It’s real. It’s rare. It’s deadly. This puff of smoke is here to stay.

Photo courtesy of Peter O’Donnell Jr. Brain Institute

“It sounds like Mahi-mahi! Is it named after the fish?”

“I’ve never heard of it…”

“What does it mean?”

Moyamoya is a Japanese word that means “puff of smoke” and describes the appearance of tiny capillaries that form to compensate for vascular blood vessels that thicken and close — the primary result of untreated Moyamoya disease. Imagine a tree stump — all that’s left after someone wielding a chainsaw removes the trunk, limbs, branches, twigs, and leaves — and the puff that consists of new saplings springing up around it. Now put the tree in place of the brain’s blood vessel network and you get the picture. If you are familiar with the Circle of Willis in the brain, you also know the location of the stump.

Our first experience with Moyamoya Disease was the day that Victor was diagnosed with this progressive — and often fatal — disease. This diagnosis came not long after he sustained a fall doing Rocky sit-ups in our hallway. The bar he was using to do a series of inverted sit-ups torqued — both fell, and Victor sustained a dent in his head that matched the one in our plaster wall, roughly three inches in diameter.

Why was he doing Rocky sit-ups? you ask. If you knew my husband prior to this event, you might think you already know the answer to this question. But you’d be wrong. Although he was known for creating crazy exercises for himself and his fitness students, this one was strictly personal. For months (maybe years?) he’d been feeling as though he needed more blood in his head, and what better way to get it there than by hanging upside down?

He had also asked for gravity boots and an inversion table, both of which I passed off as both too trendy and too costly. He never mentioned that he was in pain. Our kids, often with him in the car, thought that Tylenol and Advil were found in everyone’s glove compartment…and that the usual dose was five tablets every two hours.

In addition to the dent in his head, Victor sustained a concussion, which is by definition a traumatic brain injury (TBI). His fall occurred in late September, but a week passed before he was diagnosed with Moyamoya. The physicians at the hospital ran every test possible and when they observed the “puff of smoke” blood vessels on an angiogram, they advised my husband to “take an aspirin every day and watch for signs of stroke.” These were their exact instructions. No definition of Moyamoya included. No scans offered for review. No other information. And then they sent my husband home.

Victor’s headaches, which he already had been experiencing regularly, increased after the concussion. In March, he was finally accepted into treatment by a neurologist, who was convinced that the headaches were caused only by “post-concussive syndrome.” He treated Victor for migraines, injecting Botox as frequently as allowable, creating a Hellboy appearance that reduced tension but didn’t touch the sudden spikes, pressure sensitivity, and other extremely painful headaches that plagued my husband.

From a neurological standpoint, Victor was already experiencing deficits after his two diagnoses. When given a list of three things to remember (ball, blue, and chair), Victor came up with ball, blue, and stapler. This deficit was blamed on the concussion, rather than chronic lack of blood flow to the brain (due to the Moyamoya). When he experienced numbness and tingling in his hand, it was chalked up to elbow neuropathy, rather than a sign of impending stroke. There were many times his doctors could have made different choices, but remember, Moyamoya is rare. Many doctors haven’t heard of it, while others have Googled it only after seeing the diagnosis in a patient’s chart.

The typical Moyamoya patient is a young female of Asian descent, often with a family history of Moyamoya disease, or having another disorder, such as sickle cell anemia or Down syndrome.

Victor is Caucasian and male, with neither family history nor any other disorder, yet he is one of the 1 in 100,000 people diagnosed with Moyamoya disease every year. He is one of the many people affected by this rare disease, although percentage-wise it seems like he is one of the few.

Often, Moyamoya is diagnosed when one is searching for other causes of brain deficits, whether concussion, as in Victor’s case, or a primary disease such as Down syndrome or sickle cell anemia, as in other cases. When Moyamoya is primary, it is classified as a disease and when a secondary illness, it is classified as a syndrome. Either way, the symptoms and complications may render a person ill, disabled, or dead.

Victor was the last person who would ever be considered ill, much less disabled. He was the epitome of fitness — running half-marathons, raising funds by doing lunges for 400 meters, pushing cars for fun, teaching Athletic Training and Conditioning (ATAC), and single-handedly running No Limits classes — in addition to serving as the chief handyman and stable lackey at home. If it weren’t for his dedication to fitness, Moyamoya would likely have killed him.

He didn’t smoke. He didn’t drink. He was definitely not sedentary. He had excellent blood pressure and low cholesterol. He was healthy (so healthy) and young (too young, we were told by the Social Security office). But Moyamoya changed many of his abilities, including that of his brain to function normally.

Although Victor was diagnosed at age 40, he was 43 before he had his first brain surgery (a direct bypass) and at age 48, his second surgery (an indirect bypass) followed by his first stroke, a big one. The second surgery eliminated his headaches completely. The stroke eliminated his ability to speak as well as diminishing the use of his right arm and right leg.

Victor received full disability after the first brain surgery, retroactive to when he could no longer teach — or swim, or ride a bike. When the judge asked what would prevent him from working, his eyes filled with tears. He wanted nothing more than to work hard and play harder. Today he would love to speak and tell you all of this himself, but for now, I am the one to transform his thoughts into words and set them on paper. He used to be right-handed, but this deadly puff of smoke took away his right hand as well.

I am sure he often said (to himself) that he would give his right arm to be rid of the headaches that plagued him. Be careful what you wish for, they say. In the end, he did give his right arm. And his right leg. And his voice. But he will always have Moyamoya and its ongoing effects.

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