Health mystery: A pain too bad to bear

Some doctors thought it might be the side-effect of a drug, migraines, a dental problem, mental illness, or an attempt to obtain painkillers

Kim Pace was afraid he was dying. In six months he had lost more than 13 kilograms because a terrible stabbing sensation on the left side of his face made eating or drinking too painful. Brushing his teeth was out of the question and even the slightest touch triggered waves of agony and a shocklike pain he imagined was comparable to electrocution. Painkillers, even morphine, brought little relief.

Unable to work and on medical leave from his job as a financial consultant for a bank, Pace, then 59, had spent the first half of 2012 bouncing among specialists in his home state of Pennsylvania, searching for help from doctors who disagreed about the nature of his illness.

Some thought his searing pain might be the side effect of a drug he was taking. Others suspected migraines, a dental problem, mental illness, or an attempt to obtain painkillers.

Even after a junior doctor made what turned out to be the correct diagnosis, there was disagreement among specialists about its accuracy or how to treat Pace. His wife, Carol, a nurse, said she suspects that the couple’s persistence and propensity to ask questions led her husband to be branded “a difficult case” — the kind of patient whom some doctors avoid. And on top of that, a serious but entirely unrelated disorder further muddied the diagnostic picture.

So on July 17, 2012, when Pace told his wife he thought he was dying, she fired off an emotional plea for help to the office of a prominent specialist in Baltimore. “I looked at Kim and it hit me: he was going to die,” she said. “He was losing weight and his colour was ashen” and doctors were “blowing him off. I thought, ‘OK, that’s it,’ and the nurse in me took over.”

Her missive got results. Three weeks later Pace underwent corrective surgery for an uncommon problem that causes pain so intense and debilitating it is regarded by doctors as among the worst known.

“I knew the pain was real and I felt like my life was on the line and I just had to prove it to somebody,” Pace said.

Pace’s symptoms began in early 2012 when he developed an intermittent burning on the left side of his face and down his oesophagus. The pain was mild at first but intensified during the day. Because Pace took medication for a host of chronic conditions including Type 2 diabetes, hypothyroidism, high cholesterol and severe depression, doctors at first suspected a drug reaction; Pace had switched antidepressants a few months earlier. Another possibility was acid reflux.

By the end of March he had developed a facial twitch, and the pain was worse, especially when he chewed. “Nothing really relieved it,” he said. His family physician in Wilkes-Barre had suggested going off the antidepressant, but his psychiatrist disagreed. His symptoms were not known side effects of the medication, which was working well for Pace after other antidepressants had failed. The drug “turned my life around”, said Pace, who was reluctant to stop taking it.

On Easter Sunday, April 8, he spent the day lying on the sofa, forgoing his favourite dinner of ravioli because eating hurt too much. He seemed so weak that his wife took him to an emergency room, where he was diagnosed with burning mouth syndrome, which makes the mouth and tongue feel as though they are on fire for no discernible reason.

A few days later Pace visited his dentist, who prescribed a special toothpaste and mouthwash as well as dietary changes to ease the burning.

About two weeks after that, Pace collapsed at work and was taken to the emergency room complaining of severe chest pain. Doctors diagnosed gastro-oesophageal reflux disease, or GERD, which can cause heartburn that mimics a heart attack. Days later, in the throes of severe abdominal pain, Pace was found to have acalculous cholecystitis, an inflammatory disease of the gallbladder sometimes seen in diabetics. He underwent surgery to remove his gallbladder, which had ceased to function.

Through it all, the facial pain, which had largely morphed from burning to a series of electrical shocks, remained a constant — and baffling — problem. Because Pace’s medical records listed, incorrectly, that he was bipolar, doctors seemed to discount his facial pain as psychosomatic after scans and other tests failed to reveal anything.

By May he had lost more than 11 kilograms. “I remember putting peanut butter on a spoon and saying, ‘Just eat this,’ or making pea soup and puréeing it and coaxing him” to eat a little, Carol Pace recalled. “And I was trying to keep him hydrated the best I could.” At that point he had consulted a gastroenterologist as well as his endocrinologist, family practitioner and dentist, and he had made several trips to the ER, seeking relief from the unbearable pain.

One day in yoga class, Carol Pace was describing her husband’s symptoms when a woman said that her mother had suffered from something that sounded similar: the condition was called tic douloureux — the old name for trigeminal neuralgia, or TN.

TN is an uncommon form of nerve pain that affects the fifth, or trigeminal, cranial nerve, which is one of the most extensive in the head and which carries signals from the face to the brain.

