Archive | September 2015

Prognosis Happiness

To date, this is the most important piece I have ever written. Most important because our family can now embrace Mermaids disease with a true diagnosis  and make constructive but cautious decisions about our future with the right information.

We have so much to share you might want to pause here and move somewhere comfortable…

Ready???

This week we not only received the long anticipated biopsy results but got to meet Mermaid’s new dermatologist and I must say, she was a ray of sunshine in our cloudy little nevus world.

The next sentence is my favorite collaboration of word’s I have ever put to print. MERMAID DOES NOT HAVE CANCER. There is always that higher potential of cancerous cells developing in nevus owners but we are officially free & clear at this time. To be fair, we knew that piece a few weeks ago. Our Primary Care Dr. let us know but we wanted the final reading before sharing. Needless to say I slept a smidge better the night we found out.

Here is our official biopsy reading:

-Epidermolytic hyperkeratosis.

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

So what does this mean? Officially, this means that Mermaid has linear epidermal nevus, which occurs in 1 out of 1,000 people. However, the rare subtype of epidermolytic hyperkeratosis only occurs in 1 out of 2/300,000 people worldwide.

There is a grey area, we cannot yet rule out ILVEN.  The biopsy site did not show the specific subtype but Mermaid has had several occurences that I was able to photograph and share with her new dermatologist. She agreed it was inflammation and said it may in fact be an early onset of ILVEN. We will wait and see what the nevus does from here on.

I know not everyone understands what this means so I’m going to break it down a bit more.

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.

Linear epidermal nevus follows the lines of Blashko, which are invisible on the skin but thought to follow the paths which cells migrate as the skin develops before birth.

LEN can be linked to complications such epidermal nevus syndrome; abnormalities effecting brain, internal organs, eyes or bones. Tumors can also arise within the lesions. They may be harmless and simply need removal or may be a form of skin cancer.

We can happily report that as of this moment Mermaid does not harbour any of said abnormalities. All our tests and check ups thus far have come back clean.

If she does indeed develop ILVEN the potential treatment options and complications change a bit but the two are extremely close in comparison.

We were also told at our visit that Mermaids chances of developing a Wilm’s tumor are very unlikely as she would have already developed some of the other abnormalities effecting the organs. (I wont personally celebrate until her 6th bday!)

Mermaid occasionally gets small lesions on her scalp which we were asked to come in and have swabbed next time they occur. We were also able to discuss the possibility of Mermaids overheating being correlated. This is more common with giant nevus but it may be that Mermaids sweat glands are not working correctly in the area of her nevus. We cannot say definitively this is or is not the cause as there is no way of knowing for sure and we will continue to monitor it.

From the beginning of this journey I have been plagued by the thought of my Mermaid being teased or self-conscious about her “birthmark”. As we have learned more about her disease and gotten to know fellow Nevus owners that fear has only grown. The emotional effects can be devastating for Nevus owners of all kinds.

We have been lucky thus far that Mermaids nevus is mostly unseen by others with the exception of her arm & hand. Mermaid is very protective of her hand and rarely lets me photograph or touch it. At our appointment it was noted that her nevus is starting to come up over her palm. I can pray this doesn’t happen but on the chance it does I was FINALLY able to have that conversation about what treatment looks like in the future.

In the future, our dermatologist offered a form of cauterization in office that can help flatten and smooth the skin. We discussed both the pluses and deltas of co2 with a decision to avoid that route and of course, the talk of removal.

On the chance Mermaid’s nevus continues to thicken on her hand and move over her palm we will need to take action. At school age we will go through with removal. This can be done by our healthcare system here opposed to out of state which we originally thought.

This decision is based not only on a cosmetic and emotional basis but on the grounds of physical irritation it has caused.

I feel like it has been a struggle to get to a place where we understand our path moving forward; but we have arrived.

Our dermatologist is supportive of our trials, we will see her once every 6 months unless needed sooner. If Mermaid inflames on a more chronic basis or develops the spots on her scalp we will return. We will monitor her body temperature and physical discomfort, being conscious of potential tumors. In a few years we will revisit the surgical options and until then, we will continue our journey with LEN.

I know that our recent decision to find new care and push for more solid diagnosis and treatment information will free me from so much fear.

To all you rare and beautiful nevus owners, we hope our struggles to find the right care and options brings you a sense of direction and understanding.

Thank you to everyone for supporting us up to this point.

Thanks for walking with us today,

Mermaid’s Mommy

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Do frogs give you warts?

