For EB Awareness Week (October 25-31) 2018 I would like to touch on one of many complications of Epidermolysis Bullosa which is skin cancer: Squamous Cell Carcinoma. I believe it should be spoken about more frequently, though, I can understand the heartache that comes with any type of cancer. *Photos will be linked below.
Squamous Cell Carcinoma is a fairly common skin cancer seen in adults (without EB) due to years of sun damage and/or overuse of tanning beds. The nose, cheeks, ears, chest, upper lip, scalp, and hands are most commonly affected by this cancer. The cancer is usually treatable with little to no complications but can result in death if undiscovered and untreated. For those with Epidermolysis Bullosa, however, the cancer is not usually slow growing, especially in my experience. While skin checks are tiresome and an addition to the already load of doctor appointments, skin checks are essential to save your life. If something appears abnormal or isn’t healing, this should not be ignored thinking it’s “just EB”. Even if your doctor says they don’t believe it’s cancerous upon examination but something seems off, push for a biopsy. It has happened to me. It doesn’t mean you have a bad doctor, sometimes diagnosing ailments with EB can be challenging. SCC’s appearance can differ greatly in EB patients. SCC mixed with EB combination causes cancer to become a wildfire—it can become invasive quickly even metastasizing, unfortunately, resulting in death due to the very limited treatments (chemo usually is not an option or recommended). There are different medications that have been proven to help slow and kill cancerous cells for those with EB. I, myself, have not experienced these alternatives.
SCC has been in and out of my life for more than half my life now. You can read more in-depth my first struggles with SCC, etc: right here. I had SCC removal of my right arm back in 2016 without much complication, though, I had questionable painful (even red and some swelling) built-up keratinous areas on my feet, hands, leg, and knee. These built-up areas were abnormally tender. Even after much soaking and moisturizing, these areas grew back in a couple days. Usually, wounds become abnormal with squamous cells but later in my adulthood I discovered it’s not just opened wounds that could be cancerous—even unopened areas can be cancerous. I also had a small wound on my left arm that started to develop that wouldn’t heal, at all. It became increasingly painful over time. The pain started radiating up my arm and neck, telling me it’s definitely bothering vital nerves. The wound started out as dime sized then opened up to half dollar size. Another indication was the wound’s appearance was similar to a “perfect” circle. The edges of the wound did not have any new skin growth over time. Usually, a “healthy” wound has uneven edges displaying activity of new growth over a few days.
You’re probably wondering why I waited so long? I had been on the fence about the differences from my past tumors/skin cancer by its appearances. I also had been battling financial and legal burdens at the time. The hospital is 250 miles one way which is obviously challenging especially having to sit in a car after surgery. Unfortunately, there is limited support nearby for me especially catering to Epidermolysis Bullosa. Planning the surgery itself was tiresome because most of my past surgeries here in Utah have been done without putting me under.
I had seven areas of removal. The areas were covered in INTEGRA to serve as some protection and jumpstart healing. Six of these areas were questionable spots over the past 2-3 years. Five of the seven were positive for squamous cell. Two were invasive and the rest were in situ (means stays within location found, not invading neighboring tissues).
The very invasive one on my left arm was the most painful before removal. It still has cancerous cells meaning I will require another surgery soon. My lymph nodes under my armpit and left breast were increasingly tender and grew painful. With a dermatological disorder and constant bacterium, lymph nodes are usually enlarged in EB patients. Determining whether it is related to the skin’s normal breakdowns versus cancerous cells traveling throughout the lymphatic vessels into the nodes—comes biopsying the lymph nodes with a thin long needle. The doctor will do 3-6 passes into the lymph node under ultrasound, extracting cells to hand over to pathology.
