What is Epidermolysis Bullosa (EB)?
I am going to describe EB in my own words and terminology. Please excuse any incorrect ways I might describe EB in advanced. To clarify, I am also going to describe EB from my own mental and emotional prescriptive. I will share an example to give a small depiction of what EB entails—the bad and the good. If I say anything that offends you personally, it is not aimed to offend anyone nor am I saying this how you should live. These are my feelings.
Epidermolysis Bullosa is a genetic skin disorder that affects the connective tissue between the epidermis and dermis with constant blistering. It can also cause blistering of the mucosa, from eyes to the mouth to the anal region. The esophagus suffers blistering (for me, the most painful next to eye abrasions) which leads to esophageal strictures. A simple meal can turn deadly due to choking and blistering that can obstruct the passage. The average adult esophagus is 14-20mm, but someone such as myself with EB is only at 2mm (5mm after surgical dilation.) The eyes can have abrasions which can cause abnormality to the eyes cornea. Depending on the severity of the abrasion, it can lead to permanent damage (scarring) which can limit the sight or cause complete blindness. Eye abrasions should heal within 2-3 days but with EB it can take up to 1-3 weeks. No light should be let into the eye. The best healing method is being in a dark room with the eyes closed. Aside from the excruciating pain, the mental anguish can be crippling being further confined.
There are several subtypes, but hundreds of mutations of the EB gene. In my subtype (recessive dystrophic), I am missing the “hooks in my velcro” (collagen VII), that allows the skin to stretch with friction and touches. I am often left with scarring which has caused my hands and feet digits to become “mittened” or webbed (complete syndactyly). More commonly seen in recessive forms of EB, the scarring can limit mobility and function of limbs. In severe cases, the deformities and/or discomfort can cause limitations to walking for long periods (i.e. why I have used a wheelchair.)
There is some envy behind the simplicity of those who can get to shower within 10 minutes or dressed within 5 minutes. I don’t want to make it sound like I am completely immobile because I am not. After the years of the process day in and out, you would think one would be used to it. Never. I love showers. I love the feeling of being free of my gauze, seeing my actual skin. Mentally and emotionally, I loathe the process. Not so much the preparation of the gauze, but building my mental state to withstand the excruciating pain that jolts through me every dressing change. The rushes of adrenaline are exhausting. Even taking off day old gauze can be a detrimental process in itself. If a non-stick pad that covers my wound from sticking to the gauze shifts, sometimes I am unaware and will tear off completely stable “strong” skin around the wound. I have screamed and trembled with anxiety, nearly letting out the profanities swirling through my head at that moment. On rare days of fatigue, my emotions can range from a mixture of rage and sadness. I never want to take out my pain on anyone around me, not to say that has not happened before. No one deserves to see that side of me, the side I loathe feeling. I chose not to give too much of my precious time.
Wound care is a tiresome job for me and my caregiver (usually my husband). From preparation to finishing my wound care by the caregiver can take 3 hours, sometimes 2.5 hours on a good day. If I do it entirely on my own with unopened wound care supplies, it could take me 6 hours because of the webbing of my hands slows the process. It can be frustrating when people say, “You’re so lucky you don’t have to go out to work.” Words of ignorance but my survival of my disorder has been a full-time job. Does it impede my social life and those around me? Greatly. Growing up, people did not understand why my mom or I could not meet or talk on the whim. Even with an explanation, some people did not want to grasp or even believe it. That in itself can make you feel worthless. You can really find out who really listens and cares when you or someone dear to you has health challenges.
Epidermolysis Bullosa is much like a full-time job for both patient and caregiver. There are very rare moments when you can live life without thinking about EB. Every movement has to be planned out in my head so I don’t upset my skin somehow. Even days where I am feeling “healthy”, I have to consciously be aware of what to do and what not to. Throughout my childhood, I did not fall a lot, but when I did fall, they were very traumatic falls—with the full-on gore of blood and missing skin. Because of those falls, I have to remember how and why those falls occurred.
A major use of our toes is for balancing. EB has caused my toes to mitten due to the scarring. My toes started fusing together from the early age of two. Before different wrapping methods were practiced to slow the scarring of the digits, surgery was largely recommended when I was younger. I had surgery on my right foot to separate my toes and straighten them. The doctor put me in a cast, which over the years, doctors realized that was not a good idea for someone with EB. When the cast was removed, there was a 6-week old xeroform dressing healing into the wounds of my foot. Xeroform is a type of pliable petroleum based wound dressing. From my own personal experience, I am not fond of the dressing, especially for surgical wounds. They dry out too rapidly for me personally. The doctors did not give me pain relief for the nightmare under the cast. The xeroform that was essentially now becoming part of me, literally, was being ripped out of my wounds. Wounds that were nearly healed were reopened, forcibly. I was only two, but I will never forget my heel was completely red raw and bleeding. Lessoned learned: never wear a cast when you have a genetic disorder that constantly breaks down and needs care, every day. Not only did that cause my foot to become worse before the surgery, the surgery did not help my foot’s deformities as it healed. As I grew, it was difficult to do physical therapy on my foot without causing blisters. My other foot that I decided against surgery upon has scarring and some fusing of the toes. It is more stable, balanced, and less painful. The foot that had surgery, the toes are now in a “W” shape beneath all the scarring. My toes are basically pushed upward and toward my body. My shoe size is women’s 6 (without socks and gauze) but if my toes were stretched out they would be an 8 or 9. Doctors are usually amazed how well I can walk, though, it is limited. The metatarsophalangeal joints are constantly under strain because of the locked bent position they are in. A past doctor described it is equivalent to walking on the tips of my elbows.
Sharing the tidbits of the challenging parts living with EB, shows the “bad” about living with EB. What is the good that comes from it? With anything challenging in our lives, we come to learn the good from the bad. There are uncountable times people have tried bringing me down, telling me I cannot do this or should not do that. This only made me stronger to take chances which have given me a fulfilling life. Granted, I don’t like EB can do and has done, but I accept the challenge, to kick its butt! I love seeing the miracles that are gifted to me each day. God has shown His kindness and mercy through my life and the wonderful souls that I have grown to know. If it were not for my disorder, my life story would be completely different. For the better? Maybe. Maybe not. To me, I live in this moment of greatness given to me, making the best of each day even during those rough days. Health challenges of any sort should not define who you are, it is just a small part of you. EB seems vast, but it is just a small part of me. EB has made living challenging, but I have not allowed it to disable me.
Disabling yourself is not only physically, it is mental and through attitude. We beat EB every day.