ABC of palliative care: Special problems of children
BMJ 1998; 316 doi: https://doi.org/10.1136/bmj.316.7124.49 (Published 03 January 1998) Cite this as: BMJ 1998;316:49
- Ann Goldman
The death of a child has long been acknowledged as one of the greatest tragedies that can happen to a family, and care for seriously ill children and their families is central to paediatrics. It is only recently, however, that the needs for palliative care of children with life limiting illnesses and their families have been considered as a speciality within paediatrics: the most suitable approaches to care are still being developed, and the provision of services nationally is uneven and sometimes inadequate.
Numbers of children with life limiting illness
Annual mortality from life limiting illnesses
1 per 10 000 children aged 1-17 years
Prevalence of life limiting illnesses
10 per 10 000 children aged 0-19 years
In a health district of 250 000 people, with a child population of about 50 000, in one year
5 children are likely to die from a life limiting illness—Cancer (2), heart disease (1), other (2)
50 children are likely to have a life limiting illness, about half of whom will need palliative care at any time
Which children need care?
Fortunately, deaths in childhood that can be anticipated, and for which palliative care can be planned, are rare. A recent joint report by ACT (Association for Children with Life Threatening or Terminal Conditions and their Families) and the Royal College of Paediatrics and Child Health offers the most up to date information about epidemiology (see box of further reading).
Paediatric palliative care may be needed for a wide range of diseases, which differ from adult diseases and many of which are rare and familial. The diagnosis influences the type of care that a child and family will need, and four broad groups have been identified.
Groups of life limiting diseases in children
Palliative care may be needed from infancy and for many years for some children, while others may not need it until they are older and only for a short time. Also the transition between aggressive treatments to cure or prolong good quality life and palliative care may not be clear—both approaches may be needed in conjunction, each becoming dominant at different times.
Aspects of care in children
Child development
An intrinsic aspect of childhood is children's continuing physical, emotional, and cognitive development. This influences all aspects of their care, from pharmacodynamics and pharmacokinetics of drugs to their communication skills and their understanding of their disease and death.
Care at home
Most children with a life limiting disease are cared for at home. Parents are both part of the team caring for the sick child and part of the family and needing care themselves. As their child's primary carers, they must be included fully in the care team— provided with information, able to negotiate treatment plans, taught appropriate skills, and assured that advice and support is accessible 24 hours a day.
Assessing symptoms
Methods of assessing pain in children
Body charts
Faces scales
Numeric scales
Colour tools
Visual analogue scales
Observation of behaviour
Assessing symptoms is an essential step in developing a plan of management. Often a picture must be built up through discussion with the child, if possible, combined with careful observations by parents and staff. There are formal assessment tools for assessing pain in children that are appropriate for different ages and developmental levels, but assessment is more difficult for other symptoms and for preverbal and developmentally delayed children.
It is also important to consider the contribution of psychological and social factors for a child and family and to inquire about their coping strategies, relevant past experiences, and their levels of anxiety and emotional distress.
Managing symptoms
Many of the symptoms that children suffer and the approaches to relieving them have not been studied formally. Until definitive information becomes available, treatment is often based on clinical experience and adapted from general paediatric practice and palliative care of adults.
Faces scale used to measure pain effect in children (adapted from McGrath P. Pain in children. Nature, assessment and treatment. New York: Guildford Press, 1990)
Many of the drug doses and routes used in palliative care are not licensed for children, and responsibility lies with the clinician prescribing them. In all situations the management plan should consider both pharmacological and psychological approaches along with practical help.
Children often find it difficult to take large amounts of drugs, and complex regimens may not be possible. Doses should be calculated according to a child's weight. Oral drugs should be used if possible, and children should be offered the choice between tablets, whole or crushed, and liquids. Long acting preparations are helpful, reducing the number of tablets needed and simplifying care at home. If an alternative route is needed some children find rectal drugs acceptable; they can be particularly useful in the last few days of life. Otherwise, a subcutaneous infusion can be established or, if one is in situ, a central intravenous line can be used. Parents are usually willing and able to learn to refill and load syringes and even to resite needles.
