Maine Medical Center postponed elective surgeries at its Portland campus for two days this week in response to a patient suspected of carrying a rare but dangerous pathogen that’s resistant to sterilization.
The patient is being tested for prion disease, a rapidly degenerative illness that leads to brain damage and eventually death. The diagnosis has not been confirmed, MMC said in a statement.
The MMC patient, who was not identified for privacy reasons, underwent a biopsy that came back as suspicious for prion disease, the hospital said in an FAQ posted to its website. Final results may not come back for several days.
As a precaution, the hospital rescheduled elective surgeries set for Wednesday and Thursday at its Bramhall campus, according to the statement.
MMC is sterilizing equipment and surgical areas, going above and beyond protocols recommended by the U.S. Centers for Disease Control and Prevention, the hospital said.
Patients scheduled for surgery and their families are not at risk because potentially contaminated surgical instruments have been removed from circulation, according to the hospital.
However, MMC said it’s “too soon to tell if a small group of patients treated at the hospital prior to the original patient’s pathology report are at any theoretical risk.” If those patients are deemed in danger of contracting the illness, they will be notified, but the “risk of actual transmission in a hospital setting is extremely low,” the hospital said.
Here are the basics on prion disease:
It ravages the brain.
Prion disease is distinct from other infectious diseases in that it’s not caused by a bacteria, virus, or fungus, but by abnormal proteins called prions. They can cause other proteins in the brain to fold abnormally, essentially leaving the organ full of holes like a sponge. That brain damage leads to memory impairment, dementia, personality changes, and difficulty moving, among other symptoms. The incurable disease usually progresses very quickly and is always fatal, according to the CDC.
Prion disease refers to a family of progressive disorders that affect both humans and animals.
The most common form of it among humans is Creutzfeldt-Jakob disease, which can arise spontaneously, for no known reason. A variant form of it is caused by eating meat from cattle infected with another prion disease, bovine spongiform encephalopathy, or mad cow disease.
It can survive sterilization.
In addition to eating contaminated meat, humans can develop prion disease by inheriting a genetic mutation for it or by contracting it during a medical procedure. It can live in donated tissue, such as corneas, or on medical equipment.
That’s why it poses such a daunting challenge to hospitals: Standard methods for sterilizing surgical equipment before operations don’t protect against the disease. The infected brain tissue can persist on surgical equipment, potentially infecting other patients on down the line before doctors even know it’s there, as reported in Scientific American.
To treat metal instruments contaminated with prions, hospitals have to put them in an autoclave and heat the tools to 121 degrees Celsius for 30 minutes, according to CDC protocols. That’s much more than is required to kill bacteria and viruses. But even that doesn’t always work — prions can survive the superheating, though it does weaken them, according to Scientific American.
MMC said it is cleaning all surfaces and instruments that may have come in contact with the patient, using “a high-strength cleaning solution and extreme high temperatures to ensure adequate sterilization.”
The disease is not otherwise contagious and does not spread through the air like the flu or other infectious illnesses.
It’s hard to diagnose.
Another reason why prion disease poses such a risk is that it has a long incubation period. The time between when a person is exposed to when they start feeling symptoms can range from a year to even several decades.
That means a patient with the disease can arrive at the hospital and show no symptoms. It may not be until they’re on the operating table for another reason that doctors spot signs of trouble. Or the medical staff may spot no red flags at all, only to discover months later that the patient was infected. In the meantime, many patients could have been exposed to the contaminated tools.
Officials say that’s exceedingly unlikely. No cases of Creutzfeldt-Jakob disease related to contaminated medical equipment have been reported since 1976, according to the CDC.
In the Atlantic magazine in 2013, infectious disease specialist Dr. Timothy Lahey wrote that only four known cases of the disease have been spread through contaminated surgical instruments in all of history.
It’s rare.
CJD is estimated to affect about one out of every million people worldwide each year, according to the World Health Organization. About 250 cases are diagnosed each year in the U.S.
MMC said it has ruled out the possibility of prion disease on all of its campuses and at other area hospitals.
Even so, prion disease has posed a threat here before.
In 2014, a Kennebunk woman who worked as a nurse at Maine Medical Center died from Creutzfeldt-Jakob disease, according her family.
And in 2013, 15 people in New Hampshire, Massachusetts and Connecticut were warned that they may have been exposed to the disease through potentially contaminated medical equipment.
