Neurological symptoms will vary with the type and location of the tumour. In the palliative care setting, neurologic symptoms may be caused by a variety of factors including primary and metastatic malignancies, neurodegenerative disease and the side effects of medications. In many cases there may not be a definitive treatment available although symptoms can usually be controlled. Central nervous system tumours can also arise as metastases of other malignancies such as lymphoma. Cancer involving the central nervous system is the second most common paediatric malignancy with the most common tumours being astrocytomas and medulloblastomas. Treatment should take into account the underlying disorder and the site of any lesion or lesions.
In this section.
Diazepam should NOT be given intramuscularly or subcutaneously as it is irritant to tissue.
If the child is unable to tolerate either oral or rectal administration of anticonvulsants, midazolam can be given either IV or SC. For emergency seizure management the dose is Midazolam 0.15 mg/kg IV or SC stat then 2 micrograms/kg/min increasing by 2 micrograms/kg/min until seizures cease (max 24 micrograms/kg/min) 3.
Children with neurodegenerative conditions tend to have protracted courses. For some children a specific cause can not be identified. Individually, neurodegenerative diseases are rare but as a group they account for a large proportion of children who require palliative care. The aetiology of neurodegenerative disorders is varied but metabolic and other genetic abnormalities are major causes. Their clinical condition then deteriorates inexorably and many children spend long periods in states of high dependency. They are often well at the time of diagnosis with the slow and insidious onset of symptoms.
Early recognition and treatment are important. Often there is a prodrome in the form of restlessness, sleep disturbance, irritability and anxiety. Management includes.
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Clarity around the goals of care is vitally important in this setting as achieving adequate symptom control may reduce the ability of the child to interact. A history of open, clear communication will facilitate appropriate management and decision making. In some cases however, the adequate control of symptoms requires the use of medications and doses which reduce the child's conscious state. In this setting, the goals of alleviating suffering and facilitating the best possible interaction between child and family may come into conflict. If symptoms such as agitation become refractory the question of sedation for the child will arise. For some parents, being able to interact with their child is fundamental to quality of life and they become understandably concerned if this becomes impossible. A crucial factor here is the family's prior experience with the health care system.
Palliative care providers have particular expertise in supporting families through this difficult time. Families may need reassurance that restlessness and agitation are often a part of the dying process and also need to understand that while difficult to witness, it may not be troubling the child. Restlessness and agitation are not uncommon in the terminal phase of illness and while there may be no apparent cause, uncontrolled pain, hypoxia, anxiety and medications can contribute to the development of these symptoms. Management centres around treatment of the underlying cause (if appropriate), nursing the child in a quiet and safe environment and using medications where necessary. Support in the home setting can be provided by the family doctor and/or domiciliary nursing staff.
1 Woodruff R. Asperula, Melbourne 2002. Symptom control in advanced cancer (2nd Edition).
It is postulated that the major cause is accumulation of opioid metabolites, but the dose at which it occurs varies widely. It is also a recognised side effect of opioid use, particularly pethidine 1 and is more common where the patient has renal impairment. Myoclonus, the brief and irregular contraction of all or part of a muscle, can occur as a result of immobility, pain, or other sensory stimulation.
Compression of the spinal cord may develop in the setting of childhood cancer. Early recognition and treatment may avoid such a devastating impact on a child's quality of life. It may occur suddenly and has the potential to leave the patient disabled, dependent and incontinent.
Corticosteroids are frequently used in the management of vomiting and headache experienced by adult patients with raised intracranial pressure. Caution is exercised in children however as they appear to be more prone to the side effects of this group of medications.
The parenteral preparation can be administered directly onto the buccal mucosa or intranasally in a dose of 0.3-0.5mg/kg/dose (max 10mg). 4. Midazolam is an alternative anticonvulsant.
Sublingual absorption is rapid and the dose can be repeated. In circumstances where rectal administration is difficult or undesirable, clonazepam 0.01 mg/kg can be used orally. 2 Clonazepam is available in an oral drop preparation such that extremely small volumes can be given even if the child is having a seizure.
