By SARA ARTHURS
It’s been six years since Consolation “Connie” Shaffer first experienced symptoms of a rare neurological disorder, moyamoya disease. Now, after multiple brain surgeries, she is grateful for the support of her husband and community, and trying to raise awareness.
Shaffer, of Pandora, felt what seemed like a “shot of novocaine” in her face in September 2010. Tests found blocked arteries, and she wound up at the Cleveland Clinic where she was treated by Dr. Peter Rasmussen, a neurosurgeon. Rasmussen is a staff physician in the Cerebrovascular Center of Cleveland Clinic, medical director of Distance Health at Cleveland Clinic and associate professor at Cleveland Clinic Lerner College of Medicine of Case Western Reserve University.
Rasmussen said moyamoya usually affects the carotid arteries, the main vessels supplying blood to the brain. As they come into the skull to supply blood, they begin to spontaneously narrow. So the brain tells the body to create new blood vessels to supply it with blood. This tends to lead to one of two things, Rasmussen said: either the blood vessels aren’t formed correctly so they don’t supply enough blood to the brain, causing strokes; or they are fragile and get overtaxed, rupture and bleed.
The name “moyamoya” comes from a Japanese phrase, “puff of smoke,” because the newly formed blood vessels can form a sort of spider web which may resemble a puff of smoke on a scan.
If untreated, it can lead to recurrent strokes over time. This includes both bleeding strokes — in which blood vessels rupture and bleed into the brain — and ischemic strokes, which is when the brain’s demand for blood supply exceeds what narrowed arteries can supply.
Once moyamoya has been diagnosed, “there really is only one option,” surgical treatment, Rasmussen said. He said medications such as aspirin and blood thinners may manage the symptoms, but not really treat it.
The goal is revascularization, meaning making new pathways for blood to get to the brain.
“It’s like solving a plumbing problem,” Rasmussen said. Not enough blood is getting where it needs to go, so doctors create “new pipes,” he said. But unlike the pipes running through your house, this is living tissue, Rasmussen said. It changes and grows over time, becoming more robust. About 90 percent of the time, patients who go through these types of surgeries do not have further strokes or bleeding episodes, Rasmussen said.
Rasmussen said there are two ways of revascularizing the brain: direct and indirect bypass.
Direct bypass is similar to a heart bypass operation, when surgeons bypass the blockage. In this case, they use an artery that normally supplies blood to the scalp, cut it out of the scalp and connect it to supply blood to the brain instead.
Shaffer had this procedure done on the left side. Her husband, Ryan, said it is a 12-hour procedure. She had also, early in her treatment, had a stent put in the right side.
Rasmussen said the direct bypass is the best option if there is a sizeable artery in the scalp that can be used. However, frequently in patients with moyamoya the arteries are too small, which was the case on Shaffer’s right side. So, she had an indirect bypass on that side. This involves the large chewing muscle on the side of the head, which powerfully closes the jaw.
What doctors do is move this muscle from outside to inside the skull, removing the bone underneath so that the muscle lays on the surface of the brain. The brain will then keep sending out chemical messages indicating the need for more blood, prompting new arteries to grow, Rasmussen said. So new connections are formed as arteries grow from the muscle onto the surface of the brain.
Shaffer had this procedure done earlier this year, after she found herself feeling tired, and went back to the Cleveland Clinic.
She said she has received good support from friends, and is especially grateful to her husband.
“He’s been my rock,” she said.
They’ve also received support from Pandora churches and the greater community. Shaffer said she is thankful for the grace of God. The couple attend St. Michael the Archangel Roman Catholic Church.
Now, Ryan Shaffer said, they are working to raise awareness.
Consolation Shaffer, now 51, said she had had no warning signs. But her mother had a stroke at a young age. Moyamoya can be hereditary, and is also related to diabetes, which Connies also has.
Rasmussen said moyamoya is most common among certain ethnic groups, including people of Japanese and southeast Asian descent. It can be genetic and in some cases is related to other conditions, including thyroid disease or, in children, as a rare complication of chickenpox.
“It is a frequent cause of stroke in young adults and children,” Rasmussen said.
So he encouraged people who have a loved one who has a stroke when they are young — under age 50 — to be aware of this as a potential cause, as addressing it can reduce the likelihood of more strokes in the future.