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.
When I am feeling poorly, I love going on adventurous drives to the unknown. Nature is such a gift to us to enjoy. Earth is one big playground. It is much-like medicine to me. Even if you are not the outdoorsy type, I think it is important to take a moment to quiet the mind, to look around you. There are so many stressful scenarios amongst us, it is often challenging to take a moment to merely look at the moon and breathe. For me, I have always loved the outdoors. Everything about nature appeals to me. I remember when I was a child, I would sit myself near a window and stare at the moon for hours. Just, stare. Absolutely mesmerized in amazement. Trying to memorize each lunar crater placement that my eye could see. It quiets my spirit, giving me that stillness to listen to God while analyzing my growths and weaknesses. His creations brings me back to gratitude when I am feeling ill-tempered. It grows me each time I take an adventure.
Every day, take a moment.
The course of the last several months has been a mixture of fun and health ups and downs. Let’s cover the trying parts, shall we?
My health has been pretty stable for the past 2 years — no cancers and iron level has been stable with yearly intravenous iron therapy and supplements. The past year I have been rather quiet about an issue I have been experiencing internally. I have had a strange lump rollabout in my solar plexus region. The sensation started out feeling small and less troublesome. If I attempted to bend over, it would feel as if a marble rolled from my spine to the front of my chest. It was alarming, but did not hurt. After a few months, the sensations increased and the sternum pressure started. I went to my local hospital’s ER a few times for the fullness sensation I was experiencing in my chest. It felt like I couldn’t breathe, or my chest was craving in. Being safe, I listened to my body. I kept going into the ER and was diagnosed with different issues each time. Though I felt unsatisfied with each diagnosis, I went on living my life and hoped everything would work out itself.
Over the past year, my levels of uncomfortableness increased each day. Sleeping was now a lengthy process (I get about 3 hours of actual sleep per night lately — on a good night.) Laying down was like an elephant was upon my chest. My resting blood pressure would become menacingly low. Not to mention, eating was more of a chore than it had been. Eating binding foods such as breads, broccoli, and quinoa, for example, completely horrendous pressure afterward.
The past couple of months, I had a painful spot on my arm which did not appear to resemble Squamous Cell Carcinoma (SCC), just alarmingly painful. The simple gentleness of fabric brushing against the spot felt like razors. I made an appointment with my wonderful Dermatologist, to perform a biopsy and hopefully remove most of it in one sitting (it was positive for SCC). During the numbing process, I became lightheaded and my chest felt aggravated and heavy, but I could feel myself blacking out. My doctor immediately saw my distress, and helped bring the blood back to my cranium. I spoke with my doctor about the issues I had been experiencing the last year, and he urged me to see my last G.I. doctor, not the local doctors in my small town since they don’t have the specialty to equip my disorder.
After a pre-visit appointment with Gastroenterology, we formulated a plan best for figuring out what may be the issue. Tomorrow, I am going in for a scheduled Esophagram and on July 29, the university scheduled me for an Endoscopy procedure. There is no guarantee whether the Esophagram will answer any questions, but I am praying it does. The exhaustion — physically and mentally — from this has been leaving me on empty. What could it be? There only has been speculation from a few doctors’ and nurses’ that my esophageal strictures (a complication due to Epidermolysis Bullosa) could possibly be worsening and/or developed a hiatal hernia. Whatever the issue, I pray I continue to have faith, strength, and a calm heart.
Bryce Canyon is beautiful. I am often taken back how close I live to such surreal beauty and mystery. Places like Bryce Canyon I could stare willing for hours especially the hoodoos which are pictured below. Photos don’t do justice. I happily captured a photo of the intelligent raven. Some were a bit too friendly because of people feeding them over the years. Please, I know those lovely pleading eyes may be irresistible, but refrain from feeding the wild.
My dear bestie I often visit in California came to Utah to visit for a few days! With the sun coming back out plus Spring slowly coming into play, it really boosted my mood for the rest of the year. Truly, it is amazing medicine to see your closest friends.
The few photos I am posting here are from our first day of adventures: the Antelope Island in Salt Lake City and a few from Tribble Fork Reservoir in the Mount Timpanogas Alpine Loop. Antelope Island is the largest of the several islands of the Great Salt Lake. It is home to free-range bison, mule deer, foxes, coyotes, pronghorns, and many species of beautiful fowl. There are awesome trails and designated bike routes. If you ever visit, plan accordingly, preferably before July-August–less brine flies and rotten egg-like smell. Otherwise, I highly recommend visiting. Please be sure to visit my “Like” page for more photos I haven’t added here!
Going through grief has been a challenging roller coaster. My goal is to share my rawness of grief to others, and I am not saying my ways of grieving are correct. I have wanted to write on grieving especially when I lost one of my best friend’s, Jamie. However, life has been quite hectic followed by a few more people I have known that passed in 2014, last passing being in December. A dear person whom I was very close to, who treated me like their own blood. There is no preparing the tidal wave of emotions, survival’s guilt, and hopelessness felt afterward. The gut-wrenching heartache felt for the immediate family members and wanting to erase the pain and struggles that they may face without that person in their life.
While we all experience grief differently, we are all human. We share the similar emotions: sadness, fear, anger, joy, surprise, and love. For me, grieving is something I haven’t done well, or simply altogether avoided growing up. There is a resistance to sharing my emotions with others. I am almost afraid to cry especially in front of others. There were instances some would say I am emotionless, callous, or disconnected. When, in fact, that was the very opposite of what was going inside of me. In my case, I have had to be strong physically, mentally, emotionally, and spiritually from I could remember. My feelings run deep especially for those I care about. When sadness struck close to home, I would feel sadness followed by anger especially in death. In the past, I would usually let my tears flow in the darkness of night when everyone had fallen asleep. The next morning, I would push aside the sadness and put a smile on my face.