If the infant becomes drowsy or is unrousable anticonvulsants should be ceased, if subsequent fitting occurs then these should be managed using clonazepam. It is important to understand that the management of neonatal patients in the community setting is complex and should be undertaken with the support of a neonatal unit and skilled neonatal paediatricians. In infants who are offered comfort feeding only, seizures are rarely a problem.
Therapeutic Guidelines. Melbourne 2002. 4 Therapeutic Guidelines: Neurology.
Muscle spasm, the involuntary and painful contraction of skeletal muscle (usually flexor), generally occurs in the context of upper motor neuron lesions and has the potential to cause significant discomfort. The problem may be exacerbated by pain, constipation or other factors. Therapeutic options include.
In the setting of severe pain, sedation does not address the fundamental problem and should be used only in conjunction with interventions directed specifically at alleviating pain. Morphine and midazolam combined in a subcutaneous infusion is an effective option in this setting.
Lloyd Shield, neurologist in reviewing the manuscript. The authors would like to acknowledge the kind assistance of Dr. Peter McDougall, neonatologist and Dr.
Where the child is in the terminal phase of illness, it may not be appropriate to seek and treat an underlying cause. Even where the child seems unable to respond, they may continue to be aware of their surroundings and so benefit from having their parents cuddle and speak to them. Parents will need reassurance about what is happening and guidance in terms of how they can help their child.
Diazepam 0.3-0.5mg rectally 3 is effective. It is extremely helpful to have a supply of anticonvulsant medication in the home for emergency use by the family or support services if the child has a seizure. Diazepam should not be given rectally in children at risk of neutropenia. Rectal administration is a technique easily learnt by families and kits are available through the pharmacy at the RCH.
3. The table below shows drugs in order of preference, loading doses if appropriate and maintenance doses. However, due to differing pharmacokinetics, drugs and dosages differ from those used in older children. As with older children, the oral, oro-gastric or naso-gastric route is preferred for administration of medications. Phenobarbitone is the drug of choice in controlling neonatal seizures. Seizure management in neonates deserves special mention.
If required it may be appropriate to reduce the dose of opioid, change the route of administration or change to another drug. Benzodiazepines are useful in the management of myoclonus with alternatives including. Treatment of myoclonus may not be necessary if it is not troubling the patient.
The pain tends to be sharp and more severe when lying down or coughing. Sensory symptoms and signs in the legs develop next followed by motor impairment and sphincter disturbances. The development of back pain or a change in the character of existing back pain may indicate impending spinal cord compression. Vertebral tenderness may be found on examination at this stage. The outcome of treatment will depend on the degree of neurological impairment at the time of intervention so the child's oncologist should be consulted as soon as cord compression is suspected.
Children with primary or metastatic tumours of the brain, metabolic disturbances or genetic disorders may be at increased risk of seizures. Treatment can be separated into two components: emergency management and prophylactic treatment. It can be distressing for family members to witness a seizure and parents may find it helpful to have general information, as well as a plan of action, should a seizure occur.
Therapeutic Guidelines. Melbourne 2000. 2 Therapeutic Guidelines: Palliative care.
*Midazolam is an alternative and can be given orally, or by continuous infusion sc or iv Chlorpromazine is another alternative and may be given IV/IM/o/PR *Tolerance develops to benzodiazepines and doses may require escalation over time.
Medications used for emergency management of seizures do not have a prolonged effect, and if fitting is likely to be an ongoing problem, maintenance treatment is indicated. Midazolam has the advantage of being compatible with morphine and they can be combined in the same syringe driver thus making delivery simpler. In the palliative care setting phenytoin, phenobarbitone and carbamazepine may be helpful and can be given orally. For patients who cannot tolerate or absorb oral medications midazolam can be administered.
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Paediatric Pharmacopoeia (13th Edition). Royal Children's Hospital Melbourne 2002. 3 Kemp CA, McDowell JM (Eds).
Delirium is characterised by.
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