Story URL: http://news.medill.northwestern.edu/chicago/news.aspx?id=79817
Story Retrieval Date: 10/21/2014 7:01:33 PM CST
Imagine a pain so severe that it feels like your face has been struck by lightning — a pain that makes your face so sensitive to the touch you can’t even be kissed on the cheek.
This is the kind of pain that sufferers of trigeminal neuralgia, a rare nerve disorder, say they have to live with.
John Kerkemeyer, of Romeoville, was 22 when he was diagnosed with the disease 14 years ago. Kerkemeyer was having lunch when he first experienced pain he said must be worse than electric shock therapy.
“I bit into a chicken sandwich and had a shooting pain that wrapped around the side of my face,” Kerkemeyer said. “The next thing you know, I’m on the floor in my knees in tears and it wouldn’t stop.”
“For me it was just like somebody taking electric shock to your mouth and yanking your mouth sideways at the same time,” Kerkemeyer said. “I didn’t want to eat; didn’t want to open my mouth; didn’t want to talk; I didn’t want to do anything.”
Trigeminal (pronounced try-JEM-i-nal) neuralgia, also called TN, is a disease most people have probably never heard of. Doctors don’t know the exact cause of it, but research suggests the disease is linked to a malfunction in the trigeminal nerve, which supplies sensation to the nose, lips, ears, tongue and teeth.
“What is believed is that the trigeminal nerve is kinked around a blood vessel and as a blood flows through it causes spasms,” said Dr. Patrick Sweeney, a radiologist at the Illinois Gamma Knife Center, a facility at Alexian Brothers Hospital in Elk Grove Village that treats patients of trigeminal neuralgia and other diseases categorized as neuropathic facial pain disorders.
Jane Boles, executive director and CEO of the Trigeminal Neuralgia Association in Gainesville, Fla., said 150,000 people are diagnosed with TN every year. Most of these patients are women and tend to be over the age of 50.
There isn’t a lot of public awareness about trigeminal neuralgia because many sufferers live with the disease for years before getting a proper diagnosis, Boles said.
“It generally takes anywhere from 3-5 years for somebody to get a correct diagnosis,” Boles said. “People usually start out at their dentist's, and many people go through extensive, painful dental work — root canals, teeth extractions. When the pain is still there, they don’t know what else to do.”
The earliest description of trigeminal neuralgia dates back to 880 A.D., according to the Trigeminal Neuralgia Association. However, research data on the disease is limited. The last epidemiological study on trigeminal neuralgia was published in 1988 by the Mayo Clinic in Rochester, Minn. The study found that sufferers experienced spasms lasting anywhere from 1 day to 4 years.
Dr. Sweeney said getting a correct diagnosis is difficult because the pain could be associated with something else.
“TN is a diagnosis of exclusion, meaning you want to make sure it’s nothing else,” Sweeney said. “One of the most important things you need to do when you think a patient has a diagnosis of TN is to get an MRI to make sure there’s no other cause.”
Dawn Crook, a support group leader in Illinois for the Trigeminal Neuralgia Association, said some patients feel doctors think they are being insincere about the pain.
“People tell them it’s in their head,” Crook said. “Nobody can see it, so they don’t understand. This is a disease that if you don’t have it or if you don’t know someone who has it, then you don’t know about it.”
Trigeminal neuralgia isn’t considered fatal, but both Boles and Crook said some sufferers have committed suicide. Crook said her group counseled a man with trigeminal neuralgia for months through an online chat group, but he ultimately took his own life. Trigeminal neuralgia is sometimes referred to as the “suicide disease.”
“We talked to this man for six months and then his wife came on one day and said he committed suicide,” Crook said. “He had the surgeries and he was just at wits end. He couldn’t take it anymore.”
There is no cure for TN, but multiple treatments are available, including microvascular decompression neurosurgery and gamma knife radiation treatment. Gamma knife is a popular treatment for elderly patients because it the most non-invasive procedure, Sweeney said.
“With gamma knife you have what looks like a hair dryer with 201 beams of radiation that converge at a single point,” Sweeney said.
Though some patients’ symptoms return a few years after the procedure, Sweeney said the treatment is about 70 percent successful.
“Thirty percent of those patients are pain-free and off all their medicines,” Sweeney said. Another 20 percent have a significant reduction in pain but still require medication. Another percentage of people have some improvement in pain but are not completely pain-free and still require medication” such as painkillers and anti-seizure drugs.
Kerkemeyer, whose pain has subsided since having the gamma knife treatment in 2003, hopes doctors will discover a treatment that will permanently cure the disease.
“This disease changed my life drastically. I missed most of my 20s and it took me a long time to get myself back in shape,” Kerkemeyer said. “I’m hoping there’s a cure in my lifetime.”