This time around, the grieving was almost unbearable for me to handle. Honestly, it was the first time in my life to really feel the ache of grief physically and emotionally. There were so many lives and scenarios of people dealing with a loss, and furthermore, dealing with knowing those people are gone here from earth. I went through rage, sadness, guilt, grieving, more rage then depression. It wasn’t anyone’s fault but my own. I would cry, but suddenly stop, thinking I needed to be “strong”. Stopping my emotions only led my sadness to lead to depression. The depression was causing nightly nightmares, thoughts, and feelings so dark I wanted to run from myself. I felt guilt because I wanted to comfort and help those in the immediate loss, but it was unbelievably challenging loss after loss.
Someone I could confide in recommended perhaps I should see a counselor. Truly the thought of expressing my feelings to an unknown individual was intimidating for multiple reasons. Years ago, I hadn’t had the best experience with therapy. However, I needed to let down judgment that all therapists were like so. I prayed about it, finding a decent Christ-based counselor in my town.
I have been attending lessons for a few weeks. The sessions have been rather validating. I don’t feel “crazy” especially with certain feelings I have been experiencing within. It is one thing hearing it from someone you care about versus an outside source. It truly has helped me understand it’s OK not be OK. It is also definitely OK to cry. I had a wonderful California trip this month with my bestie. The moments to myself, I just cried and cried. Praying, being silent at that moment. Thinking of those hurting; those who have lost; and those that are gone in the physical sense. The importance is sharing what they have blessed me with internally. Life’s sessions run deep with those that have come in and out of your life—the good and bad. Showing your emotions isn’t a weakness. One of the strongest men who ever lived, “Jesus wept.” John 11:45. Shows He knows our pain and suffering with such deep sympathy. Sometimes we may find ourselves face down from being strong too long. There isn’t anything wrong seeking or asking for a hand to get back up, slowly.
If someone reading this is feeling alone in any type of grief, sadness, and/or depression, and perhaps you can’t get back up. Please, seek help, or talk to someone that will just listen. It is important to mesh with someone that will be of comfort, not judging your emotional reactions. You are not alone. There are many dealing with unresolved emotions, losses, and depression that needs attention. You will be loved. You are loved. You are important. Even if I don’t know you, I want joy for you. Take that step to healing.
Enjoying the Southwest as the summer is coming to an end. The nighttime is already nearing the high 40s, and my tomato plant can’t remain outside much longer. Mr. Tomato Plant has grown to nearly 4ft tall, and has given plenty of sweet tomatoes!
Never fear! That brown remnant is poo of the Earth, not of a mammal.
This was my view on my back home after an intimate day with friends to remember Jamie. The service isn’t something one can put simply into words. I was flooded with deep emotion seeing all of Jamie’s artistic creations, accomplishments, keepsakes, etc. I thought I could keep it in and be strong, but the overflow of emotions hit like a rock. Not necessarily sadness, the depiction of Jamie’s character put together in her memory room was absolutely beautiful. It felt like I could hug her. I wanted to hug her, but there were others to hug, bursting with Jamie’s love. Many lovely people I hadn’t even known, told me how much Jamie talked about “her Monsie”, even in the midst of her last days; she worried about me, my health, and wellbeing without question. What a beautiful soul. How’d I get so blessed to meet such a person? So blessed to know such a wonderful being. Through her passing, I met wonderful people that Jamie and her family have known, and dear friends I hadn’t seen for months, even years. Thank you for your support and laughter. It was a reunion of hope and life. It’s not goodbye, it’s see you a little later, Jamiedove.
This past month has been extremely hard for me, knowing my best friend, Jamie, would no longer be here. But more so, the unimaginable pain I know she has been enduring without compliant. I have cried for weeks, sometimes unconsciously bawling, bracing myself that she won’t be here anymore.
There are thousands of people she has touched, leaving such an unforgettable impression. I will never forget the moment I was introduced to Jamie back in 2009. She was such a welcoming person. She helped me through some very challenging obstacles in my life. Jamie helped me realize the fight I had in me, that I hadn’t been using to advocate for myself. She essentially helped many become self reliant through USeb including myself. She helped me battled head on with Medicaid as she witnessed sepsis nearly take my life.
Jamie had the sweetest moves on the dance floor during camp. Jamie made me laugh until tears on our game nights; we “oinked” into our magical pigs during the board game “Pass the Pigs”. Jamie loved rough off-roading in a Jeep as much as me, and boy, we had fun. She was incredible at making cool techno noises with her mouth. We exchanged our goals and dreams together while painting on beautiful stormy days followed by vibrant rainbows. Jamie was not only incredibly talented with a paintbrush, she had an amazing angelic singing voice, that will remain echoing in my memories. I am unbelievably blessed to have had those moments with her.
I will miss you, Jamie. Thank you for being a sacrificial understanding friend to me. You never hurt me. You were truthful and real. You never made me feel like I owed you anything. You gave your time, love, emotion, and heart without expecting a thing in return. You were sympathetic and empathetic. You felt with your heart not with your head. I wish I could have told you how I appreciated you more often. I wish my life did not have to move me so far away from you during your last days here. I know Jamie would not anyone to be sad, but we will miss her friendship, selfless presence, and beautiful soothing voice.