From sea to land – a tale of scales

2015-04-02 20.06.28~2

Below is the first post I ever wrote, I choose to keep it up as a reminder of where this all started. I now love to share other people’s stories and learn about all forms of nevus. By no means am I an expert but have come a long way in understanding what our future holds. When I start to question if I should continue this blog I come back to this post and remember the place I was in at the very beginning. Welcome, and if your new… start here!

Hello there, however you got here, I’m glad you made it. Welcome to Mommy’s Mermaid, let me tell you a little about our journey.

In 2013 our family was blessed with a beautiful baby girl. From birth I noticed this odd spot on her arm, multiple Dr’s assured me it was nothing, just a tiny birthmark and home from the hospital we went. As she grew, so did this tiny birthmark. Eventually filling her left armpit, around her ribcage and down her arm onto her hand. Now it didnt resemble a birthmark at all, more so a fungal infection. Multiple creams and a perplexed primary care Dr. Later we landed in dermatology.

I will never forget how calmly I was told that what my daughter had was called a nevus and it had no cure. A type of birthmark that could potentially take over the left side of her body and to be conscious of something called a wilms tumor. Keep it moist, avoid harsh soaps, see you later. I was so fixated on the fact that it would be there forever that I didnt ask the questions I should have.

We left and went for lunch, I got out my paperwork and googled the diagnosis shown of “melanocytic nevus” and a wilms tumor.  If your not familiar with either, as I was not, take my word for it, what begins to pop up is a parents worst nightmare. Cancer, cancer, cancer. I felt sick, confused, sad. Why was it taken so lightly? Whats going to happen now? I immediately emailed the Dr. Who said a modifier didnt populate and what she had was not melanocytic but a linear epidermal nevus. Better but still not good.

I cried a lot in the following days. Sometimes with my family, sometimes alone. I thought about what the kids would say in school, would she wear a swimsuit when she was older, what about her prom dress? Would she develop kidney cancer, how would I know? Will she be tough enough to handle the vain criticisms I knew lay ahead?  My anxiety’s plagued me. I searched night and day for anyone like me. Another Mom who felt sad or scared, a positive story about someone in adulthood with this disease and time and time again I came up empty handed.

I researched and did my due diligence on the disease and made a decision right then to start raising my daughter not to be ashamed of her “birthmark.” I jokingly said they were special scales she got to keep from when she lived in the ocean as a fish. Hey, she does love water! A friend said can’t you at least make it something better than a fish? Like a mermaid? and it stuck. Her special scales from when she was a mermaid. Mommy’s Mermaid.

I wanted to create a place for people to come for support. A place we can talk about products that have or have not helped, challenges and successes.  A place where we can help eachother understand more about the disease and feel safe. A place where families like mine can find eachother just as I needed so badly in the beginning and am sure I will need again throughout our long journey. I pray for those who’s nevus has resulted in surgery and or cancer or left them feeling alone or insecure. Please know, you have a friend in us.

I hope in time to gain followers, strength and knowledge as I publicly share our walk down this road.

Your Truly,

Mermaids Mommy

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Are you listening???

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suicide2

A friend once told me to “say it out loud” and for what felt like the first time ever, I did. I had spent years upon years keeping it in. I always reacted to the initial shock, poorly at that, then locked it up to be strong for everyone else, until it happened again and I repeated the cycle. I had terrible coping mechanisms, was unable to move on and had I not finally said it out loud, I still would. It sounds so simple now, but it wasn’t always. I now say it a lot, not to just anyone, to my inner circle and myself, but in a healthy way and understand that it’s perfectly normal. In fact, it opens the door to understanding of myself and to others. So I will say it now, on the most public forum I have ever done so and I will own it.

I am sad.

Three of the most powerful words I ever said out loud. I use to have this mantra that I needed to be the one everyone else could lean on, pretend I knew the right thing to say and that no matter how I felt inside, I had it all together and could easily move onto the next day, but it was a total lie. You see, by the time I as 17, I had put more people in the ground than most adults I knew. Death seemed to be a normal occurrence in my world and being so young I lacked the ability to understand it. I didn’t understand how all these people I was so close too were leaving me. I was damaged, and I still am, but now I have a different kind of strength. Not the strength that tells me I need to hide it and be the go to person, but the strength to speak up about how I feel about it, to openly process my feelings and admit to myself that “I am sad” and truly know that it’s ok to feel that way.

The disaster I call my youth is a whole other story for another time, what prompted this post was a fellow member of my daughters support group for her genetic disease, who reached out to me a while back with their raw, true emotions. They told me about the pain they live in – both physical and mental, the ridicule they received for the way they looked, how it caused them to have anxiety and depression, lack experience in relationships and how every now and then, they feel like it would be easier to just end it all. They went on to tell me that they know how they would do it and have sat there alone at night legitimately pondering it, this was one of those nights they were struggling with that decision. So, there I was again, feeling sad. It scared me for so many reasons. Was I  the last chance? Can I handle this pressure? For them, their family, their friends, to carry on their pain without them in a different way and I thought back to the days following Mermaid’s diagnosis when every word this person was saying ran through my head like a freight train. I listened and listened and I listened, all the while, feeling slightly selfish that it had my mind wandering back to own thoughts, but I continued to listen.

I made a decision while on maternity leave with Mermaid that I needed to make a life change. I was burnt out working in Primary Care and truth be told, it’s never where I wanted to be, but I was so mesmerized by the two people interviewing me at my first potential job in the real world that I took the job. The manager was from Canada and his accent made me laugh but he was so educated, and the RN was stunning. She was beyond put together, very proper, soft-spoken, this twinkle in her eye and I knew I could work for them. Well, almost 10 years later there I was, still in Primary Care plugging away. That very nurse had spent the first 5 years of my career teaching me right from wrong, had been a friend a team lead and encouraged me when I talked about movement. Now that I wanted to make a move it needed to be angled back at what I originally wanted, and there it was, truly – like big flashing arrows pointing at the job posting. Mental Health. I applied and shockingly I got it, I thought for sure this was going to be the greatest transition ever.

