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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.

Caring for a child with a life-limiting illness is associated with immense grief, shock, fear, suffering, helplessness and denial. It is one of the most difficult situations families/carers may experience.[1-2]

This video, Demystifying paediatric palliative care from Paediatric Palliative Care National Action Plan Project provides some insight into this experience.

How people respond to the news of a diagnosis or recognition of an illness or condition will be unique to them and healthcare professionals need to be able to support them through this time. Parents may feel a sense of failure at not being able to protect their child from illness, suffering and reduced life-expectancy. When caring for a child who is debilitated for a long period, parents may experience chronic sorrow and an increasing burden of care. Even when they have a thorough understanding of their child’s illness and prognosis, and have been preparing for the death of their child for a long time, they can still feel numbness, shock and disbelief when the child dies. [1]

Parents can experience a wide range of concerns including:[1-2]

• Fear, anxiety and stress associated with observing their child’s distress and the loss of their child
• Financial burden associated with medical management, equipment and transport costs, as well as absences from employment
• A decline in parental physical health due to stress and increased workload associated with medical management responsibilities, relationships and lack of time for themselves.

Siblings of children with life-limiting illnesses can also be affected. The sibling’s experiences can include: [3-5]

• Increased responsibilities, including trying to take on the role of their sibling
• Stress from upheaval of family routine and dynamics
• Feelings of anxiety through concern for sibling who is unwell as well as separation from parents
• Feelings of guilt about thoughts they may have towards their sibling
• Feelings of embarrassment about being seen in public with their unwell or disabled sibling.

Grief and bereavement

The death of a child is one of the most stressful situations a family can face. For parents, feelings of grief and loss can start well before the child has died. [1-3] Responses to the death of a child will vary. Some parents and siblings can experience grief that continues long after the death of a child. For many, this is a normal response to a significant loss and the continuing bonds that parents have with their child even after the child’s death.[1-3] Other individuals can experience a more complicated grief. For some, this complicated grief will require additional professional supports.[5, 6]

Grief and loss is often experienced also by grandparents, friends, community and health professionals involved in the child’s care. Grandparents can experience grief both from the loss of their grandchild and from seeing their own child experience loss.[5]

Paediatric palliative care is about improving the quality of life of infants, children and young people diagnosed with life-limiting illness and supporting those who care for them. Being cared for by palliative care does not mean that a child is dying, in fact many children with life-limiting illness live into adulthood, but palliative care helps them to live as well as possible.

Quality care in this context enables the child to live in an environment where curative treatment can be a part of their life, but not their entire focus. Palliative care aims to provide the best quality of life through a holistic approach which supports the physical, emotional, social, cultural and spiritual aspects of the child and their family.[1]

Palliative care for children and young people with life-limiting or life-threatening conditions is an active and total approach to care, from the point of diagnosis or recognition throughout the child’s life and death. It embraces physical, emotional, social, and spiritual elements, and focuses on enhancement of quality of life for the child/young person and support for the family. It includes the management of distressing symptoms, provision of short breaks and care through death and bereavement. [2]

The World Health Organisation’s definition for paediatric palliative care includes the principles:[3]

• Palliative care for children is the active total care of the child’s body, mind and spirit, and also involves giving support to the family
• It begins when illness is diagnosed, and continues regardless of whether or not a child receives treatment directed at the disease
• Health providers must evaluate and alleviate a child’s physical, psychological, and social distress
• Effective palliative care requires a broad multidisciplinary approach that includes the family and makes use of available community resources; it can be successfully implemented even if resources are limited
• It can be provided in tertiary care facilities, in community health centres and in children’s homes.

There are unique characteristics of paediatric palliative care that differentiate it from adult palliative care, including: [4-5]

• Care is child and family-centred
• The family has an increased role in decision-making
• The child usually remains under the care of their primary treating team
• Perinatal palliative care can be provided alongside antenatal care
• Care is provided across a wide range of care settings, and children can have disabilities that have a compounding effect on palliative care needs, as well as healthcare coordination and communication
• Predicting a prognosis can be difficult
• The number of children dying is smaller so health professionals may be less familiar with providing palliative care
• Each child is learning, growing and developing along a continuum toward adulthood which requires consideration of social, emotional, spiritual and cultural needs
• A child’s ability to communicate and understand varies
• The provision of education and play is essential
• When parents withdraw partially or completely from the workforce to become caregivers, this can have life-changing financial effects for the family
• Siblings are vulnerable and require individualised support
• Grandparents are at risk of ‘doubled worry’: worry about the wellbeing of the child with the life-limiting illness, and worry about their own child (the parent of the ill child)
• Conditions are sometimes familial: other children in the family may be affected by, or have died from the same illness
• There can be numerous, evolving losses experienced throughout the illness trajectory requiring grief and bereavement support prior to death
• The death of a child can lead to an experience of prolonged or lifelong grieving for the child’s parents.

Children and families affected by life-limiting illness have many aspects of life that are beyond their control. However, the care that the child and family receives is something that can be controlled. It is important for the healthcare team to promote an empowering culture in conversations about care.

