Weekday mornings in a house with school-going kids are usually a time of orderly (and sometimes disorderly) chaos. From the moment the first wake-up alarm rings until the last of the kids is deposited at school, it’s a constant whirl of activity as everybody gets dressed, fed and prepared to start the school and work day.
But in the Terry and Deep Mann household in Surrey, the mornings are far more hectic – and far less joyful – than in your average multi-kid home. They too have three kids – all boys – ranging in age from just four to 15, but their middle child, Brayden, 11, requires extraordinary attention every morning.
That’s because Brayden suffers from Epidermolysis Bullosa (EB), a rarest of rare medical conditions. Essentially, when you have EB, you have extremely fragile skin. The slightest friction causes blisters, ranging from relatively small ones to wounds where whole swaths of skin are simply peeled off the body.
Basically, Brayden’s parents wake up almost every morning to find their middle son injured – sometimes slightly, sometimes badly. Those who have children will understand how traumatic it is to have your child sustain an injury, especially a bad one.
To know that you’re going to find your child injured every morning, 24/7, is beyond imagination.
“Daily life is difficult, very difficult,” says Deep, Brayden’s mom. “Watching your son in pain every day is very hard.”
What makes the pain – literal for Brayden, emotional for the rest of his family – even more unbearable is the knowledge that a cure is nowhere in sight. “Not being able to do anything besides wound and pain management is really hard on all of us,” says Deep.
What is EB? – A simple explanation
Human skin has two layers, with the outer layer anchored firmly to the inner layer. However, in EB sufferers, due to a genetic mutation, the anchoring filaments are missing or damaged due to the absence of certain proteins. Thus, the outer layer of skin is essentially floating free above the inner layer – and any kind of friction peels it right off.
The blisters that result from these injuries are described by doctors as being similar to 2nd or 3rd degree burns.
There are three broad categories of EB (see chart), with progressive severity. There are numerous sub-categories within the three broad categories. EB occurs in every racial and ethnic group, and affects both genders. It is not contagious and does not affect mental acuity.
The condition occurs in one out of 17,000 people on average.
In severe forms of EB, common occurrences include open wounds, disfiguring scars, musculoskeletal deformities, internal and external blistering, malnutrition and deterioration of eyes and teeth. EB can be disabling and life-threatening.
Daily inspection of wounds is a requisite, with the high threat of infection. Bathing and bandaging are so agonizing that strong pain medication is often required.
And wound and pain management for Brayden is a much more complicated process than it would be for an ordinary person. A whole range of materials, including needles of various shapes and sizes, is required. “It takes two of us to do everything,” says Terry.
“Any kind of minor friction, any kind of slight rubbing of the skin can either peel it right off or cause blisters. He can even get it when he’s sleeping. It can happen even if the clothes he wears rub his skin, depending on the type of clothes he wears. The clothes he’s wearing right now has fleece lining inside. We just try and buy special clothing for him.
“The blisters are not like the blisters you and I get. His just grow and grow because the layers of skin don’t attach to each other,” says Deep.
But adversity often brings the best out of people, and the Mann family has adopted a businesslike attitude to the situation they find themselves in. Brayden bears his never-ending and excruciating pain with a stoic and even cheerful attitude for someone of such tender age, his parents go about their arduous daily task of cleaning up his blisters as just another part of their routine, while his brothers
Tajan, 15, and little Akshay, 4, interact with their brother as normally as they can.
But the effects of Brayden’s condition go far beyond the physical. The emotional and psychological toll is immense.
“It’s important to understand that Brayden cannot do all the things a kid of his age needs to do. He cannot play any kind of contact sports. If a soccer ball so much as brushes against his skin, it will peel off. He cannot indulge in horseplay with his brothers or his friends. To even show physical affection for him as a parent is extremely difficult,” says Deep.
She describes how traumatic she and Terry felt when Brayden was just a baby, and even carrying him often left him wounded. “And at that time, we didn’t know what the problem was, which really made the situation worse for us,” she says.
And neither is it easy for Brayden’s brothers. “I coach Tajan in soccer and hockey, and he wants nothing more than to have his brother playing with him,” says Terry.
Such are the needs of Brayden that Deep took extended leave of absence from her job a year and a half ago and is now full-time engaged not just in the care of Brayden, but in spreading awareness of EB as much as she can. And that’s because the family also has to deal with outsiders on a constant basis like any other set of parents.
Brayden goes to school, and it’s no surprise that he’s been the target of unkind treatment of all sorts due to the extensive bandages he has to wear as protection against skin contact and as a curative device.
“We had a real tough time last year because he didn’t want to be at school. He’s had issues with other children in the past. He was teased by another child where he wanted to commit suicide. He’s very self-conscious about it. We try to teach the kids at school about it, making people more aware about it. He wants to be normal and be like all the other kids,” says Deep.
She says part of the reason outsiders – kids and adults – often react to Brayden in unacceptable ways is simply a lack of awareness about EB because it’s so rare. In fact, it is so rare that it took a few years for Brayden’s condition to be accurately diagnosed despite regular visits to the doctor.
“The first year of his life, he was misdiagnosed several times. I even had a specialist tell me that Brayden’s condition was not painful at all and that he would grow out of it. And I asked him, if it was not painful, why is my child screaming? Imagine trying to pick up your little baby, and it peels his skin right off.
“We had no idea at that time about what to do to take care of him,” says Deep.
He was finally accurately diagnosed by Dr. Julie Prendivilleat of Children’s Hospital (of Vancouver), who at that time was the only specialist in western Canada that knew about this condition. But until today, knowledge about EB even among healthcare professionals is not that widespread.
