Archive | May 2016

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From our family to your’s…

Happy Memorial Day!!!

May we all remember what today is truly about and give thanks.

“True heroism is remarkably sober, very undramatic. It is not the urge to surpass all others at whatever cost, but the urge to serve others at whatever cost.”

-Arthur Ashe

 

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Thanks for walking with us today,

Mermaid’s Mommy

 

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A,B,C,D,EHK,LEN,ILVEN,ENS…

Navigating the world of Epidermal Nevus can be confusing. No matter how hard I try, I will never be able to forget the day I received Mermaid’s diagnosis. I know now that it’s going to be ok and we can jump whatever hurdle presents, but I didn’t always feel that way, we are constantly gaining new followers, many who feel the way we all did in the beginning. Lost. I feel like It’s been a while since we have broke down the basics, like what do all these acronyms mean? So many are used interchangeably that it may cause confusion and more fear. So, today I am going to break down 4 of the most commonly used terms surrounding EN that I see. LEN, ILVEN, EHK and ENS. I will touch briefly on other forms but highlight these 4 acronyms.

It is so important to understand that where all these terms refer to Epidermal Nevus, LEN is different than ILVEN, EHK is slightly different than both LEN and ILVEN and MOST IMPORTANTLY, having a diagnosis of Epidermal Nevus does NOT mean that you have the syndrome.

Here is a reminder of Mermaid’s official biopsy report:

-Epidermolytic hyperkeratosis.

Exam demonstrates aconthotic and hyperkeratotic epidermis with focal dyskeratosis. The differential includes linear epidermolytic epidermal nevus and epidermolytic acanthoma.

Though we were told that ILVEN has not yet been ruled out.

(linear epidermal nevus occurs in 1 out of 1,000 people. However, epidermolytic hyperkeratosis only occurs in 1 out of 2/300,000 people worldwide)

Epidermal nevus is a clinical term for a family of skin lesions that involve the outer portion of skin, the epidermis, and are distributed in a linear and often swirled pattern.  The lesions may be single or multiple and are usually present at birth.  All epidermal nevi show some changes in texture which can range from very rough, warty and spiny, and often darker than the surrounding normal or uninvolved skin (verrucous epidermal nevus), to red and scaly (inflammatory linear verrucous epidermal nevus or ILVEN),  to yellowish, rough and pebbly appearance due to proliferation of oil- or ’sebaceous’ gland-like structures (nevus sebaceous).

Epidermal nevi are genetically ‘mosaic’, meaning that the mutation causing the nevi are not found in other cells of the body. Mosaicism arises when the genetic mutation occurs in one of the cells of the early embryo sometime after conception; such mutations are called ‘somatic’ mutations.  This mutated cell, like the other normal cells, continues to divide and gives rise to mutated daughter cells that will populate a part of the body.  The linear patterning of the epidermal nevus reflects the movement of the mutant daughter cells during fetal growth.  These linear, developmental patterns are termed the ‘lines of Blaschko’.  Many epidermal cells within these affected areas harbor the mutant gene, while most or all cells from uninvolved areas do not.  After birth, the nevus “grows with the child”, although some new areas of involvement and/or extension of the nevus to new areas can occur.  ILVEN is an exception: here, lesions often do not appear until later in infancy or childhood.

Types of epidermal naevi:

The skin lesions most often referred to as epidermal naevi are due to an overgrowth of keratinocytes (horny skin cells).

  • Linear epidermal naevus
  • Epidermolytic epidermal naevus
  • Acantholytic epidermal naevus
  • Systematised epidermal naevus
  • Linear porokeratosis

However, several other conditions are also characterised by benign overgrowth of the epidermis and its appendages (organoid naevi)

  • ILVEN
  • Sweat gland naevi
  • Sebaceous Nevus
  • Comedone Nevus
  • Becker Nevus

Let’s start with LEN: Linear Epidermal Nevus:

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Linear epidermal nevus (LEN) is an uncommon skin condition. It usually affects the limbs and torso on one side of the body. The lesions are typically present at birth. LEN is the result of a genetic abnormality. It is not inherited but arises from somatic mutations in cells that occur after conception.

