Friday, November 19, 2010

Melissa's DEBRA Lifeline Appeal

Black and White photograph of Melissa showing her head and shoulders.

My name is Melissa Smith, and I'm a twenty-four year old English post-grad student, who loves all things decadent. I'm a fledging blogger, and I generally love writing, whether articles, stories or complaint letters to hospitals (they're the most fun!). When my body permits, I enjoy gigging with friends, shopping, visiting galleries, travelling and playing with my mad little dog, who completely ignores my disability and treats me like she does everyone else. If only all people could be like that!

On Sunday (21st November) at 4.35pm, BBC One is showing their monthly Lifeline appeal show, which highlights charities working to support people in the UK and abroad. This month’s show is particularly close to my heart, as the featured charity is DebRA UK, who support people with and fund research into treatments for, a genetic skin disease called Epidermolysis Bullosa (EB).

I was born with EB (of the Recessive Dystrophic sub-type) twenty-four years, and the best way I can describe its symptoms is waking up each morning with fresh second and third degree burns, and developing more throughout the day. The simplest of tasks can damage my skin, causing it to form fluid-filled sacs masquerading as blisters, or my skin will just lift off completely, leaving a raw wound. In my type of EB, this is caused by a deficiency of a protein called Collagen VII, which acts as a glue holding the layers of skin together. I have very little Collagen VII, and what I do have doesn’t work as it should. EB affects the entirety of my outer body, as well as my mouth, oesophagus, nostrils, eyeballs and inner ears. It has caused my fingers and toes to fuse and contract, limiting my dexterity and balance, and the blistering and nerve damage to my feet means I am usually reliant on a wheelchair. I am also reliant on my family, especially my mum, who is my official carer.

But that isn’t as bad as EB gets, not by a long way. I’ve seen friends of mine, with the same type, almost paralysed by the wounds and scarring, unable to straighten their legs or even sit up. I’ve lost twenty-five friends to EB, with complications including massive infections, heart failure, kidney failure, malnutrition, and most commonly, skin cancer (people with my type of EB are 70% more likely to develop a squamous cell carcinoma, and our bodies’ are often unable to prevent it spreading). Then there is Junctional EB, and it is one of the worst illnesses conceivable. Imagine thinking a new baby is teething, and realising that their gums are actually disintegrating, or having to give a child so much Morphine to make your touch bearable that they don’t recognise you any more, or watching your baby’s skin wipe off on their pillow, just because they turned their head. That is Junctional EB, and even Stephen King couldn’t dream it up.

But despite all of this, because of DEBRA, we have hope. They fund specialist adult and paediatric nurses who travel the UK giving home visits, and advising medics in hospitals; they buy equipment needed by EB patients in the hospitals that the NHS won’t pay for; they advise patients and their families on how to claim benefits, and fight the corner of those who are being challenged; and, last but by no means least, they fund research into treatments and cures for EB. For my family and me DEBRA have been like a recovery service – picking us up, sorting us out, and getting us on the road again. They supported us when our local authority said I couldn’t attend mainstream school, with our Director running rings around the officious idiot from the council, and since then they’ve encouraged us to believe that nothing is out of reach. They have done everything to support me, and now I want to return the favour. Especially as I’ve been a Trustee for the past few years!

I’m not asking you to watch the appeal in the hope that you will make a donation. I know all too well how tight money is for everyone who relies on the benefit system, and how uncertain the future is. I’m asking that you watch to learn about our little known disease, and how it affects our lives. So many people think of skin conditions as itchy rashes and minor problems, but EB kills on a far too regular basis. I don’t care for myself, I just desperately don’t want EB children to be bullied and to be stigmatised like I was. And I want them to have futures free of bandages and blisters. So if you could tune in, and ask your family and friends to watch too, I would be eternally grateful.