WARTS

We have an exciting product to trial and are patiently awaiting its arrival. It’s called WartPEEL; an acquaintance and Mommy’s Mermaid follower turned us onto this exciting endeavor. WartPEEL is a wart removal product that treats planter warts, foot warts, and other warts found on skin. It is a compound medication available only in certain locations in the United States and is typically a cash product running $79.00 per 5 grams (0.17 ounces).

In 1997 MedCara’s pharmacist founders experimented with a compounded treatment using chemotherapy drugs which is marketed as WartPEEL. This compound has been used by medical practitioners to treat more than 50,000 patients.

WartPEEL is available as a compounded medication exclusively from MedCara, LLC and it’s contracted pharmacy, NuCara. Dispensing of WartPEEL requires that an appropriate doctor-patient-pharmacist relationship be maintained. NuCara Pharmacy will not fill prescriptions for patients that do not have a valid patient-doctor relationship.

What does an appropriate doctor-patient-pharmacist relationship mean? This means you cannot call NuCara and simply purchase the product on your own. You must have a physician complete a prescription form and fax it directly to their compound pharmacy.

Where Mermaid’s Nevus is not a wart she does have a problematic spot on her wrist that is thickening. This is a particular spot she picks and says hurts – so whenever it arrives we will start with a small trial on that one designated area. Considering the medication contains a chemo agent and typically isnt used in patient’s this young we will be more cautious about the usage.

We got so caught up in our busy lives we forgot to share the results of our last trial, we took pictures and documented everything and then dropped the ball. Like other Amlactin products tried in the past we saw little to no improvement but I will still post the picture and information on my “Got any secret solutions?” page with the others.

 As a quick reminder we used AmLactin Moisturizing Body lotion and below is the decription from their website.

amlactin-moisturing-body-lotion

“AmLactin Moisturizing Body Lotion has a special formula with clinically proven 12% lactic acid that’s pH balanced for the skin. Don’t let the word “acid” concern you. Especially since lactic acid is a naturally occurring humectant for the skin with a certain affinity for water molecules to help keep skin hydrated. And the more moisture that can be retained deep within the skin, the softer and smoother your skin feels. In addition, by encouraging natural skin cell renewal through exfoliation and delivering intense hydration deep within the skin, this lotion creates a soft, smooth texture.”

Monday 8/17- Monday 8/31.

Monday 8/17/2015:

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During this time I again noticed no change in Physical appearance, she continued to speak about it just as much as before. At one point Mermaid developed a skin rash which I do not believe to be corrolated with the AmLactin product in any way but added a small complication.

Ultimately we came to the same conclusion as the other AmLactin products, they work for hydration but moving forward we will opt out of trialing any other versions for the soul fact that we know the extent of their use.

Monday 8/31:2015-09-21 12.44.09

Stay tuned for results and more information on WartPeel!

Thanks for walking with us today,

Mermaid’s Mommy

Good things come in small packages…

Nevus owners come in all shapes and sizes. The disease does not discriminate for race, religion or belief. Some cases are more severe than others but my experience thus far is that despite the subtype or severity, the owners and families of nevus owners alike, share the same levels of support for one another.

It gives me great pleasure to openly write about our journey and the journey of others on this platform to share not only our emotional struggles and human reaction to the disease; but to potentially offer knowledge to those seeking.

Today I am blessed to share a story about a little girl with ILVEN. Meet 9-year-old Rachael, a fan of literature – anything Bad Kitty by Nick Bruel is right up her alley. She has a healthy appetite and unlike myself,  has blessed her family with not being picky!

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Rachael was born with what her parents believed to be a suckling blister on the palm of her left hand and they thought it was adorable. However, by the time she was 6 months old it had spread halfway up her palm. The first dermatologist diagnosed Rachael with linear porokeratosis but he admitted he was way out of his league and sent them to a different dermatologist in Rochester,  NY.

A punch bioosy was done and Rachael was diagnosed with epidermal nevus.  The family was given a few prescriptions for creams but basically brushed off (ahhh… how I know the feeling).

At that time Rachael had growths on the bottoms of her feet that the same dermatologist insisted were warts. She attempted to freeze them off with liquid nitrogen but with no surprise to the family, failed.

Six months later they returned asking for more literature on Epidermal nevus but were given copies from a medical journal,  that I’m sure many of us can confirm,  means little to nothing without a medical degree.

Being that the family was military there same search and struggles for information continued with each  move to a different state and new Dr.

Rachael struggled crawling because her ILVEN had spread at a rapid rate and would snag on the carpet as well as place pressure on her lesions. They were so thankful when she started to walk and passed that point of despair.