An empowering culture is one in which the family is enabled to have maximum control over the resources, information, decision-making and relationships that affect them. An empowering culture seeks to understand the uniqueness of each family within their social, spiritual and cultural context and demonstrates curiosity and humility in learning about the values and beliefs that are important to the family.

Such a culture respects the reality that each family has their own strengths, resources and information and is capable of making decisions and maintaining and contributing to relationships (including their relationships with care providers). Within this culture of empowerment, families are trusted to manage their own lives. They are not perceived as passive recipients of professional services but as competent and leading partners in their child’s palliative care.[1]

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Communication with children

Like adults, children often have questions about their illness, care and dying. It is important that responses to questions are open, honest and age appropriate. Age appropriate communication with children and adolescents assists with acceptance of their condition, management of symptoms and preparation for dying. [1] Involving children as much as possible in discussions about their own care supports collaborative decision-making. Involving adolescents in their own care planning creates trust with health professionals.[2]

Age, individual developmental stages and possible neurological impairments need to be considered when communicating with children. Communication should be directed at the child though, depending on age, communication about care could also be through parents. Consider all family members who need to be involved when discussing aspects of the child’s care. [3]

Strategies when talking to children include:

• Respect the parents’ views and expertise on how best to communicate with their child
• Use words and other communication methods that are understandable to the child. Check for understanding regularly and clarify as needed
• Use age-appropriate methods of communication such as drawings, books, action-based toys
• Validate the child’s questions (eg, That is an important question to ask)
• Be honest and maintain trust, be clear with responses to questions, especially if you do not know the answer to their question (eg, I’m not sure about that. Can I think about it?)
• Take time to be together and communicate, not just about their illness
• Be curious about what the child says and questions they ask (eg, Why do you want to know about…?)
• Allow children to have a break away from family and carers
• Be prepared to listen to and revisit conversations about care. [4-7]

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Communication with family

Building rapport with a family in the early stages of care is crucial for effective ongoing support; part of this process is to understand and acknowledge the unique needs of each family. Parents  have a primary role in decision-making, including decisions regarding the extent to which their child is involved in conversations about care planning. When talking with children with a life-limiting illness and their parents together, it can be difficult to assess each person’s level of understanding, as neither may want to openly acknowledge how sick the child may be. [7]

Specific considerations when talking to families around the time of death of a child include:

• Plan the conversations in advance, taking into account timing and environment
• Assess prior knowledge and what is needed to be discussed
• Be clear, concise and empathetic when delivering information about the child’s condition
• Allow silence and acknowledge emotions
• Allow time for the family to raise concerns and questions
• Family members can have strong emotional reactions; it is important to acknowledge and validate these
• Plan for future meetings or discussions to allow families time to go away and consider the conversation. [7-8]

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Supporting siblings

Most siblings of children with life-limiting illness experience distress, but many do not share their thoughts and feelings. This may be because their parents are distracted or physically unavailable, or because they do not want to add to their parents’ difficulties. Siblings who are unable to express themselves adequately can develop behavioural problems such as attention seeking, school failure, developmental regression or physical symptoms resembling those of the sick child.[7]

The level of understanding of dying and death for children with a life-limiting illness may be different to other children their age. This understanding develops from inclusion in conversations about their illness and management, and their experience of seeing other children they know die. Age appropriate conversations around dying and death are required for siblings and friends of the child with a life-limiting illness. [7-8]

Siblings can benefit from:

• Being included in family conversations
• Being provided with education about their sibling’s condition
• Having time allocated to discuss concerns with health professionals
• Connecting with support groups or other children who are dealing with similar circumstances
• Being shown personal interest in them as individuals
• Being encouraged to express emotions such as sadness and anger
• Having these feelings and emotions normalised
• Being allowed to spend time with their sibling who is ill
• Receiving reassurance about their own health.

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Age appropriate communication

It is recommended that you employ age-appropriate communication with children. The following table outlines age appropriate communication techniques for children in different age ranges:[3, 5, 9-12]

Table 2. Age appropriate communication techniques

Children up to 6 years	Younger children are egocentric Reinforce that they are not being blamed for the illness Reassure them that they will always be safe and cared for Explore their understanding of the illness and/or death Dispel guilt by correcting misconceptions and reassuring the child that nothing they did caused the illness or death, including their behaviour (eg, don’t tell them to ‘be good’ so that they can be well).
Children between 6-12 years	Children in this age group can be concerned that they won’t be accepted by their friends and peers and for some, being different for any reason can be distressing. Children in this age group need: Relevant information aimed specifically to their level of understanding To maintain relationships with their peers To continue to participate in affirming activities including sport, clubs or social activities Ongoing opportunities to talk about comments from their peers and how these comments make them feel. Provide a simple explanation of the diagnosis and treatment options Dispel misconceptions regarding causes of illness or death, as well as contagion Help put guilt and other concerns in perspective by thinking together about the entire relationship rather than only the recent past.
Children 12 years and up	Maintain social relationships Access specific and relevant information about dying and death Facilitate opportunities to talk openly about the illness with their parents Provide information about the illness and treatment options Dispel misconceptions regarding causes of illness or death, as well as contagion Provide clear and accurate information about causes of death. Remember that adolescents may seek information from other sources, such as the internet. Encourage them to check the accuracy of this information with their parents.