11 End-of-life care

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Contents

• Objectives
• Introduction to end-of-life care
• End-of-life planning
• Managing distressing symptoms at the end-of-life
• Management at the end-of-life
• Supporting the family at the end-of-life
• Case studies

Objectives

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

1. Define end-of-life care in children.
2. Recognise when a child is dying or close to death.
3. Explain the meaning of a ‘good death’.
4. Know how to use and develop a terminal care plan for end-of-life care.
5. Discuss end-of-life management options and planning.
6. Support the family at end-of-life.

Introduction to end-of-life care

11-1 What is end-of-life care?

End-of-life care is the support and care that is provided to the child and family when a child’s clinical condition has reached a stage where the child is dying or is likely to die soon. End-of-life care may begin hours, days or even weeks before their death. This final phase of life is usually recognised by both the family and the carers. It may also be recognised by the child. Sometimes end-of-life care is called terminal care.

All staff working with children should know how to care for a dying child.

End-of-life care is the care provided to the child and family when it appears that the child is dying or likely to die soon.

11-2 What is the purpose of end-of-life care?

End-of-life care is active care with the emphasis on symptom management, comfort and dignity. The aim of end-of-life care is not to prolong life but to help achieve a ‘good death’. It aims at improving the quality of the time for both parents and child at the end of the child’s life. Just being present is often enough.

The aim of end-of-life care is to provide symptom management, comfort and dignity during the dying process.

11-3 Why is it important to recognise that the end-of-life is close?

Not recognising that a child is dying is a major barrier to providing good end-of-life care. Many healthcare providers do not know how to manage a dying child and their family. They often do not know what to say, so may avoid the situation. However, just being there can be helpful. The memories that parents have of the last days of their child’s life are affected by the quality of end-of-life care.

Aggressive life-saving treatment should be stopped when it is recognised that a child is dying. Some healthcare workers find this a difficult decision.

11-4 Can you predict when a child will die?

It can be very difficult to predict when a child is going to die. Some children’s condition deteriorates quickly and unexpectedly while others may rally for a while and live longer than predicted. Either situation can be emotionally and physically exhausting for the family, child and carers.

It can be very difficult to predict when a child will die.

11-5 How can you recognise that a child is dying?

Some indicators that the child will die soon include:

Physical signs:

• Long periods of sleep
• Decreased urine and stool output
• Deteriorating vital signs such as low oxygen saturation, slower or altered breathing and bradycardia (slow pulse).

Behavioural or emotional signs:

• Not wanting to eat
• Loss of interest in surroundings
• Decreased interaction with family and staff
• Clingy or withdrawn behaviour
• Lethargy or sudden increased awareness
• Not wanting to be left alone
• Talking about ‘going home’
• Talking of angels, lights or dead relatives
• Talking or drawing pictures of vehicles or journeys.

11-6 How would you describe a ‘good death’?

A ‘good death’ has the least amount of suffering and distress for the child and family. Characteristics of a ‘good death’ include:

• An expected death where there has been time to plan and prepare
• Achieving a sense of calm and control for the child and their family
• Maintaining the child and family’s dignity
• Strengthening or restoring relationships with loved ones
• Focusing on the dying child and not hospital routines
• Relieving the burden of suffering
• Adequate pain and symptom control
• Avoiding inappropriate prolongation of dying.

Note

The term ‘good death’ is often used in palliative care to describe a death which has been well managed. However, it should be remembered that no child’s death can be considered ‘good’ and will always be brutal and heart-breaking for the family.

11-7 Where could children die?

The place of death depends on the preferences of the child and family, available resources, access to healthcare services and the timing of the child’s death. This could be in a hospital, a hospice or in the child’s own home.

A child may move between settings, so it is important to maintain teamwork and good communication. For continuity of care, the family should be well informed of the goals of care, and a formal advance care plan should be provided to guide healthcare professionals.

Children can receive end-of-life care and die in a number of environments depending on preferences of the family and resources available.

End-of-life planning

11-8 What is the best approach when planning end-of-life care?

