Causes and management of neurological & neuromuscular symptoms
Children with severe neurological impairment (SNI) are likely to have metabolic, genetic, congenital or traumatic conditions that require support for the most basic personal care needs. Paediatric palliative care is well suited to providing support for families of children with SNI because of the uncertainty and life-limiting nature of the condition, the focus on symptom management and quality of life.
Conditions affecting the nervous system
Trisomy 13 and trisomy 18 are genetic disorders that present with a combination of birth defects. This includes severe learning problems and health problems that affect nearly every organ in the body. Most babies born with trisomy 13 or 18 die by age 1 but some babies with these disorders do survive the first year of life.
Inborn errors of metabolism
Inborn errors of metabolism are a large group of rare genetic diseases that generally result from a defect in an enzyme or transport protein which results in a block in a metabolic pathway. The central nervous system is often affected, leading to neurological disease.
Most commonly seen neurological conditions in paediatric palliative care include Duchenne muscular dystrophy (DMD) and spinal muscular atrophies (SMAs). New disease directed therapies have led to an increase in survival of children with these diseases.
There are multiple causes of acquired brain injury in children which include trauma (such as from a car accident), hypoxia/ischemia, CNS infections, metabolic disorders, tumours and vascular abnormalities.
General principles of a palliative approach to neurological and neuromuscular conditions
Attending to grief and meaning making throughout treatment and not just at the end of life.
Prepare the child and families for the future by anticipating care needs and making proactive decisions around these needs.
Try to ensure all team members provide the same medical information and recommendations on medical decisions to the child and family, being mindful of the language used.
Approach symptom management using both non-pharmacological and pharmacological strategies.
This video “Generalized Seizures in Children” is part of the Lecturio course “Pediatrics”