End of life care for infants, children and young people with life-limiting conditions: planning and management

Think about how to provide information for children and young people with life-limiting conditions, taking into account their age and level of understanding. When appropriate, use formats such as:

• one-to-one discussion

• play, art and music activities

• written materials and pictures

• digital media, for example social media.

When deciding how best to communicate with the individual child or young person and their parents or carers, focus on their views and take account of:

• their personal and family situation

• their religious, spiritual and cultural beliefs and values

• any special needs, such as communication aids or the need for interpreters.

Ask children and young people with life-limiting conditions and their parents or carers:

• if there are other people important to them (such as friends, boyfriends or girlfriends, teachers, or foster parents) who they would like to be involved, and if so

• how they would like those people to provide a supporting role.

Think about how best to communicate with each child or young person and their parents or carers:

• when the life-limiting condition is first recognised

• when reviewing and developing the Advance Care Plan

• if their condition worsens

• when they are approaching the end of life.

Ensure that all parents or carers are given the information and opportunities for discussion that they need.

When deciding which healthcare professional should lead on communication at a particular stage in a child or young person's illness, take account of:

• their expertise and ability to discuss the topics that are important at that time

• their availability, for example if frequent discussions are needed during an acute illness or near the end of life

• the views of the child or young person and their parents or carers.

Be aware that most children and young people with life-limiting conditions and their parents or carers want to be fully informed about the condition and its management, and they value information that is:

• specific to the child's or young person's individual circumstances

• clearly explained and understandable

• consistent

• up-to-date

• provided verbally and in writing.

Be aware that some children and young people and parents or carers may be anxious about receiving information about their condition.

Ask how children and young people and their parents or carers would like to discuss the life-limiting condition. For example:

• Ask which topics they feel are important and would particularly want information on.

• Ask whether there are topics they do not want detailed information on, and discuss their concerns.

• If appropriate, ask parents or carers whether they think their child understands their condition and its management, and which professional their child would like to talk to about it.

• If appropriate, ask parents or carers what they think their child should be told about their condition.

• Discuss with the child or young person and their parents or carers their right to confidentiality and how information about their condition will be shared.

• Review these issues with them regularly, because their feelings and circumstances may change over time, and they may need different information at different times.

When talking to children or young people and their parents or carers:

• be sensitive, honest and realistic

• give reassurance when appropriate

• discuss any uncertainties about the condition and treatment.

Be alert for signs or situations that the child or young person or their parents or carers need more information or discussions, for example if:

• they are more anxious or concerned

• the child or young person's condition deteriorates

• a significant change to the treatment plan is needed.

Provide children and young people and their parents and carers with the information they need on:

• their role and participation in Advance Care Planning (see Advance Care Planning)

• the membership of their multidisciplinary team and the responsibilities of each professional (see multidisciplinary team)

• the care options available to them, including specific treatments and their preferred place of care and place of death (see preferred place of care and place of death)

• any relevant resources or support available to them.

Develop and record an Advance Care Plan at an appropriate time for the current and future care of each child or young person with a life-limiting condition. The Advance Care Plan should include:

• demographic information about the child or young person and their family

• up-to-date contact information for:

  • the child or young person's parents or carers and

  • the key professionals involved in care

• a statement about who has responsibility for giving consent

• a summary of the life-limiting condition

• an agreed approach to communicating with and providing information to the child or young person and their parents or carers

• an outline of the child or young person's life ambitions and wishes, for example on:

  • family and other relationships

  • social activities and participation

  • education

  • how to incorporate their religious, spiritual, and cultural beliefs and values into their care

• a record of significant discussions with the child or young person and their parents or carers

• agreed treatment plans and objectives

• education plans, if relevant

• a record of any discussions and decisions that have taken place on:

  • preferred place of care and place of death

  • organ and tissue donation (see recommendation 1.2.17)

  • management of life-threatening events, including plans for resuscitation or life support

  • specific wishes, for example on funeral arrangements and care of the body

• a distribution list for the Advance Care Plan.

Begin discussing an Advance Care Plan with parents during the pregnancy if there is an antenatal diagnosis of a life-limiting condition. For each individual think about who should take part in the discussion, for example:

• obstetricians

• midwives

• neonatologists

• specialists in the life-limiting condition

• a member of the specialist paediatric palliative care team (see recommendation 1.5.4).

Develop and regularly review Advance Care Plans:

• with relevant members of the multidisciplinary team and

• in discussion with the child or young person and their parents or carers.

