1 The principles and practice of palliative care for children

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Contents

• Objectives
• Introduction to palliative care for children
• Categories of childhood illnesses needing palliative care
• Places where palliative care can be provided
• Levels of care
• Case studies

Objectives

When you have completed this chapter you should be able to:

1. Describe the meaning of palliative care.
2. Define life-limiting and life-threatening conditions.
3. List the goals of palliative care.
4. Define children’s palliative care.
5. Explain why palliative care for children differs from palliative care for adults.
6. Explain why children have a right to palliative care.
7. Describe the continuum of care and illness trajectory.
8. Categorise childhood diseases and conditions that need palliative care.
9. Define the different levels of palliative care.

Introduction to palliative care for children

1-1 What is palliative care?

The World Health Organization (WHO) defines palliative care as ‘an approach that improves the quality of life of patients and their families facing the problems associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial and spiritual.’

Palliative care is holistic care as it is a comprehensive approach to caring for the whole person and their families. This is very different from just treating the person’s illness. The palliative care approach should be applied to all levels of care and not just tertiary care.

Palliative care is an approach to care that improves the quality of life of patients and their families facing a life-limiting or life-threatening condition.

Note

The word palliate comes from the Latin “pallium” which means a cloak or cape. Patients receiving palliative care are surrounded, protected and guarded by a cloak of care, comfort and support. (Term coined by Dr Balfour Mount, Canada 1975).

1-2 What are life-limiting and life-threatening conditions?

These are serious illnesses or conditions that may either shorten the person’s life span or pose a threat to a person’s life.

• Life-limiting conditions: Conditions that shorten a person’s life. Examples are advanced cancer and severe congenital heart disease. Many life-limiting conditions are not immediately life-threatening as the person may still live for months or years. However, they will probably die of the condition eventually after long periods of relative wellness.
• Life-threatening conditions: Conditions where the person has a serious illness that could cause death, but survival is possible with appropriate treatment. Examples are multi-drug resistant tuberculosis or cancer that is expected to respond to treatment.

A life-limiting condition is one which may not be immediately life-threatening but will shorten the person’s life span as there is no hope for a cure. Some life-limiting illnesses can be medically controlled for long periods of time, even if not cured.

1-3 What are the goals of palliative care?

The many goals of palliative care include:

• To thoroughly assess the physical, social, emotional and spiritual needs of the patient and family
• To address the individual needs of the patient in a caring and compassionate way
• To provide relief from pain and other distressing symptoms
• To offer a support system to help the patient live as positively and actively as possible until their death
• To maximise quality of life
• To use a team approach to provide the best care possible
• To not hasten nor postpone death
• To offer a support system to the family both before, during and after the patient’s death
• To begin care at diagnosis of a life-threatening or life-limiting condition and continue through to bereavement
• To be carried out together with other forms of medical treatment such as chemotherapy or antiretrovirals.

The main goal of palliative care is to improve the quality of life of the patient with a life-limiting or life-threatening condition.

1-4 Who is regarded as a child?

There is no general agreement as to when a child becomes an adult as service providers vary in their age definition of a child, an adolescent and an adult:

• The World Health Organization (WHO) and the South African Constitution define childhood as being from birth to 18 years.
• In many health facilities in South Africa children’s wards will only admit patients up to twelve years of age. Over the age of twelve some are transferred to an adult ward. Very few hospitals have an adolescent ward for older children.

In practise, however, it often depends on the individual child’s age, maturity, stage of development and illness as well as the wishes of the parents and the guidelines of the local health facility.

1-5 What is children’s palliative care?

The World Health Organization (WHO) defines palliative care for children as follows:

Palliative care for children represents a special, closely related field to adult palliative care. 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 even in children’s [own] homes.

Palliative care improves the quality of life of patients and families who face life-threatening illness, by providing pain and symptom relief, spiritual and psychosocial support from diagnosis to the end-of-life and bereavement.

(World Health Organization, 2002)

Children are not simply little adults. They have special needs, think and behave differently to adults and require care that closely involves their family. Therefore, it is important to recognise the differences between adult and children’s palliative care.

See Appendix A for the charter of the International Children’s Palliative Care Network (ICPCN). The ICPCN’s mission is to achieve the best quality of life care for children and young people with life-limiting or life-threatening conditions, their families and carers.

Children’s palliative care is palliative care that addresses the specific needs of children, which often is different from that of adults.

Note

The ‘children’s home’ in the definition refers to the child’s own home.

1-6 What are the differences between palliative care for adults and palliative care for children?

