25 October 2013

Appearance diversity: Living Without Skin - Life with Epidermolysis Bullosa.

I first met Nikki online when she read my blog back in 2011. I think she left me a comment and I contacted her by Facebook. Nikki has a rare, extremely severe skin condition called Epidermolysis Bullosa (EB). Her condition is evidence of just how much skin affects the body's functions - her skin is raw and she's in pain a lot of the time. I can't imagine the pain she's in. The condition can be fatal, and in the time I've known Nikki, she's lost two close friends to EB, and been on the intensive care ward in hospital twice. It must be really hard to lose friends to the same condition you have while you're going through a life threatening experience.

I asked Nikki to tell her story for the appearance diversity series - and she has timed it well because today is the start of EB Awareness Week which runs from 25 - 31 October.

"Skin. The largest organ of the human body. Your body's first line of defence against injury, bacteria and infection from the outside world. The organ that everyone takes for granted...

Well I don't...

My name is Nikki and I'm 27 yrs old. I was born with Epidermolysis Bullosa or EB for short. EB is a genetic skin disorder where by the skin is so fragile that it blisters and tears at the slightest touch. An easy way to imagine it is that our skin is as fragile as a butterfly's wings (which is why EB children are often called Butterfly Children).

EB has been likened to having 2nd degree burns all over the body, except for one difference, while burns eventually heal, EB wounds are constantly coming and going. I have open wounds on most of my body and am therefore wrapped in bandages from head to toe every day of my life.

Daily life with EB can be very painful. Every day I must have my dressings changed which is a long, painful and stressful process to say the least. This involves taking off all the old bandages which can sometimes be stuck, washing the open wounds and then having new dressings applied. This process can take up to 2-4 hours. It is safe to say this is the worst time of day for children and adults with EB. You can always tell when it's getting to 'that time' of day on a person with EB because a little anxious furrow line will appear across their brow. No matter how much of a good day you are having there is always that knowledge in the back of your head that bath time is just around the corner.

(Nikki with Richard Dawkins)

When things are running smoothly I have a 3 monthly EB clinic at the hospital for a full body check for SCC's (Squamous Cell Carcinoma). I am also seen by every medical discipline all in one day as EB can affect every part of the body. I also have monthly renal clinics since having kidney failure.

My subtype of EB can be life threatening as we have about a 50% increased risk of SCC which is a very aggressive skin cancer. There is also the constant risk of infection as well.

While EB may not directly affect other organs, years of constant infections and inflammation can cause damage to other organs such as the kidneys. I have been unlucky enough to have this happen and am now on dialysis. If I have a kidney transplant I will be probably be the first EB person ever to have one which is both scary and....scary. Whether or not I would survive it...that's a whole other question.

Along with the physical pain, comes the emotional pain from constant stares and whispers of strangers. Then there's the innocent but no less hurtful comments from little kids like "Look at that girl!?, Yuck that girl's dirty!" I know they are just curious but well, kids can be cruel sometimes.

Sometimes people can mistakenly believe that EB is contagious but this is definitely not the case. EB is a genetic condition so you can hug me all you want and you won't catch anything! As long as you hug gently though!

I can go out but since I use a wheelchair mobility can be quite an issue. I usually go out with my family and friends to lunch or dinner or have my friends over to my house. During the summer though I tend to stay inside in the air conditioning as I get extremely hot with all my bandages on and cannot control my body temperature.

I studied up till yr 12 and then did 3 years of TAFE studying various Multimedia/IT/Graphic Design courses. After that my health declined and I have not studied any further, however I did teach myself Web Design and HTML and have done a bit of freelance work here and there. Things I enjoy doing to take my mind off EB include having good food with friends, Movies and DVD marathons, cryptic crosswords, building Lego, trying and failing to draw and most recently jigsaws!

I must admit its hard to stay resilient and there are often times I just want to give up. But I can't let that happen so I have to try my best to pick myself up and keep positive.

(Nikki with The Cranberries)

This week is EB awareness week and I want to use this post to educate and bring about awareness of EB to the public. For any parents reading this, teach your kids that if they come across someone with a physical difference like EB that there is nothing to be afraid of and we are just like them.

Currently there is no cure for EB and treatment is mainly based on the provision of very expensive dressings. While these dressings are funded under a very generous scheme from the Government, the EB support charity DEBRA Australia is non-government funded and relies solely on donations from the general public.

Since I was born DEBRA have been a great support to me and the EB community not only providing dressings and other medical equipment, installing air conditioners, but by offering a close knit community of other family members to share stories, tips and advice.

If you would like to help spread awareness and provide dressings and medical equipment for children and adults with EB please follow this link to DEBRA to donate. Your donation WILL make a difference as every little bit counts!"

Read Nikki's blog.

Follow Nikki on Twitter.

Read about Nikki in a Sydney Morning Herald article for Rare Disease Day.

Watch Nikki on ABC's 730.

Keep up to date with DEBRA on Facebook.

For more information about organ donation - so that one day Nikki might get her kidney transplant, visit Donate Life.


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