In palliative care, we aim to journey with a child and their family from the time of diagnosis to beyond the child’s death. By being compassionate and considerate, and having difficult conversations during times of calm, not crises, we can address many of the practical concerns surrounding the death of a child before it happens.

It is important to work at the family’s pace, and not rush the process. The child and their family have already faced many significant losses before entering the end-of-life phase, so our role is not to cause more distress.

Each journey is unique. Some families need more guidance than others. Belief systems and wishes will vary, and the time allowed for planning is different. Never make assumptions based on religion or culture but rather clarify the specific wishes of that particular child and family.

As the goals of care change, we should review all interventions to reduce the burden and relieve suffering.

Each end-of-life journey will be unique and it is important to provide care that is culturally and spiritually sensitive.

11-9 What should be considered in end-of-life planning?

When planning for the end of a child’s life, the following should be considered:

For the child:

• The decision to resuscitate or to only provide comfort care in the event of a life-threatening event such as cardiac arrest
• Limitations on other medical interventions
• Organ donation
• Where will the death occur?
• If planning for a home death, who to call and who will certify the death?

For the family:

• Who should be present when the child dies? Would the parents prefer to not be around?
• Preparing the family for altered respiratory patterns such as gasping and the ‘death rattle’
• Holding the child
• Washing the child’s body and involvement in preparing for burial or cremation.

For the healthcare team:

• Privacy in the ward
• Preparing the medical and nursing staff
• Reduce the use of monitors and nursing observations to a minimum
• Symptom control, feeds and fluids
• Allow the family as much time with the child as they need both before and after death
• Inform the family before the child’s body is wrapped
• Preparing a private space or room for the family to be after the death
• Debriefing of the team.

11-10 What is the role of advance care planning in end-of-life care?

Advance care planning involves the palliative care provider, the whole care team, the child and the family and provides the healthcare provider with guidance on managing end-of-life care. Ideally, this should be done while the child is stable. This is much easier than making decisions in a crisis or during acute deterioration in the child’s condition.

The planning process considers the wishes and preferences of the child and family, appropriate interventions that may be needed, place of care, available palliative care services and the likely clinical scenarios and end-of-life issues the child may face.

The advance care plan document and the terminal care plan (if developed) should be readily available in the child’s clinical notes, road-to-health booklet, local hospital emergency room and copies should be given to relevant family members. The advance care plan should be reviewed with the family and healthcare team on a regular basis.

The advance care plan aims to make decision-making in a crisis easier and should be readily available.

See chapters 2 and 7 for more detailed information on advance care planning.

11-11 What is a terminal care plan?

A terminal care plan (also known as an end-of-life plan) should form part of the advance care plan. It is a separate document that deals specifically with end-of-life issues and concerns.

11-12 What information would you expect to find in a terminal care plan?

Below is a checklist of the information you would expect to appear in a terminal care plan:

• Personal details:
  • Name
  • Main caregivers
  • Diagnosis
  • Home language
  • Management of pain and symptoms, e.g. excessive secretions, nausea and agitation
• Comfort measures to be taken:
  • Non-essential drugs stopped and required medication to treat symptoms started
  • Inappropriate interventions stopped, e.g. blood tests and intravenous lines, unless needed
  • Resuscitation status agreed and recorded
  • Syringe driver considered and set up, if available
  • Spiritual needs of the child and family assessed
• Communication with the family:
  • Who makes decisions within the family?
  • How can they be contacted?
• Communication with other professionals:
  • Who are the key professionals involved?
  • How can they be contacted?
• Out of hours emergency details (if the child is being cared for at home):
  • Who will the family call in an emergency?
  • What will happen?
  • Do the family know what to do?
  • Have they got enough medication?
  • Do they know how to manage the situation if the child is dying?

See Appendix J for terminal care plan checklist.

11-13 What should be considered for the funeral and bereavement?

A number of important issues need to be addressed:

• Funeral policy
• Burial or cremation plan
• Memory making
• Bereavement follow-up. Who will call and counsel the family?
• Support for siblings, grandparents and other close family members
• Developmentally appropriate therapies for children (siblings), such as art, play or music therapy.