Although adult palliative care services are available across South Africa, these services do not always meet the needs of children, as their care needs are often quite different to those of adults. Some of these differences include the following:

• Most adults are independent while almost all children are still dependent on their parents or other adult guardians (caregivers).
• Communication with children tends to be more difficult due to age differences and language abilities.
• Children’s understanding of illness, death and dying are not the same as those of adults, so different approaches need to be adopted.
• Children often fear being separated from their family, friends, school and home.
• The medicines used, their doses and side effects are often different in children.
• Symptoms can be difficult to assess in non-verbal children.
• Children under twelve years of age are not able to give consent, however they can still give their opinion and need to be included in the decision making where possible.
• Health professionals often find providing palliative care to a child particularly difficult and emotionally challenging.
• The way parents and family experience bereavement and mourn the death of a child differs from that experienced when an adult dies.
• Children tend to have a broader range of people involved in their care and so teamwork and an understanding of team dynamics is especially important.

Children are not little adults; therefore their palliative care needs are unique and dependent on their age, size, language ability and understanding.

1-7 What are some of the incorrect beliefs about children’s palliative care?

Common incorrect beliefs are:

• Children do not need palliative care.
• Children’s palliative care is the same as adult palliative care.
• Only children with cancer and HIV need palliative care.
• Palliative care is only needed at the end-of-life.
• Palliative care means giving up hope and stopping treatment.
• Palliative care can only be provided in tertiary facilities or a hospice.
• Palliative care aims at shortening life or hastening death to avoid unnecessary suffering.

1-8 Do children have a right to palliative care?

Yes. Children, have a right to basic healthcare, including palliative care.

Note

Palliative Care is a recognised part of universal health coverage (UHC). Universal health coverage (UHC) means that all people and communities can use the promotive, preventive, curative, rehabilitative and palliative health services they need, of sufficient quality to be effective, while also ensuring that the use of these services does not expose the user to financial hardship (World Health Organization).

In April 2017 the National Health Council approved the government’s policy on palliative care for all ages (The National Policy Framework and Strategy on Palliative Care) and by 2018 an implementation plan was proposed.

1-9 What does the South African Child Care Act of 2010 say about the rights of children?

Key sections of the act include:

• Decisions must be made in the best interests of the child
• Children should be given information about their health status and treatment
• If children are old and mature enough, they should be allowed to participate in discussions about their healthcare to enable them to make informed decisions
• Children should be treated with dignity and respect and be protected against discrimination
• Children’s rights need to be acknowledged.

A child may consent to their own medical treatment if they are twelve years of age or older and are of sufficient maturity and mental capacity to understand the benefits, risks, social and other implications of the management. (They must be assisted by a parent or guardian for a surgical procedure).

The continuum of care

1-10 What is a continuum of care?

It is the care and support that is provided throughout the course of a life-limiting or life-threatening condition until the child’s death. It will then continue to support the family into the bereavement period until they no longer need palliative care support. A continuum of care includes the transition of care from one place of care to another, for example, the continuation of care and support from home to hospital or another facility and then back home, if and where possible.

A continuum of care is care and support that is provided throughout the course of the illness.

1-11 Can curative and palliative care be provided at the same time?

Yes. In the past, palliative care was only started when curative care was no longer effective. There was, therefore, an abrupt change from curative to palliative care.

Today this transition is more gradual and palliative care should be started when curative care is still being offered to a child with a life-threatening or life-limiting illness. A palliative care approach should be offered from diagnosis and alongside curative care.

Figure 1-1: The transition of curative care to palliative care.

Palliative care can be offered alongside curative care from the time of diagnosis of a life-limiting or life-threatening illness.

1-12 What is an illness trajectory?

This is the course and pattern that an illness or condition is expected or likely to take. Some illnesses get worse quickly and the child does not live long while other illnesses slowly get worse and the child may live for many months or even years. The illness trajectories of childhood illnesses often differ from that of adults. Each condition has its own trajectory.

An illness trajectory is the course that an illness is expected to take.

Note

Each child’s illness will follow its own unique path and not the exact path of the expected trajectory.

1-13 How can an illness trajectory help in managing the patient?

The illness trajectory can be very helpful because it gives a broad time frame for the expected course of the condition which helps:

• Plan the goals of management and changing needs along the course of the illness
• Guide clinical assessment and choice of treatment options
• Decide when to transition from more aggressive medical treatment to palliative care
• Educate the patient and family about the course the illness is expected to take
• Recognise when end-of-life is approaching.

Trajectories can assist in identifying the changing needs and goals over time.

1-14 What factors can influence the illness trajectory?