The mental: I honestly did not want to do this surgery for a multitude of reasons. No one ever wants to have surgery but there are moments you feel like it’s just too much. Waking from surgery you experience an immense feeling of gratitude each time. This surgery was my biggest challenge I had ever experienced in my lifetime. It took away my ability to walk without the use of a wheelchair full-time, hopefully temporarily. Doing physical exercises to stretch the tightness of scar tissue forming, to prevent these areas to heal “frozen” in place. Feeling the ripping and bleeding that comes with it causes nausea. The frustrating sleepless nights due to discomfort. On top of the usual EB pains, there were now seven surgical wounds causing an endlessly loud hum throughout my entire body. It feels like a sensory overload. Unable to walk or leave the bed often causes painful bedsores, new wounds amongst areas that were stable over the years. A few weeks go by, the infection starts to set in. Taking heaps of different antibiotics wrecks my body. There were days I slept 15 hours or days I felt a force pulling my body down to sleep. I just wanted to sleep and sleep (and for once, I’m actually not anemic.) There were a few moments I wasn’t doing well—not over exaggerating, there were a few close moments. I’m still battling these infections and will require further tests to try to (hopefully) get a handle on them. Infection is sneaky and can claim a life quickly. In the midst of balancing life, health and then your emotional and mental state to calm yourself that you may be living your last day. There are many different emotions and death can be looked as a freeing experience—that’s often our perspective when we aren’t living an illness or injury. When facing hard moments physically and mentally, you have to hold to what’s good and what’s pure and try to release those fears. It doesn’t mean it won’t be scary especially when it has been your daily. In these recovery moments, in your most vulnerable and lonely days, sometimes all you can do is cry. Crying does not mean you haven’t been strong. Jesus wept. I believe that emotional response was instilled in us for a reason—to release/reveal pains, to show empathy to those hurting and even in our happiest moments we can cry. I often run from crying but it’s something I am learning to embrace in my downtime. I wish I could have told my younger self, “Just cry sweet one.” Even though EB deteriorates my physicality, it cannot rob my heart and soul.
Keep conquering. Hold dear to your loved ones. EB won’t be forever.
*DISCLAIMER: Not for the faint. If you wish to view photos of the aftermath of removing Squamous Cell Carcinoma, enter the password: ebscc at the following link: SURGICAL WOUNDS.
Seeing the #MeToo statuses are sadly no surprise to me, but it makes me feel not alone in the burdens I have carried: the feelings of shame. The reasons behind why I hated myself. The reasons that confident girl kind of disappeared. Sexual harassment/abuse is something we have endured since man walked the earth. It does not make it right. Not one human owns my body nor do I own theirs whether man or woman. In my belief, my body is a temple and there are parts of me that are and should remain sacred, or so I thought.
It’s important to raise your children with awareness interacting with others as well keeping close tabs on their internet interactions. Nothing can be 100% full-proof but knowing everyone is on the page, being aware of the warning signs can save someone from years of mental, emotional, and physical turmoil.
I’m trying to muster up sharing a couple of my stories here. There are too many harassment stories to tell and as long as I am alive, we will encounter with some kind of sexual harassment nearly every day. For me, personally, this is not about getting back at my abusers—it’s just fact. I’m not at all looking for pity or apologies from my audience. I want this to be public because my life story can be a lesson to our future or simply touching with someone who has been similar instances. One day, my disease will most likely be the reason for my end here on earth, but sometimes our death can be the start of a huge impact on others that could save a life.
As a child, my mom was teaching me some lessons of responsibility by checking the mail independently. There was a neighbor across the street who seemed friendly and would often speak to my mother, in a neighborly fashion. On my daily mission to check the mail said neighbor was acting oddly and was talking me in an inappropriate way, trying to lure me over to his apartment. I ran. From that day, he would avoid talking to my mom at all costs. I remember seeing him at a gas station, he hid from us appearing uncomfortable by the look of me. If he didn’t have bad intentions, why the shame I thought and, somehow, I felt like I was invading him.
I became friends with someone around the age of 12, someone that I thought enjoyed my company and my personality. He was a bit older than me so he was a teenager. When I grew older I tried excusing it to him being hormonal. Thing is, I did not want nor asked for these inappropriate actions. Anytime I tried hinting or sharing with anyone, “You allowed it. You knew right from wrong.” He exposed me to pornography. He exposed me to lewd acts. I didn’t want that. I wanted a friend. I wanted safety in a friendship. I was a child. I was numb and drained. It went onto until I was 18. I had been extremely excited to attend college after graduating high school. There were a couple of years I did not interact much with him because I was dealing with my cancer. I stupidly talked to my abuser online—it was nothing but sexual with him. Whatever I could do to fulfill him sexually, I did. He was attending the same college I wanted to attend. I told him I was gearing up to apply. He explained what areas of the campus he would take me and outright said, “I’m going to rape you.” I broke. I lost it. For the first time, I felt murder in my heart. I felt crushed. I never thought I wanted someone dead. The rage was more than I could endure. While I threatened to go to the authorities, I was frozen. My mind kept saying, “This is your fault. They’re not going to do anything.” I started abusing narcotics to numb my emotions. I wouldn’t sleep for a day or two at a time then sleep the day away, rinse and repeat.
