Source: www.washingtonpost.com
Author: Sandra G. Boodman

A Florida man spent months consulting doctors baffled by stabbing pain that radiated to his neck and shoulder.

For more than a year, Arthur L. Kimbrough had done everything he could think of to find out what was causing the stabbing sensation that radiated from his throat to his neck and down his left shoulder. He had seen anesthesiologists, an ear, nose and throat doctor, a neurologist and neurosurgeons in Florida and Maryland; undergone tests and scans; and taken a variety of drugs that failed to alleviate the intensifying pain that baffled his doctors.

It wasn’t until February 2022, after Kimbrough suffered a life-threatening hemorrhage in a hospital waiting room, that the cause was finally identified.

Two years later, Kimbrough, now 76, attributes his survival to being in the right place at the right time. He says he feels lucky to be alive and is not angry his illness wasn’t diagnosed earlier.

Doctors “missed some things clearly, [but] it wasn’t because they weren’t looking,” said Kimbrough, an executive coach who lives in the Florida Panhandle and owns funeral homes and cemeteries in Florida and Mississippi. “They were very responsive.”

“The blinders we had on was that it turned out to be the fundamentally wrong place to be looking,” he said.

Unusual sore throat
Kimbrough first noticed the pain — a tender spot under the left side of his tongue in the back of his mouth — in mid-December 2020. It didn’t seem like a conventional sore throat: Swallowing wasn’t painful. His family physician found no inflammation and recommended he see his ENT; both doctors are Kimbrough’s close friends. A heavy smoker for 25 years who quit in his early 40s, Kimbrough asked the ENT if he might have throat cancer.

The doctor was reassuring. “Throat cancer doesn’t generate this kind of pain,” Kimbrough remembers him saying. “It’s been so long since you smoked.”

The ENT suspected that a salivary gland might be infected and prescribed an antibiotic. When that failed to alleviate the pain, the doctor examined Kimbrough’s throat with a laryngoscope, an instrument used in office procedures. He told Kimbrough his throat looked healthy and suspected the soreness might reflect a jaw problem, possibly temporomandibular joint dysfunction (TMJ), or a pinched nerve in his neck. The latter hypothesis would guide Kimbrough’s 14-month quest.

After Kimbrough’s dentist ruled out TMJ, he began seeing a chiropractor who recommended spinal X-rays. They showed age-related arthritis in the C3 vertebrae near his jaw.

For the next two months, the chiropractor performed neck “adjustments.” At first, they provided some relief, but by the end of March, Kimbrough’s pain was worse. The chiropractor sent him to an anesthesiologist who specializes in pain management. He administered a nerve block, an injection consisting of a painkiller and a steroid to reduce inflammation. It didn’t help.

The anesthesiologist ordered an MRI of Kimbrough’s cervical spine, which showed spondylosis, abnormal wear on the neck cartilage and vertebrae that may be more common in very active people. He told Kimbrough he might have spinal stenosis, a common problem that increases with age and is caused by a narrowing of vertebrae that can affect nerves. But there was no sign of nerve compression that could explain his pain.

In June, six months after the throat pain started, Kimbrough consulted a friend who is a vascular surgeon to informally review his care. The surgeon told him it sounded appropriate.

Kimbrough, who pays close attention to his health, is a fitness devotee and regularly worked out with a group of former Army Rangers. At that time, in addition to his family physician, he consulted an “anti-aging” doctor every four months who ordered blood tests and prescribed supplements to enhance his health and fitness. Kimbrough took 50 pills per day.

‘Like a hot spear’
By July, the pain, which had spread to his left ear and eye socket, had worsened, disrupting his sleep. Kimbrough managed to maintain his busy work schedule and trained for a short-distance triathlon, his 20th, which he completed July 4. Exercise, he found, seemed to blunt what “felt like a hot spear stabbing me from my jawline, encasing my head like a vise and then radiating to my left shoulder blade.”

Kimbrough decided he needed to expand his search for an answer beyond north Florida. Through business contacts he obtained an August 2021 appointment with an expert in spinal neurosurgery at Johns Hopkins Hospital in Baltimore.

The neurosurgeon reviewed the results of his MRI and confirmed that it showed degenerative changes in his neck. But he told Kimbrough that the pain on the left side of his head was perplexing; according to the scan, it should have been on the right. He suggested that Kimbrough try wearing a cervical collar to immobilize his neck for 20 minutes every day to see if it helped. Neither the collar nor the acupuncture sessions Kimbrough decided on his own to try made a difference.

Over dinner one night in October 2021, Kimbrough’s ENT, noting the severity of his pain and his difficulty swallowing, suggested his spine wasn’t the problem. He thought Kimbrough might have trigeminal neuralgia (TN), chronic debilitating facial pain caused by a nerve injury.

He immediately changed Kimbrough’s medication to a drug used to treat TN. The pain “exploded,” Kimbrough said. “For the first time, I began to understand why some people commit suicide.” He called his primary care doctor who put him back on the previous drug and made a next-day appointment for Kimbrough with a neurosurgeon in Tallahassee.

The neurosurgeon couldn’t find anything and referred Kimbrough to a neurologist who was equally baffled. He sent Kimbrough to a second pain specialist who administered epidural spinal injections that didn’t help. Meanwhile the Baltimore neurosurgeon recommended he see a nerve pain specialist at Hopkins; his appointment was scheduled for late February 2022.

In December, Kimbrough underwent a CT scan of his neck and another test to determine why he was having trouble swallowing. The scan found “mild asymmetry” on his left tonsil but no visible mass. The radiologist suggested that an ENT perform a throat exam that can involve a biopsy; the procedure was never performed.