This is a type of birthmark that is usually present at birth, but may develop later in childhood. They are usually light or dark brown in color. They may start as a flat area in the shape of a line or as a “skin tag.” Over time, they grow and become thicker like a wart. They can be located anywhere on the body. Often, epidermal nevi follow a pattern on the skin known as the lines of Blaschko. The lines of Blaschko, which are invisible on skin, are thought to follow the paths along which cells migrate as the skin develops before birth.

Second is ILVEN: Inflammatory Linear Verrucous Epidermal Nevus:

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Inflammatory linear verrucous epidermal nevus is a special kind of epidermal nevus. Like other linear epidermal nevi, ILVEN is characterized by warty lesions that tend to group together in a linear pattern. The difference is that the lesions are red, inflamed and itchy, sometimes intensely so. The surface of the lesions may look like eczema (dry, red, scratched) or like psoriasis (red and scaly).

ILVEN most often affects one leg and may extend from the buttock to the foot. It may be present at birth, but usually arises during the first 5 years of life and spreads over months or years. ILVEN is very rarely observed during adulthood. It is somewhat more common in females.

Next up… EHK: Epidermolytic hyperkeratosis

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Epidermolytic hyperkeratosis is a skin disorder that is present at birth. Affected babies may have very red skin (erythroderma) and severe blisters. Because newborns with this disorder are missing the protection provided by normal skin, they are at risk of becoming dehydrated and developing infections in the skin or throughout the body (sepsis).

As affected individuals get older, blistering is less frequent, erythroderma becomes less evident, and the skin becomes thick (hyperkeratotic), especially over joints, on areas of skin that come into contact with each other, or on the scalp or neck. This thickened skin is usually darker than normal. Bacteria can grow in the thick skin, often causing a distinct odor.

Epidermolytic hyperkeratosis can be categorized into two types. People with PS-type epidermolytic hyperkeratosis have thick skin on the palms of their hands and soles of their feet (palmoplantar or palm/sole hyperkeratosis) in addition to other areas of the body. People with the other type, NPS-type, do not have extensive palmoplantar hyperkeratosis but do have hyperkeratosis on other areas of the body.

Epidermolytic hyperkeratosis is part of a group of conditions called ichthyoses, which refers to the scaly skin seen in individuals with related disorders. However, in epidermolytic hyperkeratosis, the skin is thick but not scaly as in some of the other conditions in the group.

Lastly… ENS: Epidermal Nevus Syndrome

The term “epidermal nevus syndrome” has generated significant controversy and confusion in the medical literature. Originally, the term was used to denote a disorder that was actually several different disorders erroneously grouped together. In the recent past, the term was used to denote a specific disorder now known as Schimmelpenning syndrome. However, the term epidermal nevus syndrome could be correctly applied to several different disorders. Therefore, the umbrella term “epidermal nevus syndromes” now represents a group of distinct disorders that have in common the presence of one of the various types of epidermal nevi. However, there is so far no general agreement how to classify the types of this diverse group of disorders, adding to the confusion within the medical literature

Epidermal nevus syndromes (ENSs) are a group of rare complex disorders characterized by the presence of skin lesions known as epidermal nevi associated with additional extra-cutaneous abnormalities, most often affecting the brain, eye and skeletal systems. Epidermal nevi are overgrowths of structures and tissue of the epidermis, the outermost layer of the skin.

In ENS, neurological involvement may include:

  • Epilepsy or infantile spasms.
  • Intellectual impairment.
  • Structural or vascular brain abnormalities.
  • Spinal lesions.

Skeletal involvement includes:

  • Incomplete formation of bony structures – eg, spina bifida.
  • Hypoplasia of bones.
  • Bony cysts.
  • Asymmetry of the skull or spine.
  • Spontaneous fractures and rickets.

Ophthalmic involvement includes:

  • Colobomas.
  • Strabismus.
  • Ptosis.
  • Nystagmus.
  • Corneal opacities.
  • Retinal changes.
  • Various other ocular abnormalities which have been described.

Endocrine features have been reported:

  • Hypophosphataemic vitamin D-resistant rickets has occurred in a number of cases.
  • Precocious puberty has been described in several cases.

Syndrome of inappropriate antidiuretic hormone (SIADH) has been reported in one case.

Other potential complications of EN:  

Complications due to Epidermal Nevus are rare; but, it is observed that there is an association with the development of basal cell carcinoma, squamous cell carcinoma, keratoacanthoma, and clear cell acanthoma.

