Author Alan Peres, RGN, is senior staff nurse, Royal Brompton and Harefield NHS Trust, London
Abstract Peres, A. (2008) Nursing patients with bronchiectasis: part one. Nursing Times; 104: 23, 42-44.
Alan Peres describes the epidemiology and pathophysiology of bronchiectasis, its most common clinical manifestations and the nursing assessment of patients who have it.
Bronchiectasis is defined by Sapey and Stockley (2004) as 'a destructive lung disease characterised by abnormal chronic dilatation of the bronchi associated with a persistent, variable inflammatory process in the lung'. The number of people with the condition is unknown. In the 1940s, the suggested incidence was 1.3 per 1,000 of the population; this figure was based on chest X-rays and, as such, underestimated the true incidence at the time (Sapey and Stockley, 2004). Some studies suggest that a third to half of patients with COPD have concurrent bronchiectasis (Patel et al, 2004; O'Brien et al, 2000), but the true picture is still unknown.
Patients with cystic fibrosis (CF) often develop bronchiectatic airways; CF, however, is a genetic disease while bronchiectasis is considered to be the result of a series of pathological processes and it is often difficult to pinpoint one specific cause of it (Fall and Spencer, 2006).
The respiratory system
The respiratory system is divided into the upper (nose, nasopharynx, oropharynx, larynx and trachea) and lower (bronchi and lungs) respiratory tracts. Its main function is to perform gas exchange, allowing the supply of oxygen to the tissues of the body and the elimination of carbon dioxide. This is an essential part of cell function.
The lungs have the largest surface area of the body in contact with the environment and, as a result, are very susceptible to damage by a variety of agents such as micro-organisms, pollutants and foreign particles, such as dust and pollen (McGowan et al, 2003). In order to prevent damage to the lungs, three main defence mechanisms are used by healthy humans:
Physical defences. These prevent entry of foreign material by filtering processes in the nose and nasopharynx. Aspiration of food and fluids during swallowing is prevented by the closure of the epiglottis. Inhaled foreign material is removed through the action of the cough reflex, mucociliary clearance (Box 1) and alveolar macrophages;
Humoral defences. Proteins such as surfactant, immunoglobulins and enzymes (antiproteases) contained in the respiratory secretions possess antimicrobial properties;
Cellular defences. These are present in the alveoli and include macrophages and neutrophils that protect the lung tissue by ingesting debris and bacteria, which are then removed by mucociliary clearance. These agents initiate an inflammatory process, recruiting cytokines and chemokines, complement components, growth factors, oxygen radicals and proteases.
In bronchiectasis, some of these mechanisms ('host defences') are defective, causing damage to the lung tissue through the following processes.
Box 1. Mucociliary Transport
The bronchial walls have a lining of epithelial cells that secrete mucus, which then sits on top of hair-like structures called cilia. The cilia beat synchronously at 1,000-1,500 strokes per minute and any particles trapped on the mucus are then continuously transported towards the mouth at a rate of 1-3cm per minute. Reaching the trachea and larynx, the mucus and trapped particles are then either swallowed or coughed up. This process is called mucociliary transport or clearance.
Aetiology and pathophysiology
The pathophysiological pattern that characterises bronchiectasis consists of a vicious cycle (Fig 1), as described by Bourke (2003). It is often difficult to identify what happens first in that process, as the interplay of all factors leads to the progression of the disease. Theoretically impaired mucociliary clearance will lead to an increased accumulation of secretions, which become more viscous, exposing the patient to bacterial infections. The infection then causes a marked inflammatory response, not only as part of the body's attempt to eradicate the invasive micro-organisms, but also caused by the presence of the bacteria. This inflammatory response is often ineffective however, and tissue damage, scarring and dilating the bronchi and further impairing mucociliary clearance eventually occurs, completing the cycle. There is often small airways obstruction, slowly leading to fibrosis of bronchioles and loss of respiratory function.
Bronchiectasis may originate from a number of different causes, for example, after a serious infection such as pneumonia, tuberculosis or whooping cough. It can also occur following an insult to the airways such as in aspiration of gastric contents or smoke inhalation, where the damage caused to the epithelium and cilia may allow bacterial growth to take hold.
Diffuse bronchiectasis is generally caused by the impairment of host defences, be they acquired or hereditary, such as Aids or other immunodeficiencies (Shoemark et al, 2006). Localised bronchiectasis, on the other hand, may be found in patients after a bronchial obstruction, for example, by a foreign body. Causes are listed in Box 2. In approximately half of all cases, investigations fail to identify a cause (idiopathic bronchiectasis) and any explanation for the condition is speculative (Pasteur et al, 2000). In children, bronchiectasis is most likely due to congenital defects, aspiration of irritants, immunodeficiencies and mucociliary clearance abnormalities. It was often misdiagnosed as asthma. In a study of 136 children with bronchiectasis, no cause was found in 35 of them (Li et al, 2005).
Box 2. Causative factors in Bronchiectasis
The physician will take a detailed patient history, asking the patient about childhood illnesses, family background of respiratory diseases, patient contact with pollutants or environmental hazards and a history of symptoms. The result of the investigations will then be interpreted. Here the focus is on identifying the cause of bronchiectasis for each particular patient and treating it, if possible. Investigations are listed in Box 3.