In 2011 the family was transferred to Virginia Beach and yet again, had to establish care with a new physician.  At this point Mom, Lauren, was more Internet savvy and had determined on her own that what Rachael had was ILVEN. Mom printed everything she had found and presented to Portsmouth Naval Hospital with her findings.

Their new dermatologist was riveted by Rachel,  completely and utterly charmed by this blue-eyed blondie.  Unlike the past the Dr. Listened to everything Mom had to say and ordered a second biopsy.  The results? ILVEN. it was such a relief to have a diagnosis and course of treatment began to change.

After being diagnosed Mom felt the struggle and knew she needed support. Thats when she decided to start a Facebook support group, which is where I came to know this angel.

Rachael’s ILVEN continued to grow. In February 2015 she had her first surgery to remove the ILVEN from her hand, knee, toe, finger, wrist and bottom of her foot.

In July 2015 she had a second surgery to correct a keloid scar ;  an overgrowth of granulation tissue at the site of a skin injury, on her left hand. She had a pin placed in her hand/finger to keep it in place.

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Rachael is a fighter. She has never been ashamed or embarrassed of her ILVEN.  She explains it to people who ask or stare in her own child like innocence and even keeps a stock of band aids on hand for when her ILVEN cracks or bleeds.

Rachael,  you are an inspiration. I yearn to teach my Mermaid to go through life with your candidacy and courage in the face of adversity.

My very first post spoke about staying up at night searching for a family like mine, someone to understand, that was the birth place of Mommy’s Mermaid.  I later found the support group Lauren had created and will forever be grateful. We are 7 years behind their struggle but thanks to Lauren’s diligence and command,  we are making headway.

Our families share a bond unlike others. Lauren and I share that need to find a sense of belonging and teach others through our own experiences. We sincerely hope that through the ILVEN support group and Mommy’s Mermaid we can be a place for others to find companionship and strength.

Thanks for walking with us today,

Rachael,  Lauren, Mermaid

and

Mermaid’s Mommy

Operation scales complete!

Exhale. Its done. Nearly two years after first asking for a biopsy it’s finally complete. Just knowing we will soon have a difinitive diagnoses brings a sense of calm.

We battled to have this done through specialty care and in the end I am ever so thankful that our primary doctor stepped up to the plate. There seemed to be a sense of hesitancy to complete the biopsy on a little one until our family doctor joined the cause. I truly believe that without the compassion and knowledge of our support group, family and Mommy’s Mermaid followers, we would still be fighting. The genuine compassion they showed us gave us opportunities to not only share what they had completed but allowed us to pick the brains of specialists across the country. We were armed with the right information to close the deal. To each of you that have spent your evenings chatting with me and sent me pertinent information, I thank you.

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So why does this mean so much to my family? Nevus comes in many forms. The treatment and complications for your specific subtype varies. Some have more opportunities for less invasive treatment and others simply result in surgery. I feel very strongly that it is our right, if the technology exists, to know the true pathology of Mermaid’s nevus. We deserve as a family to prepare and educate ourselves on whats to potentially come in the future. Could our original diagnosis of Linear Epidermal Nevus remain, sure, but it also may not.

I worked daily for a week or so to prepare Mermaid for this day. We talked about the first shot, where it was going to be and that we would then be cutting out one of her special Mermaid scales so the Dr. could make her feel better someday. Sounds silly to have an expectation that a 2-year-old would comprehend what I was saying, but I must say, it worked!

From the minute we walked into the office she was mentally prepared. Yes, there were tears with the inital injection of lidocaine but heck, when I worked in Internal medicine I saw tears from adults too! She got away once but we readjusted and from there on out it was smooth sailing. Daddy, myself and our beloved assistant kept her still and calm and it went much quicker than anticipated. She got one small stitch which I will remove myself in a week or so and by the time she had a hello kitty & my little pony sticker in hand, she had forgotten what happened. It didnt hurt that brother was there shortly after to offer another level of sunshine. I am so proud of the strength and understanding my Mermaid showed today. She really is something special.

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I cannot express what a gratifying feeling it is to have a Dr. That not only supported us doing this, supports us participating in the Yale trial, but supports all our efforts to find answers and comfort for our Mermaid. After all, he may have one of only a few children in our state with this rare disease on his case load!

In roughly 2 weeks we will know the exact pathology of Mermaids nevus and whether or not it contains cancerous cells.  One more victory in our journey!

Thanks for walking with us today,

Mermaid’s Mommy