Today I want to tell you a little bit about myself and my condition called EB.
My name is Nikki and I’m a typical 26 yr old girl living in Sydney, Australia. The one thing that is not so typical is that I was born with a rare genetic skin disorder called Epidermolysis Bullosa or EB for short.
The Worst Disease You’ve Never Heard Of
EB is often referred to as “The Worst Disease You’ve Never Heard Of”. It causes my skin to blister and tear with the very slightest touch. When I say blister, I do not mean the tiny blisters you might get on your foot every now and then. EB blisters are very different, they fill with fluid and if not lanced with a needle can grow up to the size of well, it could cover an entire limb if not lanced! After they are lanced the skin tears off and a large open wound is left. 90% of my body is covered in large open wounds similar to 2nd degree burns, the only difference being that these wounds never go away. Because of this I am wrapped in bandages from head to toe every day of my life.
Open wounds similar to 3rd degree burns cover the whole body
My bandages must be changed every second day in a bath which can take over 2 hours. First I get in the bath and soak all the bandages off which is a very painful and stressful process. Everyone knows what it’s like when you have a sore or a cut and you get water on it. It hurts right? Well now imagine that feeling on your entire body….not with a little cut or scratch, but open wounds that have no skin at all. I cannot dry with a towel because just the towel itself could cause more damage so i air dry infront of a heater. Then my mum applies new bandages which takes at least an hour and a half. I HATE bath days (all EB kids do). It is the most stressful time of day. I have done this process everyday of my life with NO painkillers. Not even Panadol! So I find it strange how people immediately take a painkiller for minor injuries.
Underneath The Bandages
Warning: These pictures are very graphic and may be distressing to some readers but I think it is important to raise awareness of EB and what it is REALLY like.
EB also affects the inside of the body aswell. I get lots of blisters in my mouth which are annoying and can make it hurt to eat. Sometimes just eating causes blisters. Some EB kids get blisters in their throats and even their eyes causing their eyes to be fused closed and they have to sit in pain darkness for a week until they heal. Luckily I haven’t had this…yet.
You have probably seen from my photos that despite me being 26 I am TINY, about the size of a 10yr old. Nutrition is a complicated thing with EB. We use so much energy repairing skin and fighting infection that there is barely anything left to help us grow. It is also nearly impossible to eat enough calories to meet our needs. To help me get enough nutrition I have a high calorie milk supplement that goes into my stomach via a Nasogastric Tube in my nose while I sleep.
EB also causes the fingers to fuse together and web into mittens. Surgeries can be done to separate the fingers but unfortunately they don’t last long. My right hand is pretty bad but I still have some fingers on my left. The skin behind my knees has contracted which means I cannot straighten my legs and it is painful to walk. When I go out I use a wheelchair, a pink one of course! I also have an electric wheelchair that gives me a little bit of independence but it is still so hard because so many places are not wheelchair accessible. Grrrr!
The big problem with EB is that as we get older (by old I mean 20′s) we have a very increased risk of getting Squamous Cell Carcinoma’s (SCC), a deadly form of skin cancer. If not caught early it can spread very aggressively. Because of this I attend regular EB clinics every 3 months where I have a skin check. I take all my clothes and bandages off and about a million drs look at me and take photos of me in the nude….FUN! This is to look for anything suspicious that could be an SCC. I also see every other team u could think of, Renal, Gastro, Endocrine, Dietician etc etc. Sometimes I have to have blood transfusions through loss of blood. Even though I hate EB clinics, I have amazing Drs and the Dermatology dept are like a 2nd family. There are not many of us EB kids so we are all very close.
It’s very hard living with EB, nothing is easy. But I just have keep pushing on. I have good days and bad days and I just have to make sure there are more good days than bad. Appreciate the little things in life and don’t take life for granted.
That is just a little about EB to start you off and to answer some of your questions. Feel free to ask me anything you like! I won’t be offended If you would like to help find a cure or treatment for EB please go to http://debra.org.au and donate!
Read more of Nikki’s inspiring, insightful blog over at: NikkaBlogger.