a living hell,” says Ritu Jain, president of Debra Singapore, which is part of a worldwide network of EB patient-support groups. “We’ve seen some children who are highly dependent on morphine to help them cope with the pain.”
Raed’s parents did not know they were both “carriers” of the recessive EB gene until he was born.
Nurzeehan Abdul Halim shares that her son had seemed perfectly healthy during pregnancy scans. But she soon realised something was amiss when the nurse did not hand him to her immediately after he was delivered.
“The doctor said it might be better for me not to see him yet. Initially, I thought he was born handicapped,” recalls Zeehan, 26, a nurse.
At first glance, Raed had appeared fine to his mum as most of his body was covered by a piece of cloth.
His exposed feet, however, told a different story. Where a protective layer of skin ought to have been, there was raw flesh.
Despite the shocking image, the stoic mum says that her nursing background enabled her to remain calm.
“Not once have I been in denial about my son’s condition. Because of my profession, I am able to accept it and try to find a way to move on,” adds Zeehan, who got to hold her baby only a month later.
Raed’s condition makes it impossible for anyone to give him a hearty hug or embrace him tightly; the pressure from those intimate, loving touches may risk painful skin tears and blisters.
Only four people – his parents, Grandma, and the domestic helper – are permitted to carry him using techniques that do not apply too much pressure on his skin.
“Up to about seven months, we were still carrying him like a newborn baby – in the cradle position. We couldn’t simply just lift or hold him the way most parents do with their babies,” shares Zeehan.
“Until now, we don’t allow friends or relatives to carry him because we can’t afford to take the risk.”
The financial and emotional toll
While Zeehan’s nursing background has helped her accept and tackle Raed’s condition more readily, it doesn’t make things easier.
For the first-time mum, “horrible” doesn’t even begin to describe the insurmountable challenges she faced in the first year of Raed’s life.
For one, EB is a rare disease which has no standard treatment or cure. The focus is often on troubleshooting symptoms, pain or infections whenever they crop up, february 2017 / 27 / young parents so his parents are constantly on their toes watching out for new wounds or any signs of infection, which can be potentially deadly.
Especially important is proper wound care, which typically involves an elaborate, time-consuming routine and is financially draining.
In the first 10 months of Raed’s life, his parents spent approximately $3,000 each month on his wound dressings, not counting the medical bills they chalked up whenever he came down with a bug or had to be hospitalised.
“One roll of dressing costs $88 and lasts only three days. You can’t even bring these products in from the US, where they are considerably cheaper, because they are considered medical supplies,” says Zeehan. These outpatient costs are not Medisave-claimable.
She also had to cope with extreme physical exhaustion from caring for Raed. After juggling nine-hour work shifts at the hospital, Zeehan would return home and devote four to five hours each day to bathe him and dress his wounds. In the day, Raed is cared for by his grandmother and helper.
“The whole process takes so long because you can’t rush it; you have to remove his dressings very slowly using forceps. You can’t even towel-dry him normally because any vigorous rubbing would tear his skin,” she shares. “Every movement has to be slow and gentle.” Bath times were particularly traumatising for Raed then, because he would “scream in pain” when the water touched his body.
“It really hurts to see your child in pain like that,” says Zeehan.
Morphine around the clock
To help Raed cope with the pain, he was prescribed morphine – to be taken around the clock – after his discharge from the hospital.
Those with severe forms of EB experience such high levels of pain that they usually do not respond to conventional painkillers, and may require strong longterm pain medication like morphine, shares Dr Priya Bishnoi, a clinical associate at KK Women’s and Children’s Hospital (KKH)’s Dermatology Service.
But pumping morphine into her son’s body continuously wasn’t something Zeehan could accept.
Instead, she did her own research and turned to alternative techniques like aromatherapy, Ayurvedic medicine and supplements, in an attempt to ease his pain levels and boost his immunity.
“EB is a lifelong disease and I do not want him to become too dependent on morphine. But I gave him a dose once a day before bath time as I knew it would be very painful and traumatising for him if I hadn’t,” says Zeehan, who weaned him off the drug by the time he was 2½ months old.
Things began to look up by Raed’s first birthday when the skin on his lower limbs eventually formed, a stage which Zeehan never thought he would ever reach given the devastating state his skin was at birth.
The toddler is now able to walk with some assistance, which is an achievement considering the fact that he had to skip the usual motor developmental stages, such as flipping and crawling during the first year of life.
Hurtful remarks the biggest hurdle
Among all the obstacles she has faced, Zeehan shares that her toughest hurdle to date is the lack of public understanding and awareness of the disease.
“Some doctors don’t even know of it,” she says. “What was particularly difficult were the negative societal perception and hurtful remarks.”
Strangers sometimes make disparaging comments or say “yuck” when they catch a glimpse of Raed’s blisters or raw wounds.
“These comments are mostly made by adults,” she says. “Initially, I was angered very often by their insensitivity, but I’ve learnt to live with it. Now, I try to return their unkind remarks with a smile.”
That is mainly the reason why Zeehan aims to parent her son in a way that would hone his confidence and self-acceptance.
She also tries not to let his condition get in the way of a fun-filled childhood. For instance, they attend family functions and have gone on overseas trips now that his condition is more manageable.
“We take along additional luggage to hold all his dressings, and he sits on our laps during the flight. I feel it is important for us to build a healthy family despite his special needs, and for Raed to experience (a happy childhood),” says Zeehan.
Looking at the bubbly and easygoing toddler now, his mother certainly seems to be right on track with her parenting style.
“He’s a very cheerful boy,” she says with a smile. “I hope that he will grow up and become a positive and resilient person who is able to adapt to his condition, and not say: ‘Why me?’”
Like all parents with EB children, Zeehan hopes that a cure might be available one day. But right now, all she yearns for is a more understanding and compassion society that will show people living with EB some kindness.
“Every individual should learn not to be so quick to make negative remarks. By offering a warm smile, you can take away some pain from the child. We all need to show that bit of humanity,” she says.
What is epidermolysis bullosa?
Imagine skin so fragile that just going down a slide, being hugged, walking several steps or wielding a pencil can cause blisters or open sores that feel like third-degree burns.
That is the horrifying reality a child with a severe form of the rare, inherited disorder – epidermolysis bullosa (EB) – faces every day.
About 15 in every one million births worldwide are affected by the genetic disorder, according to Dr Mark Koh, head and consultant at the Dermatology Service of KK Women’s and Children’s Hospital (KKH).
In Singapore, about 30 to 40 families have children who are affected by the incurable condition.
The skin has an outer and inner layer. In healthy skin, both layers are held together by “anchors”. Children born with EB lack these anchors, so any friction can create blisters and painful sores, according to Debra International’s website (www.debra-international.org). It is a worldwide network of EB patient-support groups.
The condition can be inherited from a parent with EB, or both parents who carry the gene without any symptoms.
With proper care, the majority of children with EB living in Singapore are able to work and start a family. But about one in 10 have such severe forms of the disease that they experience extensive skin blistering, painful ulceration and scarring throughout their lives, says Dr Koh, who is the medical adviser to Debra Singapore.
“This can be very painful, and the repeated blistering and scarring may even lead to amputation of fingers or toes. In severe cases of EB, babies commonly die during the first few weeks or months of life, mostly from complications or skin infections,” he adds.
To find out more, visit www.debrasingapore.org, or e-mail debrasingapore@gmail.com.
MAIN PHOTO ZAPHS ZHANG / ART DIRECTION EILANE WANG.