All of this is… incredibly difficult to post especially in the public eye. A bit before all that occurred with said “friend”. I was touched inappropriately, every morning from 5:30-6am by someone in a professional setting. A place where you are supposed to be safe and feel safe. For years it would play it repeatedly my mind, “Maybe I am overthinking this. Maybe they were supposed to do that.” Even to this day, extreme stress or grief can cause flashbacks to those moments.
In my search for decency and healthy relationships, it allowed more unhealthy instances to cross my path. I thought it was how I was supposed to be treated by men. I was their puppet and they had to be pleased. Unhealthy sexual relations for years. Deep down I knew it was not how anyone should be treated. Anytime I was the “big sister” anyone, I would become overprotective and a bit neurotic whenever they were dealing with their own relations with guys. How I wish I could have taken my own advice sooner rather later. I have come to healing and relearning the importance that sexual harassment is NOT OK. Anywhere or anytime. Toxic relationships are NOT OK. You are worthy. We are worthy.
My mom received a lot of pent-up attitude and rage from me as I was not one to express my emotions very well to her, at least when it came to the darkness and sadness. For this, I felt awful. I did not want my mother to feel like a failure. Parents, watch for signs. Talk with them. Hold their hand and say, “When you’re ready, I am here when you need me. No judgments.”
I loathed anyone’s hands on me. I didn’t matter who you were. I did not hug my best friend, Sara, until the 10th year of our friendship. There was a time I loved being hugged then it turned into something I avoided. It made me feel claustrophobic and, on hard days, it still can be challenging.
There has been much healing through prayer, therapy, and journaling. Some days are rough but I learned to hug those I trust. I love showing love and I don’t want my perpetrators to destroy the beautiful person God has built me to be. We are not obligated to share our stories, no one is. So if you are reading others stories and don’t wish to share yours or you feel bad for not sharing your story, stop right there. Do not. This is not a competition or a rule. You are just as valid. You are not unnoticed. For me, the healthy vulnerability sheds my shame. Shame is the root of my depressive meltdowns. It has been one of those things I believe we are taught not to share. Just shove it away and move on. Awareness is so important for change amongst our hearts—so we can treat one another with respect. Man or woman. Look at the heart. Next time you interact with someone, ask yourself, “Am I respecting this person?” or better yet “In what way am I not respecting this person?”
To my perpetrators: You made me hate something that was meant to be beautiful: sex and my body, overall, just me. You made question my self-worth. You made me do things I did not want to do. No does, in fact, mean no. There is no such rule, “If a woman says no, it really means she wants you.” Hatred festered in my heart, but it did not win. You did not win. You will know and understand what you have robbed of me and others. Every evil thing we do will have consequences.
But I’m not crazy, I’m just a little unwell
I know right now you can’t tell
But stay awhile and maybe then you’ll see
A different side of me
I’m not crazy, I’m just a little impaired
I know right now you don’t care
But soon enough you’re gonna think of me
And how I used to be, me –Unwell by Matchbox Twenty
“Connection is why we’re here; it is what gives purpose and meaning to our lives. The power that connection holds in our lives was confirmed when the main concern about connection emerged as the fear of disconnection; the fear that something we have done or failed to do, something about who we are or where we come from, has made us unlovable and unworthy of connection.”
― Brené Brown, Daring Greatly: How the Courage to Be Vulnerable Transforms the Way We Live, Love, Parent, and Lead
I am not sure if I will post this on my blog or not. I go in and out of being transparent and vulnerable to the internet nowadays. We can be so quick to judgment and misunderstanding with anything posted even if they are our own thoughts and feelings. Somehow you are shamed for showing emotions. It makes me question keeping this blog but there is a part of me that writes for therapeutic reasons, in hopes that another individual going through similar trials can benefit. Maybe it can help that one life to say, “They understand.”
There has been a plethora of gains in my life and accomplishments I had never seen myself doing or participating in. I have felt so incredibly grateful and gifted with new relationships developed.
