Management of the paediatric patient with bronchiectasis is ideally supervised by a multidisciplinary team lead by a paediatric respiratory physician with experience in suppurative lung disease. In most circumstances management can mirror the established cystic fibrosis (CF) care model however specific additional medical and allied health staff may be required to provide overall management of the patient. Ear nose and throat and audiology input into patients with Primary Ciliary Dyskinesia is essential as is the involvement of gastroenterologists and nutritionists in conditions such as inflammatory bowel disease and CF and neurologists and speech therapists in conditions involved with impaired oropharyngeal co-ordination.
General management principles include reduction of mucus production from the damaged area of the lung and optimisation of airway clearance.
Bronchiectatic areas of the lung will usually produce more mucus than unaffected areas of the lung. This excessive production is increased by other insults including smoke exposure and infection.
The principles of treating bronchiectasis in children are similar to that in adults which include:
control of symptoms
improving quality of life
prevention and prompt treatment of exacerbations
preservation of lung function
prevention of secondary complications, such as haemoptysis
To achieve the above, appropriate use of antibiotics and airway clearance techniques as well as attention to general aspects of chronic management are the mainstay of management. Children with a wet cough should be treated with a course of antibiotics which should be repeated if necessary.
However, as children are not little adults, treatment needs to be tailored for the age of the child and the family which includes:
different chest physiotherapy techniques
types and delivery of antibiotics
monitoring strategies (such as type of lung function)
Management strategies include:
While active smoking may be seen in some adolescents and need direct attention many young children with bronchiectasis will have increased symptoms due to secondary smoke exposure in the household. This should be specifically addressed with parents and guardians and referral to adult services for access to smoking cessation services should be encouraged.
It is recommended that all patients with bronchiectasis have regular influenza and pneumococcal vaccinations. Provision of the quadra-valent influenza vaccine to patients with significant bronchiectasis should be considered.
Medications (see Medications for Bronchiectasis)
Antibiotics – based on regular assessment of airway microbiology
Bronchodilators – bronchiectasis is not always associated with reversible airway dysfunction and determination of bronchodilator response in the pulmonary function laboratory should precede institution of long-term bronchodilator therapy.
Corticosteroids – While some conditions resulting in bronchiectasis may respond to steroid treatment including even oral steroid therapy (ABPA) care should be taken in children with bronchiectasis to ensure that regular assessment of the benefit of such therapy is balanced against the potential side effect of prolonged therapy in the paediatric patient.
Mucoactive agents – Improved airway clearance may be achieved with the regular inhalation of mucolytic agents such as hypertonic saline (6 or 7%) or mannitol. The mucolytic therapy rDNase used as a mucolytic agent in CF has not been shown to be generally effective in bronchiectasis caused by conditions other than CF and, given its significant cost, should not be used.
Macrolide therapy – Several studies have shown benefit to the use of azithromycin in retarding the progression of bronchiectasis from conditions as Pseudomonas Aeuriginosa positive CF patients and COPD in adults by preserving lung function and reducing the number of acute exacerbations. Therapy is aimed at a direct anti inflammatory action rather than an antimicrobial action and thus dosing is often taken as single dose daily or three times a week.
For further information see TSANZ Guidelines
Airway clearance program – see Paediatric Airway Clearance Techniques
Action Plan – Patients should always have an updated action plan provided to them after each outpatient appointment, which should include:
Symptoms and management strategies including a complete list prescribed medication to be taken when well
A strategy when unwell including what changes to initiate in prescribed medication and physiotherapy.
Surgery – may be considered in patients:
With localised disease that have not responded to treatment
To remove an obstructing tumour or foreign body
Where bronchial artery embolization is indicated with massive haemoptysis
In severe cases, lung transplantation may be considered.
When bronchiectasis is diagnosed early and managed appropriately in children, the prognosis is excellent. Several studies have shown that lung function can improve and stabilise over the ensuing 5 years. Other studies have also shown that early radiologically-diagnosed bronchiectasis is reversible. Thus it is very important that bronchiectasis is diagnosed early and children receive optimal treatment.
Summary of paediatric bronchiectasis – from kidshealth.org.nz
Bronchiectasis information for schools – from kidshealth.org.nz