Brayden and his parents are regular visitors to Children’s Hospital, and Terry describes how at one time up to a dozen residents (doctor interns) would crowd into the room whenever Brayden was attended to by a regular doctor.
“They (doctors) are also learning from us every time we go in. But it just got too much for Brayden (so many residents observing him). He felt like a guinea pig. So we requested that the number of residents watching him be kept at a less intrusive level,” he says.
Brayden also sees a psychiatrist, a psychologist, pain management personnel, an occupational therapist, even a dental specialist. “We spend a lot of time at Children’s Hospital. Up to four times a week sometimes,” says Terry.
Brayden also has to take various medications all the time to manage the pain.
“He takes regular painkillers like Tylenol and Advil. They (the doctors) are wanting to put him on morphine. Painkillers are going to be a lifelong thing for him. We are trying to avoid giving him opiates but that’s all you can do for pain management. He also takes medication for anxiety and to help him sleep,” says Deep.
In addition, all kinds of non-medical adjustments have had to be made to accommodate him. “Even the couches in the house have blankets on them just to prevent skin friction on Brayden. We had to remove the carpets because when he fell on the carpets, his skin would peel right off. We put hardwood in,” says Terry.
Which all adds up to huge expenses. Fortunately, Brayden’s medical and preventative needs are now covered by the government.
“We were rejected for coverage for about seven or eight years. We had to buy his supplies on our own. But then, a new nurse came to his case and she took it upon herself to press his case for coverage. Subsequently, BC’s home care program reread the file, they sent another person to come and look at him, and finally a few years ago, they started to cover the cost of all his medical supplies,” says Deep.
Terry also has extended care benefits at work.
Yet, even with the medical coverage, EB keeps extracting a high financial toll on the family, which is exacerbated by the fact that Terry is now the only bread winner in the family with Deep devoting full time to grappling with EB.
The vehicle for spreading awareness about EB is the Dystrophic Epidermolysis Bullosa Research Association of Canada, or DEBRA for short. Deep is a director of the Ontario-based organization.
“Board members and volunteers for DEBRA are all parents. We don’t know how many people have this condition across Canada, partly because there are those who don’t even know they have this condition.
“I’m on so many social media spreading awareness about EB, and I keep discovering new people who have the same condition, or who only become aware after reading about it in the postings. In that way, we at DEBRA are also able to reach out to these people and offer whatever assistance we can,” says Deep.
The condition is so rare that in the Lower Mainland, there are probably only about 10 families who have a member suffering from this condition, says Deep.
There’s a little bit more awareness of EB in the US. “There’s DEBRA America, but also other organizations,” says Deep.
There are also a number of celebrities who champion the fight against EB in the States. “Singer Eddie Vedder of the band Pearl Jam created a group called EB Research Partnership which raises millions of dollars, and Courtney Cox of TV’s Friends holds an annual fundraiser in California,” says Deep.
DEBRA also helps EB sufferers materially where it can. “For instance, they helped us get a special bed – a soft-gel bed usually used for burn victims – for Brayden,” says Deep.
But the ultimate solution – a cure for EB – remains out of reach for the near future.
“There’s a doctor who works in the US who is really involved and doing a lot of research on it. But it’s going to take a long time. Among the things they are working on is gene therapy and gene editing. But it is still nowhere near finding any kind of cure,” says Deep.
The Manns are just thankful for each day that they are able to successfully cope with Brayden’s condition, and remain optimistic and hopeful for the future. “In the long term, I don’t think it (Brayden’s condition) is going to get worse. But it’s hard to say. An infection could kill him,” says Terry.
The Dystrophic Epidermolysis Bullosa Research Association of Canada (DEBRA) holds an annual fundraiser in B.C. This year’s event will be held on Feb. 26 on Grouse Mountain. To be called Bella’s Ball, it will be a “broomball” event – a game played on ice, but with participants wearing ordinary shoes, and using brooms in place of hockey sticks to manipulate a ball around the ice.
“We supply the brooms, and participants bring their own helmets and regular shoes. The idea of wearing regular shoes on ice is to provide an indication of how difficult it is for many EB sufferers to move around easily,” says Deep Mann, a DEBRA director and organizer of the fundraiser.
This is the third time the annual event will be held. The event usually highlights one or more children suffering from EB, and this year, the spotlight will be on Jonathan Pitre of Ottawa, and Kourtney Kajawa, another child suffering from EB.
Jonathan was the subject of a major story on EB by CTV as well as by The Ottawa Citizen.
More details and a video depicting Jonathan’s condition are available at http://ottawacitizen.com/news/local-news/butterfly-child-dreams-of-the-northern-lights
(NOTE TO READERS: The video on the website contains graphic images of Jonathan’s EB-related wounds.)
Deep says that during the first fundraiser, DEBRA raised $13,000. That increased dramatically to $40,000 last year. This year, the organization hopes to raise much more.
“We’re now able to get sponsors, and that is a big help,” says Deep.
Among the sponsors this year are the host Grouse Mountain, which will donate all money raised from lift tickets on that day to DEBRA, and Scotiabank.
Deep has appealed to the public to donate to DEBRA and help battle EB.
“Most people, including doctors and specialists, are unaware about it and governments don’t fund any research. DEBRA Canada provides support and medical assistance funding for people with EB. But we would like to grow and be able to fund research for a cure to be found. Without donations and sponsors, that is not possible. All donations are tax deductible and we are always looking for volunteers because we are a non-profit, voluntary organization. New ideas for fundraising are always welcome,” she says.
More details on EB and how to donate or volunteer are available at the DEBRA website at http://debracanada.org
You can also contact Deep Mann for more information at firstname.lastname@example.org