A bizarre, fatal disease first observed to strike brain-eating cannibals may be hitting patients in the northeast -- and it’s being spread by hospital instruments that can carry the deadly proteins that cause the disease even if they have been properly sterilized.
But here’s the kicker. Patients at risk for the illness, known as Creutzfeldt-Jakob Disease, can take years to develop any symptoms and live perfectly normally in the interim. In fact, many patients exposed to Creutzfeldt-Jakob will never get the disease at all. There’s no way to know, no way to prevent it and no effective treatment.
But doctors have been telling patients who may have been exposed, leaving them to go about their daily business knowing that a grotesque, malformed protein may be burrowing around in their brains, slowly and quietly devouring their thoughts, memories, senses and identities.
A patient recently died of Creutzfeldt-Jakob Disease, at Catholic Medical Center in Manchester, N.H., officials there have confirmed. The sophisticated and expensive medical imaging system use to treat that victim was apparently also used at other hospitals around new England on 15 patients who needed high-tech neurosurgery.
Sharing expensive equipment among regional hospitals is a common, cost-saving practice in the medical industry.
The problem is, the original equipment may have been contaminated by the “patient zero” in Manchester. Getting rid of the contamination is impossible using conventional sterilization techniques, because the disease is caused by a type of misshapen protein called a “prion.”
Not only are prions malformed, but they latch on to healthy proteins and mess them up as well, If the disease takes hold, a patient’s brain becomes overrun with misshapen, monstrous proteins.
The disease was first spotted in the Fore tribe of Papua New Guinea, who ritually consume the brains of deceased tribe members. Many of the Fore have a genetic immunity to the prion-caused disease, but those who lacked the proper gene would die from eating their neighbors' brains.
Symptoms of Creutzfeldt-Jakob, which is different from, but related to Mad Cow Disease, include memory loss, personality changes, anxiety, depression, problems thinking straight and in its later stages, breakdown of muscular coordination, blindness, coma and death.
The disease affects only about one of every million human beings worldwide.
SOURCES: NBC News, Boston Globe, The Atlantic