Miggy: Welcome Corinne! Thank you so much for being here on This Little Miggy today sharing your story with us. You have a very rare skin condition called Recessive Dystrophic Epidermolysis Bullosa or EB for short. I have heard that EB is quite painful and can often be fatal. But I also know that almost every condition varies person to person, so will you please educate us about EB, and specifically how does it affect your day-to-day life?
Corinne: Thanks for having me! Epidermolysis Bullosa (EB) is a rare genetic connective tissue disorder. About 200 people each year are born with this disorder. The subtype I have is Recessive Dystrophic (RDEB). There is a mutation in the gene, Collagen VII. This means that the anchoring fibrils of skin which typically connects our Epidermis (outer layer) to our Dermis are not intact. Since I do not have the protein that holds these layers together any trauma, friction, or sheer force will result in blistering and/or tearing of the skin. This affects anything lined by squamous skin cells; so it affects my outer skin as well as corneas and esophagus. EB also can result in loss of hand function, due to the fingers fusing together over time. Anemia, Gastrointestinal Issues, and Osteoporosis are some of the secondary issues that may also occur.
There is currently no cure or treatment. My daily maintenance is quite painful and lengthy. I live independently, but each morning with the help of a caregiver, clean and bandage all of my wounds which takes about two hours. Bleach or Salt baths are commonly used to try and prevent infection. Twice a week I have physical therapy in order to maintain some mobility in my hands and arms. Throughout the month I’ll also have doctors visits to my Dermatologist, Gastroenterologist, etc. Since I have an esophageal stricture all of my diet has to be specially prepared and pureed. Shoutout to Vitamix which changed my life! I can now eat any food because it is so easily blended. My corneas can also tear while I sleep, so this often makes for some blurry days! As you can imagine the light can be quite painful, so when I was younger I used to have to wear sunglasses indoors on “bad eye” days. I would sometimes be embarrassed, but my mom would just try and tell me I looked like a movie star and sometimes she would even wear her sunglasses in solidarity!
Miggy: Now let’s go back to the beginning, while you don’t remember your birth can you tell me what you know about your early days and what it was like for your parents when you were first born? How did they first hear about the news and what prognosis were they given?
Corinne: In 1989, my parents had no idea I would be born with EB. When I was delivered, the doctors put the sticky electrodes on my chest to monitor my heart and were very surprised when my skin came off when removing the monitor. I was immediately air lifted to Johns Hopkins Hospital in Baltimore, Maryland where I was subsequently diagnosed. My parents were given a choice, since I had so many wounds in my mouth and was unable to feed, they could either place a gastrostomy tube in my stomach that would give me nutrients, or they could let me die. My parents have always said that there was never a choice (phew), and immediately placed the g-tube. I was in the NICU for about a month before I was brought home. I am very fortunate in that my dad is a physician and my mom was, well a saint. Although I can only begin to imagine the feelings of overwhelm and grief they must have felt those first weeks, I was in incredibly capable hands.
Miggy: Moving forward a bit how old were you when you first gained an awareness of your condition? What do you remember most about having EB as a child? What was your family life like, how did they support and help you over the years? How was your school and social life as a child with EB? Is there anything you wish you or your family would have done differently in relation to your condition?
Corinne: I honestly cannot pinpoint at what age I realized I was “different” because I can’t remember a time when I wasn’t aware. I was told from a very young age that I had to be extremely careful in everything I did and everywhere I went. When asking my parents why I was born “like this” I remember my mom saying “Because God made you special, and he does not give us anything we cannot handle”. Obviously as an adult you realize pretty quickly the world can be arbitrarily unfair and its not always comforting to rely on the old adage of “God’s Grand Plan.”
Throughout it all though, I was raised as a “normal” kid. My mom became my full time care giver and champion. I remember attending pre-school and she would sit in the lobby every single day just in case I got hurt or needed her. She also would write letters to my teachers and parents of my classmates, explaining my disorder which really helped me transition each year and also took away some of the questions I may have gotten from my peers if they had come in knowing nothing about me. I took dance classes and loved every second of it, even when I came home with blisters all over my feet. I was taught that I could do anything I put my mind to and that I was “just like everyone else.” There was much about this attitude that benefited me and gave me the drive, ambition, resilience, and perseverance that I still carry with me. However, as I have had the time to reflect, I realized there was a downside in striving to be like everyone else. Because, spoiler alert, I’m not. And it took me a long time to realize that is OK! It’s a very fine line persevering and not being held back by your limitations, but also honoring your body and it’s capabilities. I still, to this day, struggle with pushing too hard or too fast, in the desire to not let this disorder “Win.” I am coming to terms that having a chronic illness is not a sprint, it’s a marathon. Some days you have to stay home and recharge even when your mind is telling you otherwise!
Corinne: EB is a very visible disorder, but there are also many parts to it that are invisible. The other day someone was saying how Swans look so peaceful gliding on the surface of a pond, but underneath the water, where no one can see, they are pedaling furiously with their feet trying to stay afloat. And I thought, wow, that beautifully describes what it is like living with this disorder. For instance, I will be having a conversation with a friend joking, asking how their day went, being attentive and listening. Yet, in the background, under the surface, a million little things are happening. I’m aware of the stabbing pain radiating from my thighs, my heart palpitations are fluttering, my feet and ankles are swelling and the eyes I’m looking out of so “serenely” are blurred and in agony from my cornea tearing the night before. I’m pedaling my booty off every single day just to appear as if I have it all together. I guess in one way or another, we all are.
