Lily’s Journey with Linear Nevus Sebaceous Syndrome
Lily is a smart, active two year old and the light of my life. If you meet her, you might also notice that she has large, flesh-colored lesions on her face, head and neck.
She was born with linear nevus sebaceous syndrome. In addition to the lesions, the syndrome also affects her central nervous system and eyes. Around three months of age she started having focal seizures and then around 6 months she had infantile spasms.
I had a normal pregnancy and didn’t learn of her condition until after she was born. I of course had all of those self-doubts that many mothers have when their babies are born with a condition. What did I do wrong? What could I have done differently? I was told that nevus sebaceous has no known cause, occurs within the first six weeks of gestation, and there was nothing I did to cause it.
Once Lily received her initial diagnosis, I asked the doctor in our hometown of Whitesburg, Kentucky, if she was going to live. He said yes, there is no reason to believe that she won’t live a full life. From that point forward, I knew that so long as that’s true, we can handle whatever is to come.
Like many parents facing a very rare condition, we started the process by doing some research. We found our way to Cincinnati Children’s because we read about the experience Dr. Kara Shah in dermatology has with this rare syndrome.
Despite Lily’s condition, I don’t consider her different than any other child. She just has a few more obstacles. Let me tell you about the ones she’s been through so far.
She’s had a total of 10 or 11 small procedures as well as drug therapy to control her seizures. Her doctors in the Comprehensive Epilepsy Center were able to find a drug, Sabril, to successfully end her seizures as of July 2013.
Once the seizures were under control, we were able to start thinking about how to remove the nevi, or lesions. Because the nevi can become irritated and painful, as well as develop into skin cancer in her teenage years, we decided to embark upon a multi-step procedure to remove them.
That’s when we were referred to Dr. Sitzman in plastic surgery. He recommended that Lily undergo tissue expansion to ultimately remove the nevi. Essentially what’s involved is expanding the skin so much that there is enough for the surgeon to completely cut off the nevi and attach the healthy skin together.
The process of stretching the skin is pretty remarkable. It’s not painful for Lily at all but it does require her to sit still. It’s about a 30 minute process and it takes two of us. We first start with a numbing cream and then distract her with either the TV or the iPad. One of us holds her still while the other fills the 10 ml syringes with saline and then attaches it into the butterfly needle. The expanders are filled through a port little by little until the skin has been expanded enough for Dr. Sitzman to perform the surgical procedure.
She has undergone two surgical procedures to remove the nevi. The first was April 2014 which removed 60% of the lesions and the second on April 29, 2015. She’ll need at least one or two more rounds of the tissue expansion and surgery to remove the remaining nevus on her left cheek, a small patch on the right cheek, on her neck, chest, and left shoulder.
We feel very fortunate to have found the resources Lily needed. She may have some more hurdles ahead of her – some developmental milestones to catch up on – but we are happy with the progress she has made so far in her journey.
When people ask me what it’s like to have a child with this condition, or how I deal with it, the answer is simple. I am positive I was chosen to be her mother for a reason. Maybe it was to share her story in order to educate others about people with special needs. Maybe it’s because I am a special needs teacher and I already had perspective. Regardless of the reason, I wouldn’t change any of it for any reason. All of these different parts, all of the things she’s had to go through, make her who she is. She’s Lily, and she’s awesome.