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!
Here’s to hoping we have an otherwise eventless summer!
Thanks for walking with us today!