August 3, 2012 - 3:00 PM
Over the past few days, there have been a number of questions from the community about the case of Creutzfeldt-Jakob Disease diagnosed at Greenville Memorial Hospital in February. We would like to provide more information to address those concerns.
Creutzfeldt-Jakob Disease, or CJD, is an extremely rare disease that affects the brain and causes rapid confusion, memory loss and difficulty moving. The diagnosis is made by observing tissue from a brain or spine biopsy. There is no prevention or treatment for the disease and it always results in the death of the patient. According to the Center for Disease Control or CDC, it is diagnosed in about 1 patient per million patients per year. That means that about 300 cases of CJD are diagnosed in the United States every year and that we would expect to diagnose 1 case every other year in South Carolina.
The cause of CJD is not clearly known. It appears to be caused by a protein called a prion that can affect the brain and spine. It is not caused by germs such as bacteria or viruses. There are 3 forms of CJD. The first form called idiopathic CJD represents 85% of the cases. In this form, there is no known exposure which causes the disease. The second form is called familial CJD and represents most of the remaining 15% of cases. In this form, the patient inherits the prion from a parent. The third form is called variant CJD. It is extremely rare as only 200 people in the world are known to have had this form of the disease and it likely is related to eating contaminated animal tissues. It is related to mad-cow disease.
In theory, CJD can be transmitted through medical care. This requires that brain or spinal tissue from a patient with CJD comes in contact with brain or spinal tissue of the second patient. A recent paper published by the CDC outlines this potential risk. Most of the cases of transmission of CJD involved the transplanting of a piece of brain tissue called the dura mater or the use of human growth hormone taken from the brains of patients with CJD. There are only 6 cases ever thought to be transmitted to a second patient through surgical instrument contamination. All of these cases occurred between 1952 and 1976 in Europe. Routine sterilization processes are much improved from when those cases occurred and no cases of surgical instruments transmitting CJD are known to have occurred anywhere in the world since 1976. Additionally, non-variant CJD is not transmitted by blood and has never been transmitted by close personal contact between individuals.
While we are not allowed to share specific details of the case at Greenville Memorial Hospital because of patient privacy rights, we can share general information. A patient underwent a surgical procedure on the brain in early February. The patient had symptoms of confusion and memory loss that were consistent with the patient’s known medical conditions. There was no reason to suspect that the patient had CJD. Following surgery, the surgical instruments were cleaned using the normal, CDC approved methods for sterilization. The patient’s symptoms rapidly progressed and doctors performed a second procedure a few weeks later to attempt to diagnose the patient. They were considering a broad range of possible diagnoses at that time. The spinal fluid test for a protein that would lead doctors to consider CJD as a potential diagnosis was negative, but the brain biopsy was suspicious for CJD. Because of this suspicion, the surgical instruments were immediately isolated and the tissue was sent to the National Prion Center for definitive diagnosis. We received confirmation of the diagnosis of CJD over a month after the original surgery. We also requested further testing to determine if the patient’s CJD was familial. We received those test results back several weeks later and they were also negative.
Shortly after we became aware of the diagnosis, we were in contact with the CDC and the National Prion Center to obtain advice on any additional steps that we could take to protect our patients. The CDC advised us that the surgical instruments used in the first surgery were acceptable to use without further sterilization because they had gone through more than one sterilization cycle. The instruments used in the second case were sterilized using the more extensive sterilization process for prions as recommended by the CDC. We also identified all patients who had undergone brain or spine surgery at Greenville Memorial Hospital since the original surgery. We then systematically reviewed each case and determined which patients were exposed to the original surgical instruments during the short period of time between the first surgery and taking the instruments out of use.
Our internal investigation revealed that our policy regarding how to handle CJD cases is very good and that it was followed. Our surgical instrument sterilization process is also very good and it was followed. Thus, no patient safety error occurred and no mistakes were made. Our physicians, who are familiar with CJD, could not have made the diagnosis at the time of the original surgery and thus had no reason to isolate those instruments at that time. Additionally, the sterilization process currently in use in the United States is in all likelihood adequate to protect patients from CJD.
The investigation identified 11 patients whose treatment included using the sterilized instruments from the first surgical case. We believe that the risk to them is extremely small and probably zero. Even though there is no known effective treatment, we also believe in transparency and that they have a right to know of their possible exposure. Thus, we contacted each patient, disclosed the facts, provided them with as many answers as we could and pledged our support if issues did arise in the future. We are confident that no additional patients were exposed.
In conclusion, our investigation is complete. Our policies and procedures meet all CDC recommendations. As an added precaution, we are increasing the sterilization temperature for instruments used during all brain surgeries. This change goes well beyond CDC recommendations, but is an extra safety precaution. The risk for any patient to contract CJD from medical or surgical care at Greenville Memorial Hospital or for that matter any other hospital in the United States is extremely unlikely. The very small risk of contracting CJD from this situation is limited to these 11 patients. Additionally, there is absolutely no risk to any of our employees, even those involved in the initial surgery. We hope this answers concerns from the public and suggest that additional information can be obtained from the CDC website.
Tom Diller, MD, MMM
VP Quality and Patient Safety
Greenville Health System
About CJD (CDC)
Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment (CDC)
Guideline for Disinfection and Sterilization of Prion-Contaminated Medical Instruments (SHEA)
Creutzfeld-Jakob Disease Overview (National Library of Medicine)