When developing the Advance Care Plan, take account of the beliefs and values of the child or young person and their parents or carers.

Explain to children and young people and their parents or carers that Advance Care Planning should:

• help them be involved in planning their care and give them time to think about their views carefully

• help them to understand the life-limiting condition and its management

• help to prepare for possible future difficulties or complications

• support continuity of care, for example if there are changes in the professionals involved or in the care setting (such as a hospital admission or discharge).

Share the Advance Care Plan with the child or young person and their parents or carers (as appropriate), and think about which professionals and services involved in the individual child or young person's care should also see it, for example:

• GPs

• hospital consultants

• hospices

• respite centres

• nursing services (community or specialist)

• school and other education services

• ambulance services.

Update the Advance Care Plan when needed, for example if:

• new professionals become involved

• the care setting changes (for example hospital admission or discharge)

• the child or young person and their parents or carers move home.
  
  Discuss the changes with the child or young person (if appropriate) and their parents or carers.

Share the Advance Care Plan with everyone involved each time it is updated.

When making an Advance Care Plan, discuss with the child or young person and their parents or carers:

• the nature of the life-limiting condition, its likely consequences and its prognosis

• the expected benefits and possible harms of the management options.

Be aware that all children and young people with life-limiting conditions should have an Advance Care Plan in their medical record, and that this should not be confused with a do-not-attempt-resuscitation order.

Be aware that any existing resuscitation plan for a child or young person may need to be changed in some circumstances, for example if they are undergoing general anaesthesia.

For information on organ donation (including donor identification and consent, and when and how to discuss the topic), see the NICE guideline on organ donation for transplantation.

Talk to the child or young person and their parents or carers about organ or tissue donation, and explore their views and feelings on this.

Explain to the child or young person and their parents or carers which organs or tissues (if any) it may be possible to donate.

Involve the organ donation service if needed. If organ or tissue donation is not possible, explain why.

If the child or young person is eligible to donate organs or tissue, ask them if they and their parents or carers (as appropriate) would like to discuss this, and if so:

• provide written information if needed

• discuss how deciding to donate could affect their care, for example by changing their place of care and place of death

• explain the practical policies and procedures involved.

If the child or young person does not have the capacity to decide about organ and tissue donation, ask their parents or carers to make the decision.

Be aware that children and young people with life-limiting conditions and their parents or carers may have:

• emotional and psychological distress and crises

• relationship difficulties

• mental health problems.

Be aware that children and young people and their parents or carers may need support, and sometimes expert psychological intervention, to help with distress, coping, and building resilience.

Be aware that children and young people may experience rapid changes in their condition and so might need emergency interventions and urgent access to psychological services.

Be aware of the specific emotional and psychological difficulties that may affect children and young people who have learning difficulties or problems with communication.

Provide information to children and young people and their parents or carers about the emotional and psychological support available and how to access it.

Regularly discuss emotional and psychological wellbeing with children and young people and their parents or carers, particularly at times of change such as:

• when the life-limiting condition is diagnosed

• if their clinical condition deteriorates

• if their personal circumstances change

• if there are changes to their nursery care, school or college arrangements, or their employment

• if there are changes to their clinical care, for example if their care changes focus from treating the condition to end of life care.

Be aware that children and young people with life-limiting conditions and their parents or carers have varied social and practical support needs, and that those needs may change during the course of their condition. This may include:

• material support, for example housing or adaptations to their home, or equipment for home drug infusions

• practical support, such as access to respite care

• technical support, such as training and help with administering drug infusions at home

• education support, for example from hospital school services

• financial support.

Ask children and young people with life-limiting conditions and their parents or carers if they want to discuss the beliefs and values (for example religious, spiritual or cultural) that are important to them, and how these should influence their care. Be aware that they may need to discuss their beliefs and values more than once.

Take account of the beliefs and values of children and young people and of their parents and carers in all discussions with them and when making decisions about their care.

Be aware that:

• some children and young people and their parents or carers find discussions about their beliefs and values difficult or upsetting

• others find these discussions reassuring and helpful.

Be aware that children and young people may feel differently to their parents, carers, or healthcare professionals about how their beliefs and values should influence their care. If there is disagreement, try to make a mutually acceptable care plan, and if necessary involve the chaplaincy service or another facilitator.

Discuss with children and young people with life-limiting conditions and their parents or carers where they would prefer to be cared for and where they would prefer to die.