Well I thought wrong, at least for a while. This is the absolute truth; upon my arrival I was point-blank told by multiple other staff members that they didn’t want me there. They had no idea how I could be a piece of their process and one person honestly said to me that he thought I was delusional about working there. Don’t worry, those people no longer work there for reasons completely unrelated so I am not outting anyone in our present environment. At the time, their world consisted of Psychiatrists, Therapists and a few nurses that didn’t see their patients on a daily basis but were tucked away on the phones handling the back scene crisis situations. The department was changing and growing, adding more nurses who were slowly becoming more involved in the daily routine, Medical Assistants, Case Managers and Social Workers. I was one of the first people to arrive in an expansion continuing to this day and spent the first two weeks sitting in my chair spinning in circles watching the clock because nobody would hardly even speak to me. I knew I had to prove myself but needed help. Help is just what I got, one of the Psychiatrists who was onboard with me being there actually told me that he had helped fight for more staff. and support. He led me through some basics and continued to support my presence on a daily basis. Slowly his patients grew to like me and in time, I had a small space where I fit, but I couldn’t shake the initial distaste for my presence.

As I continued fighting , completely determined to prove everyone wrong, to show my worth, I had a whole other struggle that came to light just after my transfer. Mermaid’s complicated new diagnosis of Epidermolytic Hyperkeratosis with Linear Epidermal Nevus . I will never, ever forget those first few hours after leaving the specialists office who diagnosed her. As things slowly settled into my brain it moved into weeks and months of anxiety. I would close my office door of my new job and just cry. As if it wasn’t hard enough to walk down those halls every time I needed to get a patient and face some of the people who had made me so uncomfortable, I now had to wipe away tears and gather myself enough to not look like a complete mess. I would repeatedly tell myself, I am sad, and that is ok. At that point in time it was so new and the extent of her lesions were unknown. I had all of these anxiety’s; would it take over the whole left side of her body? Would she be embarrassed? Be made fun of? Would others see it as gross? If she got cancer, how would I know? What if I didn’t catch it in time? Will it be painful? Make her sad? Will she become depressed? Will she someday feel the sadness I feel now? I was consumed and scared. Until I figured out how to move on from that dark place where I cried nearly non-stop, at a job where I was already so alone, those anxieties remained. It was awful.

Eventually I found my footing in both World’s, I actually received a few apologies for the things people had said to me and massive compliments about sticking it out, one of them happily admitted that he was wrong for how he felt and the things he said,  I thought that was big of him to share but I have never forgotten the one person who accepted me from day one, Dr. Pavan Somusetty. I have never forgotten how he made me feel, had I not had him on my side, I certainly wouldn’t be there now,  no intentions of ever leaving, bossing people around, completely confident in my work and considering most of my teammates family. As time went on I also found my way with Mermaid’s diagnosis. Over the years we have openly shared our journey and been blessed that her nevus has not spread to the extent it could have or might still, we maintain it well and despite the controversy around the possibility of childhood kidney cancer, we are just about a year away from wiping that off the worry list all together. For now, we are both happy, healthy individuals, my initial feelings about her diagnosis often get lost in our daily lives, until that night. Our fellow Nevus owner who sought me out as a safe place to share her fears took me back to that place. That place where even though Mermaid is small and healthy now, Icouldn’t help but think again if that be the same story later? Will she one day be reaching out to someone in this very same way? Every case is different, some are simple, some complex, none of us can completely identify with another’s road, but we can agree that the more extensive cases have a very high potential to cause social out-casting, pain, anxiety & depression. That night I was reminded of my original fears and I remember how alone I felt with those fears, so I listened.

Just as I did that night, I will to continue to put this out into the world for everyone; if you are struggling, feeling sad, lost or a potential danger to yourself, reach out. Your identity and personal struggle will remain private. I will do my best to be a good listener, share what I have learned and attempt to find you resources in your area. I am happy to say that those suicidal thoughts and feelings this individual was having have passed, for now, but that may not have been the case if they remained quiet. Holding that strength to say things out loud allowed them to process their thoughts easier, and all I had to do, was listen.

I can’t tell you how many times a week I hear someone on the other end of the phone say “you probably didn’t need to know that, but thanks for listening” or asking me if I have just a few minutes for them to vent, and I always will. Where my sadness surrounding Mermaid’s diagnosis during a difficult transition to a new job never left me suicidal, it certainly left me empty until I found a healthy way to change that. I am not immune to suicide. As I took in all this person’s fears that night and reasons it would be easier to just end it, I flashed back to when I was much younger, to a classmate in the 7th grade that I use to walk home from school with sometimes, who took his life in the family home just a block away from me. I couldnt process the magnitude of that at 12-13 years old,  I thought about another friend that took her life a few years ago leaving behind a husband and children and I thought about another one of my very closest friends who was supposed to be at my house for game night but died in a car crash chalked up to being under the influence by authorities, though some people claim it was completely deliberate, and I wondered, was this all they needed? Someone to listen, without judgement? To tell them it was ok? So I continue to listen.

I’m glad I had someone on my side when I made the leap to Mental Health, someone who believed in my value adding to my decision to stay because the department and their cause are a piece of me now. I have learned so much about myself and others as a result of working in this world. I have learned that being sad, is ok, it’s normal. It doesn’t mean I am depressed or suicidal, it simply means, I feel.  That talking about the things making us feel that way opposed to hiding them, is refreshing; but more so, that suicide can be prevented.

The person who told me to “say it out loud” left this world less than a year ago, changing my life forever. The person that listened to me all those years, let me be angry when I needed to be, distant when I was incapable of closeness and taught me to say out loud that I was sad, is no longer here. She occasionally struggled herself and where she was outwardly happy, inside she was a bit lost herself, despite her own thoughts she continued to be there for me and I will never forget how she changed the way I feel. Just as I will never forget how that nurse in my initial interview all those years ago at the beginning of my career made me feel, like I wanted to be a part of that world with her, so I made the leap. A few years ago this individual decided she could no longer handle her own struggles, and took her own life. I will never forget that in a time when I was going through something so personal and difficult, while basically being told I didn’t belong, that one Physician made me feel welcome. I knew he was special from day one, his patients know he is special now and I am so proud to say that tomorrow, you can tune in to hear him yourself.