It is suggested to search for dysplastic kidney disease in patients with neurocutaneous disorders. Neurocutaneous syndromes are disorders that lead to growth of tumors in various parts of the body. One being a Wilm’s tumor. They’re caused by the abnormal development of cells in an embryo and characterized by the tumors in various parts of the body (including the nervous system) and by certain differences in the skin. It is important to understand this is very rare but something to be aware of.

Whew, that was a lot of information, I hope it brought a small amount of clarity surrounding all the terms thrown out there.

Thanks for walking with us today!

Mermaid’s Mommy

Buzz buzz little bee…

After using a product called WartPEEL on Mermaid’s thicker patch of Nevus it proved to be raw and a bit painful. I had been sent some amazing products from a company called Wild Carrot Herbals and amongst them was a salve. I personally had never used one but thought it might be the trick, within 24 hours all the rawness, swelling and discomfort was gone. I was beyond tickled and started reading more about “salves”.

Mermaid has one particularly “itchy” spot on her back so I thought I would take a swing at making my own. I researched all the ingredients that potentially soothe dry or itchy skin, a few recipes and came up with my own concoction.

I’m blessed to have a lot of savvy people in my life so most of these ingredients were gifts from people’s own labor’s of love. I included:

  • Pure raw honey – (given a jar from the neighbors hives)
  • Organic Beeswax – (given a bar made by my parents from their swarm)
  • Shae butter – (given a pure bar from a local Oregon company)
  • Lavendar oil – (a gift from Mermaid’s Grandma)
  • Coconut oil
  • Baking soda

The process in harvesting beeswax is interesting and my Mom had some pretty great pictures – here are a few.

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We gathered our ingredients:

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Started prepping them, if you have ever tried “grating” beeswax, it’s not easy, Mermaid gave up on that part and let Mommy finish.

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Melted part of them together:

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Time consuming, I learned quickly that slow and steady is required. After it was all melted we removed it from the heat and slowly added our other ingredients.

We poured it into a few containers and waited for it to cool. I feared for a minute that it might not bind, it stayed liquid for better than an hour and we continued to mix it, but eventually, it became this nice smooth product.

Given the ingredients I expected it to be slightly oily, which it is but we put some on Mermaid’s arm and it absorbed quickly with less grease left behind than I assumed would be present.

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We added a little garnish and closed up our afternoon project.

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I suppose the real test will be to see how well it really works, I have high hopes!

We are going to ship a container to one of our “itchiest” Nevus babies on the other side of the country and let them be the true judge.

We have plenty of leftover ingredients so I feel a round two of a lotion coming on.

Round one of find a natural aide complete.

Thanks for walking with us today,

Mermaid’s Mommy

 

 

Happy Mother’s Day!

Mother’s Day, by definition, is a celebration honoring the Mother of the family, as well as motherhood, maternal bonds, and the influence of mothers in society. Over the year’s, like many other holidays, it has bloomed into a bit of a Hallmark holiday. Cards, flowers, a gift, perhaps a nice gesture… and done. I question if buying someone flowers and signing your name on a card really encompasses “honoring” what Mother’s really are. So this year (yes, we still made cards and will do a nice gesture) I want to capture a little about what makes Mom’s so special.

For me, I don’t think I ever really grasped how important or hard a job it is to be a Mom until I became one myself. Sure, my Mom always asked me if I had clean underwear on while we were driving to the Dr. (what would she have done if I said no?), she fed me ice cream when I had my tonsils out, carted me back and forth to girl scout meetings, softball, basketball, volleyball, took me prom dress shopping, bought me my favorite ice cream from the schwan’s man, and let’s just be honest here…. she still loved me through my terrible teens and early twenties when I thought I knew it all, yet managed to screw up everything.

My Mom also did some things I will never forgive her for, like letting my Dad drive us to my junior high concert in a yellow 70’s something Chrysler my Grandparents had given us, it was the size of a house, literally, you could fit 17 people in it comfortably and if my memory serves me right, we got pulled over in front of the gym for everyone to see.  She took me through the bank drive thru with curlers in my hair for all the ladies to gawk at and I’m pretty sure I had a mullet for a solid 2 years. She made me sit at the table with a plate of eggplant parmesan in front of me for at least 14 hours, what kid eats that? Really, what adult eats that? Gross. She made me pick up filberts, apples and plums from the yard for hours. Ever owned a filbert tree? Your lucky. It’s a really quick way to turn your children against you. When I was a teenager, my Mom & Dad rode their bicycles through town in spandex and helmets, even to the river where I was hanging out with friends jumping out of a tree into the water cheering me on. Talk about embarrassing. Worst ever, wore matching fanny packs with my Dad in PUBLIC, once, for an ENTIRE vacation in California.

