Rare & Fatal Brain Disorder Confirmed in Patient at Maine Medical Center


Creutzfeldt-Jakob disease, a rare & fatal degenerative brain disorder, has been confirmed in a patient at Maine Medical Center.

On Friday, November 11, Maine Medical Center—dubbed as the number one Medical Center in Maine by US News and World Report for 2016-2017confirmed that a patient at the hospital has been diagnosed with an incredibly rare brain disorder: Creutzfeldt-Jakob disease (CJD).

According to the Centers for Disease Control and Prevention (CDC), CJD is a neurodegenerative disorder that is rapidly progressive. Also known as the human prion disease, CJD is not only rare, but fatal, with patients dying within one year after infection.

The CDC reports that in about 85% of CJD cases there is no recognizable pattern when it comes to transmission of the disease. In addition, according to the hospital’s official news release, CJD transmission within a hospital setting has not occurred in over 20 years.

When it comes to this particular case, healthcare officials suspected CJD after they received initial biopsy results that came in earlier in the week last week. It wasn’t until Friday, however, that their suspicions were confirmed by the National Prion Disease Pathology Surveillance Center at Case Western University.

After receiving confirmation of the fatal disease, the hospital put all of their efforts into prevention and infection control. One of the preventive steps that they took was to reschedule 150 surgeries so that the hospital could focus on decontamination efforts. The team made sure that their efforts were in accordance with safety guidelines that had been released by the CDC pertaining to the disease. Leaving nothing to chance, the team did not just rely on the tracking of surgical equipment, but took the extra step to ensure that any tools that had the potential for being infected were taken care of.

Joel Botler, MD, chief medical officer at MMC commented, “We thought it important to go above and beyond to assure the safety of our patients. 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.”

In addition to decontamination efforts, healthcare officials are also making a list of patients who had received treatment before the pathology report had been received and depending on the type of procedure performed and the surgical tools that were used, patients thought to be at any risk will be contacted by the hospital.

In the press release, Dr. Botler said, “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. 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.”

Over 100 staff members participated in the decontamination efforts, and, in order to continue performing necessary emergency surgeries, other hospitals in Maine joined the effort by lending necessary equipment to Maine Medical Center in their time of need.

Feature Picture Source: InAweofGod’sCreation​ / flickr / Creative Commons.

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