Entercom radio has put together a nationwide broadcast called “I’m listening” that focuses on breaking the stigma attached to talking about mental health. The radio program will be live at 10 a.m. ET on Sept. 10 for a two-hour, commercial-free live broadcast, featuring personal stories and a call-in number during the show so listeners can share how they’ve been impacted by suicide.  Metallica, Halsey, Bleachers and Nirvana’s Krist Novoselik will be among some of the people speaking and sharing stories.

The conversation will be led by BJ Shea from KISW-FM in Seattle and will offer guidance and resources from Christine Moutier, M.D., Chief Medical Officer of the American Foundation for Suicide Prevention; Dr. Ursula Whiteside, clinical psychologist, CEO of NowMattersNow.org; and my friend, Dr. Pavan Somusetty, Psychiatrist and Assistant Chief of Mental Health for Kaiser Permanente Northwest.

To read more about the broadcast or find your local station airing it; click the link below:

http://www.imlistening.org

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We have all been effected by someone with Mental Health issues, perhaps you have experienced a loss, been around another person you were unsure how to help or heard someone’s story that triggered your own dark places, whatever your connection, it’s time to speak up and let others know you are listening.

Tomorrow morning, at 7am my time, I will be up with a cup of coffee tuned into 94.7 for the broadcast. I invite you all to join me for that cup of coffee from the comfort of your own home and just listen. Take in the stories, the advice and remind yourself to be open, that mental health is real. Together we can bring it to the forefront of healthcare where it is treated with the same social acceptance as the common cold. A better understanding of one another.

Listen for Pavan’s interview and if you have thoughts, pop on here or my Facebook and share them.

To the ones I have lost and mentioned, thank you for being a part of my story today, you are forever in my heart.

Looking forward to our Sunday morning coffee date!

Thanks for walking with us today,

Mermaid’s Mommy

 

 

This entry was posted on September 10, 2017. 1 Comment

Hunting for facts…

It never fails, each and every time I think I have this whole nevus World figured out; another piece of research comes out, a tidbit shared from Physician to patient or some publication from a Scientist pops up that was never even considered – changing everything. Or does anything change at all??? That’s where this world becomes tricky, the information varies and sifting through facts vs theories is often a task. To boot, medicine is forever evolving and new discoveries are happening all the time, when and how those discoveries are shared is not uniform. Depending where you are in the World can change how much or how little you know.

Either way, I think everyone can agree that we all want research to continue, a lot of us want to be a part of it, having that chance to help move advancements along gives us a sense of purpose and we all want a new little morsel that gives us hope for treatment. I wish that what I was going to say next was…  GUESS WHAT?!??! I have ALL of those things for you today! But I don’t. I DO however have a few updates to some recent conversations. So let’s get to it!

I continue to hear people state that they attempted to join a study we were referred to by the Amazing Lauren (founder of the best support group ever) without resolve and I was perplexed. On your behalf, I reached out to resolve this. I realize some people are new here so I am going to take a few steps back and explain WHAT this study is first.

 Dr. Keith Choate at Yale University School of Medicine/Yale-New Haven Hospital is running several research studies for people with skin disorders. One of them is specific to ILVEN. The goal is to find out how inheritance of specific genes causes some people in families to develop skin diseases. This is done by analyzing DNA molecules. Some studies may take years to complete and they may never find the true cause of the mutation, but if they do, they may be able to develop better tests and treatments in the future.

To participate you will be required to release your last biopsy. You will need to sign a release at the medical facility holding that biopsy allowing them to send it to Yale to be examined. It includes a background on the Nevus owner. Parental consent, family skin conditions, symptoms, origin, PCP & Dermatologist information, Grandparents information and other medical history of the family. This is fairly lengthy. Next & lastly it requires a biological sample. Saliva can provide much of the same genetic information as a blood sample so depending on your location and whether you have recently had specific blood draws, they may ask for just one, or possibly both to obtain the patient’s DNA.

The DNA will then be screened for mutations and stored indefinitely for future use.

Sounds pretty cool right? Well it IS cool! What’s even more impressive is that we went into this study with the sole intentions of giving our information and truly never hearing anything again, but to my surprise, in the 2 years since we participated we have had multiple phone and e-mail conversations with Dr. Keith Choate himself. He is so kind and informative, just recently he let me know that they were just working on Mermaids sample and may potentially be able to use us in a separate study as well. Being that Dr. Choate has always been so receptive to us I took a shot and sent him an email asking if the study was still accepting new participants. My understanding is that many of you have inquired without response. Keeping with his reputation Dr. Choate personally picked up the phone and called me.  We had a quick chat about Mermaid, the study and another small tidbit I will share in just a moment. The long answer is YES, the study is still ongoing, in fact, he needs people more than ever! There were a few factors in the lack of response, one was due to some staffing issues creating an abundance of work for people not necessarily equipped to handle to volume of requests and bigger than that was the funding. Research needs money, when it’s lacking, research stops from growing. GREAT NEWS… the NIH just funded this project big time. That means it’s time to get involved! Opposed to using the old contact information Dr. Choate asked me to tell everyone to email him directly and he will get you started.

So here is a simpler view of the study and how to get involved. 

ILVEN study: 

Who? Dr. Keith Choate at Yale University School of Medicine/Yale-New Haven Hospital

What? Research how the inheritance of specific genes causes some people in families to develop skin diseases by analyzing DNA molecules in hopes to develop better tests for diagnosing and potential treatment.

How? Email:  Keith.Choate@yale.edu

Why? Because you can! 

