Facts and Feelings: Marie Elaine and Moyamoya with Dr. Connolly

When Marie Elaine suddenly lost the function in her right hand, she went to a neurologist. After taking an MRI, the neurologist sent her right to the hospital.

There, the cheerful science teacher found out that she’d had a stroke…or rather, that she’d had multiple strokes. One major stroke had affected her hand, but she had also been having “mini” strokes without even knowing it.

A self-described “science person,” married to a physician, Marie Elaine knew that strokes happen when there is a problem with blood flow in the brain. But what had caused her strokes?

As doctors ran tests to establish the facts of her situation, she just hoped for some cut-and-dried answers. “I have to tell you, my heart was in my mouth,” she recalls. “[I wondered,] can’t we just say I had a stroke but now I’m good?”

The tests revealed that Marie Elaine had a “dissected” carotid artery. This type of problem, in which the layers separate in the neck’s carotid artery can cause a stroke.

But the story didn’t end there. Dr. Dorothea Altschul, a neurointerventionalist who specializes in problems with blood flow in the brain, was one of Marie Elaine’s doctors. When she examined all the test results, something didn’t sit right. Dr. Altschul’s instinct and experience gave her the feeling that there might be more going on. She explained that she would like Marie Elaine to come back for a few more tests. Marie Elaine weighed the facts and her feelings, and then she agreed.

And it was a good thing she did. Dr. Altschul’s instincts and experience had been right on. The tests revealed something surprising: Marie Elaine had a rare disease called Moyamoya.

Moyamoya is a Japanese word meaning “puff of smoke.” The name comes from the appearance of the brain’s blood vessels on a diagnostic test called an angiogram: they have a delicate, cloudlike appearance, like a puff of smoke.

In Moyamoya, blood supply to the brain is reduced. To provide the brain with the blood it needs, the body grows new vessels. But the new vessels are thin. In an effort to meet the demands of brain for oxygen and nutrients, more and more of these delicate vessels grow and branch. This is what leads to the characteristic “puff of smoke” appearance.

Since Moyamoya is so rare, experts in its treatment are few and far between. But as luck would have it, a colleague of Dr. Altschul’s is one of those experts–Dr. E. Sander Connolly.

When Marie Elaine first met Dr. Connolly, there was a lot to discuss: the diagnosis of a type of Moyamoya, the treatment options, the prognosis. A bewildering array of facts and feelings vied for Marie Elaine’s attention. But even against that backdrop, she felt comfortable with Dr. Connolly right away.

“He’s very calming,” she says. “There’s a reassurance.” But she appreciated his candor as well as his manner. “I was so enamored with the way he spoke to me as a patient. He explained everything in layman’s terms and drew out the diagrams…He gave me everything, all the facts. He didn’t hold back. He explained everything to me, and gave me hope as well.”

Dr. Connolly described the surgery that he felt would best restore blood supply to her brain. He would be using her temporal artery–the blood vessel that passes over the temple area. He would redirect one branch of this artery onto the surface of her brain. There it could begin to form a new blood supply pathway.

Then they talked about timing. “We can have surgery next Tuesday,” he said. Or, in Marie Elaine’s particular case, they had a second option. She could continue her course of medication for two more months, and then see if there had been any improvement.

She chose the latter route. Looking back, she reflects, “I think that time helped me, believe it or not. Psychologically and emotionally.”

Those months gave her feelings some time to get used to the facts. But the facts did not change–things did not improve. So by December, it was time for surgery.

On the day of the surgery, Marie Elaine was nervous. Despite her husband’s profession, she had never been too involved with doctors’ offices or hospitals. “Never had a stitch in my life. [Before all this] I never had any tests, had never been in the hospital. It was like a whole new world.” She says, “I walked in and there were these nurses and obviously doctors and anesthesiologists. The nurses… what left an impression was…they turned around and said, ‘Hi, how are you?’…They were so pleasant.’” And her feeling of reassurance from Dr. Connolly was as strong as ever. “I felt very good in his hands.”

She felt ready for her surgery–as ready as she was going to be. She went under, and the surgery began. After being under for six hours of brain surgery, she heard Dr. Connolly’s voice gently calling her name. “Marie… Marie…”

But, “I didn’t answer,” she laughs. “In my mind, my mother’s name is Marie and I’m Marie Elaine.” She remembers that Dr. Connolly continued: “‘Marie, I told you you’d be okay. We’re done. We’re good.’ And I was like, he’s talking to me! And I woke up.”

After she woke up, Marie Elaine moved to the Intensive Care Unit (ICU) for recovery. Her husband and family visited her there, happy to be holding her hands and talking to her once again. Dr. Connolly checked in on her once or twice a day. And the nurses were outstanding–caring and personable. She recalls singing with one nurse, and taking a walk down the hall with another. “I’ll never forget the nursing staff,” she says with a smile.

Marie Elaine stayed in the hospital just three days, then headed home for recovery. Three months after surgery, she had returned to work. By six months after surgery, she was back to doing everything that she had done before. She even regained the function in her right hand.

Marie Elaine wanted to tell her story in order to thank Dr. Connolly, Dr. Altschul, their office staff, the nurses, and everyone else involved in her care. “Everything from A to Z was a wonderful experience, from something that was so terrible,” she says.

But she also wants to help other patients who may now be sorting through a bewildering array of facts and feelings. Reading other people’s stories helped her through the process, and she wants to help others in a similar way. Her advice for other patients is to “be fearless–at least try to be. It’s not easy when you’re going through it.”

Dr. Connolly is the Bennett M Stein Professor of Neurological Surgery, Vice Chairman of Neurosurgery, and Surgical Director of the Neuro-ICU at Columbia University Medical Center/NewYork-Presbyterian Hospital. He is also director of their Cerebrovascular Research Laboratory–that is, the laboratory that researches problems with blood flow in the brain. He is especially interested in Moyamoya, and has decades of experience performing surgery for this and other cerebrovascular problems.