11-14 What social concerns may arise?

Do not forget the following:

• Certificates may be needed for the employers of family members, such as compassionate leave forms
• Financial constraints such as funeral costs and loss of grant money
• Family conflicts.

11-15 What organisation tasks surrounding a death should we consider?

Important organisation tasks are:

• Death certificates
• Legal requirements to certify a death
• What are the local policies surrounding the death of a child?
• Need for a medico-legal post-mortem?
• Repatriation of the child’s body to another country
• Transport arrangements for the family to get home.

Many of these tasks can be considered before the child dies and important information can be included in the terminal care plan.

Note

There may be insufficient time to develop an advance care plan. A terminal care plan will need to be developed in cases of late referral to palliative care when the child is close to death.

Managing distressing symptoms at the end-of-life

11-16 How should distressing symptoms be managed at the end-of-life?

The five golden rules of end-of-life care are:

1. Don’t panic
2. Thorough assessment
3. Listen and communicate effectively
4. Hope for the best but prepare for the worst
5. Treat what you can treat.

11-17 What are the aims of end-of-life symptom control?

The aims of symptom control at the end-of-life include:

• To relieve the child’s suffering and distress
• To minimise the child’s pain and fear
• To neither delay nor speed up the death but to keep the child comfortable
• To reduce the distress and anxiety of family members.

You may need to explain to the family that the underlying disease process is what will result in the death of the child, not the symptom relief medication.

The main aim of end-of-life symptom control is to minimise the child’s pain and suffering and reduce the distress to the child and family.

11-18 How should pain be managed at the end-of-life?

We should recognise that not all deaths are physically painful. However, to effectively manage a child’s pain at the end-of-life the following should be considered:

• Achieve rapid pain control using the WHO pain ladder as a guide.
• Choose pain medication appropriate to the child’s needs and the underlying causes of pain.
• Opioids (morphine or other opium-like drugs) are a well-studied, acceptable and effective medication to manage pain at the end-of-life.
• Consider the best route for administration of pain medication, for example, the child’s ability to swallow or absorb medications from the gut may be reduced towards the end-of-life.
• If available, set up a syringe driver.
• Severe pain can cause anxiety, in which case it may be necessary to prescribe an anxiolytic such as midazolam (Dormicum). However, pain may look like anxiety so never sedate a child until their pain has been treated.

11-19 What is important to know when using opioids to manage pain at the end-of-life?

It is very important that opioids are used correctly. Many children die with severe pain when these are not well managed:

• The starting dose depends on whether or not the child has received opioids before. Establish if the child is opioid naïve (never had opioids) or already on opioids.
• Titrate the dose rapidly upward until the pain is controlled. The rule of thumb is to increase the dose by 1/3 more than the previous dose if more pain control is needed. Do not be afraid to titrate quickly.
• There is no maximum dose for opioids as the aim is to control pain.
• Provide additional breakthrough doses if using an oral formulation.
• Anticipate the side effects of opioids such as urinary retention, constipation, nausea and vomiting. Manage these side effects quickly if they develop.
• If titrating to pain, opioids do not cause respiratory depression.
• The role of opioids must always be to treat pain and shortness of breath and not to hasten death.

The correct dose of opioids for end-of-life pain needs to be titrated according to the level of pain experienced. Do not be afraid to increase the dose until the pain is controlled.

Note

Consider rotating opioids or adding ketamine for severe, refractory (intractable) pain.

Note

Morphine syrup can also be given rectally or buccally if the child is unable to swallow. Another alternative can be via a nasogastric tube.

11-20 How should shortness of breath be managed at the end-of-life?

There are a number of things that can be done to reduce shortness of breath (dyspnoea) when a child is dying:

• Anxiety can increase shortness of breath and vice versa. Therefore, it is important to manage both together.
• Pharmacological management with low-dose morphine often reduces the panic caused by shortness of breath. Increase the dose of morphine, if needed, or add a benzodiazepine such as lorazepam (Ativan).
• Open a window or use a fan to increase the sensation of air flow towards the child’s face (but not directly aimed at them), move the child into an upright position or get them into the most comfortable position if lying down.
• An oxygen mask or nasal cannula may provide reassurance and some comfort to the child but is unlikely to relieve the shortness of breath.

