Creating a community

Networking with other parents who have children with Moya Moya Disease saved my sanity

By Kristen O. McTigue, LSW

“She’s fidgety…..and you need to see the doctor??” That’s how our family doctor’s receptionist responded to my request for an appointment on that Monday morning almost seven years ago. I didn’t have a chance to explain to her that my daughter’s handwriting had changed, or that she was falling out of her chair at school. However, I was insistent. Something wasn’t right, and I wanted my daughter seen. With a rather exasperated sigh, she made us an appointment for that Thursday.

During the next three days, my daughter’s speech began to slur. Thankfully, our doctor, who hadn’t ever met us before that fateful Thursday because we had only moved to town two months before, really listened. He trusted “the mommy instinct.” He sent her for a CT scan, which lead to an MRI, which put us at Children’s Hospital in Boston.

Ten days and lots of weird questions later, (“Has she eaten any raw meat lately?” “Has she been bitten by any strange insects?” “When was the last time she left the country?”) my daughter had been poked, prodded and had tests I never knew existed. Finally we met with a neurosurgeon who explained that our daughter has a very rare, progressive neurovascular disease that only 1 in every 2 million people have – Moya Moya Disease.

“Moya Moya” is a Japanese phrase that, when translated, means “puff of smoke.” That’s what the diseased vessels look like on an MRI – a puff of smoke. Moya Moya causes some of the blood vessels supplying the brain to progressively narrow until they are completely closed off.

While there is no cure for the diseased vessels, surgeons can “re-route” some of the vessels from the scalp to the area of the brain affected by the diseased vessels. The hope is that the “re-routed” vessels will like their new home, and supply enough blood to that area of the brain so when the diseased vessels are totally occluded, there is no change in your brain function. However, it is kind of a race against time, and it involves brain surgery. For my child, it would involve two separate surgeries – one on the left side and one on the right. My daughter was only 8 years old, and I felt like we were living a nightmare.

Much of that time years ago seems like a blur to me now. I remember the nurses telling me how lucky we were. There are only two Moya Moya specialists in the US: one at Stanford University in California, and one at Children’s Hospital in Boston.

People from all around the world travel to Boston to see him. We drove for less than an hour. Two months before, we had been living in North Carolina. The timing was perfect! Perfect? They’re about to operate on my baby’s brain! Twice! And you think I am lucky? I didn’t feel lucky. Mad, scared, panicked, overwhelmed, nauseous..lots of things, but not lucky.

Over the next five months, our lives changed in ways I could never have imagined. Our daughter had her first surgery, and had some scary, life-threatening complications. She recovered, but needed to spend a few weeks at a rehabilitation hospital for intense physical, occupational and speech therapy before having the second one. Letting go of her hand as they wheeled her into the operating room the second time was the hardest thing I have ever done.

As I write this I have that feeling in my stomach that I had that day – one you can only imagine if your own child went in for surgery – please take me instead. The second surgery went much more smoothly. There were some minor complications, but we were finally discharged to home on Christmas Eve. We had been in Boston since the middle of October.

In February, we had our second daughter, the identical twin to the first, tested for the same disease. While the surgeon had been assuring me no genetic link to the disease had been found, and the statistical probability of her having it too was nearly impossible, my “mommy instinct” couldn’t let it go. I wasn’t sleeping. It’s all I could think about, and I was obsessed. Finally, the surgeon relented and ordered the test, more to reassure me than anything else.

Not only did my second daughter also have the disease, but it had progressed further than her twin’s. One in two million people have this disease, and I have two in my family.

I think going into surgery was harder for my second daughter. She had seen all the possible complications that could happen when they happened to her sister. While it all looked cool before – the ice cream, the staying up late to watch movies, the hospital clowns, alone time with mom – now she was seeing all of the IV’s, the no eating, the EEG leads glued to her head – this wasn’t so fun after all.

I am happy to report my second daughter’s surgeries went off without a complication. She had two separate brain surgeries, and was in and out of the hospital in eight days. She was on the road to a seamless recovery, and the race against time with this disease was on.

I wish I could say my emotional health recovered as easily. I had now become scared for my girls. The “what-ifs” were keeping me awake at night. What if they hit their heads? What if they become dehydrated? What if other kids tease them? I became really overprotective. T

he girls’ doctors, teachers, our friends, all tried to reassure me. They tried to tell me to relax. They offered to babysit so I could have some time to get away. As wonderful and loving as they were, and as well meaning as their intentions were, getting away to relax wouldn’t help me right then. That would only leave me alone with my “what-ifs”.

I felt as if no one got it. No one could really relate to my feelings, because they hadn’t gone through a situation like that. I needed to talk to another parent, one whose child also has this disease. However, as far as I knew from the girls’ surgeon, the closest family to us with Moya Moya Disease lived in Buffalo, N.Y. We couldn’t exactly get together for coffee.

I don’t remember how I first found it. I’m pretty sure it was by accident. But truly, it saved my sanity.

A gentleman, who has since become a good friend to our family, started the website MoyaMoya.com because he too, was diagnosed with Moya Moya Disease. He was also overwhelmed to learn there was no one remotely close to him with this same medical condition. And he needed someone to talk to that “got it.” Honestly, I thank God every day that he did.

What a difference this one website has made for our family. To be able to talk to other families living with the same medical condition has provided us with knowledge, insight and experience we couldn’t have gotten elsewhere. We have made some amazing friendships. It has been so helpful for my girls to meet other folks with Moya Moya – to see that they’re not alone. To see strong, intelligent, independent women and men go on to lead normal, happy, healthy lives, and that Moya Moya hasn’t stopped them.

For me, meeting other Moya Moya parents has changed my life. I learned how normal my reactions after the girls’ surgeries were. With the help of other MM families, I was able to lighten up a little, and be less overprotective. I was able to stop obsessing over the “what-ifs” and gradually go back to having typical kid experiences.

With the help of grants from the New Hampshire Council on Developmental Disabilities and in the past, the Harry Gregg Foundation and CCACHC, three times since my girls were diagnosed, our family was able to attend a gathering of families of people with Moya Moya Disease.

I cannot describe how amazing it is to network with other parents. It was so helpful to hear from them how they approach certain things my girls have difficulty with – they had perspectives we could only get from a family with this disease.

Families who saw the opposite surgeons (one in California, one in Boston) were able to trade tips – for instance, the surgeon in California recommends no sky diving, our surgeon recommends no soccer. We’ll be avoiding both now, thank you very much.

It was so helpful for my girls to commiserate with other MM patients, about their moms nagging them to drink more water than you can ever imagine – over an over and over again. And to compare their scars, and even, as the case might be, whose surgeon’s assistant was cuter? (Yes, my girls are now 15 and terribly boy crazy.)

Meeting other Moya Moya parents has really helped change my whole way of thinking. While there is still some uncertainty in my girls’ future – the disease had only been discovered 10 years prior to my girls’ diagnoses, so there are not a ton of long-term studies – I now consider that uncertainty to be a gift instead of a burden.

There are parents who know for sure their children won’t live to be a certain age because of a devastating illness. And while it’s true – maybe my girls won’t live to be 99 year old little old ladies in their rocking chairs – maybe they will.

Thank you, fellow Moya Moya parents, for giving me the hope, the understanding, the incredible patience, and for giving me my sanity back.

Kristen and her husband are the parents of three children – - twin daughters, and a son. They live in southern New Hampshire. Kristen is a social worker and works for Partners in Health in Nashua.