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.

General

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

Malignant

• 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

Respiratory

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.

Genetic

• 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
  

Metabolic

• 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

Renal

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

Gastrointestinal

• 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

Neonatal

• 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

Antenatal

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

Cardiac

• 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