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Respiratory symptoms

  • Dyspnoea

    Dyspnoea, the sensation of shortness of breath, is a relatively common symptom in children receiving palliative care. It is often accompanied by considerable anxiety in both the child and the family. Dyspnoea is a subjective phenomenon and reported symptoms may not match respiratory signs or pulmonary function tests. A visual analogue scale may be a useful way of measuring dyspnoea and monitoring the response to treatment.

    Children with malignancies

    Dyspnoea occurs in 40-65% of children with malignant conditions ®1,2,3 and may occur as a consequence of

    • Tumour involvement of lung parenchyma- primary or metastatic
    • Tumour involvement of airways leading to obstruction/collapse
    • Pleural effusion/haemothorax/pneumothorax
    • Pericardial effusion
    • Infection
    • Effects of radio/chemotherapy
    • Anaemia 
    • Respiratory muscle weakness
    • Hepatomegaly 
    • Cardiac failure as a consequence of chemotherapy
    • Pain
    • Anxiety

    The sudden development of dyspnoea, headache, swelling and distension of the veins of the face, chest and upper limbs suggests the development of superior vena caval obstruction. A paediatric oncologist should be consulted immediately.

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    Children with neurodegenerative conditions

    Children with neurodegenerative conditions may experience dyspnoea as a consequence of

    • Respiratory muscle weakness
    • Scoliosis
    • Cardiac failure (eg. cardiomyopathy in Duchenne muscular dystrophy)
    • Infection (eg. Pneumonia)
    • Lung collapse
    • Anxiety

    Initially hypoventilation may only be present during sleep giving rise to symptoms such as irritability, nausea, headache and daytime somnolence. Patients with these symptoms may benefit from non-invasive ventilation overnight.

    Admission to hospital with an episode of respiratory compromise due to pulmonary infection (commonly complicated by lung collapse) should prompt discussion of decision making options for this episode. Recovery should be a time for reflection about the options available for future episodes in the context of the child's quality of life.

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    Cystic fibrosis

    Children with cystic fibrosis (CF) experience progressive impairment of pulmonary function and pancreatic malabsorption. The condition is usually diagnosed in infancy and although, no cure exists, a multitude of treatment strategies are available to relieve symptoms and maximise longevity. These include chest physiotherapy, antibiotics, pancreatic enzyme supplementation, and attention to nutrition. The majority of children now survive to adulthood with a median age of death of 31 years ®4. Insulin dependent diabetes develops in 25% of patients who reach adulthood ®5. Other complications include pneumothoraces, haemoptysis, hepatic cirrhosis, arthropathy and ileal obstruction.

    In the context of gradual loss of lung function, there is usually no clear transition to a totally palliative focus of care in cystic fibrosis ®6. As with children with neurodegenerative conditions, children admitted to hospital with a severe respiratory exacebation may die during as a result of acute deterioration. More commonly however, they recover, and return to their severely compromised background respiratory state. Commonly, a mixed model of care is adopted in which opiates, anxiolytics and increased emotional and spiritual support is added to the day to day treatment regimen.

    When it is clear that someone is dying, enzyme replacement remains appropriate for symptomatic control of malabsorption and modified physiotherapyis generally useful in managing respiratory symptoms. The decision whether to use antibiotics or not should be consistent with the goals of care.

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    Management of Dyspnoea

    The first principle of management is to identify and treat any underlying cause of dyspnoea. This may not always be possible or appropriate in the setting of palliative care and needs to be considered on an individual basis. Investigation and treatment are generally indicated for children who are otherwise enjoying a reasonable quality of life, providing these measures do not bring significant burdens in the form of pain or lengthy hospital admission. Even quite invasive measures such as drainage of a pleural effusion can be undertaken if the benefits to the child outweigh the burdens given the stage of illness. There may be a role for palliative radiotherapy to reduce the effect of tumour load in children with malignant disease. If bronchospasm is present, bronchodilators may be helpful. Steroids can also be useful in alleviating bronchospasm and may reduce inflammation around pulmonary metastases. They should not be used for long periods however, as side effects such as weight gain and behavioural change can be problematic even in children needing palliative care.

    Some causes of dyspnoea respond to simple measures. Anxious children will benefit from the presence of confident and reassuring family members and staff. Breathing exercises (eg. long, slow breaths), appropriate positioning (ie. upright) and relaxation training may also be helpful.

    Opioids are very effective in treating dyspnoea although there may be no effect on respiratory rate, effort, oxygenation or pC02. They can be commenced at a low dose (half the usual starting dose) and increased as required to reduce symptoms (eg. for a child aged over six months, start with oral morphine 0.1-0.25 mg/kg/dose q4h orally). ®7 Nebulised morphine may be effective in some patients with dyspnoea. It has the advantage of being rapidly effective and produces fewer systemic side effects. The starting dose is 2.5-5 mg morphine (injectable solution) via nebulizer 4/24. ®8 Caution is needed in using nebulised morphine as it can cause bronchospasm in some children.

    Many children will be frightened by dyspnoea. Benzodiazepines may be helpful in conjunction with such measures as relaxation training and guided imagery.

    • Clonazepam 0.01mg/kg orally q8-12h ®9
    • Diazepam 0.05-0.1 mg/kg orally q4-6h ®9
    • Midazolam 8-30 micrograms/kg/hr subcutaneously ®9

    Oxygen is useful in alleviating dyspnoea in some children and can be provided in the home where necessary. The development of headache, nausea, or confusion in the setting of dyspnoea may indicate hypoxia. These symptoms are also consistent with Co2 retention. Oximetry can be used to confirm this in the hospital setting although some children with normal oxygenation may also benefit from oxygen. Many children will use it only intermittently. Use of a portable oxygen concentrator rather than oxygen tanks, at home, is recommended, although the flow rate is limited to a max of 5 L/min via a single concentrator. Two concentrators can be used in parallel to provide a greater flow rate. Concentrators are quite noisy and the use of a long length of oxygen tubing will allow the child some mobility within the house and facilitate the placement of the concentrator away from the living areas of the house. The family should also have at their disposal, portable oxygen tanks that can be used outside the home for short periods. A size "C" cylinder will last 2-3 hours at a flow rate of 2L/min.

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    A symptom that frequently accompanies dyspnoea is cough. Cough can result from irritation to the upper or lower airway, pleura, pericardium and diaphragm. Tenacious or thick secretions can be loosened with nebulised saline thus allowing the child to remove them by coughing. Physiotherapy can also be helpful. For upper airway irritation, it is worth seeing whether cough lozenges or a simple cough linctus can sooth the throat and alleviate a dry, irritating cough. Bronchospasm may also contribute to cough and treatment with salbutamol may be helpful.

    Children with a persistent dry cough may benefit from opioids which work via their action on the central nervous system.
    Eg. For children aged over 2 years codeine orally 0.25-0.5mg/kg/dose q6-8h ®7
    Morphine can also be used.
    Children already receiving morphine for analgesia may need the dose increased.

    Significant coughing can be a very distressing symptom for children. Opioids are generally effective at reducing coughing and the distress associated with coughing.

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    The authors gratefully acknowledge the assistance of Professor Susan Sawyer, Royal Children's Hospital, Melbourne in reviewing this manuscript.

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