Epidermal Nevus effects roughly 1 in 1,000 people worldwide. Each subtype i.e. LEN (linear epidermal nevus), ILVEN (Inflammatory epidermal nevus) or verrucous epidermal nevus, holds a different value of people effected. Less common, effecting roughly 1% of the population are congenital epidermal nevus and/or melanocytic nevus. Within an individuals type of nevus can be additional subtypes, such as Mermaids diagnosis of “epidermolytic hyperkeratosis;” which effects 1 in 2-300,000 people worldwide.
I know, because until Mermaid was diagnosed, that unless you have a personal connection to this disease, it is not something the general public is aware of. I had never in my life even heard of this disease, let alone met someone with it, and I have worked in the medical field for nearly 12 years! Hence the fact that they are considered rare genetic diseases.
When I started Mommy’s Mermaid it was out of pure desperation for contact, support and answers from others living with this disease. Throughout this process I discovered a few support groups and have gained my own following here, on my website, of both people who themselves have a form of the disease or know someone with it.
To possibly imagine that Mermaid and I would be graced by God to have another nevus owner in our own backyard was unfathomable. It seemed that everyone we had contact with was Midwest or East Coast. Graces were granted and after months of communicating via Mommy’s Mermaid, our Facebook support group and text messaging, we finally got to meet face to face with a fellow local nevus owner.
Before meeting I could never even begin to express to you the gratitude I held for our crossing paths. We are relatively new to this disease and our cohort is an adult who has been living life managing her symptoms long before we even knew it existed. The opportunity to pick her brain, absorb her knowledge, hear her courage; To place my Mermaid and another nevus owner – with significant enough struggles to match our own – in the same place, wow – words cannot describe.
The days leading up to our meeting I was nervous, like my husband and I’s first date kind of nervous. Who was I? I’m just a small town, working Mom that put together a website in hopes of finding others. I have written one other piece on a fellow nevus owner and her daughter and took a stab in the dark that maybe, just maybe, our new found companion would be willing to meet with us, share her story, and trust me to share it publically in a tactful way. I honestly expected a no. I wasn’t asking her to answer a few questions from the comforts of her home, but to physically meet in person, give us her own valuable time. She doesn’t know me from Adam, how long could we really talk? What was I going to ask her? At what point did I go from sharing our own experiences to dragging other people into this crazy ride acting like a reporter of some type?
Well folks, she said yes.
It was Mermaid’s 3rd birthday. I had taken a few days off to celebrate my little blessing and what better way could we have done that than to spend it with a fellow nevus owner.
We opted to meet at a local kids play place. Given the age of our children and the weather, you really couldn’t have found a better location. It’s really kind of comical, our stories were interrupted by crying children, hungry children, diaper changes, snotty noses and just about every other possible kid related need. It sounds kind of awful but really, that’s what both are lives are like on a daily basis so it was normal and delightful to us. It felt comfortable. I only hope between all the needs I was able to capture Monica’s story correctly!
Monica is the 2nd of 5 children, a teacher by trade, a wife and a Mother of two. She has taught an array of ages and in many settings but my praise goes to her work as a special education teacher. Specifically with the Life Enrichment Education Program (LEEP); which provides specially designed instruction and related services for eligible students ages 5-21 years with special needs. The curriculum focuses on personal management, communication, practical living skills, vocational skills, leisure/recreation activities, and social skills. Throughout her teaching career she found the humor in her disease and when asked by students what was on her leg she would drum up stories of what it was, things like she got it saving animals from a burning building, always something that made her a hero. Her stories made me laugh and I adore the ability to have fun with it. Though I have to say Monica, in my eyes, your involvement both as a teacher and time with those that have special needs, makes you a hero. Saving cats is pretty cool too though.
As far as Monica knows, her nevus was not present at birth. Her first real memory of its presence was grade school, with a busy Mom, remember – there were five children – it may have been present prior to that but without her speaking up about it being bothersome it may have been overlooked as just a normal benign skin condition. At that time her nevus was present only on her left leg. Around age 11 it became more prevalent and began to cause problems so she was taken in and officially diagnosed with Inflammatory linear epidermal nevus (ILVEN).
Over Monica’s lifetime she has had 3 biopsies. The first when they lived in Northern California at age 11, which was where her initial diagnosis of ILVEN began, again in her 20’s here in Oregon and lastly, in her 30’s. So why so many? Over the years her nevus has spread, now her nevus rears its head from her left knee down to her foot – around her big toe, with a small spot on the bottom of her foot and up the inside of her thigh. In more recent years Monica has found her ILVEN to be more bothersome than ever before. The previous biopsies were old and locating the dermatologists that performed them proved more difficult than just completing another one. The purpose was to have a clear diagnosis with her current dermatologist to move forward with treatment options. What’s interesting to me is that I have noticed the small spot on Mermaid’s under arm where her biopsy was performed has never produced new nevus, Monica has noticed the same thing. All places where biopsies have been done have not produced new nevus, which leads us both to have faith in the excision process. As Monica put it “a light bulb moment” which made the path she wanted to pursue very clear.
There is no clear reason for why it has become more active; i.e. spreading, becoming inflamed, and itching; however, in more recent years Monica has given birth to two adorable baby angels (really, I mean adorable – her 2.5 year old son was my friend within 60 seconds of meeting me and her infant smiled nearly the entire visit, not one whimper) and she has experienced more activity with hormonal changes, especially postpartum, both times. It has come to a point where she can confidently state that it is effecting her quality of life. Itching being the biggest hinderance, which I know so many of you can relate too.