Here’s to vulnerability and transparency…
I have been dealing with an unknown feeling of unwellness for the past 3 or 4 years. Concidently, it all started with my esophageal strictures and these “feelings” increased over the last 2 years. The last several months has been a rollercoaster. I have been to a few doctors; in and out of the emergency room leading to extensive tests. It is most infuriating to hear comes back normal yet you cannot function on the daily. When you are already have limitations, not only due to your disease but situation and location, it can be isolating. I cannot and am too afraid to leave my house. There are days I have panic and anxiety attacks out of nowhere. I read, mediate, reflect on fulfilling verses. Embarrassing but sometimes I sit in a dark closet to calm my sensory/emotional overload. Fighting infection on my small wounds are becoming problematic more each day. Sometimes my thought process is foggy, forming simple sentences can be a challenge. Sunlight feel like a daggers throughout my nervous system once the light hits my eyes. 1500 mL of water does not keep my hydrated, my skin suffers from lackluster. My tongue feels like sandpaper if I utter a few words. My extremities constantly tingle. I feel fatigue and sleepiness after any activity even eating. Low grade fever every day. Migraines are every other day. My abdomen is tender and sore, hard to the touch at times. My blood sugar is all over the place yet I am not a diabetic. The constant urges to vomit but you cannot due to strictures and preventing the excuriating tearing of the esophagus which lead to bleeding. I can’t exercise like I used to and it makes me feel not-so-great about myself
Crying is nearly a daily occurrence for me. I remember there were years crying did not come with ease until my dear friends passed then the repeat, I did not cry for months. Now, it’s like if I don’t have my crying session each day, I feel like I cannot face anyone… not even my happy bundle of joy, my Lunabug.
I have needed oral surgery as of 2013.
There are pre-cancerous and cancerous spots that need attention.
On top of that, my life is going through a full transition. A transition that has been a hard choice to come.
I have only felt guilt and shame because I just… do. While many are so over the moon proud of me: why can’t I, for once, be proud of me?
Feels like I have and am being completely stripped from inside out. In every loss, you can only hope there will be gain.
I am known for not always really saying how I am truly am because my heart gravitates toward those less fortunate, but taking my own advice and the advice of others, pain is pain.
This was not easy, but here’s to healing (again…)
Another trip to Yosemite with my bestie completed. Every year we are surprised by the differences each year. It just does not get old for me. Some years have been dormant and desolate, needing its thirst quenched. A season like this, the thirst is being quenched with purifying waters which should reveal the enrichment of life—whether new or old. It makes me reflect upon the transitions of life. There are moments in life where everything is beautiful, seemingly unflawed until the storm or fire rolls through, pruning the good and the bad. I view Yosemite quite like how I see life each day. There are so many mysteriously beautiful parts yet there are bruises from the battles of the elements much like life itself. It all depends on perspective and attitude on how you view things. I try to strive to find the beauty in everything.
I can’t say enough how incredibly blessed I am to have a friend that has chosen me to travel with her. I am thankful for keeping dear to me selfless close friendships. Those are rare to find. They keep me smiling and laughing—to remember who I am truly am and strive to be.
Life lately for all of us has been mind numbing, blinding… rough is putting it lightly. I am not going to get too personal publicly, but there have been instances in my life and others I never thought I would witness or experience. As cliché as it sounds, never say never. Any scenario in life is possible, but it is how you respond and learn from such trials and transitions. Grow from it. Don’t let pain or hate callous you. We have plenty of that swarming amongst us.
The sun shines not on us but in us.
Thank you for loving concern, prayers, thoughts, etc. Today was a long day for everybody. My dilation was delayed due to unexpected surgeries that took priority over my procedure. I was under for 2 hours for a 30-45 minute procedure because my wonderful GIs went very at a slower pace. This was my first official “real” dilation in my entire life with EB. They were terrific. It was also my first time being intubated by an anesthesiologist, too. Everything was done with a wonderful tender hand. I feel like a new person. Back story: I had an attempt for dilation in Utah with the best GI doctor in the state, but he felt was not experienced enough in Epidermolysis Bullosa. Furthermore, he felt he would perforate my esophagus. While feeling hopelessness after his decision, I am very thankful he turned me away for future procedures. I’d rather be miserable awaiting that special person to help me than deal with irreversible consequences. His heart was in the right place.
What I am about to say is not a cry for attention or pity: It has been a challenging 6 years, honestly. I have shed many tears. Had many counseling sessions. There were many scary moments within my own head. I know a multitude of us have gone through numerous events in life. Things we never thought we’d go through or live through. Little by little, my social life has been shorten just trying to survive. Thankfully, I have friends’ that have chosen to stick close even when I just wanted to hermit. I have not been the best daughter, friend, stranger, etc.
