Sophia

So today seems as good day as any to give the full story about Sophia's condition (get comfortable if you're interested!)

She was born with a birthmark, called a giant congenital melanocytic nevus. Basically, it's a mole. It is made up of the same cells that make up your moles. The thing is, it covered her entire chest - from her belly button to her neck, and from side to side to her back. She also has hundreds of "satellites," which are smaller nevi (plural of nevus) scattered all over the rest of her body. These range in size from a freckle to about five inches in diameter. They all look like moles, including the giant one. Most of them have hair on them, some of which is darker and coarser than normal hair (we shave it).

This is a 1 in 200,000 random thing. It is not genetic or hereditary, nor has it been linked to nay cause. No one really knows why or how this happens, but sometime around the 8th week of pregnancy, these melanocytes (which are the pigment cells in your skin) normally "scatter" throughout your body. They are supposed to deposit evenly throughout your skin and determine how dark you are. In Sophia's case, a whole bunch of these cells just globbed together on her chest to form her birthmark.

The main problem is that, as with all moles, there is the risk that malignant melanoma (the deadliest form of skin cancer) could develop in her nevus. Because the number of mole cells are so much more numerous than most people, she has a much higher chance of getting melanoma. Because her nevus is so large and her grandfather (Davis's father) had melanoma, her dermatologist estimates her chances around 20-25%, but it's really a guess. This condition is too rare and affects kids too differently to get really good statistics. I've read all the studies myself, and there are all over the board.

If caught early, melanoma can be successfully treated (Davis's dad is a survivor!!) The biggest things we can do to prevent it are (1) remove as much of her nevus as possible, (2) sunscreen her like crazy and keep her out of the sun, and (3) watch her closely for signs of change in her skin.

Additionally, because the melanocytes originated in the neural crest (along with brain cells) during development in the womb, there is a chance that some of these extra melanocytes ended up in Sophia's central nervous system. This can lead to a condition called neurocutaneous melanosis, which is basically when the melanocytes start growing in the nervous system (usually brain). This is almost always (95%) fatal. About 1/3 of children with a giant nevus get neurocutaneous melanosis, but again the figures are sketchy. Generally kids with a nevus on the scalp or back are at highest risk, though one study showed that the biggest risk factor was the number of satellites. There is not really anything that can be done to test for, prevent, or treat this condition. The fact that she's made it past the three year point is good though. As she gets older, the chances of her developing this get lower.

Because Sophia's chest nevus is so huge, the best way to remove it is with tissue expansion. Plastic balloons are placed under the healthy skin surrounding her birthmark. Slowly over a few months, we inject saline into the expanders so that they grow like balloons being blown up. This stretches (and actually makes new skin) the healthy skin. Her plastic surgeon then removes the birthmark and uses the stretched skin to cover the area. Other than the surgeries and recovery time, this is not a painful process. We numb her skin prior to the injections. The stretched skin feels like pregnant belly skin - a little tight and itchy and sensitive, but not painful. The cosmetic result of this procedure are amazing. Because you are replacing belly skin with belly skin and it is the adjacent skin (so it is never actually cut off) it heals perfectly and all that is left are the seams (which are beautiful - we are so lucky to have such a talented surgeon!)

When Sophia was fifteen months old, we had her first set of expanders placed. We had to travel out of state to find a plastic surgeon to do it, and we are blessed to have found him. Because we live far away, we did the expansions ourselves at home, instead of having the doctor's office do them every week. We expanded her for four months last time and had them removed when she was 19 months old. Her surgeon was able to remove about 70% of her birthmark. Sophia had a lot of complications from her initial surgery, but after that, she did great. She is such a trooper, and was really the biggest source of strength for us going through all of this.

Because her skin needed a year to recover, we placed her second set of expanders this April, and we've been expanding ever since. Medically, things have been going excellent this time. She had no complications from the surgery and was only in the hospital for three days. Unfortunately, the expansions have been really hard on her emotionally this time. She just dreads her "shots" and we can tell that it is causing her a tremendous amount of stress. She wakes up every morning and asks "we're not doing my shots tonight?" She bursts into tears several times a week, saying "I don't want any more shots." She has nightmares every night. It's just been a lot harder on her this time around.

We had two surgery dates set to remove the expanders this time - July 2nd and July 30th. I took the girls to Texas yesterday so that Sophia's plastic surgeon could tell us whether we had enough skin to remove them on the 2nd. Based on how her skin looked compared to her last expansion, I was sure that he would remove them on the earlier date. Even the resident who came into the room before her surgeon said he felt sure that they would need to be removed soon. Well, I was shocked to find out that not only did her doctor want to keep going, he wanted to continue on indefinitely until one of the expanders burst. This was not a possibility we had ever discussed.

A tissue expander "bursting" is not nearly as scary as it sounds - it doesn't explode or anything, it just tears and the saline leaks out of it. This isn't immediately dangerous, but it would require immediate removal (as her surgeon said "maybe in the middle of the night"). We always knew this was a complication that could arise at any time. We were not prepared to "plan" for this to happen. We are not comfortable with that option at this point, mostly because (1) her plastic surgeon is out of town all the time and (2) we live 6 hours away, and it would be really difficult to just pack up and drive (or fly) down there with no warning. For now, we are planning on sticking with the July 30th surgery date, but we're doing some thinking, praying, and consulting to make up our minds for sure. The goal of course is to get as much skin as possible, so that hopefully we will not have to repeat this next year. But I wonder if physically and emotionally we should give Sophia a break. Her surgeon suggested we might continue until October. Well seven months is a LONG time in a three year old's life, and every week gets more traumatic for her.

Well, this is where we are today... I returned home with the girls today (I took both of them so Davis could work) weary, but certain that we will figure it out and get through this.

God is my strength and power: and he maketh my way perfect.
2 Samuel 22:33