So ya, My Mom did the great normal Mom stuff, the embarrassing, I will never forgive or forget stuff… but she did two other things and these are the things I want to recognize today, as we honor our Mom’s.

#1. My Mom created stuff and let me be free. She did and still does all those things that are a dying breed. She painted, did ceramics, quilted, canned, made jam, salsa, sewed my Halloween costumes, gardened, took us to the river and let us run free while she enjoyed the sunshine and a good book, let me float the creek with friends and walk on the country road back home with no parents, let me ride my bike all around town with friends for hours, play kickball in the middle of the road with the whole neighborhood, go on a road trip with the neighbors, travel to Canada with my aunt and uncle, sleep in tents in the yard, be bussed to a school 20 miles outside of town in the middle of nowhere with about 20 kids where we learned about the forest, hiked and got to play hockey in the gym with the Portland Winterhawks… she taught me how to be creative and make things and live outside the box. She stayed close, but let me wander.

I look around my house today and see things she has created, and things I have created. Had she not done those things, I probably wouldn’t have either. I truly believe my expression through writing stems from feeling a sense of freedom for so long. The world feels like a different place now and some of the things we were free to do as kids may not pan out but I am grateful that at that time in my life, my Mom recognized that it was ok to let me find my own adventures. I already see myself doing the same things with my own daughter. Teaching her to cook, taking her to the river for the entire day while she explores, creating lotions and scrubs for her skin, it all stems from my own Mom’s creativity. For that, I feel honored.

#2. I selfishly resented my Mom for this decision for a long time, but stay with me, it makes sense, I promise. Up until a certain age my Mom was always around. She was there when I got home from school, took me everywhere I needed to go and tucked me in at night.  She had gone to college when she was younger and graduated but didn’t find a good use with her degree, then came marriage and kids, for so long we were her world, and she was mine.

She made a decision when I was in grade school to go back to college to become an RN. Suddenly she was gone, or studying and wasn’t all mine anymore. After she finished and started her career she was gone nights, weekends, holidays… paying her dues as a new nurse, but all I knew, is that she wasn’t there as much. I don’t know that until this very moment that I have ever actually admitted that I was jaded about it for a while. When your young, you don’t always understand the reasons for adult decisions. I wasn’t able to see that as much as she needed her own identity, she did it for us, her family.

As an adult I now understand and respect that decision. I can’t imagine our lives had she not made that decision, but I didn’t always make it easy on her, I became a rotten and at times ungrateful teenager and for that, I am sorry. As I look back now, and from a “Mom” perspective, I can’t even begin to imagine how hard that was. To juggle family, home, work and school, it’s a tough feat. Something I certainly do not long to do. As a result of her decision she has done some of the things that make me admire her the most. Like working in hospice. A job where you are there to make people comfortable in their end days, you become attached to their families, to the patient, only to have to let go. A job I truly believe only an angel can do.  She worked her way through the ranks managing many different units and teams, worked in critical settings making life decisions and made career choices that ensured a better future for all of us.

I didn’t like college, more than a few times I changed my path, stopped going, wanted to quit… but I knew if she could do it with all those other factors in play, I could too. My Mom gave me the strength to finish, and for that, I am honored.

It’s important for me to recognize the things I did not always understand at the time because one day, I may be in the same boat with own daughter. On the flip side, it’s equally important for me to share what these things mean to me today. I am much better at putting things in writing than saying them in person so I dont give my Mom the credit she deserves on a daily basis but need her to know her worth.

Our Mom’s are so much of who we are, I only hope that when my Mom looks as me, it’s with the same pride and honor as I have when I look at her.

There is no truer statement than “it takes a village”, I had surrogate Mom’s that took me in as well, Aunts galore; Cindy, Leora, Pam, older cousins, My Mom’s best friend – my Aunt Miss, my Godmother Peggy my best friend’s Mom’s Tammy and Maryellen, they all fed me, kept me in line… called my real Mom when I was hanging out with boys I shouldn’t have been or throwing parties when my parents were gone, thanks for that by the way, but they too helped plant seeds of freedom, creativity and hard work. As I look back I feel so thankful for my village.

