A patient treated recently at Maine Medical Center in Portland has Creutzfeldt-Jakob disease, a fatal degenerative brain disorder caused by an infectious type of protein, the hospital said Friday.

Maine Med announced Wednesday that it had postponed elective surgeries at its Portland campus after it suspected a patient had a rare, pathogen-based type of prion disease, in the same family as “mad cow disease.” It continued to suspend elective surgeries Thursday while hospital staff performed specialized sterilization of equipment and surgical processing areas. Surgeries resumed Friday.

Hospital officials said they learned this week that a patient appeared to have the rare condition based on an initial biopsy result. The National Prion Disease Pathology Surveillance Center at Case Western University in Cleveland tested the biopsy sample and confirmed the diagnosis Friday, according to a Maine Med news release.

The hospital said it is now looking at the procedures it performed and the instruments that were used prior to the initial biopsy to determine which patients should be contacted because they might be at risk.

“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 Maine Med, said in the release. “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.”


Also known as transmissible spongiform encephalopathies, prion diseases include bovine spongiform encephalopathy (mad cow disease) in cattle; Creutzfeldt-Jakob disease in humans; scrapie in sheep; and chronic wasting disease in deer and elk, according to the National Institutes of Health.

Prion disease is not contagious. It can occur spontaneously, be inherited, or be transmitted through contaminated food, usually meat, or through medical and surgical procedures, the National Prion Disease Pathology Surveillance Center website says. There is no cure for prion disease, which occurs in about one out of every million people worldwide per year.

Transmission of Creutzfeldt-Jakob disease in a hospital setting is extremely rare, the hospital said, with no confirmed incidence of such transmission in more than 20 years. Still, the hospital said it decided to take “extraordinary steps to assure patient safety.” Roughly 150 elective surgery cases were rescheduled while the hospital sterilized surgical equipment and facilities in accordance with guidelines set for prion disease by the U.S. Centers for Disease Control and Prevention, Maine Med said.

“We thought it important to go above and beyond to assure the safety of our patients,” Botler said.

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.

“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,” he said.


Maine Med spokesman Clay Holtzman said he could not disclose the infected patient’s condition, or whether the patient was still at the hospital. However, Holtzman said, “There is zero risk for transmission for any Maine Med patient right now.”

Botler said more than 100 Maine Med employees worked around the clock to sterilize the surgical equipment. In the meantime, other Maine hospitals loaned the hospital equipment so it could perform the emergency surgeries that are required of the state’s largest medical center and Maine’s only Level 1 trauma center.

“We want to thank our amazing team here at Maine Medical Center,” Botler said. “This is an especially rare circumstance in medicine, and the men and women here responded with the utmost professionalism and dedication to make sure we continue to provide our patients with excellent, safe patient-centered care.” Botler also thanked the hospital’s patients for their understanding and apologized for any inconvenience and concern the situation caused them and their families.

“Finally, we’d like to thank all of our colleagues in the statewide healthcare community who have come to our assistance,” he said.


Only subscribers are eligible to post comments. Please subscribe or login to participate in the conversation. Here’s why.

Use the form below to reset your password. When you've submitted your account email, we will send an email with a reset code.

filed under: