By Emily Spurrier
From her Blog: Dys-Located

This is the fifth in a blog series on Epidermolysis Bullosa (EB) as part of EB Awareness Week (October 25-31).

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Following the demise of my first serious relationship, I figured honesty was still the best policy with those I had dated. With the first potentiality, however, intimacy was not an option, for he feared injuring me due to my EB. I was crushed. My worst dating fear had come true. I reverted back to my “Don’t ask, don’t tell” personal policy.

The following April, I had no choice but to tell.

My roommate and I had some friends over for dinner and beer. Around midnight, I tripped on an air mattress we had on the floor, fell against the sofa and completely dislocated my left knee. I landed, stuck between the sofa and the air mattress, on top of the feet of one of our guests. We called an ambulance and for the next three hours, he and I talked about the wonderful time we were having.  Though the ambulance showed up within a short amount of time, my morphine allergy prevented them from giving me anything for the pain except nitrous oxide. Inch by inch, they moved me to the gurney. By the time they loaded me onto the ambulance and got me over to the hospital I was delirious.

Once at the hospital, I was given an IV with pain medication. I insisted that they not use adhesives on me, and it was listed on my medical record to not use them at all. I begged for a tourniquet and gauze to hold the IV in place. “Oh, it’s OK,” said the nurse as she taped the IV to my arm, “It’s hypoallergenic tape.” I didn’t have the consciousness about me to rebut her any longer. Next thing I knew, the doctors and nurses were counting to three and they popped my knee back into place. I later learned that my screams could be heard all the way in the lobby, where my roommate and male guest were waiting. The second scream came about half an hour later, when they ripped the tape off my arm. It was then that they discovered just how serious I was about my ‘no tape’ policy. I later told my male guest that he should have come back with me, to translate my persistence into English. He stayed at my house for another two days to make sure I was OK. We watched Monty Python together and he laughed at my Vicodin-induced antics. Two years later we were married.

I can’t wear heels of any form. Most nice flats only last a couple of hours on me before my knees give out, blisters form around the outsides of my feet and my lack of ankle support. I was married in sequined slippers. Thanks to them, I was able to walk down the aisle, dance with my father, husband and Man of Honor. I did the Electric Slide, the Macarena and only remember sitting down for dinner and removal of my garter. I did have some significant trouble moving the next day. We were forced to cut our camping trip short because massive blistering from the stress and activities of the past week and being one with nature do not combine well. But hey, I achieved my goals.

Now, my biggest worry is staph. Since moving to Arkansas in 2005, the heat and humidity have caused it to come more often. In fact, three years ago, my right elbow swelled to the size of a golf ball, I had blistering all up and down both legs, and I was breaking out into cold sweats and shakes. I was placed on Doxycycline, which we quickly discovered I have adverse reactions to. The pain was intense and I was throwing up with violent frequency.  A trip to the doctor showed that I was near systemic. I immediately switched antibiotics. The stresses at home and work had impacted me to the point where it made me sick. I was put on an aggressive round of Dicloxacillin and missed a month of work. Finally, while I was still on leave from work, my husband took me on a fishing trip to Southern Missouri. After a few sunny days on the boat, I was feeling much better and my skin almost immediately cleared up. I started searching for another job, but felt that the only thing I could do was more customer service. I would be right back in the same unhealthy position. On the flipside, however, a sitting job was what was best for me. It was a long process of finding exactly the right job.

In February 2009, I had one more foot surgery to remove a spur on my left foot. It was hurting and hindering what ability I had to walk. I was put in a  soft cast and was given crutches to use. I couldn’t use the crutches, as they immediately caused severe blistering on my arms and hands, making me even further immobile. The pain caused me to move slowly, thus making it impossible for me to make it to the bathroom, or wherever I had to go and back to my desk in the allotted time I had while working in a call center. In such an environment, any form of tardiness could result in disciplinary action.