A number of factors can impact the illness trajectory:

• The natural history of the illness and what is expected to happen
• The patient’s general state of health and how advanced the illness is
• Any unexpected complication
• Treatment goals and decisions such as when to stop treatment
• The health services and resources available
• Other unrelated illnesses or conditions (e.g. malnutrition).

The health services and resources that are available can have an important impact on the illness trajectory.

Categories of childhood illnesses needing palliative care

1-15 What is the value of placing children’s illnesses into different categories?

Classification of illnesses into different categories can help in identifying children who may benefit from a palliative care approach to their management. It will also assist when determining the expected trajectory of the condition or illness, developing care plans and deciding the appropriate level of care and treatment needed.

Categorisation of an illness can assist in identifying children’s palliative care needs, deciding on the level of care required and on how aggressive treatment will need to be.

1-16 What are the four categories where children’s palliative care may be needed?

The four categories are:

• Category 1: Life-threatening conditions where curative care may still be successful but may fail.
• Category 2: Life-limiting conditions where the child is expected to survive for a long time and receive prolonged medical treatment. The medical condi­tion can be controlled but not cured.
• Category 3: Progressive conditions that cannot be cured and palliative care may be needed over months or years.
• Category 4: Conditions which are not progressive but cannot be cured.

Note

Categories 1 to 4 were proposed by the “ACT” palliative care charity, now known as Together for Short Lives, in the United Kingdom.

1-17 Which conditions fall into category 1?

Examples of life-threatening conditions where curative care may still be successful, and the child may survive, include:

• Children with cancer that is expected to respond to treatment
• Children with tuberculosis, especially drug-resistant TB.

Both curative and palliative care can be given side-by-side. However, if it becomes obvious that curative treatment is failing, the main management can be transitioned to palliative care.

Figure 1-2: Category 1. A life-threatening condition which goes into remission or is cured with treatment.

Category 1 conditions are life-threatening where curative treatment is still possible but may fail.

1-18 Which conditions fall into category 2?

Life-limiting conditions where the child is expected to survive for a long time and receive prolonged medical treatment but will probably eventually die of the condition. These include:

• Cystic fibrosis
• Complex congenital heart disease which requires palliative care surgery
• Inherited muscular dystrophy.

Again, both curative and palliative care can be given side-by-side until it becomes obvious that curative treatment is failing and the main management can be transitioned to palliative care.

Figure 1-3: Category 2. A life-limiting condition with prolonged medical treatment before death.

Category 2 is a life-limiting condition where an early death cannot be avoided.

1-19 Which conditions fall into category 3?

Progressive conditions that cannot be cured and palliative care may be needed over months or years. These include:

• Chromosomal disorders such as trisomy 13 and 18
• Inherited metabolic conditions
• Inherited neuromuscular conditions
• Advanced cancer
• Inoperable cardiac disease.

The role of medical treatment is very limited and the focus is on comfort care.

Figure 1-4: Category 3. A progressive deterioration of the condition.

Category 3 is where only palliative care can be given, as the condition cannot be cured and progressively deteriorates.

1-20 Which conditions fall into category 4?

Conditions which are not progressive but cannot be cured. For example:

• Severe cerebral palsy
• Multiple disabilities such as brain or spinal injury.

Again, the role of medical treatment is limited and the focus is on palliative care.

Figure 1-5: Category 4. A non-progressive but incurable condition.

Category 4 conditions are non-progressive but irreversible.

1-21 Can categories of illness needing palliative care change?

Yes. Categories may change in different countries or settings depending on resources. For example, a child in chronic renal failure will be placed in category 1 if a renal transplant is available, category 2 if only renal dialysis is available and category 3 if no lifesaving treatment is available.

Categorisation may also change if the child’s condition worsens, if treatment fails or severe complications develop.

1-22 What is perinatal palliative care?

Perinatal palliative care focuses on an approach that assists, guides and supports families in decision making and planning in the event of a diagnosis of a life-limiting condition during pregnancy, labour (a stillbirth), a premature delivery or an infant born at term with a poor prognosis. It allows families to spend quality time with their infant, bonding and creating positive memories. Grieving will start at the time of hearing the bad news of the health or prognosis of their fetus or infant. Therefore, perinatal palliative care includes bereavement support of parents, siblings and the extended family.

Perinatal palliative care focuses on assisting families in decision making and planning if a diagnosis of a life-limiting condition is made during pregnancy or soon after birth.

1-23 When is palliative care needed for newborn infants?

