Mysterious Symptoms: Intense Heartache and a Sore Behind
Patient: Marvin, a 55-year-old chemical engineer
Doctor: Dr. Brian Goldman, a Toronto ER physician, CBC host and author of The Secret Language of Doctors
One recent winter afternoon, a middle-aged man walked into the emergency room at a hospital in downtown Toronto, holding his head. Marvin had experienced headaches, but nothing like this. The pain came on gradually, then grew more intense over several hours until it throbbed across his forehead. He also felt fluish and kept rubbing his left buttock. “Aches and pains,” he told Dr. Brian Goldman, who was on call that day. “Must be getting old.” Marvin had no fever, and no one around him was sick.
“I’m an emergency physician, so I always think worst-case scenario,” Goldman says. “This was not a headache guy, so that factor made his case unusual.” Because of the intensity and atypical nature of the pain in his patient, the doctor immediately wondered whether he had a tumour or subarachnoid hemorrhage (a brain bleed often caused by an aneurysm). The other possibility was meningitis.
Goldman started with a physical exam. Marvin’s heart rate was a little elevated. His ears, nose and throat showed no signs of flu or viral infection. “I checked his neck for stiffness, which is a sign of meningitis, but it was normal,” Goldman says.
The only definitive test for meningitis is a lumbar puncture to look for white blood cells in the spinal fluid. But in a patient with intracranial pressure due to bleed or a tumour, the procedure would direct that pressure downward, squeezing the brain toward the base of the skull. “He would lose consciousness, go into a coma and eventually stop breathing,” Goldman says. The doctor ordered an emergency CT scan, which showed that all was well in Marvin’s brain, making it safe to proceed with a lumbar puncture.
Goldman carefully inserted a long needle in the middle of Marvin’s back, between the fourth and fifth vertebrae. The fluid went to the lab, which confirmed the absence of red blood cells, helping the doctor rule out a hemorrhage. However, there were white blood cells-a clear sign of infection. The test showed 95 white blood cells pure cubic millimeter-not a lot, but enough to reveal that Marvin did have meningitis.
The illness, an inflammation of the membranes around the brain and spinal cord, comes in several forms, including bacterial and viral. “Bacterial meningitis is rapidly progressive and often kills young adults,” says Goldman. The viral form has subtler symptoms and is also less likely to be fatal-but it’s most common in kids under five. “I had no idea why he had viral meningitis,” Goldman says. “Then I remembered his bottom.” The doctor asked to take a look.
There, on Marvin’s left buttock, was a red rash with groups of blisters. “The medical mind looks for the connection,” Goldman says. “It doesn’t assume there are two different illnesses.” Suddenly the viral meningitis made sense. In addition to a horrible headache, Marvin had shingles.
The illness is caused by the varicella zoster virus, one in a group of enteroviruses that tend to circulate in summer and early fall. Others include mums and West Nile-and all of them can cause meningitis. Goldman says it’s not uncommon for varicella zoster to lead to shingles, but he’d never seen shingles and viral meningitis occurring simultaneously.
Marvin was admitted and put on acyclovir-the only intravenous antiviral drug that treats meningitis. Two days later, the headache was gone and he made a full recovery. The moral of the story, Goldman says, is to look for a connection. “You never know what you’re going to find.”
Mysterious Symptoms: Chest Pain, Fever and Fatigue
Patient: Angeline, a 32-year-old administrative assistant
Doctor: Dr. Anne McCarthy, chair of the Committee to Advise on Tropical Medicine and Travel and professor of medicine at the University of Ottawa
In 2013, Angeline visited her doctor. She’d had a fever for several days, her chest felt tight, and she was exhausted. The physician noticed that her lymph nodes were swollen and that she’d lost some weight. He decided to refer her to the Ottawa Hospital for a CT scan and biopsy, which led to a diagnosis of non-Hodgkin’s lymphoma.
The fifth most commonly diagnosed cancer in Canadian adults, non-Hodgkin’s lymphoma has a good prognosis when it’s caught early-especially if the patient is under 60. Angeline was given medication to support her immune system, chemotherapy to target the cancer cells and steroids to bolster the treatment. A week later, she became tired and nauseous, and her blood-cell count dropped dramatically. These can be side effects of chemo, but in this case, they were unusually pronounced.
Within days, Angeline’s fever spiked and she had trouble breathing. Bacteria had entered her bloodstream, and she’d developed an infection in her lungs and abdomen. The patient was admitted to the ICU and given antibiotics, but the drugs didn’t help. Instead, Angeline’s breathing worsened, and she was put on a ventilator.
That’s when Dr. Anne McCarthy was called in. The infection disease specialist found traces of a rash on Angeline’s abdomen. When she learned the patient had grown up in Haiti and immigrated to Canada eight years earlier, McCarthy immediately thought of strongyloides.
Strongyloides are a type of roundworm common in Southeast Asia, South Africa and the Caribbean. The larvae are small-about the size of a mustard seed-and are transmitted to humans through contaminated soil. They can penetrate unbroken skin (often through bare feet) and migrate through the body to the small intestine, where they lay eggs.