TN can be caused by a blood vessel pressing on the nerve, or an injury to the nerve during sinus surgery or a dental procedure. It can also affect people with multiple sclerosis, which causes deterioration of the myelin sheath that protects nerves. TN, which affects only one side of the face, is more common in women and people older than 50.

“The intensity of the pain can be physically and mentally incapacitating,” according to the National Institute of Neurological Disorders and Stroke. That is one reason the disorder has been nicknamed “the suicide disease”.

Morphine and other opioid painkillers are not typically effective, but some anti-seizure drugs may help. If those don’t work, several invasive treatments are available, including brain surgery.

Because many other conditions can cause facial pain, including tumours, shingles and temporomandibular joint disorders that affect the jaw, these need to be ruled out before TN is diagnosed.

When Carol Pace suggested that her husband might be suffering from TN during an emergency room visit in late May, a doctor dismissively told her, “Don’t look for zebras,” medical parlance for a rare disorder. In his notes he wrote that Pace “has been on every SSRI known to man” referring to a class of antidepressants, and that lab tests showed the presence of benzodiazepines, often-abused tranquillisers “the significance of [which] is unknown”. But the same records show that Pace had told the doctor he was taking Xanax, a benzodiazepine prescribed by a doctor to help manage his pain.

The couple say that the doctor’s dismissive attitude and his insinuation that Pace was either crazy or a drug seeker was the last straw, reinforcing their view that he needed to find care elsewhere. Doctors in their home town seemed unable or unwilling to help.

In June, Pace and his wife drove two hours to Philadelphia to see a neurologist at a teaching hospital. The doctor said he suspected trigeminal neuralgia, but he also noted that Pace had “atypical features” including headaches that had recently started. He recommended that Pace undergo a dental workup to rule out other causes of pain and see an ear, nose and throat specialist because of swallowing problems. Pace began taking neurontin, an anti-seizure drug commonly prescribed to treat TN.

The drug was ineffective, and more specialists seemed to generate more questions. A CT scan found a possible periodontal infection that might be the cause of his pain, and a surgeon questioned the TN diagnosis; he thought Pace had “atypical facial pain”.

No one seemed to be in charge, nor was there a clear treatment plan. A scan failed to show nerve compression that might indicate TN. Pace was becoming increasingly desperate: because swallowing was so painful, he was drooling.

For weeks, a cousin had been urging Pace to seek a second opinion at Johns Hopkins, which has a Trigeminal Neuralgia Center. Carol Pace had readily agreed. Pace had asked a surgeon for a referral to Hopkins, but the doctor refused, for reasons that are unclear.

“I thought you need a doctor’s referral to go to Hopkins,” Pace said, adding that he subsequently learnt that was not the case. Several weeks later, when her husband told her he feared he was dying, Carol Pace sent an e-mail pleading for help to Carol James, then the physician assistant to neurosurgeon Ben Carson, who was affiliated with the Trigeminal Neuralgia Center. (Both James and Carson, until recently a Republican candidate for president, retired from Hopkins in 2013.)

A few hours later James called and spoke to Kim Pace for about half an hour. She then asked him to fax his records, and she sent his case to Carson’s colleague Michael Lim.

At their first meeting on July 26, Lim, an associate professor of neurosurgery, recalled that Pace had the shocklike pain characteristic of TN. Pace seemed very distressed about his condition and said he favoured brain surgery over injections and radiation, which are also used to treat the disorder. The operation, known as microvascular decompression, offers the most durable results: about 80 per cent of patients who undergo it remain pain-free for 20 years or more.

Surgery involves opening the skull, exposing the trigeminal nerve and locating the cause of compression — often a blood vessel — then inserting a tiny cushioning sponge that isolates and protects the nerve from pressure. The procedure, a difficult and delicate operation, poses significant risks including stroke, paralysis and infection.

Pace was not fazed. “I’m a scrapper,” he said, adding, “When you’re in that much pain, you’re willing to do anything.”

The operation, performed on August 10, 2012, proved to be more complicated and difficult than Lim expected. Two arteries — not just a lone blood vessel — were compressing the nerve, which was deeply scarred. The cause of the nerve compression remains unknown. Pace, a former high school and college football player who suffered multiple concussions as a youth, wonders if those played a role.

“He did great,” said Lim, who has performed more than 600 such surgeries in the past nine years.

Pace said his pain vanished after the surgery and he returned to work about six weeks later. He has since had to undergo an additional procedure to correct a problem related to the decompression, and he is again pain-free, although his lips are numb. He regards that as a small price to pay.

Both he and his wife remain deeply grateful to James and Lim, who listened to his story, took his complaints seriously and did not reflexively attribute his pain to depression.

“There are people out there suffering from this who don’t know they have it,” Pace said. “That worries me.”