Creutzfeldt-Jakob Disease Strikes Again In MaineNovember 15, 2016
CJD Often Misdiagnosed As Alzheimer’s Disease
Maine Medical Center confirmed Friday that a patient treated at the hospital carried a rare, dangerous brain illness to which a “small number” of other patients may have been exposed, though the hospital called that risk “exceedingly low.”
The patient has Creutzfeldt-Jakob disease, or CJD, a degenerative brain disorder caused by an infectious type of protein, the hospital said in a statement. CJD is a form of prion disease, which is fatal.
Hospital officials suspected earlier this week that the patient, who was not identified for privacy reasons, carried the pathogen, based on an initial biopsy result. The National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland confirmed the diagnosis on Friday, according to the statement.
“We are in the process of reaching out to the small number of patients who we think should be notified based on the details of their specific case,” Dr. Joel Botler, chief medical officer at MMC, said in the statement. “Our staff members have been fielding calls from patients who have legitimate concerns and questions about their care. Let me be clear, only a small number of patients who have had surgery at MMC have been exposed to any degree of risk, and that risk is exceedingly low approaching zero.”
Transmission of the disease in a hospital setting is “extremely rare,” with no confirmed cases in more than 20 years, the statement said.
Humans can contract prion disease from medical procedures, as the pathogen can survive on surgical tools and other equipment even after standard sterilization. Brain tissue from an infected patient potentially can infect subsequent patients before doctors know it’s there.
MMC is determining which patients were treated before the initial pathology report showing Creutzfeldt-Jakob disease and should be notified.
“While the hospital does track surgical equipment used in each case, the decision was made not to rely on tracking and instead make sure that any piece of equipment that could potentially be affected was treated,” Botler said in the statement. “Now that we know this case is confirmed, we can see that our response was 100 percent appropriate and that patients should feel confident in the safety of their care at Maine Medical Center.”
As a precaution, the hospital rescheduled approximately 150 elective surgeries Wednesday and Thursday while staff decontaminated surgical equipment and facilities in accordance with guidelines set by the U.S. Centers for Disease Control and Prevention, the hospital said.
In the meantime, other Maine hospitals loaned equipment so MMC could perform emergency surgeries.
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 a report in Scientific American.
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 months to years. 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 later that the patient was infected. In the meantime, other patients can be exposed to the contaminated tools and more.
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 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, also known as mad cow disease. (Editor’s Note: In deer, elk, moose and reindeer, prion disease has been dubbed chronic wasting disease. It’s time to put the species-specific names aside. A deadly prion is a deadly prion.)
CJD is rare, 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. but thousands of other cases are suspected to go undiagnosed and misdiagnosed.
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.
Editor’s Note: The most common forms of neurodegenerative disease include Alzheimer’s disease, Parkinson’s disease, ALS and Creutzfeldt-Jakob disease–the most aggressive and infectious of them all. According to Nobel Prize Laureate Stanley Prusiner, these brain diseases are on the same disease spectrum-prion disease. It’s also known as transmissible spongiform encephalopathy (TSE). The bodily fluids of TSE victims are infectious and deadly. This infectious waste is now an environmental nightmare.
Prion disease is a spectrum disease that varies in severity. It also varies depending on which region of the brain is impacted first. It affects most, if not all, mammals. Prion disease causes memory loss, impaired coordination, and abnormal movements. It’s not known which patients with brain disease become infectious or when, but both CJD and Alzheimer’s patients are being mismanaged. The most savvy neurologists won’t touch patients with these symptoms because of the risks. They are making diagnoses from across the room. Unfortunately, caregivers aren’t warned accordingly.
CJD behaves like Alzheimer’s disease on steroids,” said Dr. Jennifer Majersik, an associate professor of neurology at the University of Utah.
Experts claim that at least 25 percent of Alzheimer’s diagnoses are not Alzheimer’s disease. Millions of misdiagnoses are actually CJD, which is further up the prion spectrum. Millions of patients and caregivers are being misinformed, misguided and exposed to an aggressive prion disease. Misdiagnosis and misinformation regarding prion disease is a matter of life and death.