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Singer shares experience with rare brain malformation

When the MC of a recital said some of the scheduled singers couldn't perform for various reasons such as allergies, a virus - and brain surgery - he was serious.
Grace Cutsforth of Maidstone and daughter Bonnie Cutsforth-Huber, a member of the faculty at this year's Summer School for the Solo Voice in North Battleford. Cutsforth-Huber is recuperating from brain surgery and shared her story with the students of the school in a special lecture July 10.

When the MC of a recital said some of the scheduled singers couldn't perform for various reasons such as allergies, a virus - and brain surgery - he was serious.

Mark Turner, a member of the faculty of this year's Summer School for the Solo Voice in North Battleford, was referring to Dr. Bonnie Cutsforth-Huber, who joined the staff for the first time this year.

While she wasn't allowed to sing, under doctor's orders, she was certainly able to teach. She also gave a presentation on her journey through a disease that has meant two surgeries on her brain, one of which was in May of this year, and six surgeries on her spine.

Originally from the Maidstone area, Cutsforth-Huber holds a Bachelor of Music in vocal performance from the University of Saskatchewan, a master's degree in vocal performance from Southern Illinois University and a doctorate in musicology from the University of Kentucky, and she currently serves as assistant professor of music at Penn State Altoona in Pennsylvania.

She accomplished all this and built an international career in opera despite a malformation of the brain that blocked messages to her vocal cords, restricted her ability to breathe, caused pain and partial paralysis and eventually brought her to death's door. It was not until she was 34 that a diagnosis was finally made.

Thursday, July 10, at an evening presentation, Cutsforth-Huber used a contagious sense of humour and the expertise that comes from living with a condition such as Chiari malformation to share her story with fellow faculty and students of Summer School for the Solo Voice.

"I am so truly thankful for every single noise that comes out of my mouth," she said, "because those are all things I'm not supposed to be able to do."

Chiari malformation is a neurological disorder where part of the brain, the cerebellum, descends out of the skull into the spinal area. This results in compression of parts of the brain and spinal cord, and disrupts the normal flow of cerebrospinal fluid, the clear fluid that protectively bathes the brain and spinal cord.

Medically speaking, it is unknown why Cutsforth-Huber is able to sing, or even to talk, after the damage done by her long-undiagnosed condition. Doctors say it might be muscle memory; she had to do incredibly difficult physical work just to sing in the first place. She points to extreme stubbornness as a likely reason as well.

"I come from a tough bunch of folks who are pretty 'dig your heals in and get going' type of people," said one of the two daughters of Donald and Grace Cutsforth of the Maidstone area.

While she is still recuperating from her latest surgery, she said, "I'm going to continue to sing, I'm going to continue to fight and I'm going to continue to be who I am because I'm from Saskatchewan and that's what I do."

Cutsforth-Huber began her story by explaining that as a vocal student she experienced severe tongue tension.

"Other people could do tongue exercises and they got better," she said. "With me, it never got better."

She had tests done and all appeared to be normal.

The experts said, "Everything's fine, she's just a stiff little person."

She also had a problem with what singers call their "break," or passagio, the point within their vocal range where the voice passes from chest voice, where the sound seems to vibrate in the throat and chest, to the head voice, where it resonates in the head.

Many singers have problems with their break, but for Cutsforth-Huber it was a "nightmare" for which there was no explanation.

With much frustration and practice, she got to the point where she could get through it, but it was always a struggle, she said, and it was never pretty.

Then, she added, there was the issue of breathing. With Chiari malformation, the respiratory system does not function correctly.

"My teacher would say, 'Breathe!' but my ribs would not move."

Everything was locked down tight, she said.

She was in her second year of university, studying vocal performance, when she decided to have a heart to heart with herself.

"Maybe this wasn't what I was meant to do," she thought. "This seems to be a lot harder for me than everybody else, so maybe I shouldn't be doing this."

But her answer was, "No, I was put on this earth to sing. If there's one thing I know for sure, it's that."

She told herself, "Whatever is wrong with me, whatever is causing 'cute little tight girl,' there is something there and I have to find out what it is."

There was "no sense blubbering about it," so she carried on, despite the increase of symptoms that became increasingly alarming.

She developed severe back pain, but x-rays showed no reason for the pain. She was told, 'there's nothing wrong, it's probably just stress,' and she was given muscle relaxants. They didn't help.

Meanwhile, her career was progressing because she was so determined to sing.

"I worked harder than everybody else and worked around all these inadequacies in my voice."

She was 22 and studying in the United States when the continuing back pain prompted her to see a chiropractor. He x-rayed her spine and diagnosed scoliosis, an abnormal curvature of the spine, saying she must have had it since she was a child.

Her mother, who she phoned with the results of the x-ray, said she'd had full body scans done at six months, two years, five years and 11 years and there was no scoliosis ever present at these times.

The chiropractor's response was to suggest, "The Canadian health system is different. They probably just check your x-rays differently."

What no one knew was that her condition could not be diagnosed by x-ray. It could only be diagnosed by magnetic resonance imaging, an MRI. But none was ever ordered. (This, says Grace, is due to the insurance system in the United States. She is confident a diagnosis would have been made if her daughter was still living in Canada.)

