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Chronic illness in paediatrics

Clinical Discipline(s)/Organ System(s)
Paediatrics, Nervous System, Neurology, Orthopaedics, Respiratory Medicine, Musculoskeletal System, Respiratory System
Progress Test Topic(s)
Child health
Description
A 12 year old Samoan girl presents to Emergency Department with her mother after a fall from her wheelchair during a seizure. They have a recent outpatient clinic letter which outlines her past medical history to include spastic quadriplegic cerebral palsy from birth; she is wheelchair-bound and fully dependent for all self-cares. She has epilepsy and global developmental delay and is nonverbal. She has had a gastrostomy for night feeding since 4 years of age and has orthopaedic problems including scoliosis and tendoachilles contractures. She has bronchiectasis with two previous episodes of severe pneumonia, the last of which 6 months ago required a prolonged admission and she now remains on nocturnal VPAP (variable positive airway pressure). She attends the local intermediate school via satellite class and has multiple community services involved in her schooling, transport and therapy.
Progress Test-Type Questions:   Question 1 | Question 2
Applied Science for Medicine 
   - Anatomy of the thoracic cavity
   - Pathophysiology of cerebral palsy
   - Pathophysiology of bronchiectasis; aspiration, gastro-oesophageal reflux, recurrent infections
   - Developmental and DSM-IV criteria for intellectual disability
   - Pharmacology of antispasmodics including botulinum toxin, anticonvulsants (valproate, phenytoin, carbamazepine, benzodiazepines, lamotrigine)
Clinical and Communication Skills 
   - Elicit a history from parent or carer of a child with chronic illness and intellectual disability with no verbal communication including assessing for physical signs of chronicity (clubbing, chest deformity, contractures, dentition, nutritional status)
   - History and physical examination of functional status and development
   - Interpret relevant investigations: anticonvulsant levels, imaging (in context of deformity and osteopenia)
   - Management of complications such as epilepsy, muscle spasms, orthopaedic, nutritional status, respiratory and dental
   - Prognosis of severe cerebral palsy with significant lung disease and mobility issues
   - Indications for medical interventions such as noninvasive ventilation, gastrostomy, orthopaedic surgery
   - Role of early intervention services and community agencies such as child development team, home care nurses, IHC New Zealand, Taikura trust
Personal and Professional Skills 
   - Communicate, in an empathetic manner, with patient and their family/whānua
   - Best interests principle
   - Social and financial impact of chronic illness and disability in child for family/whānau
   - Transition of childhood chronic illness to adult services and palliative care
Hauora Māori 
   - Ensure a culturally safe environment and interpret symptoms in an appropriate cultural context
   - Understand inequities in access to social and health services care for different ethnic groups
Population Health 
   - Epidemiology of intellectual disability
   - Availability of health and educational services in New Zealand for people with disabilities
Conditions to be considered relating to this scenario
Common
epilepsy, intercurrent infection (upper respiratory tract infection, lower respiratory tract infection, urinary tract infection), progressive respiratory failure, pain from contractures or undiagnosed fractures and other orthopaedic causes (e.g. back pain from scoliosis, hip subluxation), obesity leading to secondary problems with mobility/pressure areas
Less common but 'important not to miss'
non-accidental injury and neglect, co-morbid mental health problems (e.g. depression)
Uncommon
neuropathic and regional pain syndromes
Related Scenarios
[Labour], [Chronic sputum production]