Curriculum standards
Knowledge guides
LG 14: Life-limiting and life-threatening conditions
Key presentations and conditions
Advanced Trainees will have a comprehensive depth of knowledge of these conditions.
Epidemiology, pathophysiology, and clinical sciences
Advanced Trainees will have a comprehensive depth of knowledge of the principles of the foundational sciences.
Investigations, procedures, and clinical assessment tools
Advanced Trainees will know the scientific foundation of each investigation and procedure, including relevant anatomy and physiology. They will be able to interpret the reported results of each investigation or procedure.
Advanced Trainees will know how to explain the investigation or procedure to patients, families, and carers, and be able to explain procedural risk and obtain informed consent where applicable.
Important specific issues
Advanced Trainees will identify important specialty-specific issues and the impact of these on diagnosis and management and integrate these into care.
Non-malignant conditions, including but not limited to
- Cardiac conditions, including:
- cardiac disease, congenital
- cardiomyopathy, acquired
- cardiomyopathy, familial
- Congenital health problems, including:
- birth complications:
- hypoxic brain injury
- genetic conditions, including:
- trisomy 18
- prematurity-related
- birth complications:
- Dermatological conditions:
- epidermolysis bullosa
- Gastrointestinal conditions, including:
- liver failure
- refractory feeding intolerance, generally associated with another presentation, including:
- severe neurological impairment
- short gut syndrome
- Immunological conditions, including:
- immunodeficiencies being considered for or treated with bone marrow transplant, including:
- severe combined immunodeficiency (SCID)
- immunodeficiencies being considered for or treated with bone marrow transplant, including:
- Metabolic conditions, including:
- mucopolysaccharidoses
- Pompe disease
- Neurological conditions, including:
- brain injury
- neurodegenerative disorders:
- Batten disease
- Duchenne muscular dystrophy
- leukodystrophy
- spinal muscular atrophy
- static encephalopathies:
- severe cerebral palsy
- Renal conditions, including:
- bilateral renal agenesis
- renal disease, end stage
- Respiratory conditions, including:
- cystic fibrosis:
- timing of consideration for lung transplant
- cystic fibrosis:
Malignant conditions, including but not limited to
- Brain tumours, including:
- diffuse midline glioma
- glioblastoma multiforme
- Haematological malignancies, including:
- patients with haematological malignancy entering transplant with high disease or comorbidity burden, such as high-risk transplants
- relapsed / refractory leukemia / lymphoma, and high-risk leukemias
- second bone marrow transplant
- Relapsed resistant solid tumours with poor prognosis
For each presentation and condition, Advanced Trainees will know how to:
Synthesise
- recognise the clinical presentation
- identify relevant epidemiology, prevalence, pathophysiology, and clinical science
- take a comprehensive clinical history
- conduct an appropriate examination
- establish a differential diagnosis
- plan and arrange appropriate investigations
- consider the impact of illness and disease on patients and their quality of life when developing a management plan
Manage
- provide evidence-based management
- prescribe therapies tailored to patients’ needs and conditions
- recognise potential complications of disease and its management, and initiate preventative strategies
- involve multidisciplinary teams
Consider other factors
- identify individual and social factors and the impact of these on diagnosis and management
For the above conditions
- Disease- and patient-specific factors in prescribing potential therapies:
- organ dysfunction
- susceptibility to medication side effects
- Epidemiology
- Expected trajectory / trajectories of conditions
- Intent of potential therapies, including:
- attempt at cure
- life prolongation
- symptom management
- Pathophysiology
Clinical assessment tools
- Symptom assessment scales for verbal and nonverbal patients, including:
- face, legs, activity, cry, and consolability (FLACC) scale
- numerical rating
- Wong–Baker faces
- Tailored to pathophysiology, prognosis and goals of care
Investigations
- Blood tests
- Imaging
- Tailored to pathophysiology, prognosis, and goals of care
Disease- or syndrome-specific procedures (understanding of the indications, benefits, and burdens of disease-related procedures, including the following)
- Cardiac:
- disease
- surgery for congenital cardiac disease
- ventricular assist device insertion
- Gastrointestinal:
- nasogastric insertion
- percutaneous gastrostomy
- Neurological:
- baclofen pump insertion
- cerebrospinal fluid diversion
- palliative radiotherapy
- Orthopaedic
- Renal:
- renal replacement therapy
- Respiratory:
- non-invasive ventilation
- oxygen therapy
- tracheostomy
- video-assisted thoracic surgery (VATS) and pleurodesis in the setting of recurrent malignant pleural effusions
- Surgical interventions:
- fracture fixation in the setting of osteopenia of disability
- scoliosis surgery in the setting of severe neurodisability
Clinical considerations
- Anticipatory prescribing
- Complex family discussions, encompassing priorities and goals of care of patients, their families, whānau, and/or carers
- Investigations and monitoring in the context of illness stage and goals of care
- Options for place of care
- Patients’ and their families’, whānau, and/or carers’ preferences for communication and degree of:
- information shared
- involvement in decision making
- Uncertainty in prognosis and trajectory
- Understanding and having treatment strategies for the psychosocial effects and psychosocial drivers of symptoms
Ethical and legal issues
- Advance care planning
- Capacity assessment in the young person
- Disagreement within families about treatment decisions
- Disagreement between families and clinicians about treatment decisions
- Nutrition and hydration in the context of illness stage and goals of care
- Withdrawal and/or withholding of life-sustaining treatment
Procedures
- The understanding and ability to communicate the relative risks and burdens to children and families
- Informed consent
Self-care in palliative care
- Personal impact of dealing with incurable illness, death, and dying
- Physician burnout