Despite the scoliosis diagnosis, Cutsforth-Huber's career continued to develop, with singing gigs coming her way, but her health kept getting worse. She began having trouble swallowing and her breathing problems worsened. She would wake at night unable to breathe, and her husband would rush her to the emergency department.

She was diagnosed as having allergies, and for four years was taking 12 allergy shots a day. The epinephrine helped open things up, but it always came back, she said. Again there was no explanation, except to say she was probably stressed.

They confided to her husband, "Make sure she takes care of herself because she obviously doesn't handle stress well."

Her husband, she said, knew better, saying she could handle stress better than 10 people together.

But as far as the medical world knew, "she looked too good" to be really sick.

It was 2007 when things reached dire straits.

That summer she was in Rome, singing the part of Marcellina in Mozart's the Wedding of Figaro. She got up one day and couldn't feel her left leg. She fell on the Italian tile floor.

After she sat for a while, wondering if she should call someone, the feeling started coming back and she was able to get up. But she was dragging her leg as she walked.

She was hobbling around at rehearsal, and because she was playing the part of an old housekeeper, the director thought the limp was deliberate.

"Great character choice!" he called out.

She also noticed, when working with props, she couldn't hang on to anything with her left hand, and she would drop her knife when eating.

Could she have put her back out, or was the scoliosis taking a sudden turn, she wondered.

She also found herself unable to keep food down, and lost 20 pounds in six weeks - a major loss for someone with her tiny frame.

"Clearly, something was very wrong," she said.

She was due to visit her parents, and when she hobbled off the plane, her mother, a nurse, said, "You look like you just got out of a death camp!"

Her daughter was grey, her face was gaunt and she was significantly underweight. She extracted a promise that when Cutsforth-Huber returned to her home in the United States, she would go back to her doctor and not leave the office until an MRI had been ordered.

"So that's what I did."

This time it could not be said she looked too good to be sick.

"I looked like I was dying, because I was."

She didn't know it yet, but for years her brain had been herniating down into her spinal cord, blocking vital nerves and causing the build up of a fluid filled tumour called a syrinx that stretched from the base of her brain to 95 per cent of the length of her spine.

The suspicion at that time, however, was that she had MS, multiple sclerosis, and an MRI would be needed to confirm the diagnosis.

On the day of the test, she told her husband, "I hope they find something so people will stop telling me I'm crazy."

This was a reaction she'd heard since she was about 12 years old.

Hers was the first appointment of the day and was expected to be about 15 minutes long. First, however, the radiologist explained he would be doing a quick, complete sweep of her spine to make sure the machine was "warmed up" and ready. The "real" test was to involve only for a portion of her spine as approved by her insurance carrier.

"There was a reason that man was there that day," said Cutsforth-Huber, and it saved her life.

Once he had done the sweep, "there was just silence," she said.

"I thought, what's going on? I'm lying in this very confined little space with that Hannibal Lecter thing over my face, and there's just silence."

Finally his voice came over the speaker, apologizing for the delay, but saying he had called her doctor to get her insurance carrier to authorize a scan of the entire spine and the brain and that he was waiting for a call back.

Her 15-minute test had turned into a three-hour examination. She knew they had found something.

Of course, they couldn't tell her what they'd found. She heard from her doctor the next morning as she arrived for work. She needed emergency brain surgery; they weren't sure how much time she had.

The soonest she could get in to see a neurosurgeon was two weeks. What should she do in the meantime, she asked her doctor?

"I'm not really sure, but it's probably a good idea not to make any sudden moves," she was told.

Two weeks later, the neurosurgeon said to her, "I'm looking at a dead woman walking."

He had never seen a Chiari malformation so severe on a living, breathing human being, he told her. He wondered how she could be an opera singer.

"How can you sing? Doesn't your head feel like it's going to blow off?" he asked.

She answered, "Sometimes, but I just push through. I'm from Saskatchewan, that's what I do."

He told her she shouldn't even be able to talk, and he marvelled that she'd been able to earn a PhD with such an acute illness.

As it turned out, her situation was so critical she would not have survived the wait for an operating room. So, a colleague had been approached and agreed to give up some of his operating room time for her, or she would surely die.

She was cautioned she may not survive the surgery and that, if she did, she may have no memory of who she was or of anyone she knew when she woke up.

She told her neurosurgeon she would have the surgery on one condition.

"You have to find a way that I can come back because I have a two-year-old little boy and he doesn't deserve to grow up without a mother, and he also doesn't deserve to grow up with a mother who doesn't know who he is, and most of all he doesn't deserve to have a mother who is a vegetable."

She told him, "I know you will find a way to bring me back because I'm not done yet."

A week later, allowing for her family to come down from Canada and for her to make final arrangements in case she didn't make it, she was undergoing emergency brain surgery. Her mom was right outside the door. In case something went wrong, she could come in and say goodbye.

As grave as things had appeared before the surgery, once it was underway things looked even worse. The procedure that was supposed to take an hour and a half took five hours to complete. When it was over, all they could do was wait to see if she would wake.

When she did, she knew the names of all present, including her own.