Agree the preferred place of care and place of death with children and young people and their parents or carers, taking into account:

• their wishes, which are personal and individual

• their religious, spiritual and cultural values

• the views of relevant and experienced healthcare professionals

• safety and practicality.

If possible, services should ensure that children and young people can be cared for at their preferred place of care and die at their preferred place of death.

Explain that the place of care or place of death may change, for example:

• if the child or young person and their parents or carers change their minds or

• for clinical reasons or

• due to problems with service provision.

When discussing possible places of care or places of death with children and young people and their parents or carers, provide information about:

• the various care settings (for example home, hospice or hospital care)

• the care and support available in each setting

• practical and safety issues.

If the child or young person and their parents or carers prefer care at home, take into account and discuss the practical considerations with them, such as the possible need for:

• home adaptations

• changes to living arrangements

• equipment and support.

If it is suspected that a child or young person may die soon and they are not in their preferred place of death, think about whether rapid transfer is possible and in their best interest. Discuss this with them and their parents or carers.

When planning rapid transfer to the preferred place of death, review and if necessary update the Advance Care Plan in discussion with the child or young person and their parents or carers and with the healthcare professionals who will be involved following the transfer. The updated Advance Care Plan should include a record of:

• any intended changes to care and when they should happen

• care plans that cover:

  • the final hours or days of life

  • what will happen if the child or young person lives longer than expected

  • support for the family after the child or young person dies

  • care of the child's or young person's body after death

• the professionals who will be involved and their responsibilities

• the professionals who will help with the practical and administrative arrangements after the death.

When planning rapid transfer of a child or young person to their intended place of death:

• be aware that the course of their condition may be unpredictable, and that they may die sooner or later than expected

• discuss any uncertainties about the course of their condition and how this could affect their care with them and their parents or carers

• ensure that relevant changes to the Advance Care Plan are implemented.

Think about using a rapid transfer process (see recommendation 1.5.8) to allow the child or young person to be in their preferred place of death when withdrawing life-sustaining treatments, such as ventilation.

Before rapid transfer, agree with the parents or carers where the child's or young person's body will be cared for after their death.

Involve the specialist paediatric palliative care team if a child or young person has unresolved distressing symptoms as they approach the end of life (see recommendation 1.5.4 for who should be in this team).

If a child or young person with a life-limiting condition is approaching the end of life or is dying, discuss how to manage their fluid needs with them and their parents or carers.

If a child or young person is dying, encourage and support them to drink if they want to and are able.

If a child or young person is dying, continue to provide them with lip and mouth care.

If a child or young person is dying and cannot drink, discuss with them (as appropriate) and their parents or carers whether starting or continuing enteral tube or intravenous fluids is in their best interests.

Be aware that enteral tube and intravenous fluids may have a significant effect on care, may be a burden for children and young people, and may mean the place of care and place of death need to be changed.

If a child or young person is given enteral or intravenous fluids, review this decision regularly to make sure it continues to be in their best interests.

If a child or young person is approaching the end of life or is dying, discuss how to manage their nutritional needs with them and their parents or carers.

If a child or young person with a life-limiting condition is dying, encourage and support them to eat if they want to and are able.

If a child or young person is dying and they are receiving enteral tube feeding or intravenous nutrition:

• discuss with them (as appropriate) and their parents or carers whether continuing this is in their best interest and

• review this decision regularly.

For children and young people with life-limiting conditions who are approaching the end of life:

• be aware that there is often uncertainty around when they are likely to die

• be aware that there are various symptoms and signs (individually or in combination) that indicate they are likely to die within hours or days

• take into account the wider clinical context.

When assessing whether a child or young person is likely to die within hours or days, be aware that the following signs are common in the last hours or days of life, and monitor these non-invasively as far as possible:

• a change of breathing pattern (for example noisy, laboured or irregular breathing)

• impaired peripheral perfusion (which can be indicated by a pale or grey appearance, or a prolonged capillary refill time), including temperature instability

• loss of interest in or ability to tolerate drinks or food

• a marked and unexplained fall in urine output

• an altered level of awareness (for example reduced consciousness, alertness or responsiveness, excessive sleeping, or confusion)

• intractable seizures that keep occurring even with optimal management

• new onset of profound weakness

• increasing pain and need for analgesia.

When assessing symptoms and signs to decide whether a child or young person is likely to die within hours or days, take into account the wider clinical context, including:

• their normal clinical baseline

• past clinical events (such as previous episodes of temporary deterioration)

• the overall progression of their condition.

