Fibreoptic equipment and prion disease

A 43-year-old-woman was referred to us for airway management due to worsening upper airway obstruction following a neck haematoma. Her past history included hypopituitrism secondary to a craniopharyngioma, which had been treated surgically and with cadaveric growth-hormone replacement 22 years previously. She had a severe learning disability and was cared for by her sister. She had been admitted to hospital on this occasion with renal failure from nephrotic syndrome requiring haemodialysis. A 11 French gauge double lumen dialysis catheter had been inserted into the right internal jugular vein by the nephrology service. There was no obvious arterial puncture and a chest X-ray showed a correct position. The patient was anticoagulated and underwent dialysis. Over the next 24 h, she developed a swelling in the right side of the neck, which increased sufficiently to compromise her breathing. As the Renal Unit was on a remote site without resident anaesthetic cover, the anaesthetic service from the supporting hospital was contacted for advice regarding airway management and interhospital transfer. Examination revealed a patient who, despite being slightly drowsy, needed to invest considerable effort to generate a meagre inspiratory airflow. She was tachypnoeic and had inspiratory stridor. The trachea could not be palpated due to the haematoma, which extended inferiorly to the level of the right nipple. Oxyhaemoglobin saturation was 94%, despite receiving high-flow oxygen via a facemask, and her chest X-ray was unremarkable. She was known to have marked prognathism as a result of her developmental abnormalities. In addition, her mouth opening was 2 cm, Mallampati was class 3 and neck movement was limited due to the haematoma. It was clear that the patient needed urgent tracheal intubation prior to interhospital transfer to the Intensive Care Unit, and an awake fibreoptic intubation technique was considered to be the best means of achieving this. An inhalational induction was considered but rejected because any degree of sedation would be likely to suppress the intense effort she was investing in maintaining her airway and generating airflow. Having made this plan, we were now faced with an unusual dilemma. The patient's case-notes were marked with a hazard label to the effect that, since she had been treated with cadaveric growth hormone 22 years ago, she was considered to be at risk of transmitting new-variant Creutzfeld-Jakob disease (which has previously been reported following prion transmission via cadaver-derived growth hormone and pituitary gonadotrophins [1,2]). We were able to take advice from the microbiology department, which confirmed that all equipment used in this patient was to be incinerated. We considered that once a management plan has been decided based upon clinical grounds, it would be illogical and indefensible to revise this merely on the grounds of our reluctance to sacrifice valuable equipment. After some thought, we decided to use an old LF-2 (Olympus®) flexible fibrescope which had been decommissioned from clinical use because of minor mechanical damage, but was kept by one of us (MP) for teaching purposes (on manikins). The patient was transferred to the operating theatre, accompanied by her sister in view of her severe learning disability. Intravenous glycopyrronium and topical anaesthesia was given prior to the procedure and no sedation was used. Tracheal intubation was achieved with a 7-mm reinforced tube passed nasally after a ‘spray as you go’ technique through the working channel of the fibrescope. The patient was then transferred to the Intensive Care Unit in the John Radcliffe Hospital. As prions are not inactivated by conventional disinfection or heat sterilisation, particular precautions have to be taken with all affected patients and those at risk of carrying infection [3]. Prions are highly resistant to standard physical and chemical methods of inactivation and decontamination but fibreoptic equipment is too expensive to be disposable. This case highlights the problem of finding suitable fibreoptic equipment for a patient at risk of CJD. In our department, it was possible to find an alternative solution on this occasion. However, having sacrificed this ‘teaching scope’, we would not be able to do so again. It would be interesting to hear views from colleagues in other departments about managing such a case.