Keep in mind, you may never hear anything at all. You are agreeing that Yale may use your samples solely for research and that is all. If it is medically necessary, they will contact you. Please limit the email’s to inquiring about the study, he has been so open and kind that we do not want to burn this bridge as a contact, trust me – if there is a need, he will get in touch. Yale covers the cost of shipping all materials back. If you want to see pictures of what the paperwork or specimen tube looks like you can click my link below from when we submitted our package. Now get to it! The more the merrier and we NEED your DNA! 

https://mommysmermaid.com/2015/08/28/signed-sealed-delivered

Here is the other small tidbit. While on the phone with Dr. Choate I wanted to inquire if he had any information on a potential break through in the past few weeks in the UK. It’s a long shot but when the opportunity presents, I try. It was mentioned on a forum that a Physician in the UK had just made some break throughs in the past two weeks. That this specific Physician was able to isolate 2 specific genes that may be responding to treatment. This was all still in the research phase but gave a lot of hope to our small little Nevus world. 

Dr. Choate first stated that this specific Physician is a brilliant scientist and absolutely has the capabilities to do this, but did he know anything specific to what I was speaking? No. However, he did inform me that he recently gave a talk that included information on the mutation that causes CHILD syndrome and it’s possible, it is related to this. CHILD syndrome (an acronym for congenital hemidysplasia with ichthyosiform erythroderma and limb defects)  is an inherited disorder, affecting primarily women, that is characterized by ichthyosis-like skin abnormalities and limb defects on one side of the body. The skin is usually dry, itchy, red and scaly and it comes with a list of potential side effects. Sound familiar? A little like ILVEN? That’s because the two can be misdiagnosed as the other. They both have swirled linear patterning, however, in CHILD syndrome the mutant gene is in every cell, but that gene is active in only parts of the body. The gene in CHILD syndrome is NSDHL, which is related to ones cholesterol. Their research has found that those with CHILD syndrome may benefit in a treatment combination of topical and oral cholesterol medications. It’s interesting for sure, we all know how easy it is to be misdiagnosed but the important note here is that ILVEN is NOT CHILD syndrome. 

I appreciated the information either way and found it fascinating that the two were so similar, it was also a good reminder that Dr. Choate works with all kinds of genetic disorders, not just ILVEN; but I still wanted to try to get an answer to these 2 mystery genes that had been mentioned. I’m sure you can guess what I did, I reached out to the UK of course! I really expected a dead-end but to my surprise, I got a written response from the Physician named in this work. The response will explain why I am protecting this individuals identity. As I touched on above, research costs money. No money, no trials, no publication. If an individual seeking funding from larger or national organizations spills the beans about their findings to the public, the funding could be cut, or in some cases, not backed to begin with. In this particular case I was informed that currently, they are lacking funding. 

Without funding the response I received was guarded, they informed me about the lack of funding and alluded to a potential compromise if information shared in private with participants or those inquiring were to be made public. I was told point-blank that there have been no recent breakthroughs in the past few weeks. That over a few years there have been SOME but nothing ready to share or be published. The Physician was kind and in fact, offered to share the publication with me when funding allows that to happen potentially in 2018. I did respond asking if I could quote a few things mentioned or share their name but that was not spoken too. So out of respect for the Physician I will not share their name or personal information.

My take away is this – Yes, there is research happening in the UK with a brilliant scientist. With more information and funding there may be specifics to share next year about findings that occurred over the past few years. In the past few weeks there has been no new movement. As much as I would like to tell you that I confirmed 2 specific gene types associated with ILVEN are currently responding to experimental treatment, I just can’t. I will continue to dig, keep my ears open and as always – share anything I come across. 

I pride myself in presenting the most accurate facts and I hope you have all placed your trust in me, I assure you I spoke directly to two very large figures in the US and UK performing this work. If you have something to add or know another source I can follow-up with, by all means, send it my way and I will dive right in. I apologize for the delay in response with some of this information but I wanted to make sure it was as accurate as possible. 

So tonight, I leave you with this… reach out to Dr. Choate and inquire about participating in the study, tell him we sent you! Know you are doing your part to further education and research and who knows? Maybe this time year I will be sharing some exciting new treatment from the UK! 

As always… Thanks for walking with us! 

Mermaid’s Mommy

 

 

Fish out of water Part duex…

Ask and you shall receive, maybe not the first try, but try, try again we did! We recently reached out asking for advice from our readers about their knowledge and/or experience with nevus owners overheating. We received a lot of good information but nothing lined up with Mermaid’s “LEN” so I began doubting the cause. I started researching and honestly, came up a bit empty handed so I went back to ground zero and looked at her biopsy again. I often forget that the official pathology report came back as Epidermolytic Hyperkeratosis WITH Linear Epidermal Nevus, not vice versa so I restarted my search on the EHK and wouldn’t you know, it was everywhere!

EHK is a form of Ichthyosis and the information linking the two with potential overheating was abundant. It was relieving to know that my original gut feeling that the cause was related to her genetic disease but a small piece of me needed validation. After all, what if I was wrong and ignored signs of some other underlying condition. The chances seemed slim since she has otherwise been a healthy thriving child but I reached out anyway.

Our first (and I anticipated only) connection was with Mermaid’s general dermatologist. I was shocked that without a doubt not only did he say that her LEN had zero connection but he stated that I possibly misunderstood her biopsy and she did not in fact have EHK. I spoke to him about a plethora of information we had received from the children’s hospital directly related to her EHK and he admittedly stated that this was out of his wheelhouse but stood by his stance that there was no connection. Perplexed and frustrated I reached out to her Primary Care Doctor as the dermatologist advised to begin the search for an underlying cause.

Primary Care seemed as equally perplexed as I was and again eluded to this being a bit out of their wheelhouse, they were slightly taken back at us being directed to them but did some research and found a few possibilities. There were a few “conditions” that also had links to dermatological conditions that could be the underlying cause so we agreed to go forward with testing. Her PCP felt obligated to search for anything he could and being that we were led his direction we were going to travel that road with him without question. That same night with the promise of SEVEN new toys (thank the lord for the dollar store) I carted a screaming Mermaid in for blood tests and her chart was sent to neurology for a chart review. Most of the tests were straight forward and results poured in less than 24 hours later. Negative after negative after negative. I was happy, but still felt frustrated to have no concrete answer or validation.