When assessing whether a child or young person is likely to die within hours or days, take into account the clinical judgement of healthcare professionals experienced in end of life care.

If the child or young person or their parents or carers feel that they are likely to die within hours or days:

• be aware that they may be correct

• discuss their concerns with them.

When a child or young person is likely to die within hours or days, support them and their parents or carers by:

• listening to any fears or anxieties they have and

• showing empathy and compassion.

When a child or young person is likely to die within hours or days:

• be aware that they or their parents or carers may not express their feelings openly, and may:

  • have intense and varied feelings such as fear, hopelessness or anger or

  • become more accepting of the inevitability of death

• give them and their parents or carers opportunities to talk.

If a child or young person is likely to die within hours or days, explain to them and their parents or carers:

• why you think this is likely, and any uncertainties

• what clinical changes can be expected

• whether you think the treatment plan should be changed.

When children and young people become seriously ill and are likely to die within hours or days, provide care as specified in their Advance Care Plan and review if needed.

Be aware that children and young people may have difficulty asking directly if they are going to die or are dying. Explore and discuss their concerns if you think they want to talk about this.

Be aware that parents or carers may have difficulty asking directly if a child or young person is dying. Explore and discuss their concerns if you think they want to talk about this.

If a child or young person may be approaching the end of life and they or their parents or carers want to be involved in making decisions about their care, discuss and review their Advance Care Plan with them.

When a child or young person is approaching the end of life, discuss with them and their parents or carers and with relevant healthcare professionals:

• any available invasive treatments that might be in their best interest

• any interventions they are currently receiving that may no longer be in their best interest.

If withdrawing a treatment for a child or young person who is dying, explain to them and to their parents or carers that it is often difficult to tell if or how this may affect them, or when they will die.

When a child or young person is likely to die within hours or days, ensure that they can have private time with their parents or carers.

Children and young people with life-limiting conditions should be cared for by a defined multidisciplinary team.

As the child or young person's circumstances change (for example if they change from having care primarily to manage their condition to having end of life care), the membership of the multidisciplinary team should be adjusted accordingly.

Depending on the needs of the child or young person, the multidisciplinary team may include:

• healthcare professionals from primary, secondary or tertiary services, including specialists in the child's underlying life-limiting condition, hospice professionals and members of the specialist palliative care team (see recommendation 1.5.4)

• social care practitioners

• education professionals

• chaplains

• allied health professionals (for example physiotherapists, occupational therapists, and psychological therapists).

The specialist paediatric palliative care team should include at a minimum:

• a paediatric palliative care consultant

• a nurse with expertise in paediatric palliative care

• a pharmacist with expertise in specialist paediatric palliative care

• experts in child and family support who have experience in end of life care (for example in providing social, practical, emotional, psychological and spiritual support).

Explain to children and young people and their parents or carers:

• who the multidisciplinary team members are and how they are involved in their care

• how the multidisciplinary team membership will change if the care that is needed or the care setting changes.

Think about involving children and young people and their parents or carers in multidisciplinary team meetings (when appropriate).

Think about having a named individual from the multidisciplinary team to act as a first point of contact for the child or young person and their parents or carers.

In collaboration with local hospitals, hospices, and community, primary care and ambulance services, ensure there is a rapid transfer process for children and young people with life-limiting conditions to allow urgent transfer to the preferred place (for example from the intensive care unit to their home or to a children's hospice). See recommendations 1.3.15 to 1.3.19 for the planning and practical arrangements of this transfer.

For children and young people with life-limiting conditions who are approaching the end of life and are being cared for at home, services should provide (when needed):

• advice from a consultant in paediatric palliative care (for example by telephone) at any time (day and night)

• paediatric nursing care at any time (day and night)

• home visits by a healthcare professional from the specialist paediatric palliative care team (see recommendation 1.5.4), for example for symptom management

• practical support and equipment for interventions including oxygen, enteral nutrition, and subcutaneous and intravenous therapies

• anticipatory prescribing for children and young people who are likely to develop symptoms.

Services should have agreed strategies and processes to support children and young people who are approaching the end of life and are being cared for at home. These services should be based on managed clinical networks, and should collaborate on care planning and service delivery.

Services for children and young people who are approaching the end of life and are being cared for at home should be able to support parenteral drug administration (for example continuous subcutaneous opioid or anticonvulsant infusions).