I waited 3 years for a proper diagnosis from hospitals back at home to explain why my chest was giving me issues (since I had not experienced a very small stricture–I was stumped.) I was just given pills or diagnosed with anxiety. I had anxiety and some panic attacks, but I wanted to know why. I only received 3 hours of sleep per night. Food pooled in my upper chest. I felt an elephant on my chest constantly. I was “hangry” all the time. All the time. I suffered dehydration. My stomach grumbled every hour around the clock. Yes, it has sucked. On top of that, I have had dealt with personal battles from different directions. I would rather not delve into it publicly online just yet, or at all, but I am letting you know, you will preserver.
Sometimes I only post happy shiny posts. Social media really allows us to filter our lives to “perfection”. People get this idea I have money, that I can travel whenever I want. Again, all blessings of dear people who have been beyond generous. My travels have been pleasure, but mostly medical as of late. As of now, I get $17.15 per month for my personal income. It takes me over a year just to save for small selfish purchase for myself. I am not complaining, just being raw. This is my reality. I sometimes forget, too. There’s no such thing as perfection. I simply wish to show positive more than negative; it’s what I hold dear to most often. We deal with a lot negative in our world today. Without some of those flaws, bumps, and cracks, we would not have a new stepping stone/hurdle to climb to a decent outcome and outlook on our strength. [Un]fortunately, discomfort is a necessity to get ahead and teach us something new–rebuild grow, renew. Without a climb, how do we build muscle? Speaking within self. Through such negative or discomforting moments, I usually refrain from spreading it, or become calloused by the discomforts of life. It causes a cycle of a hardened shell. I am not guiltless of this. It happens. I would much rather smile through it than be unhappy.
Stanford Hospital experiences, for the last 2 weeks, all around have been phenomenal. Every doctor, nurse, coordinator, social worker… truly a godsend. They kept telling me how blessed they felt coming across me as a patient, but I felt they were the angels. I saw their sacrifices, not just for their families but their “hospital family” as well. With with my depression and lack of self worth the last several years, I thanked God wondering how I deserved such personable loving people? The true medicine was the laughter we shared, however, just listening to each person’s story was an eye opener leavinng me inspired.
I am going home with a full heart…
Sometimes things in life are not always fluffy bunnies, rainbows, and butterflies. I try to thrive on writing about positive things in my life, but that would not be practical or relatable.
There have been awakenings in my life that are becoming more prominent with age. I have held onto rather unhealthy relationships in my life, we all have or experience them. My life does not equate as a higher placement than anyone else does. However, I do believe I have issues being an enabler of people’s weaknesses’ especially when it comes to using a person for their benefit. Trust me, close friends’ have told me I need to stop “being so nice” over the years. I am learning a healthy boundary amongst certain people in our life is okay. Years ago, I felt as if I was letting a person down if I did not bend over backward for them, though, I was feeling major discomfort. In most instances, I felt as if my actions to please that person was from my heart and would suffer whatever discomfort I endured. Thing is, would that person do the same for me? The likelihood was null. That does not mean I expect something in return every time I do something from my heart for someone. You know the genuine difference when that person’s intention or need is authentic. However, if that relationship is a one-way street, that can be incredibly dangerous to all parties.
There had been an uncomfortable and disappointing situation I was put in the middle of a few months ago. I was angry, hurt, disappointed, but of course, I was going to do anything for that person. It was revealed to me vividly in my heart, I need to speak up for myself, finally. One of the said parties ended the relationship between us because they were not getting what they needed. The old me would have blamed myself endlessly, trying to win the approval of that person back. I allowed myself to feel the emotions for a good solid 2 days before replying with a respectful letter, but strictly standing my ground, in love.
This journey has been extremely painful to deal with because that person was dear to me, but I hope I can see I am capable of loving even when sometimes we must part ways, whether with a family member or friend. We won’t please everyone in this lifetime. There are times we have to feed them with a long handled spoon, spoken wisdom of my grandmother.
