Coronial
WAhospital

Inquest into the Death of Child MD (Name Subject to a Suppression Order)

Deceased

Child MD

Demographics

2y, male

Coroner

Coroner Urquhart

Date of death

2021-05-12

Finding date

2023-11-17

Cause of death

multiorgan failure in a boy with clinically suspected sepsis and Cockayne syndrome

AI-generated summary

A 2-year-old boy with Cockayne syndrome (a rare life-limiting genetic condition) died from multiorgan failure due to clinically suspected sepsis following hospitalisation for acute illness with vomiting, lethargy and poor oral intake. He was admitted to Perth Children's Hospital paediatric ICU after initial presentation to a regional hospital. The coroner found that emergency department management was appropriate, including antibiotic selection (single agent ceftriaxone per guideline) and decision not to escalate to consultant—neither impacted outcome. A medication error occurred where acetylcysteine was given intravenously instead of by nebulisation on two occasions, but this was determined to be safe. The child received quality, compassionate medical care from dedicated staff. The child had been in appropriate Department care since birth following pre-birth planning due to maternal risk factors. A minor system issue regarding acetylcysteine formulation packaging was subsequently corrected to prevent recurrence.

AI-generated summary and tagging — may contain inaccuracies; refer to original finding for legal purposes. Report an inaccuracy.

Specialties

intensive carepaediatricsemergency medicinegeneral surgeryrehabilitation medicineneurologyENT surgeryophthalmologygastroenterologypaediatric surgeryoccupational therapyphysiotherapydieteticsspeech pathologypharmacy

Error types

medication

Drugs involved

ceftriaxoneacetylcysteineparacetamolfentanylantibiotics

Clinical conditions

Cockayne syndromesepsismultiorgan failureacute liver failureacute kidney injurysevere respiratory failurecoagulopathyhypotoniabilateral vertical talusarthrogryposismicrocephalymicrophthalmiacongenital cataractssevere hearing impairmentglobal developmental delaygrowth failurebronchopneumoniadiffuse alveolar damageveno-occlusive disease/hepatic sinusoidal obstructionacute bronchiolitisfungal infectionmiddle ear effusions

Procedures

intubationmechanical ventilationpercutaneous endoscopic gastrostomy (PEG) insertioncataract surgerymyringotomy and grommet insertionbilateral orchidopexyorthopaedic surgery for vertical talus

Contributing factors

  • Cockayne syndrome—a rare life-limiting genetic disorder affecting DNA repair mechanisms leading to progressive organ dysfunction
  • clinically suspected sepsis with mixed bacterial and fungal infection
  • acute liver failure
  • acute kidney injury
  • severe respiratory failure
  • coagulopathy
  • underlying hepatic dysfunction pre-existing in Cockayne syndrome
  • fungal infection of left kidney (Candida tropicalis)
  • middle ear effusions with bacterial and fungal elements

Coroner's recommendations

  1. Continuation of revised dispensing procedures at Perth Children's Hospital regarding acetylcysteine nebulised formulation to prevent further incidences of intravenous administration error
Full text

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