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

• When communicating with a child or young person with a life-limiting condition and their parents or carers, healthcare professionals should:
  • Consider the age and level of understanding of the child or young person. 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).
  • Focus on the views of the child or young person and their parents or carers and: 
    
    • Consider their personal and family situation; religious, spiritual, and cultural beliefs and values; and any special needs, such as communication aids or interpreters. 
    • Explore with them whether, based on their beliefs and values, there are any aspects of care about which they have particular views or feelings.
  • Be sensitive, honest, and realistic.
    • Reassure when appropriate.
    • Discuss any uncertainties about the condition and treatment. 
  • Think about how best to communicate at different stages, such as:
    • When the life-limiting condition is first recognized.
    • When reviewing and developing the Advance Care Plan.
    • If their condition worsens.
    • When the child or young person is approaching the end of life. 
  • Provide information that is:
    • Specific to the family's circumstances.
    • Clearly explained and understandable.
    • Consistent.
    • Up-to-date.
  • Provide information verbally and in writing.

Basis for recommendation

This information is largely based on the National Institute for Health and Care Excellence (NICE) guideline End of life care for infants, children and young people with life-limiting conditions: planning and management [NICE, 2021] and the Together for Short Lives (TSL) charity document  A core care pathway for children with life-limiting and life-threatening conditions [TSL, 2013].

Organ and tissue donation

• Talk to the child or young person and their parents or carers about organ or tissue donation.
  • Explore their views and feelings about organ and tissue donation.
  • Explain which organs or tissues (if any) may be possible to donate.
  • Involve the organ donation service if needed.
  • The National Institute for Health and Care Excellence (NICE) guideline on Organ donation for transplantation has detailed information on organ donation, including donor identification and consent and when and how to discuss the topic.
• If organ or tissue donation is not possible, explain why.
• If the child is eligible to donate organs or tissue, ask them if they and their parents or carers would like to discuss this. If they do:
  
  • Discuss how deciding to donate could affect their care, for example, by changing their preferred place of care and place of death.
  • Explain the practical policies and procedures involved.
  • Provide written information if needed.
• If the child does not have the capacity to decide about organ and tissue donation, ask their parents or carers to make the decision.

Preferred place of care and place of death

• Discuss and agree with the child or young person and their parents or carers about a preferred place of care and place of death. During the discussion, consider:
  • Their wishes, which are personal and individual.
  • Their religious, spiritual, and cultural values.
  • The views of relevant and experienced healthcare professionals.
  • Safety and practicality.
• Explain that the place of care or place of death may change, for example:
  • If the child or young person or their parents or carers change their minds.
  • For clinical reasons.
  • Due to problems with service provision. 
• Provide information on:
  • The various care settings (for example, home, hospice, or hospital care).
  • The care and support available in each setting.
  • Practical and safety issues.
• If care at home is preferred, consider and discuss the practical issues, such as the possible need for:
  • Home adaptations.
  • Changes to living arrangements.
  • Equipment and support.
• For children who 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 (for example, for symptom management).
  • Practical support and equipment for interventions, including oxygen, enteral nutrition, and subcutaneous and intravenous therapies. 
  • Anticipatory prescribing for children who are likely to develop symptoms.
• If it is suspected that a child or young person may die soon and they are not in their preferred place of death, consider whether rapid transfer is possible and in the child or young person's best interests. Discuss this with the child or young person and their parents or carers.
  • When planning a rapid transfer to the preferred 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/carers.
    • Ensure that relevant changes to the Advance Care Plan are implemented.
  • Think about using a rapid transfer process 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 body of the child or young person will be cared for after their death. 

Basis for recommendation

These recommendations are based on the National Institute for Health and Care Excellence (NICE) guideline End of life care for infants, children and young people with life-limiting conditions: planning and management [NICE, 2021].

Basis for recommendation

These recommendations are based on the National Institute for Health and Care Excellence (NICE) guideline End of life care for infants, children and young people with life-limiting conditions: planning and management [NICE, 2021].

Symptoms and signs common in the last hours or days of life

• Symptoms and signs that are common in the last hours or days of life include:
  • 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.

Basis for recommendation

These recommendations are based on the National Institute for Health and Care Excellence (NICE) guideline End of life care for infants, children and young people with life-limiting conditions: planning and management [NICE, 2021].