There are a number of reasons newborn infants may need palliative care:

• Major congenital abnormalities may be detected by routine ultrasound examination during pregnancy. Some parents may choose to terminate the pregnancy. Other infants may be born alive but are not likely to live more than a few days or weeks.
• Some very preterm infants may be too immature to survive and therefore not given intensive newborn care such as ventilation.
• Other infants may be born at term with a major physical abnormality, suffer a lack of oxygen during labour or have a severe condition which is life-limiting.
• Some infants may be delivered with problems due to immaturity, infection or major physical abnormalities but not have access to tertiary care and are therefore likely to die.

Even if an infant is not expected to live long and does not receive lifesaving management after birth, palliative care should still be provided. It is important that the parents feel their infant is receiving comfort care and is not suffering, even when a decision has been made not to give any intensive curative care.

1-24 Do we need to provide palliative care in a neonatal intensive care unit?

Yes. It is essential that palliative care be provided in neonatal intensive care units, especially if the infant has a poor prognosis and is not to be given extensive curative care. No infant should ever be abandoned without care.

1-25 What is adolescent palliative care?

Adolescence is the period when children grow, develop and mature into adults. The definition of adolescence is widely debated both locally and internationally. The World Health Organization (WHO) defines an adolescent as anyone between the ages of ten and nineteen years, while in South Africa children over the age of twelve are usually admitted to adult wards. Therefore, these adolescents will need to be transferred to adult palliative care services, except if they are actively dying. Good communication is needed between children’s and adult palliative care services so that the transfer is well planned.

Places where palliative care can be provided

1-26 What number of children are in need of palliative care in South Africa?

In 2013 an international (UNICEF/ICPCN) study estimated that 801 155 children needed generalist palliative care and 304 441 needed specialist palliative care in South Africa.

The need for childhood palliative care in South Africa is great.

1-27 Where can palliative care be provided?

The settings where a child may need palliative care and support at a given time may vary and can include the child’s home, community centres, clinics, children’s residential homes, hospitals and in-patient hospices.

1-28 What is a hospice?

A hospice is an organisation that provides palliative care to persons living with life-limiting or life-threatening illnesses. This care can be in the patient’s home, community centres, in-patient units in a hospice facility (if available) or they may offer consultative palliative care services to hospitals. Hospices are usually non-profit organisations and care is provided by a multidisciplinary team of palliative care specialists. There is a great need for more hospice-based children’s palliative care in South Africa.

A hospice is an organisation that provides palliative care to persons living with a life-limiting or life-threatening illness.

Note

Umduduzi, Hospice Care for Children in Durban, Paedspal in Cape Town, Sunflower Children’s Hospice in Bloemfontein and Lambano in Johannesburg are examples of organisations that offer specialist children’s palliative care services in South Africa.

Levels of care

The needs of children receiving palliative care differ and are likely to change throughout the trajectory of their illness, therefore the levels of care and support provided will also change.

1-29 What are the levels of care?

Children’s palliative care can be successfully implemented at all levels of health services even if resources are limited. However, the palliative care needs of each child should be matched to the level of care required. Healthcare services are divided into three levels:

1. Primary care: Primary care is provided at district hospitals (level 1 hospitals) and local clinics by nurses or medical officers. Primary care can also be provided in the community by a home-based care team.
2. Secondary care: Secondary care is provided at regional hospitals (level 2 hospitals) by general specialists such as paediatricians. Staff providing secondary care to these patients with more complex clinical problems should have a higher level of palliative care training.
3. Tertiary care: Tertiary care is provided at large central and teaching hospitals (level 3 hospitals) by sub-specialists such as paediatric oncologists or specialist palliative care teams within the hospital.

The level of palliative care needed by an individual patient should be matched to the level of the healthcare service.

1-30 What services are available at the different levels of care?

The services available will range from a palliative care approach which can be provided at home by home-based care services or at a community and clinic level, to a generalist palliative care service provided at district hospitals and to a specialist palliative care service provided at regional and tertiary hospitals or by specialist palliative care providers for example, a children’s hospice.

1-31 Do the levels of palliative care required change over time?

Yes. The principles of palliative care (the palliative care approach) should be available at all levels of care. Children with less severe illness and only mild symptoms can be cared for at a community or district clinic. However, as their pain and symptoms worsen, they may need to be transferred to a level 2 or 3 hospital. The transfer of a patient between different levels of care is called the referral pathway. It is important to continually assess the patient’s needs so that these can be met at an appropriate level of care.

The level of palliative care needed may change over time and requires transferring the child along the referral pathway to a higher or lower level of care.

See chapter 4 for more details on referral pathways and levels of care.