Specific problems
Pain
Children and pain
Children's nervous systems do perceive pain
Children do experience pain
Children do remember pain
Children are not more easily addicted to opioids
There is no correct amount of pain or analgesia for a given injury
The myths perpetuating the undertreatment of pain in children have now been rejected. However, most doctors lack experience in using strong opioids in children, which often results in excess caution. The World Health Organisation's three step ladder of analgesia is equally relevant for children, with paracetamol, dihydrocodeine, and morphine sulphate forming the standard steps.
Opioids—Laxatives need to be prescribed regularly with opioids, but children rarely need antiemetics. Itching with opioids in the first few days is quite common and responds to antihistamines if necessary. Many children are sleepy initially, and parents should be warned of this lest they fear that their child's disease has suddenly progressed. Respiratory depression with strong opioids used in standard doses is not a problem in children over 1 year old, but in younger children starting doses should be reduced.
Analgesic doses
Paracetamol
Oral dose 15 mg/kg every 4-6 hours
Rectal dose 20 mg/kg every 6 hours
Maximum dose 90 mg/kg/24 hours, 60 mg/kg/24 hours in neonates
Dihydrocodeine
Ages <4 years 500 μg/kg orally every 4-6 hours
Ages 4-12 years 500-1000 μg/kg orally every 4-6 hours
Morphine
Immediate release preparations
Ages <1 year 150 μg/kg orally every 4 hours
Ages 1-12 years 200-400 μg/kg orally every 4 hours
Ages >12 years 10-15 mg orally every 4 hours
Titrate according to analgesic effect and provide laxatives
12 hourly preparations
Ages <1 year 500 μg/kg orally every 12 hours
Ages 1-12 years 1 mg/kg orally every 12 hours
Ages >12 years 30 mg orally every 12 hours
These are guidelines to starting doses, but many patients may start at higher doses after titration with immediate release morphine preparation every 4 hours
Diamorphine
A third of total 24 hour dose of oral morphine
Subcutaneous 24 hour infusion
Adjuvant analgesics—Non-steroidal anti-inflammatory drugs are often helpful for musculoskeletal pains in children with non-malignant disease. Caution is needed in children with cancer and infiltration of the bone marrow because of an increased risk of bleeding. Neuropathic pain may be helped by antiepileptic and antidepressant drugs. Pain from muscle spasms can be a major problem for children with neurodegenerative diseases and may be helped by benzodiazepines and baclofen. Headaches from raised intracranial pressure associated with brain tumours are best managed with gradually increasing analgesics. Although corticosteroids are often helpful initially, the symptoms soon recur and increasing doses are needed. The considerable side effects of corticosteroids in children—rapid weight gain, changed body image, and mood swings—usually outweigh the benefits. Headaches from leukaemic deposits in the central nervous system respond well to intrathecal methotrexate.
Feeding
Being unable to nourish their child causes parents great distress and often makes them feel that they are failing as parents. Sucking and eating are part of children's development and provide comfort, pleasure, and stimulation. These aspects should be considered alongside a child's medical and practical problems with eating. Children with neurodegenerative disorders or brain tumours are particularly affected. In general, nutritional goals aimed at restoring health are secondary to comfort and enjoyment, although assisted feeding, via a nasogastric tube or gastrostomy, may be appropriate for those with slowly progressive disease.
The emetic process—pathways of emesis and the neurotransmitters involved
Nausea and vomiting
These are common problems. Antiemetics can be selected according to their site of action and the presumed cause of the nausea. In resistant cases combining a number of drugs that act in different ways or adding a 5-HT3 antagonist can be helpful. Vomiting from raised intracranial pressure should be managed with cyclizine in the first instance.
Neurological problems
Watching a child have a seizure is extremely frightening for parents, and they should always be warned if it is a possibility and advised about management. A supply of rectal diazepam at home is valuable for managing seizures. Subcutaneous midazolam can enable parents to keep at home a child with severe repeated seizures. Children with neurodegenerative disease will often already be taking maintenance antiepileptic drugs, and the dose and drugs may need adjusting as the disease progresses.
