Moya-Moya Huh? What is that?

This is a common question that we have been recently asked. When we first heard the term is was about a year ago (May 22, 2009). It was after Mattie had her strokes and the doctors at the time "suspected" she had a rare disease called Moya-Moya, it affects about 1 in 2 million. At first Dan and I could not comprehend it and we called it every name in the book from Maui-Maui to Mooey-Mooey. Over the past year Mattie has had one cerebral angiogram and recently a MRI/MRA. Last week she was officially diagnosed with the disease Moya-Moya.

So what does this mean?

Moya-Moya syndrome is a progressive disorder that affects the blood vessels in the brain. It causes stenosis (narrowing) of the carotid artery in the brain. The carotid artery is responsible for delivering blood to the brain. When the stenosis occurs, it makes it difficult for blood to pass throw the artery and the result is a stroke. At the same time tiny new blood vessels form and open up to attempt to supply blood to the brain. These tiny vessels are called Moya-Moya. Moya-Moya is a Japanese word for "puff of smoke". On a brain scan these tiny vessels look like a puff of smoke. As stated in the beginning it is a progressive disorder and there is no cure for it and as patients get older who are diagnosed with Moya-Moya they will eventually experience side affects because there brain is not getting enough blood. Such side affects are headaches, TIA's (mini-strokes), major strokes, bleeding on the brain, numbness and seizures.

There are a few surgeries that can be performed to help with re-directing the blood flow to the brain and the surgery would help with reducing the side affects of the disease.

Mattie will eventually have the surgery we are just not sure when it will happen and more than likely she will have side affects to Moya-Moya but we don't know when. It could happen today, tomorrow or 3 years from know. My husband likes to describe it has a little bomb in her head and we never know when it will go off. Of course Mattie is so young and because communication is hard for her she may have experienced something but we don't know. It is up to Dan and I to watch her and her behavior to make sure she is acting ok and if we suspect anything seriously wrong we are to take her to the hospital.

From what I understand the surgeries have been very successful and allow patients of Moya-Moya to live normal lives.

The other questions we get is how did she get it?

The answer to this question is we don't know and no one knows. It is known that the disease is genetically hereditary in the Japanese cultural but what causes it is unknown.

Mattie has secondary Moya-Moya and that means that the disease occur ed because she has another underlying genetic condition, which for her is Down-Syndrome. With this there was something in her brain that just did not form correctly.

There are also healthy people who all of sudden experience some problems and they are diagnosed with Moya-Moya, it could basically happen to you. The chances are rare but it could happen. The chance for Mattie to get Moya-Moya is one in 2 million. Crazy Huh!

At this time Mattie is doing great and we feel that she has not had any side affects. She has re-gained the use of her left side and is showing great strength and use of the left side. She takes 1/2 tablet of low dose aspirin every day to help keep her blood thin. She has another MRI/MRA in 6 months to see if there has been any changes with the Moya-Moya vessels and the stenosis of the artery.

So that is the update on Mattie and what is going on with her and our family. It is something that is very hard to understand and there are numerous times during the day that we think about it. We just never know what the next step will be. If anyone has any questions please feel free to ask. It is a new learning experience for us and questions are what helps us learn and get to know the disease a little bit better.