Monica recently met with her surgeon at our famously known teaching hospital; Oregon Health and Science University (OHSU) who is a specialist in ILVEN. It is difficult for me to write this next piece of information because I have struggled myself to find someone local with a real extensive background and experience with ILVEN or LEN, but her current surgeon/specialist told her that of the TWO prior cases she had treated, Monica’s presented as the most extensive case she had seen. I understand the disease is rare but there seems to be a lack of Physician’s in our area that have dealt with ILVEN/LEN to the extent we read about in larger cities and East. I have no doubt that said doctor is proficient in her abilities and perhaps our region just does not house a high number of patients with ILVEN/LEN; which would explain only seeing two previous cases and confirm the definition of “rare genetic disease”, but it is still a bit of a surprise that Monica will only be her third treated case.
As they discussed treatment options, the opportunity for laser therapy came up and her physician stated that both cases treated with laser, grew back. The treatment was done on one patient’s finger and another, on their face. I really have not heard of anyone having long-term success with CO2 treatment. It seems to be a fairly painful process with short-term results. Which goes back to the “light bulb moment” that excision was the right decision. To move forward – Monica will need to not be pregnant or nursing, so time is the only barrier at this point.
It was such a blessing to sit down with an adult nevus owner and pick her brain about her experience thus far. We are so new to the disease that it was nice to hear the positives and negatives she had experienced thus far. Most interesting to me was a study she participated in. When Monica was in her twenties she voluntarily participated in a study at OHSU in trade for care. A group of members with skin conditions met with Physician’s and shared details about their condition. They were not allowed to tell them their official diagnosis. They shared details such as age, symptoms, and treatments tried. With that information the panel of Providers tried to decifer what skin ailment they had. After knowing the official diagnosis they made recommendations and offered free treatment. Their recommendation at the time was cauterization. The bonus to voluntarily being there is the ability to decline treatment, which she gladly did. The idea of painful burning of the skin that smells atrocious was a pass. Interestingly enough, Mermaid has also been offered this option, which we as well, declined for the exact same reasons. Nonetheless; it was fun to hear about her adventure in guinea pig land!
I was told that nevus growing on hands and feet can cause joint pain, I asked Monica about this being that her nevus grows on her big toe and thankfully, she has not had this experience. From time to time it may crack, causing pain but only exterior pain. Monica swims on a weekly basis and has found that the warmer the water, the more she itches. The pool she attends is salt water and has not found any therapeutic connection with her nevus. Like so many nevus owners she has tried her fair share of creams and ointments with limited results but is currently using an ointment called Clobetasol Propoimate 0.05% and has found it to be extremely helpful. This medication is used to treat a variety of skin conditions (e.g., eczema, dermatitis, allergies, rash). Clobetasol reduces the swelling, itching, and redness that can occur in these types of conditions. This medication is a very strong (super-high-potency) corticosteroid. She applies a layer of the ointment and covers it with a sheet of tagaderm, leaving it until it falls off, roughly 24 hours later. She has found that it not only relieves that pesky itching but reduces the thickness of her nevus.
I asked Monica what the biggest hurdle was coming up in school and I got the exact answer I expected. Trying to explain to others that it was not contagious but would never go away proved difficult; as well as feeling as if it effected her socially at a younger age. She wisely reminded me that bullies will be bullies, if it wasn’t her skin, they would have found something else to be mean about, they are just mean, end of story. Monica then followed her answer with a statement that I have thought about a lot since that day:
“We as people are way stronger and resilient than we give ourselves credit for.”
You are right.
Monica noted that as a Mom, it probably hurts more than being the one with the actual disease, it causes them heartache, and I think she nailed it. I often feel sad and helpless, a sense of heartache; when Mermaid is that resilient, strong person that acts as if it doesn’t exist.
The end of our conversation was about finding people who fit your need at the moment, whatever that moment is. She spoke about recently losing her Father and finding herself drawn to friends she had not spent much time with recently, but they were in the same place, and there is comfort in that. Her piece of advice to everyone new to the disease?
“Knowing you are not alone is sometimes all you need, find people with the same situation to comfort you, make that connection.”
Isn’t that the truth, after all; that’s what started Mommy’s Mermaid, the need for that connection with others in our same situation. She could not have chosen a better piece of advice to offer.
I captured a pretty special moment during one of Mermaid’s breaks from the play structure. Monica asked Mermaid if she would like to see her scales, “the ones like you have” and kindly lifted her pants to share. As that Mom that feels the heartache, this moment means the world to me. To show Mermaid that she is not alone, that other people share her special gift, to make that connection… I cannot ask for anything more.
As Monica showed, every case is unique. Some proving to be bothersome from day 1, others later in life. There seems to be this never-ending circle for all nevus owners of trying product after product until you find the one specific to your own need. I am hoping to get in touch with OHSU and learn more about their blind trial, it would be fun to know what kind of conclusions they drew from it.
Monica – Thank you for sharing your story with us and allowing me to bring it to life. Thank you for being such a kind gentle soul. I truly felt as if we were old friends and will forever think of you that way. You will remain special to us for so many reasons. Our first connection in live flesh to another nevus owner, a reminder to keep our sense of humor around the disease and proof, that this disease does not define who you are.
We wish you all the luck moving forward with excision, we will be there to support and pray for quick healing and relief. We will continue our connection along this journey.
Today, Monica, you are an honorary Mermaid and we are the ones lucky enough to have you on our team.
Thanks for walking with us today,