Wednesday 17 January 2018

Living with EB- The painful condition that leaves 80pc of Emma's body covered with open sores

Born with the distressing skin condition EB that leaves 80pc of her body covered with open sores, Emma Fogarty has never had a day free from pain

Emma Fogarty was born with the skin condition EB, also known as ‘butterfly skin’. Photo: Philip Lang.
Emma Fogarty was born with the skin condition EB, also known as ‘butterfly skin’. Photo: Philip Lang.

Áilín Quinlan

Last March Emma Fogarty was admitted to hospital. It was a visit that neither she, nor those trying to care for her, will easily forget.

Emma has a rare, painful and extremely distressing genetic skin condition called epidermolysis bullosa (EB), also known as butterfly skin.

Her skin blisters and tears at the slightest touch, and the condition has left 80pc of her body covered with open sores.

There is no cure for EB, and the only form of treatment is constant, painful bandaging of the skin.

"I have always been in pain, all my life," she observes. "There has never been a day when I have been free of pain as far back as I can remember."

Read more: Severe skin condition makes learning to walk agony for brave Casey (2)

In mid-March, when Emma was admitted to hospital, her wounds were so bad that the bandaging took twice as long as normal - six hours.

"I was in horrific pain, screaming, crying shaking. I always have wounds, but on that occasion there were a lot more than usual. I had 16 on my rear alone."

Even a routine bandaging process, which can take three to four hours, can be so painful that Emma is often not the only one who is distressed.

During a previous hospitalisation, she recalls, the infection was so bad that even extremely strong painkillers, including morphine and ketamine failed to touch it.

"I was crying, screaming shaking, even praying," she recalls, adding that everyone else in the room, her mother, her personal assistant, the nurse and a student nurse were in tears at the pain she was experiencing.

A former mortgage advisor, Emma, who has a particularly unpleasant type of EB - Recessive Dystrophic Epidermolysis Bullosa - normally has to get her bandages changed every second day.

This is a process that usually takes three-and-a-half to four hours.

"The bandages cover about 80pc of my body, shoulders to toes. I'm on about eight different painkillers every day but during the bandage change I have to take two more painkillers because it's so painful."

The 31-year-old from Abbeyleix, Co Laois is now a patient ambassador for Debra Ireland, an organisation established in 1988 to provide patient support services and drive research into treatments and cures for those living with the genetic skin condition.

Although she was born with the condition - she emerged from the womb with no skin on her left foot and right arm, and was diagnosed with RDEB at just two weeks old - Emma managed to attend primary and second-level school, attending college and later landing a job as a bank mortgage advisor.

Then, seven years ago, and completely without warning, one of the by-products of her condition - osteoporosis - intervened.

At the time she was about 24.

"One day I was walking home from work and I heard a crack - and suddenly there was a fracture in my foot. Into the wheelchair I went and never really came out of it since."

After that, she recalls, she had to resign from her job.

"The fracture didn't heal well and in the end, I realised that I'd have to let my job go. That was a big thing. I was so proud of being independent and of being able to live and work in Dublin - and then to be put into a wheelchair almost overnight and to have to come home, live at home, was a blow. I lost a lot of my independence."

It is a feature of the condition, that as the patient gets older, problems increase.

"As I got older it slowly got worse. The wounds were bigger, more painful and more extensive," says Emma, adding that people with her form of the disease often do not survive past their late twenties.

Inevitably, hospital has been a big part of Emma's life - as a child, she recalls, she had to have surgery virtually every three months. EB severely affected her throat, leaving it blistered and bleeding and thickened by scar tissue, which had to be periodically removed.

Although the condition of her throat has improved, there is the ever-present fear of skin cancer.

Because of the presence of constant wounds, patients with a severe form of EB like Emma are susceptible to a very aggressive form of skin cancer, from as early as their teenage years.

She recently spent five weeks in hospital, undergoing 12 biopsies to determine whether there was an infection or skin cancer, or simply any other underlying issue.

"The wounds were not healing and they felt there must have been some problem."

Thankfully all the tests were negative, and a few weeks later, she decided to start a blog. "I'd always wanted to do a blog, but I didn't have the confidence, I didn't know if it would be interesting or if people would want to read it."

However, she noticed that when she went into her local town of Portlaoise "a lot of people would stare at me and they didn't know what it was." She wanted to inform people about EB.

The first blog attracted more than 1,000 views over a period of just 24 hours and since then it has settled into a steady 300 views per entry.

EB inevitably affects her social life - Emma has a personal assistant who brings her out and about three days a week and occasionally goes out with friends at the weekend. However, she must be very cautious and remains on the alert at all times.

"I have to be careful about going into pubs or nightclubs because people can be clumsy. You have to judge how much alcohol they've had because they could fall on top of me and even bump into me, which could mean spending two months in pain and with a wound.

"I can only be touched very gently. That's why it's called butterfly skin because if I'm not touched very gently the skin will come off or I will get a blister."

She's surprisingly sanguine about the condition which has dominated her life.

"There's no cure for it. There's a lot of research ongoing, but currently it's a case of managing the condition as best you can."

It's touch and go whether she will be able to travel to Dingle this Friday (June 19), for the hugely popular three-day Dingle Mingle Challenge, in which Ireland's most eligible and active singles are being asked to sign up to raise money for Debra Ireland's three-day Kerry Challenge.

As patient ambassador for Debra Ireland, Emma is due to attend the Kerry event, along with Love Hate's Robert Sheehan.

Participants will be joined by the actor and his brother Brendan for three days of trekking though the Dingle Peninsula, followed by nights of fun and craic in Dingle's lively pubs.

The event costs €300 per person, which includes two nights' accommodation in the four star Dingle Skelligs Hotel, all meals and snacks, transport to and from the route each day, on-site physiotherapy and the celebratory dinner and prize giving.

Participants are asked to raise a further €350 for the charity, which provides support services for those living with EB.

"I'll be going to cheer them on if I'm well enough," declares Emma. "I'm very excited about it."

* Emma's blog:

* See for information on the condition and the Kerry Challenge

10 things to know about EB

1. Epidermolysis Bullosa (EB) is the worst disease you've (probably) never heard of.

2. Children with EB are known as "butterfly children" because their skin is as fragile as the wings of a butterfly.

3. Skin blisters are the major symptom of EB (EB skin is so fragile that even minor rubbing may cause blistering).

4. Some patients have blisters internally as well (the linings of the mouth and oesophagus).

5. EB is not contagious.

6. The slightest friction can produce agonising wounds that can cover up to 80pc of their frail bodies.

7. Patients have to have their skin bandaged most of the time to avoid infection (bandage change is extremely painful and can take several hours to complete).

8. There's currently no effective treatment or cure for EB.

9. People with the more severe types of EB also have a high risk of developing skin cancer.

10. One in 227 of us has the defective gene that causes EB.

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