Diagnosis may influence management considerably (Li et al, 2005). For example, patients identified with bronchiectasis as a result of CF, may be prescribed medications to treat the specific factors in the disease process in CF such a DNAse. This mucolytic drug is often used in CF to loosen secretions, but is contraindicated in patients with non-CF bronchiectasis.
In the community, those patients who are undiagnosed may initially complain of a severe infection from which it is difficult to recover, followed by recurrent chest infections.
Patients admitted to hospital with bronchiectasis will often present with an acute exacerbation, the main symptoms of which are:
Increased sputum production - sometimes the volume of sputum decreases if it becomes too sticky and difficult to clear;
Pleuritic chest pain.
Patients may also present with other less-common symptoms, including wheeze, rhinosinusitis, joint pains, tiredness, haemoptysis, atelectasis (collapse of all or part of the lung), halitosis, weight loss, difficulty concentrating and depression.
Some complications of bronchiectasis can lead to death. These include respiratory failure, sepsis and cor pulmonale (Wilson, 2003).
Box 3. Investigations in bronchiectasis
When assessing patients with bronchiectasis, nurses must observe their breathing - its pattern, depth, rate and the effort required. There may be audible secretions when they cough or speak; most will have crackles or a wheeze that is audible on auscultation.
The peripheral oxygen saturations of patients - determined by pulse oxymetry - are an important determinant of the need for supplemental oxygen, with a normal range being 96-100% on air. Some patients, such as those with COPD, may retain CO2 more easily, so nurses should ask patients for a previous history of such problems, recurrent morning headaches or daytime somnolence. A normal PaCO2 will be in the range of 4.7-6.0 kPa.
By analysing the colour, amount and consistency of sputum samples, nurses can assess the severity of the infection (Stockley et al, 2001) as well
as how hydrated patients are. These samples may be sent for microbiological cultures and sensitivities and for acid-fast bacilli (AFB), a microscopy technique used to isolate and identify mycobacteria. The sputum may be yellow, green or brown, according to the severity of the infection. Asking patients to collect sputum for 24 hours can provide a good measure of infection and inflammation. There may be a noticeable increase in the amount of secretions produced over a day, with a thicker consistency and darkening being an indication of active infection.
Patients can also be encouraged to keep journals, writing down their symptoms alongside perceived causative and/or alleviating factors (Wilson, 2003). Most commonly, they will report increased productive cough, disturbed sleep pattern, decreased appetite (including feeling nauseated), feeling breathless on minimal exertion, reduced mobility and having difficulty concentrating.
Blood samples will be taken for various laboratory investigations. Some of the most relevant blood tests are C-reactive protein (CRP), differential white-cell count (WCC) and erythrocyte sedimentation rate (ESR). All of these are important markers of inflammation (Wilson et al, 1998) and will be raised in a bacterial infection; as patients get better, these should return to normal levels.
Assessment should also identify the degree to which individuals have been affected by the disease process. Some patients will never have had pleuritic chest pain while, for others, this may be a chronic problem.
By taking into consideration factors such as self-care ability, social support systems and coping mechanisms, nurses will be able to address specific problems and start the discharge-planning process early on. This will buy valuable time to make necessary arrangements for patients.
The successful planning, implementation and evaluation of nursing care will depend on a detailed assessment of the needs of each patient being conducted on admission and throughout the episode of care.
Bourke, S. (2003) Lecture Notes on Respiratory Medicine. Oxford: Blackwell Science.
Fall, A., Spencer, D. (2006) Paediatric bronchiectasis in Europe: what now and where next? Paediatric Respiratory Review; 7: 4, 268-274.
Li, A. et al (2005) Non-CF bronchiectasis: does knowing the aetiology lead to changes in management? European Respiratory Journal; 26: 1, 8-14.
McGowan, P. et al (2003) Crash Course Respiratory System. London: Elsevier Science
O'Brien, C. et al (2000) Physiological and radiological characterisation of patients diagnosed with chronic obstructive pulmonary disease in primary care. Thorax; 55: 8, 635-642.
Pasteur, M. et al (2000) An investigation into causative factors in patients with bronchiectasis. American Journal of Respiratory and Critical Care Medicine; 162: 4 (Pt1) 1277-1284.
Patel, I. et al (2004) Bronchiectasis, exacerbation indices and inflammation in chronic obstructive pulmonary disease. American Journal of Respiratory and Critical Care Medicine; 170: 4, 400-407.
Sapey, E., Stockley, R. (2004) Bronchiectasis. Medicine; 32: 2, 153-158.
Shoemark, A. et al (2006) Aetiology in adult patients with bronchiectasis. Respiratory Medicine. DOI: 10.1016/j.rmed.2006.11.008.
Stockley, R. et al (2001) Assessment of airway neutrophils by sputum colour: correlation with airways inflammation. Thorax; 56: 5, 366-372.
Wilson, R. (2003) Bronchiectasis.
In: Gibson, G. et al (eds) Respiratory Medicine. Amsterdam: Elsevier Science.
Wilson, R. et al (1998) Systemic markers of inflammation in stable bronchiectasis. European Respiratory Journal; 12: 4, 820-824
Part 2 will explore the treatment of bronchiectasis and the nurse's role in managing care, discussing prevention strategies and promoting patient self-management of the condition.