I now have my own daughter, and she too, has her own village. Where I try to instill all the wonderful things my village gave me, I am a working Mom, I love my job, I sometimes contemplate if I’m doing the right thing, that perhaps I should stay home with her, but the truth is, I am my best me when I have more than one responsibility. I was nearly 9 years into my career before she came so in a sense, it’s my baby too.

When I cannot be there she is in the hands of her teachers who have helped raise her since she was 4-months old. A group of Women who I trust whole heartedly to do right by my precious gift and encourage her free soul. She has Auntie Nay and Grandma who step in when needed. She has Mommy’s friends Jen and Molly who treat her as their own and she has this whole network of people here, from around the world, rooting her on in her uniqueness.

My favorite thing about being a Mom is watching the world through Mermaid’s eyes. With the exception of believing swiper fox will steal everything she owns, she is unscathed. Everything is amazing and exciting. The tiny villages of ants in our yard are a precious world to her. She thinks the bees live here to make honey just for her. There is no concept of time outside of light and dark and responsibilies are an after thought. She believes she is a ninja, a mermaid and a chef, so I believe she is too.

This Mother’s Day, opposed to just a card or a nice gesture, I challenge you to tell your village those things they did that make you proud, the things that make you feel honored to have them. The things that added value in making you who you are today.

To my Mom and my village, where I know I did not always make the best choices, I came out on the upside because of who you all have been for me. Mom, I am proud and honored by each and everything you have taught me, each and every decision you have made for me, be it easy or hard, and thankful that you were chosen to be mine.

Happy Mother’s Day to all my fellow Mommy’s.

Thanks for walking with us today,

Mermaid’s Mommy

 

Isotretin what?

ok… it’s Accutane, otherwise known as Isotretinoin or Roaccutane, depending on what part of the world you are in. Most of us just think of it as acne medication but has recently been suggested to a friend for ILVEN. So let’s take a closer look at how it works and the risks.

Accutane is a form of vitamin A and is part of a class of medications called retinoids. It was originally marketed as a chemotherapy drug.

Exactly how Accutane works on a cellular level is unknown but we do know that it affects all four ways that acne develops.

  • 1. It dramatically reduces the size of the skin’s oil glands (35%-58%) and even more dramatically reduces the amount of oil these glands produce (around 80%).
  • 2. Acne bacteria live in skin oil. Since oil is dramatically reduced, so is the amount of acne bacteria in the skin.
  • 3. It slows down how fast the skin produces skin cells inside the pore, which helps pores from becoming clogged in the first place.
  • 4. It has anti-inflammatory properties.

 

Number 3 and 4 are probably the most important factors when considering this drug for ILVEN.

The most common side effects of Accutane are dry skin, itching, rash, dry nose, nosebleeds, cracks in the corners of the mouth, dry mouth, dry lips, cracking or peeling skin, inflammation of the whites of the eyes, dry eyes, joint pain, back pain, dizziness, drowsiness, nervousness, or changes in your fingernails or toenails.

There are more severe side effects but they are very uncommon and let’s honest here, all medications come with a laundry list of side effects, however, here is one of the more significant ones.

Accutane can cause birth defects in babies, so women should never take the drug if they are pregnant. I found some literature stating that women have to sign an agreement to take two different forms of birth control and take pregnancy tests while they are on Accutane, to make sure that they do not get pregnant while taking the drug. Because of all of the negative side effects that Accutane can cause, the negative aspect of the drug has been played up significantly.

There is no scientific evidence proving that Accutane can cause infertility later on years after taking the drug, but there are lots of online forums and message boards that indicate that many women think that Accutane is the reason for their fertility difficulties. I visited many of these me boards and asked for feedback, to my surprise, several people wrote back that they did in fact get pregnant in time.

I must have read 10 different case studies that offered little to no information about why Isotretinoin did not work as therapy for ILVEN but all cases suggested minimal effects. Minimal meaning, it DID in fact have some effect, just not enough to continue.

To my fellow readers, I am curious to know if you have tried this form of treatment and if so, what were the results? Do you have any advice for those being offered this as a potential treatment? Thanks for your feedback!

Thanks for walking with us today,

Mermaid’s Mommy