One day, still unable to put pressure on my left foot, I attempted to get up from the sofa using only my right foot. My knee slipped out of place and I fell to the ground. Slowly, and in tears, with the help of my husband, I made it back to the sofa. I took another leave of absence and requested approval for a wheelchair.  It had been fifteen years since I’d used one and the pushing of a manual chair was causing blisters on my hands. I couldn’t go on like that. I urged approval to go ahead and push me ahead to go into a walking boot as my husband could no longer help me get around. The walking boot, however, was no picnic, either. The constant rubbing on my leg from the walking boot caused blistering that no amount of cloth barrier between it and my leg could prevent. Within four weeks, instead of six, I was out of the boot and walking normally. I still took another two weeks off, however, to ensure my amount of walking was limited and to give myself time to heal from the infection that the boot kindly gave my leg. I was on Ciprofloxacin for two weeks.

Over a year later, however, I was still working for the same company and was taking FMLA leave often. By June of 2010, I was having anxiety attacks combined with uncontrollable infections. My health was my first priority. I had to leave. I left my job of five years. I left my mid-range five-figure income and stayed home with my daughter living off of savings. I hadn’t felt so healthy in years.

Since the birth of my daughter, I had been getting inexplicable rashes in random places. They’d appear after wearing fleece, but we ruled it out as being an allergy as I’d also get them after picking her up. They’d erupt late at night. We ruled out detergent and red dye.  I’d get them on the tops of my feet. We eventually ruled out my socks. My mother had breakouts as well and she was eventually diagnosed with eczema, which is hereditary. I asked my dermatologist and he confirmed that I had indeed inherited it from her. I had eczema in addition to my EB. The two are treated completely differently and yet, one is always causing an outbreak in the other. Sweating, slight friction and stress cause my eczema to flare. Scratching the resulting hives, more stress and friction causes the rashes to develop into an EB-related mess. Today, it’s a constant mix and careful balance of creams, antihistamines and antibiotics (most of which I’ve become resistant to over the years).

I am working a job that causes significantly less stress and is much more accommodating to my health needs. As an added bonus, I’m working in the field I went to school for again. EB isn’t as much of an issue as it was as a child, obviously, but it’s still hovering above me.

This past Friday, as a matter of fact, I was on my feet from 6 am to 10:30 pm. I took my daughter to a carnival and though I could feel my feet swell from an erupting blister I let her go on “One more ride, mommy.”. Sometimes the reward of seeing her joy is so worth the sacrifice I make, even if it is painful.

Tomorrow will be my last installment for EB Awareness week… and probably the most emotional journey I had to take on this road to date. Stay tuned, as you’ll notice I left out a pretty significant event in my life in this entry…

By Emily Spurrier
From her Blog: Dys-Located

This is the fourth in a blog series on Epidermolysis Bullosa (EB) as part of EB Awareness Week (October 25-31).

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By the time I was fourteen, I was able to walk long distances, but could not do so in shoes. I was in an adapted physical education class because I was not able to do pushups. Pull-ups would shred my hands. Asking me to run sprints was like asking a turtle to play fetch. It was impossible. I had days where I would come to school in slippers because my feet were so swollen and sore. Other days, I would walk a mile.

That same summer, I had my first of many orthopedic surgeries. Because the pain involvement on my left foot was often greater than that on my right foot, I had a tendency to walk on the ball of said foot, while the right foot remained pronated. My achilles tendon was lengthened to help bring the left heel down. Now, I was wheelchair bound with a cast for the next six weeks.

Shortly afterwards, we moved from the Los Angeles area to Minnesota. Almost immediately, my skin started healing faster and my endurance was higher. As previously stated, however, I was depressed and immensely self conscious. My feet were my biggest issue. Shortly before my sixteenth birthday, I had a second surgery to completely reshape my right foot.   Years of walking incorrectly had collapsed the arch on my right foot and caused several of the bones that are naturally on the top of a foot to shift to the inside. I spent another six weeks in a cast, found out I was allergic to morphine and learned that I could never use crutches. A week later, my aunt died suddenly.

