Appendix 2 - Suggested triggers to commence Step 1 of ACP discussion

  • These are suggested conditions that should trigger the commencement of advance care planning with children and families (Step 1). Please see Appendix 3 for suggested triggers to progress the discussion to steps 2 and 3.


    Any child or family who expresses a desire to discuss advance care planning.


    • Malignant disease with inevitable fatal outcome (for example diffuse intrinsic pontine glioma)
    • Actively progressing metastatic disease
    • Malignant disease with progression on best therapy
    • Relapsed malignant disease
    • Malignant disease with predicted outcome ≤ 40 per cent survival with best treatment
    • High-risk bone marrow transplant


    Compromised respiratory status and:

    • child with cystic fibrosis  considering lung transplant/at the time of transplant
    • child with cystic fibrosis with forced expiratory volume in 1 second (FEV1) <30 per cent predicted for age
    • child with cystic fibrosis with ventilator dependence or those ineligible for lung transplant
    • bronchiolitis obliterans
    • central hypoventilation syndromes
    • patients who are chronically ventilator dependent.


    • Trisomy 18, 13
    • Potter syndrome
    • Epidermolysis bullosa (junctional)
    • Osteogenesis imperfecta type 3/4
    • Other rare chromosomal anomalies with likely poor prognosis

    Neurological / neurodegenerative / neuromuscular/ neurodevelopmental

    • Progressive neurodegenerative conditions
    • Duchenne muscular dystrophy
    • Spinal muscular atrophy type 1
    • Severe brain injury secondary to trauma, anoxia or infection
    • Persistent vegetative state
    • Batten disease
    • Metachromatic leukodystrophy/adrenoleukodystrophy
    • Brain malformations including anencephaly, hydranencephaly, lissencephaly and severe schizencephaly
    • Static encephalopathies, including cerebral palsy, with progressive complications (for example, recurrent respiratory tract infections, scoliosis)
    • Severe infantile encephalopathies
    • Rett syndrome


    • Krabbe disease
    • Hunter syndrome
    • Hurler syndrome
    • Niemann-Pick disease
    • Menkes syndrome
    • Infantile Pompe disease
    • Sanfilippo syndrome
    • Tay-Sach disease
    • Fabry disease
    • Sandhoff disease
    • Severe mitochrondrial disorders
    • Severe metabolic disorders for which bone marrow transplant is a therapeutic consideration


    • Neonatal polycystic or severe structural renal disease
    • Neonatal oligo/anuric renal failure
    • Renal failure with complex comorbidities or other life-limiting conditions


    • Short gut syndrome without prospect of curative therapy
    • Biliary atresia without prospect of curative therapy
    • Multi-visceral organ failure
    • Feeding tube under consideration for any progressive or severely disabling neurological condition with no expectation of improvement


    • Extreme prematurity with concomitant severe complications such as bronchopulmonary dysplasia, grade IV intraventricular haemorrhage, periventricular leukomalacia.
    • Hypoxic-ischemic encephalopathy (moderate to severe)
    • Prematurity complicated by major congenital malformations


    • Any antenatally diagnosed condition likely to be incompatible with life
    • Any antenatally diagnosed condition likely to result in a significantly shortened lifespan


    • Advanced heart failure symptoms attributable to congenital or acquired cardiac disease
    • Cardiomyopathy (acquired, dilated, restrictive or hypertrophic)
    • Failing single ventricle physiology:
      • hypoplastic pulmonary arteries
      • single ventricle failure
      • plastic bronchitis
      • protein-losing enteropathy
    • Severe primary or idiopathic pulmonary hypertension
    • Multiple or end-stage secondary organ failure following cardiac interventions
    • Combination of cardiac diagnosis and underlying life-limiting neurologic/ chromosomal diagnosis