My most tangible struggles I face day-to-day is coping with intense chronic pain, functioning without “proper” hands, and the frustration and anger that I can’t just go and accomplish what I would like. I’ve always joked that I’m a Type A personality in a type EB body.
My dreams and goals for my life have had to adapt and change, as my disorder has changed over the years, which can sometimes be very frustrating. However, I have always known that whatever suffering I have been through has also been my biggest teacher and the greatest thing I could ever do was be a hand in the dark, or a voice for someone who feels they have none. Words are powerful and there have been so many authors over the years who have made me feel less alone, or whom I felt understood by. To make people laugh, to make people feel, is a gift. I am a writer and speaker, and would love to publish a book and speak all around the world especially to women. I just want others to know for themselves, what has taken me a long time to figure out, that there is no shame in having an illness or disability, that nothing is wrong with us, or broken, and owning all parts of yourself is a beautiful thing! I’d also love to become a mother one day.
Miggy: Now for a lighter question, I’m a big believer in seeing the humor in life and learning to laugh, so have you ever had any funny conversations/moments you never imagined due to your special needs situations?
Corinne: There are so many funny moments when living with a chronic illness, you have to look for them sometimes just to keep your sanity! A few years ago in 2013, I had a squamous cell cancer lesion on my foot. Once it was removed I was unable to walk and wheelchair bound for five months. Haha, isn’t that hilarious?! Just kidding, I’m getting to the funny part. During this time my friends got tickets to a Justin Timberlake concert. He has been my boyfriend for most of my life, so I knew no matter what I would be there! The concert was in an arena and our seats were about fifteen rows down a flight of stairs. My friends stood next to my wheelchair at the top of the stairs and looked at me with a question on their faces. I then proceeded without hesitation to shimmy and sit down on the (incredibly grimy) stair case with people walking all around me and step by step slid down on my butt until I reached our row. Absolutely no shame to my game. Justin if you’re reading this, look how far I’ll go for our love!
Miggy: How can people best approach or respond to you? Is there something you wish other people knew so as to avoid awkward or hurtful situations?
Corinne: Over the years, as is the case with many in the “disabled” community I have gotten insensitive questions and uncomfortable stares. “Did you get burned?” “What’s wrong with you?” “What happened to your hands?” “Is it contagious?” “Is it painful?” And sometimes plain avoidance can be just as painful. When I was briefly in a wheelchair my friend was wheeling me into the movies and teenage girls passed by warily and quickly. My friend commented, “why did it look like they’re scared of you?” It’s a strange thing, sometimes people are so uncomfortable with facing differences, it touches a nerve that most likely has nothing to do with you. When I was younger I just wanted to blend in and would cringe with embarrassment if I was asked what happened or to explain my disorder. As I grew up I realized people are only afraid of that which they do not know or understand. And here’s the thing, it is certainly not your job to make everybody in the room comfortable or share you story from the rooftops if you are not ready. I have learned though that the more comfortable I am in my own skin and the more I am able to articulate my needs, that in turn gives everyone else the permission to relax and do the same. The worst is when someone calls you out in front of a crowded room of people, so if you do have a burning question about my body please have the respect to come to me quietly, one on one. Also, EB sounds scary when described, but you can touch us without us crumbling, Hugs and other appropriate forms of touching are welcome!
Miggy: Lastly, is there anything else you’d like the world to know about EB or disability in general? Do you have any advice for a family who is just starting on this EB journey?
Corinne: First off, I just want to share that I am just one perspective with EB, we all have unique voices and ways of experiencing the world. I would say it is very important to learn what is right for you and what feels comfortable. It may be getting super involved in the EB community, but it may not, and you don’t have to beat yourself up over that. It’s also extremely important to learn to be your own advocate. From medical appointments, to school systems, to relationships, it unfortunately goes with the territory that we have to work a little harder. In order to succeed and be safe we must acknowledge and articulate our needs. This isn’t always easy, but it is empowering and vital.
I just want others to know for themselves, what has taken me a long time to figure out, (and is still a work in progress.): There is no shame in having a illness or disability, I actually prefer the term uniquely-abled. You can either choose to focus on all that you are “lacking” and what sets you apart in a negative way, or you can start to uncover all of the wonderful ways in which your circumstances have made you unique, and empathetic and powerful. Sometimes in the day-to-day I’m guilty of focusing on how much pain I’m in and how it’s going to hold me back from doing what I want, that I don’t even realize I’m doing things and living my life. Right now! I’ve gone to college and grad school, I’ve been a bridesmaid, a best friend, a performer, an artist, a counselor. I have a memory bank of moments where I’ve laughed so hard it hurts, and misbehaved, and witnessed incredible things.That is my life, EB’s just another part of the puzzle.
Great spotlight, thank you Corinne and Miggy. I've seen profiles of children with EB, and Corinne's perspective as an adult living with EB was interesting and thought-provoking. I appreciate her candor and enjoy her spirit.
Corinne, you're an awesome writer! Thank you for sharing your experience and insights with humor and depth.
Corinne, Kudos to you for your courage in sharing your experiences as a unique person. Thank you for being so open and optimist about life with EB. If you are able to make one person laugh, to help one person cope, to help the world understand uniqueness, what a gift you have provided. Thank you so much!