Thanks, love Melissa

Info link: DEBRA

Note from BendyGirl: Whilst my friend Melissa is too nice to ask you to donate to DEBRA, I have no such scruples! Money is tight for us all, but if enough of us donate just a few pence it'll make a huge difference. Donate to DEBRA here


e said...

I have never before heard of this, and went to the link and website to see about it. As I live in the U.S, I thought perhaps they might not accept my card, but the donation went through.

Best to you both, Bendy and Melissa.

Anonymous said...

juxtaposed with the previous post.. i like it, in a kinda ironic way...

Melissa said...

Hello e,

Thank you so much for your donation! DEBRA UK does work alongside DEBRA US and DEBRA International, which means that we don't just fund research within the UK. Your donation will help to do a huge amount of good.

All the very best
Mel x

Melissa said...

Hi Anonymous,

I'm not entirely sure how this is ironic, but maybe I'm just being dim today.

Like many other disabled people, I am reliant on benefits, despite my desire to work. My mother is my carer, but that was not an easy decision to make, as we are far from a wealthy family. My dad is a self-employed manual labourer, and thanks to the current economic climate, he has to take each day as it comes. My mum was working part-time locally, but my needs became more time consuming, and there was no one to help me but her. We asked our district nurses, but they said I was far too much work, and declined to help except in "an absolute emergency", and then it would only be for a day or two. We asked agency nurses, but only one visited, and then she decided the same. Nurses, whether NHS or private, don't care for how unpredictable EB is, and that I can never guarantee being able to run to their schedule. I am acutely aware that caring for me is a great strain on my mum, though she would never admit it, and am asking friends if they would learn to care for me, in order to give my mum a break now and then.

In this sense, my problems support the previous post. Once benefits are cut, and I can't pay my friends to help, I will be relying on their charity, rather than having a working relationship. I already rely on the charity of my family, and from the reactions of various nurses, I would be completely stuck without them. DEBRA's nurses are wonderful, but coming into homes to change dressings on a regular basis is not part of their job, nor should it be. They come in to give advice and support, particularly when the patient is too ill to attend the hospital, but that's as far as they can stretch.

If there is anything else I can clarify, please just ask.

Lisa said...

I did not know the EB was another collagen disorder. I shouldn't be surprised, of course, all the other conditions relating to the body falling apart are collagen disorders (for example osteogenesis imperfecta, which I have and which causes bones to break easily, is a collagen disorder. EDS, which BendyGirl has and causes joints to dislocate easily, is a collagen disorder).

Melissa said...

Hi Lisa,

I wish more people knew how much trouble Collagen can cause, and that it's not just something to have injected into wrinkles.
As a knock on of EB, I also have Osteoporosis.
Strangely, an EB researcher was diagnosed with EDS by a fellow researcher who specialised in all Collagen related disorders!
Let's hope we all have treatments soon x

Lisa said...

As a knock on of EB, I also have Osteoporosis.

My mum developed osteoporosis as a result of her osteogenesis, so I guess that's something I have to look forward to one day.

Though osteoporosis gets caused by most impairments that reduce mobility: My dad has osteoporosis as a result of not being overly mobile due to his cerebral palsy.

a fellow researcher who specialised in all Collagen related disorders!

There are such beings? In some ways these days I almost more closely resemble someone with EDS than OI because I dislocate much more frequently than I break (though I don't for a second dispute my diagnosis; I have all the clinical features of OI like the triangular face and the funny-shaped ribs, plus as a child I broke my bones every 6 weeks to 2 months). But it seems like OI research at least is very insular and doesn't look to other collagen disorders at all.

It's a shame that OI research as least is so insular. Earlier this year I had a reaction to a cream my dermatologist gave me which was in keeping with the EDS reaction to local anaesthetics (not staying local but spreading through the surrounding tissues because of the poor collagen). If I didn't have so many friends with EDS I'd have never worked out what was happening!

monkey typist said...

hi melissa, im sorry ive already missed the program. is EB the condition that jonny kennedy also had?