The following morning as soon as I started seeing the negative results I made one last attempt at answers and reached out to Oregon Health and Science University where the Pediatric specialist for dermatology we previously met with resides. On our luck he had a same day appointment and off we went.

I left happy, aggravated yet understanding on all fronts. On our third connection we were assured that yes, Mermaid DOES in fact have a bit of a double whammy. She has EHK & LEN, just as I thought, EHK is a form of Ichthyosis and BOTH of them have the potential to cause overheating depending on the location. The vast majority of Mermaids fills her left armpit and as a result has clogged her sweat glands preventing her body from cooling itself properly. Essentially it causes her to internally heat up quicker. I suppose this sheds a small light on my midget sized nudist.

So now what? Well, the PNW has had some higher than normal temps and we have definitely learned that above 90 in direct sun she enters her danger zone. In the absence of a body of water we are limited to where we go if it the heat reaches that range. We started using Tazorac for a few weeks to see if we can thin the thickening near her sweat pores to allow some breathing room. There is no guarantee but smack dab in the middle of summer, any small relief is a huge feat. We followed up with all entities to ensure them it was in fact a direct result of her genetic disease and also advised that a body temperature above 105 is directly to the ER opposed to attempting to cool her ourselves at home. I guess I should replace that thermometer she broke.

We appreciate everyone that reached out to us and shared your wisdom, I can assure you that the moment you see your little one turn purple, vomit and fall asleep so deeply it’s hard to wake them, you too would be in a bit of a panic. I can’t possibly begin to imagine how she felt. I compare it to those of us without an underlying condition attempting to run a marathon in 105 degree weather while wearing a sweat suit and no water to drink. I’m so proud of how smart my Mermaid is to recognize when she has been out in the sun long enough. She is very vocal in telling me it has been enough and time to go, brings herself inside to cool off knows to continue staying hydrated. She has had so much to learn about in her short 4 years on this earth and always adjusts without a hitch. Maybe Mermaid’s are smarter than we ever knew!

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Here’s to hoping we have an otherwise eventless summer!

Thanks for walking with us today!

Mermaid’s Mommy

Fish out of water…

My Goodness, It’s been ages since we have given any kind of update, which might lead one to assume that all has been calm on the bay front but it’s actually the complete opposite! Spring led us to Doernbecher Children’s Hospital where we obtained some very useful information but between her short baseball season and additional appointments we have been noticeably absent from our virtual home.

We have many posts coming soon about all Mermaids new discoveries throughout Spring but today there is one main issue at the forefront of our fins. I distinctly remember one of our very first appointments a brief mention that “she may have some blockage of her sweat glands” and that was that; until now there has been very little thought placed on that statement but I never forgot. Summers have always been fine, she often claims she is “hot” on a regular basis and keeping clothes on her is a mission in itself but honestly, I chalked it up to being normal with NO concern whatsoever.

About a month ago we were at an outdoor birthday party, the day was especially hot for our area and the yard offered little to no shade. They had games with water balloons, plenty of cold fluids and ice cream so even though it was a scorcher, everyone tolerated it just fine. Mermaid has long been asking for a new play kitchen and the little girl celebrating her big #5 just happened to have one in the house along side her Mom’s real kitchen. Throughout the party I kept finding Mermaid in the house near that kitchen, My initial thought was that she just wanted to sneak off and play with it so I continuously led her back out to the party in the yard. She participated in a game where they placed water balloons on a spoon and had to run to the end of the yard and back without dropping their balloon. As I stood at the end taking pictures I noticed she seemed more flush than anyone else. When they were done she quietly went and sat on some steps by a bubble machine. I snapped a picture and when I set my phone down I noticed her color had drastically changed to a purple shade and her breathing seemed somewhat labored. I went from this is  just normal hot weather behavior to “Oh my Gosh what is happening”  in 2.7 seconds flat. I scooped up Mermaid and ran her in the house, she had no energy and I took her dress off as quickly as possible. For the first time in our lives I instantly knew what was happening. She was truly overheating. The left side of body where her nevus lives was bone dry and the right side was drenched. She begged me not to put it back on and began to cry. Within minutes I apologized and rushed her to the car and blasted the a/c. By the time we got home, almost 45 min later she was still roasting and eventually threw up. I felt terribly guilty for having her out in the heat like that and prayed it was a one time fluke.

Not so lucky, a few weeks later we were at a family members graduation party, again, it was a hotter than normal day for the PNW and we were outside. We were at my brother’s house and there was a bit more shade than the previous party plus they have a small pond that the kids typically flock too to catch frogs and tadpoles. More aware of her potential to overheat I kept a closer eye on her and just like clockwork, she went from fine to that scary shade of purple again within seconds. This time family was present and keeping an eye on her as well and because she refused to miss the chance of catching a frog we made her continuously return to the table for drinks of cold fluids, gave her ice cubes to hold and a wet cold rag to hold on her face and neck. Just as before the concern grew to the point that we chose to make an exit but this time left before it got so bad she threw up.

Below is a picture from the 2nd event, not the most flattering but imagine, the first time it happened her face looked WORSE than it does here!

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Just yesterday we went to visit my parents at an outdoor market where they had a booth set up, it was significantly cooler than the other two days that incidents occurred but still hot and I was very aware of the potential. She started out by sitting in my parents chairs eating some lunch we brought but shortly found a table in the shade behind their booth at a local restaurant. She seemed happy there but a worker quickly approached us and asked us to leave the table. I kindly explained that she would be done eating in 2-3 minutes and pointed out that there were multiple other open tables so I  bought Mermaid a short window but her retreat from the direct sun ended quickly.  We were not there long before she sat on the ground and proceeded to try to take her clothes off. I quickly stopped her and said No baby, not in public, when she grew weepy and said she couldn’t wear her clothes anymore. She proceeded to tell everyone she was too hot and that was my queue, off we went. Once in the car and a/c she used my water bottle to hold on her face and neck and kept asking me to turn the air on, despite the fact that it WAS on and cold enough I was looking for a sweater. Within minutes of being home she threw up. Daddy and I go her some cold ice water, a fan and had her lay down to rest while continuing to cool off.

