Prion contamination in the emergency room

prion conversionHere is a follow-up to last week’s article that described a case of variant Creutzfeldt-Jacob disease in a Texas resident caused by ingestion of BSE-contaminated beef 14 years ago.

A 59 year old male patient was admitted to the trauma unit in Lancaster, PA with a self-inflicted gunshot wound to the head. There was substantial bleeding and brain tissue extrusion from the bullet exit wound. While the patient was intubated, examination of his electronic health records revealed a previous diagnosis of Creutzfeldt-Jacob disease (CJD). After discussion with his family, the breathing tube was removed and the patient expired.

After discovering that the patient had CJD, TSE (transmissible spongiform encephalopathy) decontamination protocols were initiated. Equipment and surfaces that had been exposed to highly infectious brain tissues were identified. Because prions are extremely difficult to destroy, it was decided to incinerate many pieces of equipment costing tens of thousands of dollars. This decision was taken to protect workers in the trauma unit and future hospital patients from hospital-acquired CJD.

The usual sterilization conditions (121 degrees Celsius for 20 minutes under high pressure) do not destroy prion protein infectivity. Consequently the World Health Organization recommends incineration of potentially contaminated materials. While environmental transmission of prion diseases has not been reported, WHO suggests rinsing surfaces with sodium hydroxide or sodium hypochlorite for 1 hour, followed by flooding with water, to remove prions.

This case illustrates the problems associated with an unusual infectious agent, the prion, that is difficult to inactivate. It also shows the value of electronic health records. Without such readily accessible information, the discovery that the patient had CJD would have been substantially delayed, leading to further contamination.

Creutzfeldt-Jacob associated deaths have increased slowly but steadily in the US since 1979. The number of cases will likely continue to increase until early diagnosis tests become routinely available, and drugs are developed that can cure the disease.

1 thought on “Prion contamination in the emergency room”

  1. Elizabeth Jonas Garcia

    I am very happy today to testify the goodness of God in my
    life. I want you all here to be grateful to Doctor Uwadia Amenifo for me; he is
    indeed a wonderful man. Just last month i read some testimonies from the
    internet by a man named “James Watt” he testified of how his cousin
    was cured of Creutzfeldt – Jakob disease by a herbal doctor. At first i doubted
    because i have been suffering from the said disease for over five years, and my
    doctor said there is no cure for it. But i was under medication as my life was
    dwindling by the day. But after i read his testimony, he included the contact
    details of the herbal doctor so ii emailed the doctor and explained to him how i
    have suffered from Creutzfeldt – Jakob disease for over 5 years. But he assured
    me that he can cure me with his herbs and told me how he have cured so many of
    the said disease, well i accepted what he said to me, and followed all the
    procedures he gave me. after all, he
    prepared the herbs and sent it to me here in Australia i took it as prescribed
    just after three weeks, there was tremendous improvement in my state of health,
    and my doctor begin to wonder how greatly i am re covering. And just in the
    fourth week of taking the herbal mixture, i became totally healthy, and today i
    am perfectly OK, and free from Creutzfeldt – Jakob disease. So i contacted
    Doctor uwadia Amenifo and told him that I am ok now and he congratulated me,
    and asked me to help him tell the world about his herbs. Please i implore you
    all that have same or similar disease to have hope that a remedy have come. Please
    if you wish to contact Doctor Uwadia Amenifo, here is his contact details email
    ( or you can also reach him on his cell phone
    number (+2349052015874). So I want you all to please be happy for me, and also
    try to contact him if you have any health issues.

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