Agitation and anxiety may reflect a child's need to express his or her fears and distress. Drugs such as benzodiazepines, methotrimeprazine, and haloperidol may help to provide relief, especially in the final stages of life.
Uses of antiemetic drugs;
Support for the family
Support that every child and family should expect
To receive a flexible service according to a care plan based on individual assessment of needs, with reviews at appropriate intervals
To have a named key worker to coordinate their care and provide access to appropriate professionals
To be included in the caseload of a paediatrician in their home area and have access to local clinicians, nurses, and therapists skilled in children's palliative care and knowledgeable about services provided by agencies outside the NHS
To be in the care of an identified lead consultant paediatrician expert in individual child's condition
To be supported in day to day management of child's physical and emotional symptoms and to have access to 24 hour care in the terminal stage
To receive help in meeting the needs of parents and siblings, both during child's illness and during death and bereavement
To be offered regular respite, including nursing care and symptom management, ranging from parts of a day to longer periods
To be provided with drugs, oxygen, specialised feeds, and all disposable items such as feeding tubes, suction catheters, and stoma products through a single source
To be provided with adaptations to housing and specialist equipment for use at home and school in an efficient and timely manner without recourse to several agencies
To be helped in procuring benefits, grants, and other financial assistance
The needs of children with a life threatening illness and their families are summarised in the report by ACT and the Royal College of Paediatrics and Child Health. Families need support from the time of diagnosis and throughout treatment as well as when the disease is far advanced. Professionals must be flexible in their efforts to help. Each family and individual within a family is unique, with different strengths and coping skills. The needs of sibling and grandparents should be included.
The family of a child with an inherited condition have additional difficulties. They may have feelings of guilt and blame, and they will need genetic counselling and information about prenatal diagnosis in the future. When an illness does not present until some years after birth several children in the same family may be affected.
Families who maintain open communication cope most effectively, but this is not everyone's pattern. Children almost always know more than their parents think, and parents should be encouraged to be as honest as they can. Play material, books, and other resources can be supplied to help with communication, and parents can be helped to recognise their children's non-verbal cues.
Sick children need the opportunity to maintain their interests and to have short term goals for as long as possible. Education is an essential part of this, as it represents their normal pattern and continues relationships with their peers. Providing information and support to teachers facilitates this.
Bereavement
Grief after the death of a child is described as the most painful and enduring. It is also associated with a higher risk of pathological grief. Parents suffer multiple losses. Siblings suffer too and may have difficulty adjusting; they often feel isolated and neglected, as their parents can spare little energy or emotion for them.
Communicating with children about death
Factors to consider
Child's level of understanding
Of illness
Of death
Of own situation
Child's experience
Family's communication pattern
Methods of communication
Verbal
Play
Drama
Art
School work
Stories
The loss of a child
Multiple losses for parents
The child who has died
Their dreams and hopes
Their own immortality
Their role as parents
Stress on marriage
Change in family structure
Grief of siblings and grandparents
Helping the bereaved family involves
Support and assessment through the tasks of normal mourning—Most families do not need specialist counselling but benefit from general support and reassurance, supplied if possible by those who have known the family through the illness
Information—Such as support groups and the Child Death Helpline (freephone 0800 282986); many parents value the opportunity of talking with others who have also experienced the death of a child
Referral for specialist bereavement counselling if needed
Gradual withdrawal of contact.
Further reading
ACT, Royal College of Paediatrics and Child Health guide to the development of children's palliative care services. 1997. Available from ACT, 65 St Michael's Hill, Bristol BS2 8DZ (Tel 0117 922 1556, Fax 0117 930 4707.)
Royal College of Paediatrics and Child Health. Prevention and control of pain in children: a manual for health care professionals. London, BMJ Publishing, 1997. (Tel 0171 383 6185, Fax 0171 383 6662.)
Notes
Ann Goldman is CLIC consultant in palliative care at Great Ormond Street Hospital for Children, London.
The ABC of palliative care is edited by Marie Fallon, Marie Curie senior lecturer in palliative medicine, Beatson Oncology Centre, Western Infirmary, Glasgow, and Bill O'Neill, science and research adviser, British Medical Association, BMA House, London. It will be published as a book in June 1998.