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15 patients may have been exposed to deadly brain disease

Every surgery carries a risk. When you consent to allow a doctor to cut into your skin and perform some procedure involving your internal organs, there is a potential for risk, ranging from that of a minor infection to something life threatening. Few patients, here in Hawaii, or in New England, where this story originated, expect they risk being exposed to Creutzfeldt-Jakob Disease (CJD) during a surgery.

In a horrific case of negligence and medical malpractice, a surgical tool used by multiple hospitals may have exposed 15 patients to CJD. The tool was used on a patient who recently died, and the autopsy confirmed that a neurosurgery patient did have CJD. 

CJD is a disease that is caused by a protein that is known as a prion that is misfolded and its presence triggers other proteins to misfold. This results in spongiform encephalotpathy, or holes developing in the brain. In cattle, the disease is known as bovine spongiform encephalotpathy (BSE) or commonly, mad cow disease.

How prions behave is little understood, but the CJD has no treatment or cure, and the progression of the disease leads to death. The patient with the confirmed case of CJD appears to have developed it spontaneously, and was not exposed to beef contaminated with BSE, a frequent cause of CJD.

The hospital failed to adequately disinfect the surgical tool, as tools contaminated with prion cannot be disinfected by normal procedures. Because CJD can only be confirmed by an autopsy, the Joint Commission has recommended that hospitals handle any case where a patent exhibits possible symptoms of CJD as a case of CJD and sterilize or dispose of the tools appropriately.

Source: NBC News, "Fatal rare brain disease confirmed in N.H. patient; 15 possibly exposed," JoNel Aleccia, September 21, 2013

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