Beautiful, beautiful Yosemite we meet again. I can recall seeing Yosemite semi-snowy back in 2010 and 2012. When the drought started taking a toll on the west, Yosemite just appeared in need of precious moisture. My friend, Saries, has experienced one proper snowfall in Yosemite in the 90s, but I had not experienced it myself. Cold, cold nights, wind, rain, but never a proper snowfall in my home state, California. We both wanted to experience a wonderful proper snowfall, a snowfall that actually stuck. The forecast was not promising a few days before my arrival. We arrived in Bass Lake where Saries’ timeshare is located. There were patches of snow on the ground. Still, we did not get too excited quite yet. The next day, we drove to Yosemite Valley, it was mostly cloudy, cold, and some low clouds. The air was extremely brisk…
The following day, I woke up extremely ill, unfortunately. I was so disappointed as I was looking forward to exploring much more of the park. I could hear the relaxing pitter-patter of continuous rain upon my room’s window. Though I was completely weak and unmotivated to move, hearing the simplicity of rain put a pleased smirk on my face. I exhaled in bliss, “Finally…” Resting in my room the entire day, the rain continued into the depths of the night. The next day, I felt a surge of energy and less fevered. I was not going to allow illness ruin our trip. We left early AM since sundown came early. We gassed up and headed onward from Bass Lake. I vaguely noticed two men dressed in bright yellow, but my mind did not want to comprehend the possibility. I did not say anything to Saries because I didn’t want to cause excitement then it was nothing spectacular. As soon as we hit around 4,000-4,500 feet, “Are these… flurries?” Saries asked. “Yes…” I could feel the tension of childlike excitement between the both of us. As we climb higher in elevation, the road starts to steadily turn white. The flurries were transitioning into snow flakes. Up ahead, there was a highway patrol vehicle and a ranger directing traffic to put on snow chains! Remember those two men dressed in bright yellow attire I mentioned earlier? They were who I thought they were. Chain installers. We headed back down that way so they could install chains upon her car’s tires. After the successful installment, we headed back up. We were super quiet for a few minutes, and if we are both quiet while together, something is brewing. Saries finally let out a high-pitched scream of excitement. I could literally feel it brewing and was not surprised by her exclamation. She screamed enough for both of us, honestly. I laughed so hard. Snow flakes. Large small hand sized snow flakes falling. I pictured us in this snow globe. It was completely dreamy…
There had been some medical business I had to attend to in Northern California back in November. I had asked if my bestie if she could accompany me and drive me up from Los Angeles to my destination up north. Of course, with her kind heart, she agreed to the long travel. We, of course, made it a small road trip of great adventure coming back down to Los Angeles. For several years, we have been wanting to do a proper coastal trip from Los Angeles all the way up the Oregon coast. This was a last minute trip for me, so obviously we could not extend our trip, but we could not just pass along such beautiful spots.
Let the photos speak…
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 in 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 Justin). 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 what 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.
I try not to allow EB define me as someone that is just “a disable”. Put your all in anything, you will overcome.
We beat EB every day.
A year without our amazing Jamie Hartley here on earth, but she is still very much remaining in our thoughts and hearts. I am in awe how a year has passed already. Trying to continue life without that person can be so challenging. Some days, it can feel almost impossible, but it is possible. Holidays and birthdays; the special events that bring people together, those memories you have to hold dear. Even in the midst of problematic moments in your life or theirs, remember how you had each other. My plethora of thankfulness to know such a genuine soul—had I not known Jamie, my life would be certainly different, in a negative way. The rawness of Jamie’s passing is still there for me, but each day what felt like a non-healing wound is slowly healing. I have reminded myself how I know Jamie would not want me sad, but it does not lessen the fact I miss her, immensely. If I hear any version of Ave Maria (follow the link to hear her version), I usually have to switch the song because I will weep right there, no matter what the location. It is one of my favorite songs Jamie covered with her beautiful singing voice. It’s just too bittersweet. I have not been a big flower person, but now I appreciate poppies and orchids as they were Jamie’s favorites. Any time I spot one in the wild, I smile.
Gifted with a huge part of Jamie’s life this April, was her dog she affectionately called “Poochini” also known as Ali. For me to have her, I cannot put into coherent words, just endless feelings of love. My dog, Luna, has known Ali since Luna was a puppy. They have been bonded for years, so the transition went exceedingly well. Ali has her own wonderful persona, but there is the likeness of Jamie’s gentleness, concern, intelligence, and joyous smiles instilled within Ali. Jamie did well training such a faithful dog. I thank Taylor and Jamie for putting more joy into my life. I hope Jamie would be proud of how I am caring for her Poochini.
Although I miss you, Jamie, I am happy you are no longer in pain. You fought hard. You never gave up. I am so happy you are no longer suffering. You were just called to the next chapter of something wonderful.
We all love you, Jamie.