Managing agitation

• As children and young people with life-limiting conditions approach the end of life, they may:
  • Become agitated (shown by restlessness, irritability, aggressive behaviour, crying, or other distress).
  • Show signs of delirium (such as confusion, disrupted attention, disordered speech, and hallucinations).
• If a child becomes agitated or delirious: 
  • Make sure that they are safe from physical injury.
  • Look for causes and factors that may be contributing to this, including:
    • Medical disorders and conditions, such as pain, hypoxia, anaemia, dehydration, urinary retention, or constipation.
    • Psychological factors, such as fear, anxiety, or depression.
    • Adverse effects of medication.
  • Be aware that in children and young people with a neurological disability, signs and symptoms of agitation or delirium can be mistaken for the signs and symptoms of seizures or dystonia.
• If a child or young person who is approaching the end of life needs treatment for agitation:
  • Identify and, if possible, treat any medical or psychological conditions that may be contributing to it. 
  • Consider non-pharmacological interventions, such as:
    • Calm speaking, reassurance, distraction, and physical contact (such as holding and touch).
    • Changes to the environment to make it more comfortable, calm, and reassuring (such as reducing noise and lighting, maintaining a comfortable room temperature, and providing familiar objects and people and relaxing music).
    • Religious and spiritual support if this is wanted and helpful.
  • Consider pharmacological interventions (beginning with low doses and increasing if necessary), for example: 
    • Benzodiazepines (such as midazolam, diazepam, or lorazepam).
    • Neuroleptics (such as haloperidol or levomepromazine).

Managing pain

• Assess for the presence and severity of pain. 
  • Be aware that various factors can contribute to pain, including: 
    • Biological factors, such as musculoskeletal disorders or constipation.
    • Environmental factors, such as an uncomfortable or noisy care setting.
    • Psychological factors, such as anxiety or depression.
    • Social, emotional, religious, spiritual, or cultural considerations. 
  • For children and young people who have pain or have had it before, regularly reassess for pain and severity even if they are not receiving treatment for it.
• When assessing pain in children and young people:
  • Use an age-appropriate approach that takes account of their stage of development and ability to communicate.
  • Try to identify what is causing or contributing to their pain. Be aware that:
    • The pain may not be related to the life-limiting condition.
    • Pain, discomfort, and distress may be caused by a combination of factors, which will need an individualized management approach. 
  • Take into account the following causes of pain and distress that might have been overlooked, particularly in children and young people who cannot communicate:
    • Neuropathic pain (for example, associated with cancer).
    • Gastrointestinal pain (for example, associated with diarrhoea or constipation). 
    • Bladder pain (for example, caused by urinary retention).
    • Bone pain (for example, associated with metabolic diseases).
    • Pressure ulcers.
    • Headache (for example, caused by raised intracranial pressure).
    • Musculoskeletal pain (particularly if they have neurological disabilities).
    • Dental pain.
• Consider non-pharmacological interventions for pain management, such as:
  • Changes that may help them to relax, for example:
    • Environmental adjustments (such as reducing noise).
    • Music.
    • Physical contact (such as touch, holding, or massage).
  • Local hot or cold applications to the site of pain.
  • Comfort measures, such as sucrose for neonates.
• When tailoring pain treatment, consider the views of the child or young person and those of their parents or carers on:
  • The benefits of pain treatment.
  • The possible adverse effects of analgesia for moderate to severe pain (for example, unwanted sedation, reduced mobility, or constipation).
• Consider using a stepwise approach to analgesia based on pain severity and persistence:
  • For mild pain, consider paracetamol or ibuprofen sequentially and then in combination if needed.
  • For moderate to severe pain, also consider one of the following options:
    • Low-dose oral opioids (such as morphine).
    • Transmucosal opioids.
    • Subcutaneous opioids.
    • Intravenously infused opioids (if a central venous catheter is in place).
• When using opioids, titrate treatment to find the minimal effective dose that will relieve and prevent pain.
  • Calculate opioid doses using weight rather than age because the child or young person may be underweight for their age.
  • Titrate treatment to provide continuous background analgesia; prescribe additional doses for breakthrough pain if this occurs.
  • If treatment with a specific opioid does not give adequate pain relief or if it causes unacceptable adverse effects, consider an alternative opioid.
• In addition to background analgesia, consider giving anticipatory doses of analgesia to children or young people who have pain at predictable times (for example, when changing dressings or during moving and handling).
  • Do not include anticipatory doses when calculating the required daily background dose of analgesia.
• If neuropathic pain is suspected and standard analgesia is not helping, consider a trial with one of the following:
  • Gabapentin. 
  • A low-dose tricyclic antidepressant (for example, amitriptyline).
  • An anti-N-Methyl-D-aspartate (NMDA) agent (for example, ketamine or methadone [used under guidance from a specialist]).

Managing respiratory distress

• If a child has respiratory distress, breathlessness, or noisy breathing:
  • Explain that these symptoms are common. 
  • Discuss the likely causes or contributing factors.
  • Discuss any treatments that may help.
• Consider and, if possible, treat the likely contributing factors or causes. If these are likely to be caused by:
  • Anxiety:
    • Discuss why they are anxious. 
    • Reassure them and manage their anxiety accordingly. 
    • Consider breathing techniques and guided imagery. 
    • Consider anxiolytic drugs.
  • Physical discomfort:
    • Consider what could be causing the discomfort (for example, their position) and, if possible, help them with it.
  • Environmental factors:
    • Consider environmental changes, such as changing the room temperature.
  • Accumulated airway secretions:
    • Consider repositioning, airway suctioning, physiotherapy, or anti-secretory drugs.
  • Medical disorders, for example, pneumonia, heart failure, sepsis, or acidosis:
    • Use appropriate interventions, such as bronchodilators, nebulized saline, opioids, or oxygen supplementation.
• If further assessment is required, consider referral to an appropriate specialist (for example, a respiratory or cardiac specialist).

