Date: February 4, 2013
Treatment for a rare spinal condition
In July 2011, Nancy Fangue broke her right ankle. So when she had trouble walking a year later, she assumed the old ankle injury was to blame.
But then the aches arrived, followed by fever, vomiting and frequent urination. By late August, weakness consumed her. An urgent care clinician diagnosed the 68-year-old with a strep infection and placed her on antibiotics. She visited several specialists and a hospital emergency room, where doctors found her kidneys were beginning to malfunction. Despite these visits and changes in medicine, her symptoms did not subside.
“Every day I got progressively worse,” Fangue says. “I could not even walk to the car to get to the hospital.”
Frustrated, in pain and without answers, Fangue wondered if she would ever return to the full life she loved with her fiancé, Phil, and their grandchildren.
The final blow came in October, when her legs gave out and she fell in her Dundalk, Maryland, home’s bathroom. Fangue arrived at Johns Hopkins Bayview Medical Center via ambulance with an almost 103-degree fever, searing pain in her legs and tingling, burning feet.
“It was just like somebody was taking a knife and stabbing me in the leg and foot,” Fangue says.
After multiple tests, Hopkins doctors diagnosed Fangue with a spinal dural arteriovenous fistula (SDAVF)—a rare condition where an abnormal connection forms between an artery and vein near the spinal cord. Left untreated, SDAVFs can cause paralysis of the legs and lower body.
In Fangue’s case, the SDAVF kept blood from draining out of her spinal cord and caused extreme swelling.
“Her spinal cord was in really bad shape,” says Richard O’Brien, M.D., chairman of neurology.
Luckily, Johns Hopkins Bayview has a multidisciplinary team trained to tackle complex neurological conditions like Fangue’s. The team, made up of neurology, neurosurgery and rehabilitation experts, determined Fangue needed an immediate endovascular procedure to eliminate the fistula and prevent her body from further deterioration. She also needed an intense rehabilitation plan to regain her walking skills and return to her pre-SDAVF life.
“This thing would not get better on its own,” says Alexander Coon, M.D., a Johns Hopkins neurosurgeon who specializes in interventional neuroradiology.
To discover the root of Fangue’s symptoms, Hopkins doctors performed a series of tests, including a nerve conduction study and an MRI scan. Some patients need additional testing like angiograms to officially diagnose SDAVFs, Dr. O’Brien says. But Fangue’s fistula was so large, it could be seen on her MRI scan.
Still, the diagnosis puzzled Fangue. She remembers thinking, “How can there be a connection between my feet, kidneys, bladder and spine?”
Fangue also had never heard of SDAVFs.
Not many people have, Dr. Coon says. “It’s probably misdiagnosed all the time.” he says. “That’s because SDAVFs are a mysterious condition. Studies show there are about five to 10 cases per million people each year. A majority of patients are male, experiencing the condition while in their 60s or 70s.
Symptoms can include lower extremity weakness, sensory abnormalities, difficulty urinating and pain. But these symptoms can be subtle and mistakenly attributed to other spinal problems, Dr. Coon says. There also is no definite cause.
“It can be spontaneous or traumatic,” he says. “We just don’t know. Often times in medicine and neurovascular (care), we don’t know what causes something.”
A Team Approach to Treatment
What the Hopkins team does know is how to fix SDAVFs. Before embarking on Fangue’s treatment plan, Drs. O’Brien and Coon, along with Richard Zorowitz, M.D., chairman of physical medicine and rehabilitation at Johns Hopkins Bayview, reviewed every aspect of her case. Communication between all specialists is essential in making a safe, comprehensive treatment plan, Dr. Zorowitz explains. Under the team approach, patients also can have shorter stays and fewer complications, he says.
A spinal fistula glue embolization had to be performed first by Dr. Coon. The endovascular procedure essentially closes the fistula with a substance similar to industrial-strength glue.
Some SDAVFs require open surgery, which can involve complicated dissection around the spinal cord and a painful, lengthy recovery. But Fangue’s SDAVF could be repaired at Hopkins endovascularly, meaning less pain and a smaller incision, Dr. Coon says.
To reach the fistula, Dr. Coon sent a catheter through a 2-millimeter opening—about the size of a pin head—in Fangue’s leg. Slowly, he made his way through smaller and smaller vessels until he reached the fistula. Once there, he injected the glue-like substance to close the fistula from the inside.
“The goal is that the normal blood drainage resumes as before, and the swelling goes down,” Dr. Coon says.
Within just a few hours, Fangue was back in her hospital bed, with only mild discomfort at the incision site. Within two days, she began her rehabilitation. Fangue’s daily schedule included three hours of physical and occupational therapy, all to literally and figuratively get her back on her feet.
In her first days of rehab, Fangue says it took all of her strength just to sit and stand. But with each day, her strength increased and the swelling in her spinal cord went down. “The rehab process is a continuum,” says Dr. Zorowitz. “I tell patients, ‘What’s lost quickly is gained back slowly.’”
By mid-November, Fangue could walk more than 140 feet with the aid of a walker. By Thanksgiving, she returned home.
“This year, I really have something to be thankful for,” she says.
While her rehabilitation continues, Fangue is thankful to feel healthy again, without the intense pain she experienced for so many months. She’s grateful to be cooking in her own kitchen, as well as walking and socializing with friends and family again. And she is especially appreciative for the dedicated team of doctors and support staff at Johns Hopkins Bayview who put an end to her fistula and helped her return to the life she loves.
For more information or to schedule an appointment with a Johns Hopkins neurologist, call 410-550-0592.
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