Case study 1

A 13-year-old boy has been diagnosed with acute myeloid leukaemia which is a serious condition. He is currently in grade 8, an active member in his Sunday school group, and excited to have started playing cricket at school. He lives with his mother, father, and 15-year-old brother in a small town outside of Durban. He has been admitted to the male medical ward for his second chemotherapy treatment and is complaining of severe abdominal pain and nausea and says he is scared of the men in the ward. The doctor has suggested referring him to the palliative care team but his parents say that he is not dying and they are not giving up hope that he will be cured.

1. Do you think he requires palliative care?

Yes, he has been diagnosed with a life-threatening illness. Children’s palliative care is concerned with the treatment of children with life-limiting or life-threatening conditions by maintaining quality of life, not just in the dying stages, but also in the weeks, months and years before death. Palliative care can be offered alongside curative treatment.

2. What would be the goals of care for this boy?

The goals would be:

• To treat his pain and nausea
• To use a team approach to provide the best possible physical and psychosocial care and support to him and his family
• To maximize his quality of life
• To offer a support system to allow him to live as actively as possible.

3. Why has this boy been admitted into the adult male ward and not the paediatric ward?

In the South African healthcare system, many children’s wards will only admit children up to the age of twelve years, even though our constitution defines childhood from birth to eighteen years.

4. What are two incorrect beliefs about palliative care you can identify in this scenario?

Two incorrect beliefs are:

• Palliative care can only be provided at the end-of-life once curative treatment has been stopped
• Palliative care means giving up hope.

5. How would you respond to the parents’ concerns?

Explain to them that palliative care can be given alongside curative treatment throughout the course of his illness, and that providing palliative care does not mean that he is dying or that there is no more hope for him. The goals of palliative care are to improve quality of life and relieve pain and suffering using a team approach.

Case study 2

A 7-month-old girl has been admitted for onset of seizures and failure to thrive. On examination the girl has decreased tone of her arms and legs and is unable to sit or hold her head up. A series of tests are performed and after several weeks the results are returned and she is diagnosed with Tay-Sachs disease (a severe metabolic disorder) which is not curable and is progressive with a short life expectancy.

1. Would you consider referring her for palliative care?

Yes. Palliative care can be provided from the time of diagnosis of any life-limiting condition. Palliative care is provided along the continuum of care whether she requires active interventions or not and will continue to support her family into the bereavement period once she has died.

2. Into what palliative care category would you classify her condition and why?

Her condition is progressive and not curable with a short life expectancy, therefore her condition can be classified as category 3.

3. How can knowledge of her illness trajectory assist in her care?

This knowledge can assist in educating the family about the course the illness is likely to follow and help identify the changing needs. Understanding the course her illness is likely to take will assist with the planning of goals of care and decision making around to what extent the family will pursue aggressive active treatment, if any, and assist them in recognising the course of her condition that may indicate that at some point she is at the end-of-life.

4. What level of palliative care will be required and why?

She will require palliative care by a team of children’s palliative care specialists as her condition is complex and she is likely to experience pain and other symptoms as her illness progresses. This team will be able to provide her with holistic care that will help relieve or reduce any suffering she may experience and help support her family throughout her illness trajectory and into the bereavement period.

5. In what setting can palliative care be provided to this child?

The settings may vary depending on the need and the progression of her illness. Her care will most likely be at home with visits from the home-based care team. She may need intermittent admissions to hospital if she is needing further care, for example, pain and symptom management. If the local children’s hospice has an in-patient unit the child could be cared for there and at the same time give the mother some rest.

Case study 3

A single mother who is 24 weeks pregnant with her third child has been informed that her baby has been diagnosed with anencephaly (a severe brain abnormality). She has been given the option to terminate her pregnancy but has declined a termination and says she wants to give her baby whatever chance she can. She is in your clinic room today for her 24-week check-up and wants to know what support she can receive to help her cope through this difficult time. Her other children are six years and three years old.

1. Who should you consider referring the mother to for further care and support?

The baby has been diagnosed with a life-limiting condition and is likely to die soon after birth, therefore the mother should be referred to a children’s palliative care team for perinatal palliative care.

2. Why should you refer the mother to the palliative team now?

Perinatal palliative care should be provided from the time of diagnosis as this will allow an opportunity to establish goals of care for each stage of her pregnancy, the labour and delivery, documented in a birthing plan, as well as allow her a chance to make decisions regarding end of life care for her baby, such as pain and symptom management and withholding of medical treatments.

3. When will the mother require bereavement care and support?

The mother is likely to start experiencing grief from the time the bad news is broken to her about her baby’s condition. She will need bereavement support through the continuum of the infant’s illness trajectory, through the death and months to even years after the death.