She said, "Of course I know who I am, but I have a really bad headache."

She was told they had done what repair they could on her brain, but the tumour was too big to remove. It was hoped the cerebral spinal fluid in the tumour would go down on its own with a better flow established by the repair.

She was told she could start vocalizing six weeks post operative and go back to singing after three months.

"The very first vocal session I had with myself, I went all the way to a high B flat" she said. "I burst into tears because I couldn't ever do that."

At 34, it was now a matter of rebuilding her voice from square one.

"My body had absolutely no clue," she said. "All those cranial nerves were turning back on and they didn't know what to tell my muscles because they had always been compromised."

Until then she hadn't realized she'd had not a bag of tricks, but a trough of tricks, to get around her vocal inadequacies.

"My vocal cords couldn't vibrate like a normal human being, and I had no clue because I was never normal."

Undoing all the muscle memory that had established itself before the surgery was one of the hardest things she had to do.

It took two years of baby steps and "just being stubborn" to rebuild her voice.

"After I did, everything started to fall into place," - a Carnegie Hall debut, a Lincoln Centre debut, a trip to Vienna, back to Italy twice, and every year an MRI to monitor the tumour in her spine.

"Then six months ago, I went to get out of bed and couldn't feel my left leg," she said. "I thought, 'Oh, crap!"

Her husband asked what's wrong. She told him. He bolted upright in bed shouting, "Chiari!"

The call went out to the neurosurgeon. An MRI showed the left side of her brain was re-herniating and the syrinx had grown almost 40 per cent in one year. It was beginning to grow outwards, so nerve damage would be happening quickly, she said.

May 14 of this year, seven years after the first one, she had her second brain surgery. Over 10 hours the surgical team removed the old brain graft and repaired the opening between brain and spine and removed some bone at the base of the scull, as well as one vertebra and a multitude of adhesions.

When it was over, she needed a walker, then a cane, until about a week ago.

"I wanted to be walking on my own two feet by the time I came to summer school," she said.

"The body is really amazing, because when you want something badly enough, when you want something with every fibre of your heart and soul, your body finds a way to do it," she told her audience. "I shouldn't be able to talk, I shouldn't be able to sing, I shouldn't be able to do half the things I do, and yet I do them, because I wanted them so badly that my body found a way to overcome all these problems and do it."

She said, "I'm having a lot of fun in my career. I'm singing in very big and very major concert spaces all over the world. I'm watching my son grow up."

Most Chiari patients are never able to have children. The pregnancy and labour were both difficult, although she did not know at the time what danger she was in, especially when an epidural given during labour could have fatally punctured the syrinx in her spine.

She told herself, "Suck it up! Women have been giving birth for millions of years, so stop being a wimp."

She went on, "I'm so glad I had that heart to heart with myself and decided this is why I am on the earth, and if I have to fight for it, so be it. I am from Saskatchewan and that is what we do."

Cutsforth-Huber said she is thankful in some ways for what happened to her.

"If it hadn't had these challenges, I wonder if I would have discovered how much I truly love teaching."

If she had stayed strictly a performer, she would have had a hole in her life, she said.

"That is such an important part of who I am and I would have missed out on that piece of myself, because when you go through something like that, believe me, you know the voice and you know the body."

She added, "And you know what's important and what isn't."

People ask her if it's scary to go out for auditions every chance she gets.

"No," she tells them. "There is nothing in the world that those auditioners can say to me, or a critic can say about me in a column, there is nothing that anybody can say to me that is worse than, 'You're dying,' and I've already heard that."

She said, "I refuse to live my life in fear, because if you're living in fear, you're not living."

Neither does she dwell on what might have been, how much further along in her career she would have been without those challenges.

"I don't think that way because you can only play your very best with the hand that you're dealt, and that's not the hand I was dealt," she said. "I don't think it's healthy to think, 'what if.'"

Cutsforth-Huber has a caution for people who find themselves faced with challenges such as she did.

"When you have challenges in your life, it's so, so easy to let those challenges turn into excuses."

She said her hope and wish for everyone in the audience was that, when they do have challenges, they face them head on and learn something important about themselves.

"They are not there to make excuses, they're there to help you realize how strong you are," she said.

Cutsforth-Huber also believes, whether something good or bad happens, every human being should try to make the world a better place. She has agreed to become part of an international study on Chiari malformation sponsored by the United States National Institute of Health. She is to be monitored for the next five years in hopes that what they learn from her can help someone else.

It's the first major study to be done on Chiari malformation, she said.

"I want to be part of it."

Although Chiari malformation is hereditary and can vary in symptoms and severity, there is no one in her family pin-pointed as having had it, but "it will be in there somewhere," she has been told.

Thankfully, neither her son nor her nephews have it, she said.

Cutsforth-Huber said she is feeling better right now than she has felt in a long time. The syrinx has shrunk by four inches since the second surgery, and it's hoped there won't have to be any more.

Looking forward to her first back-to-singing gig Oct. 3, a fully-booked 2014-15 season and plans for the 2015-16 season, she said, "I try not to think of what I've lost. I try to keep my eyes moving forward and being excited about how much I still have to gain."