A low dose of morphine can often reduce shortness of breath.

11-21 What are some of the underlying causes of restlessness and agitation?

Common causes include:

• Uncontrolled pain
• Anxiety
• Hypoxia (low oxygen) or hypercapnoea (high carbon dioxide)
• Urinary retention
• Constipation
• Infection, especially pneumonia.

11-22 How should restlessness and agitation be managed at the end-of-life?

To manage agitation and restlessness at the end-of-life:

• Rule out possible causes of agitation and treat accordingly
• Address reasons the child might be anxious or afraid
• Haloperidol is the medication of choice to treat restlessness and agitation and is also effective if the child is hallucinating
• Sedation with benzodiazepines, such as midazolam (Dormicum), may be needed to manage sudden agitation and restlessness.

It is important to look for and address underlying causes of agitation.

11-23 What is the ‘death rattle’?

The ‘death rattle’ is the noisy respiration that occurs in the final days or hours in semi or unconscious patients who are unable to swallow saliva or cough out secretions. It can be distressing for the family to hear, who may think the child is choking or drowning in their own secretions. It is important to reassure the family that the noise is more distressing to them than to their child.

11-24 How should excessive secretions be managed at the end-of-life?

Excessive secretions can be managed by doing the following:

• Position the child by sitting them up, or turning them to one side if lying, to facilitate drainage of secretions. Avoid suctioning if possible.
• IV hyoscine butylbromide (Buscopan) or sublingual (under the tongue) atropine may be used to decrease secretions and prevent the ‘death rattle’ due to excessive secretions.
• Stop or reduce intravenous fluids, if possible, as fluid overload could increase the amount of secretions produced.
• Treat pulmonary oedema with furosemide (Lasix).

11-25 What could cause seizures at the end-of-life?

Seizures (fits) may occur at the end-of-life and can be distressing for the family to witness. They may be due to:

• The primary underlying illness
• Metabolic and electrolyte problems
• Hypoxia
• Acidosis
• Hypoglycaemia
• Liver or renal failure.

11-26 How should seizures be managed at the end-of-life?

Seek treatable underlying causes. Prepare and counsel the family on how to manage seizures in the event of these occurring at home and provide them with the appropriate medication. Medicines could include:

• Buccal midazolam (Dormicum) or rectal diazepam (Valium) that can be administered immediately, even at home
• A midazolam infusion may need to be started if the seizures continue.

11-27 How should massive haemorrhage be managed at the end-of-life?

Although this is rare, some conditions may cause a child to bleed excessively at the end-of-life. This can be very distressing for the child and for the family. The family should be adequately prepared to manage the situation, as the child is likely to die quickly. Haemorrhage may present as haemoptysis (coughing up blood), upper gastrointestinal bleeding (vomiting blood) or bleeding from the mouth or nose (mucosal bleed).

A plan should be in place if haemorrhage is expected:

• Tranexamic acid may be used as prophylaxis against bleeding
• Keep nasal plugs available for epistaxis (nose bleeding)
• Give sedation if the child is awake and afraid
• Use dark sheets, towels and pyjamas to lessen the appearance of blood.

Management at the end-of-life

11-28 How should fluids and feeds be provided at the end-of-life?

When the child is no longer able to swallow, certain decisions need to be made about fluids, feeds and medication. Children often lose their thirst and appetite towards the end-of-life, and their metabolic needs decrease. Management options include:

• Ideally, give fluids orally rather than through an intravenous line
• Do not feed the child if they are not hungry.

Do not force a dying child to eat or drink.

Note

Starvation causes ketosis which leads to the release of endogenous endorphins, which diminish hunger, cause euphoria and enhance the effects of analgesia medications. Providing dextrose-containing IV fluids may prolong the dying process so is not recommended.

