Rare Brain Disease Kills NH Man; Exposes Risk - NECN

Rare Brain Disease Kills NH Man; Exposes Risk



    Creutzfeldt-Jakob Disease is caused by a protein that eats away at the brain (Published Sunday, Jan. 26, 2014)

    (NECN: Jackie Bruno) - It’s an illness so horrifying, it sounds like it’s out of a movie.

    Creutzfeldt-Jakob Disease, or CJD, is caused by a protein that mutates and eats away at the brain. You can have the protein for decades before symptoms like dementia, loss of memory, and bad balance develop, but once they do, there isn’t much time left.

    Catholic Medical Center CEO Dr. Joseph Pepe said, “Once symptoms appear average time of death is four months… no screening test.”

    What doctors know about CJD is limited. It’s not a virus. It’s not a bacteria, and it can sporadically occur and doctors don’t know why.

    That’s why when an elderly patient came into Catholic Medical Center in Manchester, N.H. to have a cyst removed from his brain, there was no way for doctors to know he carried this mutated protein. Last month he returned with symptoms, and preliminary tests came back positive for CJD.
    Now, doctors are worried that eight other people could have exposed to the illness since the same surgical equipment was used on them.

    N.H. Public Health Director Dr. Jose Montero said, "The issue is that after you diagnose CJD you know that regular standard type of cleaning doesn't always get rid of the particles that cause CJD."

    And more people could be affected because that equipment was on loan from a company called Medtronic.

    Montero said, "And after that piece of equipment was sent outside New Hampshire and used in other states and different hospitals."  

    Medtronic has released a statement saying, “We are assisting the hospitals and the appropriate state health authorities as they manage this situation. We have also notified the U.S. Food and Drug Administration (FDA).  The hospitals are coordinating all communications with patients potentially involved in this situation.”

    Experts say the general public should not be worried about CJD. There have only been four cases in the world that were actually contracted because of surgical instruments. These four cases were all before 1976 and were outside of the United States. Doctors say you only have a one in one million chance of contracting CJD, none the less this is a very scary situation for those people in N.H.