So here we are, a random overcast day and I am reaching out. My gut says that this reaction is linked more to the EHK than LEN and is just now coming to life as her Nevus has slowly spread & thickened over her sweat glands on the left side. I can not find any direct information linked to her LEN that suggests this is a potential side effect but most Ichthyosis articles have share a direct link to overheating. Additionally, I read an interesting piece stating that the rapid turnover of cells in individuals with Ichthyosis require more energy thus meaning the host needs more calories to grow. Maybe, JUST maybe, this could be a link to Mermaid’s insatiable need to always be snacking like a teenage boy. I also cannot say that any of our followers with ILVEN or LEN have spoken to this directly and perhaps those with congenital, Giant or Ichthyosis can shed more light on this topic?

Mermaid falls in this weird category where she has three (One we recently received @ Doernbecher that we will share later) separate diagnosis working together to complicate the understanding and treatment. Is it simply that we need to acclimate her body leading up to summer or trips to warmer climates or is there truly no way to prevent it? Will sports be an issue? I am reaching out today looking for your expertise, tips & tricks to help us in our newest conundrum. I am at a loss as to who we should go to first to discuss it. Her PCP, her dermatologist or her genetic specialist @ the children’s hospital. Help guide us!

Coming up we have a new trial of a natural homemade cream thanks to a local friend, will dabble in some more salve making ourselves with intentions to get some out to a few of our special followers and a post about some other new discoveries added to Mermaid’s ever-growing list of diagnosis.

If you have experience in the overheating world please share your thoughts! We look forward to hearing your expertise and jumping back in to some nevus focused time!

Thanks for walking with us today,

Mermaid’s Mommy

A new beginning to an old problem…

 Those of you that have been following us since day One, know that we have had our ups and downs with our healthcare system regarding Mermaid’s nevus. For the most part we feel taken care of and have access to care in many forms. However, we do not have access to a pediatric dermatologist or one who specializes in “Genetic skin diseases”; which has left us perplexed at times and receiving a variety of responses and care plans that range from aggressive to very passive. Several years ago I requested to see a specialist at Oregon Health and Science University and was denied. In recent months Mermaid has developed new spots and symptoms that sent us searching for another avenue to make our way up the hill.

This past week, our persistence and patience paid off. At the recommendation of a fellow Nevus Mommy’s pediatric Dermatologist on the East Coast, we got our day with Dr. Kroll. A pediatric Dermatologist that specializes in genetic skin diseases. Leading up to today I knew what we wanted answered specifically and got those answers, plus some. The main concerns I wanted addressed were… What are these new spots that don’t appear to be nevus? Will she have to have surgery at school age? Despite the biopsy not showing ILVEN, is it still a possible diagnosis? and what is her real risk factor for a Wilm’s Tumor?

I will start with what we learned followed by the answers to our questions and then our treatment plan.

Mermaid’s main diagnosis was Epidermolytic Hyperkeratosis (EHK) with Linear Epidermal Nevus (LEN). We have always focused on the LEN and put little to no thought into the EHK, aside from it being a bit more rare than the LEN. Well, news to us… the EHK comes with its own potential complications. We learned that sometimes, a female with EHK on one side of the body may have it on that parallel ovary. If/when she chooses to have children she runs the risk of passing on that gene in a full body form called Ichthyosis to her children. This is a small possibility, studies have shown that it’s a 2-10% chance but we were advised to have her be seen in genetics as a late teen to further discuss this and they hope with medical advancements that they will have a test to better diagnose whether or not the disease is actually on her ovary.

He felt certain that she does not now, nor ever will she, have any of the larger complications of her LEN resulting in the syndrome. Those symptoms typically arise at a very early age, with the exception of potential learning disabilities such as delayed speech, which has never been an issue, trust me – she speaks just fine and knows more than she should to be only 4!  He reminded us that any small lump beneath the skin should be checked as there is a risk for squamous cells to develop.

As we already knew, he reiterated that there is no effective preventative medical treatment, we should see the extent of her disease by puberty and along the way there are two different laser treatments that may help in minimizing some of her nevus but the only true treatment would be complete excision with the trade-off of scars.

With some new and old but reassuring information we got the chance to clarify some of our burning questions…

What are Mermaid’s new spots that don’t appear to be nevus?

Unfortunately, Mermaid got cursed with two genetic diseases. What we originally thought was eczema, then a potential reaction to a soy allergy has now been confirmed as Psoriasis.

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At the time of her appointment Mermaid had roughly 27 spots like the one above spanning head to toe. Including her face. If you saw our previous post about the mystery spots, it was also confirmed that the scales beneath her eye are Psoriasis.

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Will she have to have surgery at school age?

The long and short of this question is still, we don’t know. The area of concern is Mermaid’s hand. Her Nevus grows thickest at her wrist and top of her left  hand. This is also the only area that has cracked and bled. When her Nevus breaks open she is at risk of further infection. It can also cause pain and limited movement when it grows over the joints. His initial feeling is no, we will NOT be doing surgery next year. This was a huge relief, the thought of putting my child under and performing a very painful excision at such a young age was worrisome. The reason we cannot get a direct yes or no to the surgery question is that Mermaid’s form of Nevus grows with no rhyme or reason and putting a definitive answer on the future is nearly impossible. If it remains as is or has only a small amount of growth we are in the clear. If it grows extensively in the next year we run a higher chance of following through with surgery. One of my fellow Nevus Mommy friends shared pictures and experiences of her daughter’s hand surgery and it truly broke my heart. She is incredibly strong and did great but it was far from a pleasant experience for her or Mom.  Please, please, please… keep your fingers crossed!

