If you met my six-year-old daughter today, you would see right away is that she loves singing, dancing and all things girly. You would also see that she has the world’s best smile because she uses her whole face to show how happy she is. You would definitely see that she is one of the gentlest creatures on this earth. If you looked closely, you might see the two seven inch scars running along each side of her head from two brain surgeries. You might see that she wears a leg brace to help her walk more steadily.
What you would not see is that she has a rare cerebrovascular disease called Moyamoya.
Evangeline, or Evie, was adopted from China when she was almost two years old. Her medical file noted that she had tight limbs, which was assumed to be a form of cerebral palsy. We immediately began physical therapy, and while she was able to start walking, it became apparent that something larger was at play. Evie began having horrible migraines, which would leave her incapacitated and vomiting for several hours at a time. We requested an MRI in June 2012, hoping for some answers, but we did not like the answer we got. Our world was rocked as our precious Evie was diagnosed with Moyamoya disease. She had already had two strokes and was at serious risk for another at any time.
When a person has Moyamoya, one or two of the four arteries that carry blood to the brain become occluded, or close off. The brain tries to compensate by growing a new set of tiny, fragile blood vessels to bypass the blockages. This puts the affected person at risk for many issues, including cognitive impairment and strokes (with all the issues those can cause). There are no drugs that can treat Moyamoya disease. The only treatment is surgery. The surgeon must bypass the blockages by introducing new healthy arteries.
So, our first critical step was to find a qualified neurosurgeon. Because of the rarity of Moyamoya and the delicate care needed not only during surgery, but post op, we were not comfortable with anything but the best for our daughter. The two hospitals with the most case experience are Stanford and Boston Children’s Hospital. Literally the day after her diagnosis, our heads were spinning and our hearts were heavy, but we met one of the world’s foremost Moyamoya experts, Dr. Steinberg, at Stanford’s annual Moyamoya picnic. We had to fight our insurance company to be seen out of network at Stanford, and took our case to the media and the Department of Managed Care. We won, and Evie had two brain surgeries a week apart performed by Dr. Steinberg in August of 2012.
Because she received treatment at the hands of someone with so much experience, Evie came through the surgeries without having any additional strokes or damage. While we had to wait a full year to get the MRI and angiogram to find out if the surgeries were successful, we knew within a couple of months that she was healing. Mercifully, her migraines stopped. Almost overnight, she became more articulate, and at age 3 ½, finally learned her colors.
When she did have her first post-op imaging done, it showed that the surgeries were successful. The new arteries that Dr. Steinberg had placed to bypass the blockages were healthy and bringing in the required blood flow. However, the disease is progressive, meaning the original vessels that were affected by Moyamoya will continue to occlude and die off, and we saw this in the images. But as long as the new arteries remain in place and stay healthy, this should prevent more strokes and cognitive impairment. Evie will have another set of imaging done at three years and then ten years post-op. If all continues to look good, then she won’t have to have any more surgical treatment.
So, what is day-to-day life like for us now? We are almost three years post-op at this point. Evie takes asprin daily to help maintain easy blood flow. She also has to stay very well hydrated for the same reason. On rare days that we don’t keep up on her hydration, she gets headaches, but they are nowhere near the severity they used to be.
The biggest daily difficulties we face are the effects of her strokes, which mainly affected her left leg. Despite regular physical therapy, Botox treatments, and wearing an orthotic on that leg, she does not have a normal gait. She walks slowly and with great effort. Her leg is very tight and often after walking just a short distance, it causes her pain. At this point, Evie is still small enough to be in a stroller when we go places that require a lot of walking. However, as she gets older, we are pretty certain that she will require a wheelchair for long days at places like theme parks or zoos.
Because Moyamoya can also cause cognitive or learning impairments, Evie is also slightly delayed in some academic areas. We, and her teachers, feel she will catch up eventually, at her own pace. And, while this cannot directly be attributed to the Moyamoya, she is also very slow at many things. This is likely an effect of the strokes. She speaks slowly, as it takes her just a little longer to form her thoughts into words. She gets dressed slowly, writes slowly and eats slowly (unless sushi is involved).
But she does it. She does it all.
As long as access to the proper surgical treatment by an experienced team is available, I feel that this special need is very manageable on a day-to-day basis. We can give her aspirin and extra fluids. We can help her put on her leg brace. We can take her to physical therapy and guide her through her stretches. We can take her to her neurology and imaging appointments. We can spend a few extra minutes helping her with homework. We can listen patiently as she tries to find the right words to tell us what’s on her mind. We can push her in a stroller now, and in a wheelchair later if need be. These are very small things. We do them, and we do them with great honor.
If you met me today, you would see that I have a face full of pride. You would see that my heart is full of joy and gratitude. If you looked closely, you would see that even though I have a few extra tasks on my plate, I count myself among the luckiest of parents. If you met my six-year-old survivor today, you would see why.
A fantastic, comprehensive resource for anyone who would like to learn more about Moyamoya can be found here. However, I also realize that some questions can only be answered by someone who lives daily with the disease. So if you are someone considering a child with this special need or if you have just received this diagnosis, and feel I may be able to help you in any way, please do not hesitate to email me.
— guest post by Lindsey
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At NHBO, we love Family Stories. And we know our readers love them, too. Beyond being encouraging and informative, Family Stories shared here have actually resulted in children coming home to a forever family. If you’ve adopted a child through China’s special needs program, we hope you’ll consider sharing your family story. It just may be the reason another family considers that very special need.
Lovely post!! I truly appreciate the posts highlighting some of the lesser-known needs, and how open the parents are about the day to day reality of them.
Yes, I understand this! Our 12 year old from China has this as well. She also has NF1. It can be a long, hard road, but what a blessing to go through it with someone you love, who otherwise might not have anyone to care. We use Chicago’s Lurie Children’s Hospital.
Thank you for sharing this update!
Caroline,
Evie also has the clinical diagnosis of NF1. We are hoping the Moyamoya is the worst she will experience because of it. I totally agree with you…we are so humbled and grateful to share this journey with her, come what may. This child is a treasure.
Sending hugs to your family, Lindsey
Lindsey, it’s great to hear that your daughter’s treatment was successful and that she is doing so well. A friend’s son, also adopted from China, has Moya Moya as well. His surgery was done several years ago in Boston. Does NF1 sometimes go along with Moya Moya? One of my children is being watched for NF1.