Managing seizures

• If a child has a seizure, look for and, if possible, treat or remove any potential causes, triggers, or contributing factors, for example:
  • Fever.
  • Electrolyte disturbances.
  • Drug reactions.
  • Sleep deprivation.
  • Pain.
  • Excessive environmental stimulation.
• If a child is thought to be at increased risk of seizures (for example, because they have had seizures before or because of an existing brain disorder): 
  • Include seizure management in their Advance Care Plan.
  • Consider the benefits and drawbacks of specific seizure treatments and:
    • Take into account how any decisions could affect the choices available for the preferred place of care and place of death.
    • Discuss this with the child or young person and their parents/carers.
  • Explain to the child or young person and their parents or carers:
    • How likely it is that they may have a seizure.
    • What they might notice if a seizure happens.
    • That seizures can be frightening or upsetting.
    • What parents or carers should do if a seizure occurs at home (for example, place the child in a safe position).
• Be aware that abnormal movements (such as dystonic spasms) might be mistaken for seizures.
  • If in doubt, seek specialist advice.
• If parents or carers have been provided with anticonvulsive treatment (such as buccal midazolam):
  • Ensure they know how and when to use it if the child or young person has a seizure at home.

Ensuring adequate hydration

• If a child or young person with a life-limiting condition is approaching the end of life or is dying (likely to die in hours or days):
  • Discuss how to manage their fluid with them and their parents/carers.
  • Encourage and support them to drink if they want to and are able to.
  • Continue to provide the child with lip and mouth care.
• If a child cannot drink, discuss whether starting or continuing enteral tube or intravenous (IV) fluids is in their best interests.
  • Be aware that enteral tubes and intravenous fluids may have a significant effect on care, be a burden for the child or young person, and mean the preferred place of care and place of death need to be changed.
  • Review the decision to give enteral or IV fluids regularly to ensure it continues to be in their best interests. 

Ensuring adequate nutrition

• If a child or young person with a life-limiting condition is approaching the end of life or is dying (likely to die in hours or days):
  
  • Discuss how to manage their nutritional needs with them and their parents (or carers).
  • Encourage and support them to eat if they want to and are able to.
• If the child or young person is receiving enteral tube feeding or intravenous nutrition:
  • Discuss whether continuing this is in their best interest and review this decision regularly.

Basis for recommendation

These recommendations are based on the National Institute for Health and Care Excellence (NICE) guideline End of life care for infants, children and young people with life-limiting conditions: planning and management [NICE, 2021].

Emotional and psychological support

• 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, and mental health problems. 
    • May need support (and sometimes expert psychological intervention) to help with distress, coping, and building resilience. 
  • Children and young people with life-limiting conditions may experience rapid changes in their condition and so might need emergency interventions and urgent access to psychological services. 
  • Children and young people with learning difficulties or problems with communication may have specific emotional and psychological difficulties.
• 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 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.

Social and practical support

• 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.

Religious, spiritual, and cultural support

• Ask children and young people with life-limiting conditions and their parents/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. 
  • Take account of these beliefs and values in all discussions with them and when making decisions about their care.
  • The charity Together for Short Lives has developed a Diversity Toolkit to support professionals and organizations to consider diversity and cultural issues in children’s palliative care.
• Be aware that:
  • Some people find discussions about their beliefs and values difficult or upsetting, while others find them reassuring and helpful. Some may need to discuss their beliefs and values more than once.
  • Children and young people may feel differently from 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.

Bereavement support

• When a child or young person with a life-limiting condition is approaching the end of life:
  
  • Discuss the bereavement support available with parents or carers and provide written information.
    • The NHS website has a searchable database of bereavement support services. 
    • The Together for Short Lives charity has a downloadable booklet (After your child has died) with useful information and advice to assist families during the early days of their bereavement.  
  • Talk to the parents or carers about available psychological bereavement support groups.
  • Offer bereavement support from a professional with appropriate expertise to the parents or carers both before and after the death of a child or young person. 
  • Be aware that siblings should also be offered bereavement support because not only are they affected by the loss of a brother or sister, but they also have to cope with the impact it has on the family and the relationships within it. 
• When planning bereavement support for parents or carers:
  • Talk to them about the support that is available, and explore with them what they would find helpful and acceptable.
  • Consider what support different professionals could provide (for example, their GP or healthcare professionals who know the child and are involved in their care).
  • Think about the role of individual professionals in providing specific aspects of support.
  • Inform the multidisciplinary team about the support plan.
• When making a bereavement support plan with parents or carers, discuss possible options with them, such as:
  • Opportunities to talk to the professionals caring for the child or young person to:
    • Discuss memories and events.
    • Answer any concerns or questions they may have.
  • Home visits from the healthcare professionals caring for the child. 
  • Bereavement support groups.

Basis for recommendation

These recommendations are based on the National Institute for Health and Care Excellence (NICE) guideline End of life care for infants, children and young people with life-limiting conditions: planning and management [NICE, 2021].