In the Quiet Moment

May 23, 2016 Family Stories, Kristi, port wine stain, Sturge Weber Syndrome, Vascular System 1 Comments

On March 20, 2009 I spent the quiet moments of nap-time looking at waiting child programs with various adoption agencies. Home from China just four months with our second child, we weren’t ready to expand our family just yet, but as a planner I wanted to have ‘all my ducks in a row’ for when the time came.

I did not realize it at the time, but my actions that day would soon change the makeup of our family. One search took me to a waiting child list and as I scrolled down, I saw the ‘slightly blurred for privacy reasons’ photo of a chubby baby girl. Keep in mind that when I saw it, this photo was blurred, but somehow she looked familiar; and I felt as if I had been looking for her for a long time.


Kristi-1


Her information was sparse, listing a birthdate, the fact that she was a smiley baby, and a diagnosis of ‘neurocutaneous syndrome‘, which was something that was completely unfamiliar to me. A quick Google search lead to much confusion, as well as some very scary terms, but then there was that face… So I sent an email to my husband that read something like, “All I ask is that you look at her information and pray…there is something about her.”

In the weeks that followed, after getting more information and contacting several specialists, it was determined that her specific neurocutaneous syndrome was likely Sturge-Weber Syndrome. Up until this point I had never even heard of SWS, so the learning curve was high. What we found is that Sturge-Weber is a relatively rare syndrome presenting with several common symptoms and a huge window of severity. The following information from the Sturge-Weber Foundation’s website gives the best summary I’ve seen.

“SWS has no clear genetic pattern, and two affected individuals almost never arise in the same family. The syndrome presents in all races and with equal frequency in both sexes. Port wine birthmarks occur in 3 of 1000 newborns.

In a patient with a facial port wine birthmark, the overall risk of having SWS is only about 8% to 15%. The risk of having SWS increases to 25% when half of the face, including the ophthalmic division of the trigeminal nerve is involved and rises to 33% when both sides of the face, including the ophthalmic division of the trigeminal nerve are involved.

Seizures occur in 72% to 80% of SWS patients with unilateral brain lesions and in 93% of patients with bihemispheric involvement. Seizures can begin anytime from birth to adulthood, but 75% of those with seizures begin having them during the first year of infancy, 86% by age 2, and 95% before age 5.

Glaucoma occurs in 30% to 71% of patients.”

We spoke with a local neurologist who previously ran a Sturge-Weber clinic, one of the nation’s leading pediatric glaucoma specialists, a highly recommended dermatologist known in the area for treating facial port wine stains, and a fellow adoptive mother from our area’s adoption group.

Basically we came to realize that moving forward was going to require a leap of faith because from the limited information available to us, it was difficult to tell exactly where this little one fell on the spectrum. We could see the port wine stain, and glaucoma was highly suspected, but the information from the MRI that had been done left the neurologist scratching his head due to the vocabulary used to describe the images that someone across the world had interpreted. Basically he told us that while the information that no seizures had been recorded to date was promising, there were some confusing terms in the MRI report and he wasn’t sure if lesions on the brain were being confirmed or denied.

I think the turning point in our decision was speaking with our pediatrician, one who realizes that there is an Ultimate Healer. As he reviewed what limited information we had and painted a picture from best case to worst case scenario for us, he shared some words that moved me profoundly: “…just remember that you are not considering adopting a condition, you are considering adopting a child, THIS child. Pray, and if God gives you peace, then He will give you what you need to parent THIS child, whatever the road brings…” And so it was after much prayer that we were led to the decision to make her a part of our family.

Exactly 11 months from the day after I first saw her picture, we walked into a cold civil affairs office in Hangzhou, Zhejiang Province and there she was.

Our daughter.

I was smitten at first sight, but my sweet girl, at not quite three years old, was already self conscious of what we have come to refer to as her ‘red cheek.’ When she began ducking her head and trying to hide from us I came undone, but by the end of our time together we began to see glimpses of the girl to come, and she went from shy and avoiding the limelight to a little girl willing to strike a pose for the camera.


Kristi-4


We arrived home on a Thursday night and hit the ground running with appointments. Monday to the pediatrician, Tuesday to ophthalmology, Wednesday neurology, and Thursday dermatology. In that first week we learned that she had advanced glaucoma in her right eye, we’d begin treatments for her port wine stain in the fall, there was no apparent brain involvement in her case, and she was “unofficially” diagnosed with Type II Sturge-Weber Syndrome (meaning the port wine stain and glaucoma were present but with no detected brain involvement).

Within her first month home she had her first eye surgery to help alleviate the high pressure in her eye. Six months later was the first pulse-dye laser treatment for her port wine stain. Going forward there would be two more eye surgeries, and to date she is in the 20s for number of laser treatments (I’ve honestly lost count). She has a daily regimen of three different types of glaucoma eye drops. With her eye, skin, and craniofacial orthodontist appointments (her complex ortho case is most likely related to tissue overgrowth in her jaw area due to the port wine stain) we drive to Duke at least once a month, but it is manageable…most days she’s just another third grader.

It was about a year after she was home and settled that I was contacted by an adoption advocate who asked if I would be willing to help as she advocated for another little girl with the same condition. Her adorable little face tugged at my heart, and while we were getting ready to travel to bring home our fourth child, I agreed to be a resource for people with questions about Sturge-Weber.

I emailed, texted, Facebook messaged, and spoke on the phone with a number of families, but none of them were fully at peace with making her a part of their family, and about 18 months later it became apparent why…because she belonged in our family.

Afterall, we knew the symptoms, we had the doctors lined up, and we had a girl who had once asked for a sister “with a red cheek like me.”

Fourteen months later, we were in Changsha, Hunan Province becoming a family of seven, and just a few months after that we began our double appointments at Duke.


Kristi-3


Yes, the medical appointments are many. Yes, we are on a first name basis with several specialists at Duke Children’s Hospital and I have their personal cell phone numbers stored in my contacts. But beyond all of that, they are your “everyday girls” and an integral part of our family.

We realized early on that Sturge-Weber Syndrome is one aspect that describes our little girls, but it is NOT what defines them. One is a free spirit, brilliant, loves art, a good friend, loyal, a dancer who gets lost in books. The other is our firecracker, a take charge gal, a math whiz with an amazing imagination, a limit pusher, and in the rare moments that she slows down enough, the world’s best snuggler.

Both just happen to have SWS and we wouldn’t change a thing.

– guest post by Kristi: facebook || email || blog



One response to “In the Quiet Moment”

  1. Tabitha says:

    Hi Kristy,

    Can I use your quote from your doctor for my organization’s social media? Beautiful post!

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