Despite the biopsy not showing ILVEN, is it still a possible diagnosis?

In the big scheme of things the difference in diagnosises is minimal and doesn’t mean a whole lot. So why would I care? Up until now, I have been told that ILVEN can be treated with steroids to reduce discomfort, redness and inflammation. I have asked before about using it for her bothersome areas but was told it is not a treatment used for LEN. Being seen on the hill was my goal because they are the specialists and just as I originally suspected, he said that even IF another biopsy showed ILVEN, it would NOT change the treatment plan. He stated that ILVEN & LEN are treated the exact same and we would begin treating it today.

and last but certainly not least… That lingering fear since Day one of her diagnosis…

What is her real risk factor for a Wilm’s Tumor?

The very first day Mermaid was diagnosed we were told point-blank that we needed to be aware of something called a Wilm’s tumor, childhood kidney cancer. She would be at risk until age 5. Since that day I have probably prayed harder than any Mom out there for her 6th birthday. I have vowed it would be the party of the century because that day, she would no longer be at risk and I could lay my anxieties surrounding this cloud to rest. Along the way the other dermatologists did not all agree but they also didn’t dispel the possibility. Nobody seemed to have numbers or facts supporting it so we continued to be aware.

Dr. Kroll finally put a stop to the worry. He seemed a bit surprised as we told him about this and very matter of fact stated that he has never heard this, especially in an otherwise healthy child without the syndrome. He said that if she had some of the big components of the syndrome such as one side of her body being larger or boney structures there may be a study or two to support a link, but in her case, No.

I’m sure you can imagine both my frustration and relief. Letting go of my frustration was easy, after all; this is why we pushed to be seen there.

So overall we learned of some potential side effects Mermaid’s EHK, that LEN & ILVEN can be treated the exact same, that we will continue to watch her hand’s growth over the next year, that she is now the proud owner of EHK, LEN, AND Psoriasis and best of all, that the risk of a wilm’s tumor is no higher than that of any other child.

Up until now we have not treated her skin issues in any way. We have done a few prescription strength creams such as Nystatin and 1% Hydrocortisone but mostly use home remedies to doctor what we can. That has all changed.

Hanging on my fridge is a crazy calendar marking out what medications need applied what day. We are starting a retinoid called Tazorac to be used specifically on her wrist and hand 2-3 times a week, Triamcinolone Ointment for her torso twice daily and Tacrolimus Ointment on her face and neck 5 days on 2 days off.

In one month we will re-evaluate its effects and create a plan from there. It’s a lot, it feels slightly daunting but I am beyond thankful that we are finally beginning a treatment plan and hopefully bringing her some relief. I am also thankful for our amazing insurance that covered the $750 cost of a months medications.

I have no doubt that in a few short weeks we will be reporting about success!

Moving forward we will now be followed @ OHSU for annual check ups and any major changes. For non urgent concerns and medication refills we will still utilize her other dermatologist. It feels like we are finally on a satisfying path.

Stayed tuned for happy updates!

Thanks for walking with us today,

Mermaid’s Mommy

Mystery spots…

It’s been a confusing 8 weeks in Mermaid’s world when it comes to her skin. About 2 months ago she had what appeared to be a black eye arise out of nowhere. As time went on the skin beneath her eye bubbled up and eventually peeled off. It has since remained rough and scaly, much like an older spot on her back. Then, a few small dots on her back turned into a round blistery patch and just like her eye, peeled off and has since remained rough and scaley. The presentation of both spots is not exactly like her normal nevus but has some similarities.

The timing was impeccable considering we already had a series of routine appointments set up in the coming weeks, so I didn’t worry much knowing we would have answers soon.

Eye#1

Without knowing exactly what Mermaids new spots were and one of them being present on her face I wanted to be careful with what I chose to put on them as far as medication goes. With a few weeks to our first appointment I chose to just use a salve that we had made over the summer.

Mermaid’s Grandparents have bees so we have access to all the wonderful benefits bees bring us. Last summer we experimented with making some of our own products with beeswax. One of our endeavors was an anti-itch salve with Beeswax, Shae butter and a few other secret ingredients.

I was pleasantly surprised with the level of improvement our salve provided. Specifically, the spot on Mermaid’s back that she was asking me to scratch 8,432 times a day, stopped being bothersome during times of its use. I am happy that the salve has prevented further itching but we still didn’t know what we were really dealing with.

First up, we had contact with our dermatologist. He seemed pretty confident that what Mermaid has is not spreading of her Nevus but either Eczema or Seborrheic Dermatitis. He suggested we use 1% Hydrocortisone and let him know if it didn’t clear up. Honestly, I hesitated with the hydrocortisone because I had read about it not being the best for use on the face. Her Dermatologist even mentioned that too strong of a medication could potentially cause the pigmentation to stay, despite his suggestion I simply opted to wait.

Our next appointment was with our Primary Care Provider, once again he stated that he did not believe these were related to her nevus and agreed with dermatology that it could be Eczema but also thought it resembled Pityriasis Rosea, if that were the case she may develop more of a rash on her body and needed to let him know, though it is not contagious. He supported using the hydrocortisone 1% both on her back and face. With 2 people agreeing on its use I picked up a tube and for no other reason than being busy that evening I never got around to using it.

The following day we headed in for one last appointment with the eye specialist. The first thing she noted when entering the room was Mermaid’s face. I appreciated that it bothered her because it had been driving me crazy for nearly 2 months! She asked what it was, to which I replied honestly – I have no idea but nobody seems overly concerned. Seems like it might be eczema….? This particular day it was angry. Red, swollen, extra flakey, so she really got a good version of what it looked like. I shared the previous encounters and the plan to which she strongly disagreed. She felt that the medication they were suggesting was fine for her back but too strong for her face, as I initially felt concerned about. In turn she ordered a steroid cream to pick up and promised in 3-5 days we should see some improvement.