By mid-February 2022, Kimbrough was in bad shape. He had lost more than 20 pounds and was unable to swallow anything other than clear liquids. His pain varied from tolerable to “like a blowtorch” and was barely controllable despite the maximum dose of the prescription painkiller OxyContin. And none of his doctors seemed to have a clue about what might be wrong.

Kimbrough worried he had a brain tumor. “I was just wandering around the morass of doomsday scenarios,” he recalled.

A few days before his appointment with the Hopkins pain specialist, he and his wife flew to Arizona for a family celebration. The night before their flight, Kimbrough experienced a nose bleed that stopped quickly. It was a harbinger of what would happen the next day.

Drowning in blood
The Baltimore appointment began with a neurological exam. The anesthesiologist asked Kimbrough to stick out his tongue, then requested that he stick it straight out. When he said he had, she handed him a mirror. It revealed that his tongue curved markedly to the left.

The doctor told him that pressure on a nerve or a mass in his throat might be causing the deviation and asked if he could stay in Baltimore for more tests. When he said he’d stay as long as necessary, she left to schedule an urgent MRI.

Sitting in the waiting room, Kimbrough started sipping a Coke. Without warning, blood began gushing out of his mouth and nose. Someone handed him a stack of napkins; it was drenched in seconds. As he coughed and spat out some of the blood and blood clots that were cascading down his throat, Kimbrough remembers thinking, “I’m drowning in my own blood.” For years, he had taken a blood thinner to treat an irregular heartbeat; the drug can exacerbate bleeding.

Kimbrough was quickly surrounded by doctors and nurses and hustled off to the emergency department. “They were so calm I never felt any real fear,” he recalled.

“The worry was that he could die of asphyxiation” by aspirating his own blood, said otolaryngologist R. Alex Harbison, the head and neck surgeon who met him in the ER. Harbison examined Kimbrough and saw a huge six-centimeter mass — at its widest point, the height of an egg — extending from the roof of his mouth over his tonsils to the back of his tongue.

He suspected the mass was cancerous and that it was caused by the human papilloma virus (HPV). The mass, which had been growing for more than a year, had become entwined with a nerve and had irritated the left lingual artery in the throat until it ruptured, triggering the bleed. Pathologists would soon determine that Kimbrough had Stage 3 squamous cell throat cancer caused by HPV-16, the most common type.

HPV, which infects virtually everyone, is spread through sex. Most infections clear on their own, but high-risk HPV, including HPV-16, can cause several cancers later in life, including cervical and throat cancer. A vaccine approved in 2006, and usually administered in childhood before a person is sexually active, can prevent the vast majority of HPV-related cancers that account for more than 37,000 cases annually in the United States. Doctors recommend the vaccine for some adults up to age 45.

HPV oral cancer, which is growing rapidly among men, is the most common head and neck cancer in the United States. (Non-HPV oral cancer is typically caused by smoking and alcohol use.) It often responds to chemoradiation, radiation combined with concurrent chemotherapy, particularly if detected early.

Harbison told Kimbrough what doctors had found and ticked off the steps they would take to try to stop the bleeding. The doctor spoke frankly: If anything went wrong, Kimbrough was unlikely to survive. How aggressive did he want doctors to be? Harbison asked.

“I said, ‘Do whatever you’ve got to do,’” Kimbrough remembers replying.

Kimbrough was intubated, received blood transfusions and underwent an embolization, a procedure that plugged the artery with a coil to stop the hemorrhage. “After that, it’s pretty much hold your breath and wait,” Harbison said. “The level of anxiety was very high” because of the chance of another bleed.

Because Kimbrough was severely malnourished, doctors also inserted a feeding tube in his stomach. A few days later, a tracheostomy tube in his neck was inserted to protect his airway. Within a week of his emergency admission, his condition appeared to have stabilized.

The Hopkins team recommended chemoradiation. Because Kimbrough and his wife knew no one in Baltimore, they opted for treatment at Washington University in St. Louis. They had lived in the city for 20 years and one of their sons still did.

Kimbrough arrived in St. Louis on March 10 with feeding and tracheostomy tubes in place. The medical team there told Kimbrough they believed chemoradiation had a 60 percent chance of eradicating his cancer. But even if they succeeded, they warned him he might always need a feeding tube.

Playing the trombone
On that, he proved them wrong: The feeding tube was removed at the end of July, a month after he finished cancer treatment and a month before he went home to Florida. Kimbrough remains unable to swallow more than a few bites of very soft food; his diet is mostly liquid. So far, his scans have shown no sign of cancer. He has returned to work and is able to speak normally and can play his trombone.

Harbison, who is now an assistant professor of otolaryngology at Washington University (he left Hopkins to return to his native St. Louis eight months ago), noted that the characteristics and location of Kimbrough’s tumor made it harder to spot, which may have helped delay his diagnosis.

Kimbrough said his ENT recently told him that as a result of Kimbrough’s experience, he is more aggressive about performing biopsies on patients with similar symptoms and recently diagnosed another man with HPV-related cancer whose throat pain radiated to his shoulder.

“Art’s presentation is extremely rare,” said Harbison, who has treated about 200 patients with HPV-related oral cancer, which often appears as a neck lump.

But someone with “persistent throat or ear pain should be investigated by an expert,” he said. It’s possible, he added, that the cancer was missed on the 2021 MRI.

Kimbrough says he wants other men to benefit from his ordeal by learning about HPV, vaccinating their children and questioning assumptions that may turn out to be erroneous, as they were in his case.

Although he now regards it as crucial, Kimbrough said it simply didn’t occur to him to get a second ENT opinion for his sore throat, partly because the focus had been on his spine.

“Everyone was doing their best with the best of intentions,” he said. “There was a fork in the road and we didn’t go down that other path.”