Focus Topics

topic 3 | Activity 1: Impact of life-limiting illness

Life-limiting illness in childhood

The prevalence of children and young people (aged 0 to 21 years) with a life-limiting illness in Australia is increasing. It is estimated that by the year 2033, approximately 44,500 children and young people aged 0-21 years may benefit from paediatric palliative care in Australia.[1]

Some key statistics are important to consider:[1]

  • The prevalence of conditions is greatest for children less than one year of age, with a large proportion represented by the neonatal population (28 per 10,000 population, compared with less than 5 per 10,000 population for 1-21 year olds).
  • The number of children and young people with a non-oncological condition was far greater than those with an oncological condition.
  • The prevalence of life-limiting illness for children and young people who identified as being Aboriginal and/or Torres Strait Islander was greater (78.6 per 10,000 population) than for non-Indigenous Australians (53.4 per 10,000 population).

Although a small but significant number of children and young people die, it is important that they receive meticulous end of life care. It seems beneficial that ‘early referrals’ are made and that there is time to develop relationships and care plans before end of life care is required.[1]

Life-limiting illness in children can include a varied and diverse range of illnesses and health disorders, including genetic or congenital disorders, cancers, and various organ system disorders. [2]

These illnesses can be divided into four broad groups that are associated with differing trajectories: [3]

Group Examples
1. Life-threatening conditions for which curative treatment may be feasible but may not be successful Children with cancer when curative treatment is not successful (eg, progressive brain or solid tumours)

Irreversible organ failure not amenable to transplantation or if transplantation is unsuccessful
2. Conditions where premature death is inevitable; however, there may be long periods of intensive treatment aimed at prolonging life and allowing participation in normal activities Examples of this category are evolving and changing with emerging therapies (including medical, surgical and genetic therapies)
3. Progressive conditions without curative treatment options, where treatment is exclusively palliative and commonly extends over many years Neuro-degenerative conditions Metabolic conditions (eg, mucopolysaccharidoses and Niemann-Pick Type C disease)
4. Irreversible but non-progressive conditions causing severe disability, leading to susceptibility of health complications and likelihood of premature death. Complications that may cause death include severe recurrent pneumonias or intractable seizures Severe cerebral palsy (disease of the nervous system)

Rett Syndrome (pervasive developmental disorder)

Multiple disabilities, such as following brain or spinal cord injury (eg, hypoxic ischaemic encephalopathy)

Illness trajectories for children with life-limiting illnesses are often uncertain, as healthcare advances are rapidly altering outcomes. This uncertainty can cause significant distress for children and their families. It can also defer appropriate early referral to a palliative care service. [4]

Many sub-specialties of paediatrics may need to refer to palliative care (eg, oncology, neurology, metabolic, cardiac, gastrointestinal, respiratory, dermatology and renal). It can be difficult for the treating team to know when to refer to specialist palliative care. The surprise question, ‘Would you be surprised if this patient died within the next 12  months?’, can be a helpful indicator of when palliative care would be appropriate. If the answer is ‘no’ then, in consultation with the family, a referral should be made.[5]

Impact of life-limiting illnesses

Life-limiting illness impacts physically, emotionally, spiritually, socially and culturally on children and their families. The impact of life-limiting illness often extends to friends, schools and communities. [2-4].

For example, some life-limiting illnesses are associated with symptoms such as pain. These symptoms can affect the child’s ability to attend school and socialise with other children, with parents and carers becoming children’s main social contacts. [2, 6] Isolation at different stages of a child’s life can affect their physical, psychological, cognitive and social development.[12] Older children who have absences from school often report anxiety about their academic performance.

Children can also be affected by altered self-image, often causing them to strive for normality. [13]

A child’s experience of living with a life-limiting illness is influenced by their age and developmental stage. It is important to assess each child’s understanding of their illness, as a range of factors including personal experience with illness can influence their experience. [7]

The following table summarises common features of the child’s concept of death at different ages, although it is important to consider this as a guide as considerable variation can exist. [7-11]

Table 1. Children’s age related understanding of dying and death
Age 0 – 2 years:

  • Sees death as separation or abandonment
  • Has no cognitive understanding of death
  • Feels despair from disruption of caretaking.
Age 2 – 6 years:

  • Often believes that death is reversible, temporary
  • May perceive death as a punishment
  • Engages in magical thinking that wishes come true – may feel guilt for negative feelings toward the person who died, and think that was the cause of death.
Age 6 – 11 years:

  • Shows gradual understanding of irreversibility and finality of death
  • Demonstrates concrete reasoning with ability to comprehend cause and effect relationship.
Age 11 years or older:

  • Understands that death is irreversible, universal, and inevitable
  • Has abstract and philosophical thinking.

 

Children with life-limiting illness can experience needs across many domains of health, requiring a comprehensive assessment approach, using language and techniques appropriate to their development stage. Key areas for assessment include:[2, 6]

Physical concerns
  • Identify pain or other symptoms, using strategies that are age or developmental stage appropriate.
Psychosocial concerns
  • Identify the child’s and family’s fears and concerns
  • Identify coping and communication styles
  • Discuss previous experiences with death, dying or other traumatic life events
  • Assess resources for bereavement support.
Spiritual and cultural concerns
  • Discuss spiritual and cultural needs.
Planning for end-of-life care
  • Identify decision-makers
  • Discuss anticipated illness trajectory
  • Identify goals of care
  • Consider concerns near end of life
  • Consider acute resuscitation plan if appropriate.
Practical concerns
  • Plan communication and coordination of healthcare team
  • Identify child and family preferences for location of care
  • Discuss child’s home or school environment
  • Assess child’s current and future functional status
  • Identify possible financial consideration on family.
  1. Consider the following conditions and for each, describe possible illness trajectories:
    • Spinal Muscular Atrophy type 1
    • Infantile Battens disease.
  2. In what ways does a child’s developmental stage influence their understanding of dying and death?
  1. Bowers, A et al, 2023. Paediatric Palliative Care Need in Australia.
  2. Paediatric Palliative Care Australia and New Zealand. 2023. A Practical Guide to Palliative Care in Paediatrics.
  3. Together for Short Lives. 2024. Together for Short Lives: Categories of life-limiting conditions
  4. Haines, ER et al, 2018. Barriers to accessing palliative care for pediatric patients with cancer: A review of the literature. Cancer 124(11): 2278-88.
  5. White, N, et al. 2017. How accurate is the ‘Surprise Question at identifying patients at the end of life? A systematic review and meta-analysis. BMC Medicine 15(139).
  6. Therapeutic Guidelines. 2023. Principles of Paediatric Palliative Care. Melbourne.
  7. Howe, C, 2009. Peer Groups and Children’s Development : Psychological and Educational Perspectives. Wiley-Blackwell: Chichester.
  8. Cancer Council Australia. 2023. Talking to kids about cancer: A guide for people with cancer, their families and friends.
  9. Kopchak Sheehan, D, et al. 2014. Telling adolescents a parent is dying.Journal of palliative medicine. 17(5): p. 512-520.
  10. CareSearch. 2021. Talking with children.
  11. Meier, DE. 2018. The Human Connection of Palliative Care: Ten Steps for What To Say and Do.