So really… what do we know? Mermaid has something that might be Eczema or maybe it’s Seborrheic Dermatitis or possibly even Pityriasis Rosea or maybe they are all off and its new scales developing as we speak! With the lack of a clear answer I decided NOT to use either medication advised by the Physicians and have stuck with just our “Bee honey” as Mermaid calls it. It truly keeps her from scratching and seems to keep it moisturized enough yet obviously fails to actually clear anything up. I trust all of our Physicians and we are not dealing with anything serious where my decision to not use the medication right away would cause harm but I truly want to be confident in what we are treating before treating it.

I am only confident in the decision to avoid medication therapy at this point because coming up in a few short weeks we will be going to Doernbecher Children’s Hospital, a unit at Oregon Health & Science University to see a Physician who specializes in Pediatric genetic skin diseases. With the variation of answers I would rather be certain about what we are treating before putting anything on Mermaid’s face. With the variation of “possibilities” and discrepancies in treatment we are sticking to our daily routine of  “bee honey” in hopes that the specialist has some concrete answers to what these new spots are.

Praying that Mermaid’s new spots are NOT Nevus and impatiently awaiting our big day at Doernbecher!

Does anyone have anything that looks similar to Mermaid’s pictures above or have a form of nevus accompanied by other severe skin issues? Looking forward to hearing all your thoughts!

Thanks for walking with us today!

Mermaid’s Mommy

 

A new shell…

This is my life book. It has been by my side for almost 6 years now. It is always near me. I carry it in my bag and it’s rare the two of us are far apart. When I get to work, I take it out of my bag and set it next to my computer.

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It’s tattered, torn and hanging by a thread, but you know what – it’s full of so much love. Inside this book are my ideas. Those pop in your head, don’t want to lose them ideas. It has over 5 years of finances carefully listed and checked off month after month. It has party invites, my step son’s 8th grade graduation program, Mermaid’s official pathology report from September 2015, a picture of a friend I just lost, stuck through the back pages is a guardian angel pin gifted to me. Some of the pages are burnt as I was writing in it one night by the fire when I was pregnant and a hot ash caught it on fire. It holds a card my husband made me, a goodbye note from a Physician & friend I highly respected that moved away, a list of all the places I want to visit and a recipe my Mom hand wrote.

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Just last week the entire cover started tearing off and I taped it up with scotch tape. One would think I could just go buy a new one but it holds sentimental value. As I was taping it up I thought to myself, this is kind of like my life right now. Barely held together, full of so much love, but in need of a new shell.

You see, these past few weeks have been more trying than I can even begin to explain. Mermaid had what I thought was nevus starting to grow on her face, the moment it started to bleed I finally took her to urgent care, with no answers we were led back to dermatology and thank the Lord, it just ended up being eczema but we are now battling that. I have had some challenges at work and been faced with making very difficult decisions, I have had to allow myself to be vulnerable, fight anxiety and search within for what was the right thing to do. A few days ago I woke up overcome with grief. I sat down in the shower and sobbed, missing my friend who passed a few months ago. Her passing is still fresh and has completely altered my life. I barely made it to work and while there, I wished I cold just get in my car and drive to heaven to have a cup of coffee with her. That being completely unrealistic, I contemplated driving anywhere, just going, clear my head, but I didn’t. I stayed, I held up my responsibilities and tried to power through.

Mid-week I told myself to shake it off. I have so many blessings and hard times will come and go. Find a new shell.

That morning when I got to work, there was a package from a friend. It contained a Mermaid journal, a coffee card and a letter with 9 reasons why she sent it to me. So once again, I sat there crying. This time, happy tears. She spoke to work, my Mermaid and the loss of my friend, but #9 was my favorite. “Just because!”

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She didn’t know about my life book until I told her that morning, she just knew I was struggling to find peace.

She gave me my new shell.

When  I told her how much it meant, she kindly said “See, people are listening.”

It’s easy to forget our blessings, to dwell and struggle to find our footing; and that’s ok because somewhere along the way, something small will remind you. Give you a breathe of fresh air and a chance to start new.

I put some of my hard decisions in motion, I started yet another nightly routine for Mermaid’s skin and I talked to my friend driving home that day. Ok, so I talked to myself, but I know she heard me. I told her to help me find a way to let go, not forget, just let the grief go.

Yesterday Mermaid was suppose to be cleaning her room but instead she made me a bracelet. She was so proud of herself and asked me to wear it to work. When we got home today she found it on the table and said “Mommy, you forgot to wear your bracelet to work” – ashamed, I apologized. A few minutes later she came out of her room with a different bracelet and said it’s ok, you can wear this one, it’s your favorite. This was not my favorite bracelet, it’s not even mine, it’s hers. She can’t possibly remember where it came from and why she said that, or chose it, I can’t explain; but I remember where it came from. A few year’s back, my friend that passed, gave it to her while we were visiting in Seattle. Maybe it was her small way of reminding me of the good times, telling me it’s ok and that she heard me.

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This weekend as I transfer my life book to it’s new shell I will embrace the new beginning and here are 9 reasons why:

1.Because I have an amazing family that supports my every move.

2.Because I know deep down inside, the hardest decisions are often the right decisions.

3.Because as of now, Mermaid has no complications as a result of her disease & we can jump any other hurdles thrown our way.

4.Because I have 18 amazing years of memories with my late friend to smile about, not cry about.

5.Because when we think nobody is listening, they are.

6.Because today, I felt the sunshine on my face for the first time in a while and it reminded me that the clouds always clear.

7.Because the positives I gain through work outweigh all the negatives.

8.Because I am slowly learning, 35 years into this life, that it’s ok to stumble as long as you get back up.

and

9. Just Because!

My Dear Kathy – I thank you for my new shell and your humorous but powerful words. I thank you for listening. I thank you for reminding me of the good. My promise to you is to listen to someone else, and when they least expect it, send them a new shell.

Today, you are an honorary Mermaid.

Thanks for walking with us today!

Mermaid’s Mommy