Outside of school, anonymity had become my friend. I acquired a pen-pal through an international service. I consciously chose not to tell him about my EB. I didn’t want him to treat me any differently than he would treat anyone else. I held my tongue often when he would ask me about outside activities, but still shared with him stories about my friends, trips to the mall, and most any other typical teenage angst story that was common for anyone my age. Within a short time, we were sending audio letters back and forth. He talked me through getting over the death of my aunt, the loss of a close friend and several other issues that contributed to my depression, but never did I mention to him that I had EB. We remained close until we were 18 and then, the letters stopped. To this day, I credit him for being that person to talk to and open myself up. It was him who helped crack my shell.

By the beginning of college, I was no longer using the wheelchair, at all. I walked to all of my classes (in shoes… sometimes boots) and conquered the Mall of America on foot. When this new-found freedom came boys, parties and the college and life experience that I personally felt I had been hindered from for so many years. I rarely told people about my condition, and felt that it was only a need-to-know basis. At the same time, however, I’d wear shorts or tank-tops that obviously revealed my scarred legs. This became an issue at one point a few years later… One that I never thought of.

I attended three colleges between 1992 and 1997. I had many friends of both genders. My best friends, however, were male. They were my protectors, my rocks and my brothers that I never had. None of us ever talked directly about dating each other. It was sort of an unspoken rule between us all that we never would.

While in college, however, I managed to dislocate my knees twice. Once, it required an ambulance ride and an immobilizer. My years without walking had weakened my knees and caused my kneecaps to grow higher up than other people’s. This instability led to countless rounds of physical therapy until my muscles strengthened enough just from walking to keep my knees stabilized. Additionally, arch supports had become mandatory. Without the support in my foot, my ankles still turned inward, causing the rest of my lower body to become misaligned, which, in turn, caused my knees to ‘slip’ in and out of place.

Then, I turned 21. While enjoying one of my only “quiet” evenings of the week in my friend’s dorm, I was asked out by one of her friends. Perhaps it was my rookie status in the dating scene, but we only lasted two weeks. I never told him about my physical health. Shortly afterwards, I accepted a date request from someone I had previously turned down. At the end of the date, I opted to open up to him and tell him about my EB. If it was going to turn him off, it might as well be then… before anything became serious. I approached the topic cautiously, telling him there was something I wanted to tell him about myself and that if he changed his mind about a second date, I would understand.  “I figured that’s what it was from day one,” he replied. “We studied about it in genetics class.” I was hooked. We were together for four years.

In those four years, he witnessed one more emergency orthopedic surgery, and several infections that come naturally with EB. He also witnessed me question my initial diagnosis of RDEB.

With the increasing popularity of the internet came the accessibility to directly speak to others with EB. I discussed my symptoms with others, including my hypermottled pigmentation, elongation of wrists and vast improvement with the onset of puberty. These were all characteristics of those who had EB-Simplex, not Recessive Dystrophic EB as I had been diagnosed with at a very young age. In fact, back in the 70s, many people I spoke with who had EB-Simplex had been misdiagnosed with RDEB around the same time and questioned their diagnoses themselves as they, too, were still alive. Second biopsies had confirmed for them the misdiagnoses. My first biopsy at the age of 2 was traumatic and consequentially caused a phobia of such procedures. Thus, I opted against getting a clinical re-diagnoses and settle for a personal one of EB-Simplex Dowling Meara.

Following my graduation from college, came the question of vocation. EB, naturally, limited my choices for employment. I couldn’t work with my hands. Waitressing was out of the question, as there was little chance my feet could endure an intense eight-hour shift. I assumed I wouldn’t have made much money as an exotic dancer. My potentiality at a career in professional sports was shot at an early age and I would have never survived boot camp had I entered the military.  Sarcasm aside, my choices were limited. I chose journalism, which later led to two years managing a book store and then ten years in customer service.  While at the book store, someone noticed my skin and scarring. The customer pulled me aside and provided me information on domestic violence. She told me that if I needed a place to go, she could help me, that it was never OK for my boyfriend to beat me. I explained to her that he never laid a finger on me, that I’d never been beaten by anyone and that I was born this way. Embarrassed, she apologized profusely and thanked me for understanding…