11-29 How do you manage the use of prescribed medications at the end-of-life?

This will depend on the individual child:

• Aim to minimise the burden of treatment and only give treatment when needed.
• Stop supplements or any medications which will not affect the child’s outcome.
• Rationalise and continue analgesia and symptom control drugs.
• Review the use of medications that address the underlying condition, such as diuretics for heart failure, as they may still be useful at the end-of-life.

Keep administering the useful medications, discontinue the rest.

11-30 How should medications be administered at the end-of-life?

Intravenous access may be very difficult, particularly towards the end-of-life. Multiple IV access attempts can also cause unnecessary suffering. Therefore, consider alternative routes, such as oral (if tolerated) or via nasogastric tube, buccal, transdermal patches, subcutaneous infusions or rectally. Avoid intramuscular injections if possible. Use the best available route for the drug and the child.

11-31 What is a syringe driver?

A syringe driver is an infusion pump that delivers a constant amount of medication subcutaneously through a butterfly needle over a 24 hour period. Its purpose is to assist with pain and symptom control when the patient is unable to tolerate oral medications or when the level of consciousness is decreasing. This is a very useful method of providing a continuous infusion.

A syringe driver delivers a continuous infusion of medication subcutaneously through a butterfly needle.

11-32 Which medications can be used in a syringe driver at the end-of-life?

The following medications can be used:

• Opioids, e.g. morphine sulphate
• Anti-emetic, e.g. haloperidol, metoclopramide
• Sedatives, e.g.midazolam and phenobarbitol
• Anti-cholinergic, e.g hyoscine butylbromide
• Corticosteroids, e.g. dexamethasone
• Anti-histamines, e.g. promethazine

11-33 Which medications should not be used in a syringe driver?

The following medications should not be used in a syringe driver:

• Diazepam (Valium) as it is oily and too thick to pass through a narrow needle or cannula
• Chlorpromazine (Largactil) as it can cause painful subcutaneous inflammation
• Prochlorperazine (Stemetil) as it can cause subcutaneous inflammation.

11-34 Which medications are compatible in the syringe driver?

The following medications can be combined in one syringe:

• Morphine sulphate
• Haloperidol
• Hyoscine butylbromide
• Midazolam
• Metoclopramide.

Note

Dexamethasone can be added as long as the solution is diluted with sterile water. It is preferable for Dexamethasone to be given through a separate syringe driver.

See appendix I for instructions on the use of a syringe driver.

Supporting the family at the end-of-life

11-35 How can you support the family as the child enters the end-of-life phase and dies?

Supporting the family can include the following:

• If in a hospital or hospice, there should be no restrictions to visiting for close family and friends. Check with the family who else should be allowed to visit.
• Reassure the family that they are doing a good job of caring for their loved one.
• Explain that their child’s breathing may change, become laboured or sound like they are gasping for air but that this is part of the normal dying process.
• Give information in advance about the procedures to be followed when the child dies to help relieve some of their anxiety.
• Family members need to be allowed to spend time alone with the patient during the dying phase, encouraged to say goodbye and even be encouraged to give the child permission to ‘let go’ and tell them that ‘it’s okay for them to die’.
• Explain what you are doing throughout the time to help allay fears.

Provide the family with information in advance about the procedures to be followed when the child dies and explain what you are doing at the time to help allay fears.

11-36 What information does the family need to know that death has occurred?

It can be difficult to always know when death has occurred, especially if the child has had prolonged Cheyne-Stokes (breathing where they stop and start) for a few hours or days. Therefore, it is important to explain that the following will mean death has occurred:

• There is no breath or heartbeat
• Eyes do not move, even if they are open
• Pupils may be large (dilated)
• Mouth may fall open as the jaw relaxes
• There may be a release of bowel or bladder contents.

Death has occurred when there is no breathing or heartbeat and the eyes do not move if they are open.

Note

If the family want to spend time with their child’s body for a period after the child has died it is important to inform them of the changes that will happen to the body once death has occurred and reassure them that these changes are normal.