In this case, the wavy rash on Angeline’s stomach was a clue: it can appear in patients with strongyloides. When McCarthy checked the patient’s stool sample, she says, “the larvae were everywhere.”
Because there are no obvious symptoms, people can be infected for years without knowing they have the parasite. Angeline had experienced mild gastrointestinal issues at times, but otherwise wealthy, apart from her recent cancer diagnosis-until she was given some steroids as part of her treatment. “Strongyloidiasis is a sleeping disease,” McCarthy says. “It can exist in your body and do relatively little harm until you give it an advantage.” Doctors aren’t entirely sure why, but steroids stimulate the parasite to reproduce faster.
Those drugs had indeed unleashed the bug throughout Angeline’s body, a condition known as disseminated strongyloides, which causes death in 50 to 90 per cent of cases. Unfortunately, it takes time to get treatment because the two anti-parasite drugs that fight the disease (albendazole and ivermectin) aren’t licensed in this country, though they are used in the United States. McCarthy had to request permission from a special program through Health Canada. Although she remained in the ICU for almost a month, the patient received the drugs and made a full recovery, at which point she was able to resume treatment for the non-Hodgkin’s syndrome.
Like most people, Angeline had no idea she could be at risk for strongyloides. Even physicians often know very little about the parasite. McCarthy, who chairs the advisory committee that’s developing strongyloides guidelines, encourages practitioners to adopt a few key queries into their preventive care routine: Where were you born? Where have you lived? Have you travelled anywhere for six months or longer? “There is treatment for strongyloides and people can survive it,” she says. “We just need to ask the right questions from the beginning.”
Mysterious Symptoms: Hiccups, Headache and Vomiting
Patient: Olivia, a 24-year-old university student
Doctor: Dr. Anthony Traboulsee, a neurologist at UBC Hospital in Vancouver
Three years ago, Olivia began hiccuping-and couldn’t stop. The bouts, which lasted up to an hour, would dissipate for a similar amount of time, then reappear. Her head ached. A couple days later, she started vomiting. A hospital visit resulted in anti-nausea pills, but the queasiness didn’t stop. Because throwing up can signal a brain tumour, her next trip to the ER two days later led to a CT scan (the results were negative), and a lumbar puncture to rule out meningitis.
A couple days after that, Olivia’s vision blurred, and she had trouble getting out of bed. Still stumped, doctors put her on antibiotics in case her problems were the result of an infection, then monitored her in the ICU. By the next morning, the patient couldn’t see and was paralyzed from the neck down.
“She was fully awake and aware and very frightened,” says neurologist Dr. Anthony Traboulsee, who was called in after an MRI revealed evidence of inflammation, swelling and damage along Olivia’s brain stem and spinal cord. The other doctors suspected acute disseminated encephalomyelitis (ADEM), a brief, intense inflammation in that area, resulting from a bacterial or viral infection. But ADEM is more common in children and is often mistaken for multiple sclerosis (MS)-which is itself often confused with Neuromyelitis Optica (NMO). Traboulsee suspected this last condition was the culprit.
Much like MS, NMO is a relapsing inflammatory disease that occurs when a person’s immune system attacks the cells in their central nervous system. It affects the optic nerves and spinal cord, and sometimes the brain.
The disease is rare-while about 100,000 Canadians have MS, only about 1,000 have NMO, Traboulsee estimates. “It’s more aggressive than MS, like a forest fire raging through the nervous system,” says the doctor. Symptoms can include blindness, weakness or paralysis, spasms, vomiting, hiccups and loss of bladder or bowel control due to spinal cord damage. In Canada, the only lab equipped to do the blood test required to diagnose NMO is in Calgary. It generally takes two weeks to get the results.
Traboulsee sent the patient’s blood to the lab but didn’t wait to hear back. “It’s like when someone is drowning,” he says. Without treatment-ideally within 48 hours after symptoms appear-most NMO sufferers wind up in a nursing home.
The doctor immediately started Olivia on a plasma exchange to target the inflammation. A day or two later, she could see shapes and wiggle her fingers and toes. He added in chemotherapy, and Olivia’s recovery was quick and dramatic. “The plasma exchange removes the sparks, but the chemotherapy puts out the fire by removing the abnormal immune cells that are creating the destructive antibodies in the first place,” Traboulsee explains.
After a week of receiving the combined treatment, Olivia regained her sight and could walk again. She’s now on long-term preventive treatment and takes azathioprine, an immunosuppressant, twice a day. There have been no relapses since the initial attack. (When it’s not treated, NMO has a 100 per cent rate of recurrence.) Olivia was able to finish her degree and hopes to someday get married and start a family.
“Like with any rare illness, the key is early treatment and preventive care so people can lead normal lives,” Traboulsee says. But not every doctor is aware of every rare disease. “Because NMO is frequently misdiagnosed and can be suddenly severe, patients are sometimes written off. We can rescue them and give them their lives back.”