11-37 How can you support the family in the hours after death has occurred?

The family is likely to need a lot of reassurance and support and you should be prepared to deal with the family’s grief which may manifest as guilt, anger, hysteria, sadness or even physical symptoms, such as fainting when the child dies.

It is important to allow the family some time with their child’s body after the death. The family should be allowed to follow cultural practices regarding preparation of the body before it is taken away so that they have the opportunity to express their feelings and say goodbye. Ask the family if they want to take a lock of hair or hand and foot prints as part of memory making. Assist the family with the completing of the required documents, such as a notification of death form, as well as contacting the funeral parlour that will be collecting the body.

Allow the family time to stay with the child’s body after death has occurred in order to express their feelings and say goodbye.

Case study 1

A 4-year-old girl with a complex congenital cardiac abnormality has been admitted to your ward. The cardiac condition is inoperable, and she has been on diuretics her whole life. She used to be a happy, playful child, but now prefers to lie in bed and watch TV or sleep. She does not engage much but cries for her mother if she is not nearby. For the past few days she has eaten very little, and passes small stools and scanty volumes of urine, despite diuretics. Now she starts to refuse her meals, and gags when given her vitamins. The nursing staff are worried that she is not getting enough fluids. When she tries to get out of bed she becomes agitated and short of breath, and cries if any of the doctors enter her room.

1. What are the signs that this girl may be dying?

She does not want to eat, has decreased urine output, gets short of breath on minimal effort and sleeps a lot. In addition she has lost interest in her surroundings, shows withdrawn behaviour, is afraid and cries for her mother.

2. Why might the girl become agitated?

Possible causes include shortness of breath, constipation, uncontrolled pain or fear.

3. Should a nasogastric tube be inserted for feeds?

No. She should be fed or given fluids orally, and only when hungry or thirsty. Forcing her to eat or drink can cause abdominal discomfort, nausea and vomiting.

4. Should intravenous fluids be given if she will not eat?

No. Inserting an intravenous line is painful, and intravenous fluids will only be harmful to a child in heart failure. Providing dextrose may prolong the dying process.

5. What about her vitamins?

These are not useful at the end-of-life. Rather give the medications that will be helpful such as her pain medication.

Continuation of case study 1: You arrive at work the next morning and find that the girl’s monitors are all alarming, and the other patients in the ward are complaining about the noise. When you see the girl, she is unresponsive and breathing shallowly. The doctor on duty is preparing to resuscitate her if she stops breathing. Her mother is screaming and confused about what is going on.

6. What could have been done to avoid this situation?

Advance care planning, with a plan for end-of-life care and limitations on resuscitation should have been made. The child could have been moved to a private ward or space and the child and her family should have been counselled and prepared for her death.

7. What can you still do?

A number of steps need to be taken:

• Switch off the monitors.
• Create a private area for the child and her family.
• Calmly explain to the child’s mother that she is dying and is not suffering.
• Assist by calling the family members who should be present.
• Counsel the parents and family, or just be there with them.
• Avoid painful procedures or anything which will prolong the dying process.

Case study 2

A 12-year-old boy has leukaemia which has not responded to chemotherapy. He is admitted with severe pain for end-of-life care. He has been on morphine for several months and the doctor feels his dose is already too high, so is reluctant to increase it. As a result, he cries for his mother at night, sleeps poorly and keeps begging to go to his home in a far-away rural area.

1. Is his pain only physical?

No. Although the physical pain is severe, the boy also has emotional (psychological) and social pain. It is important to address these issues as part of his ‘total pain’ management.

2. Should this boy be allowed more morphine?

Absolutely! There is no maximum dose of opioids, and the dose he is already on should be titrated up until his pain is controlled.

3. How should the dose of morphine be titrated upwards?

Increase the dose by 1/3 of the previous dose. This can be done quickly, even from one dose to the next.

4. The boy was previously able to swallow his medicine, but now finds it too difficult and vomits quite often. How else can you give the morphine?

Opioids can be given intravenously, subcutaneously or via patches.