Management of Respiratory Diseases

Respiratory diseases can be broadly divided into obstructive and restrictive types. 1. Obstructive diseases include conditions in which there is a resistance to airflow either through reversible factors such as bronchospasm or inflammation, or through irreversible factors such as airway fibrosis or loss of elastic recoil owing to damage to the airways and the alveoli. 2. Restrictive disorders are characterised by reduced lung compliance leading to the loss of lung volume which may be caused by disease affecting the lungs, pleura, chest wall or neuromuscular mechanisms. These diseases are therefore different from the obstructive diseases in their pure form, although mixed restrictive and obstructive conditions can occur. Obstructive diseases are by far the most common and are secondary only to heart disease as a major cause of disability. They are: chronic bronchitis emphysema chronic bronchitis and emphysema together asthma bronchiectasis cystic fibrosis. Secondly, as the changing pattern of respiratory disease has resulted in opportunistic pneumonias which are a common presentation in patients with acquired immune deficiency syndrome (AIDS), the restrictive disorders and their management will then be considered. They are: pneumonia pleurisy pleural effusion pneumothorax acute respiratory distress syndrome (ARDS) fibrosing alveolitis. There are other lung disorders that fit into neither of the first two categories but need to be included owing to their prevalence within the community or hospital environment. They are: lung abscess pulmonary tuberculosis bronchial and lung tu

CHRONIC OBSTRUCTIVE PULMONARY DISEASE (COPD)

Chronic obstructive pulmonary disease (COPD) is an ill-defined term that is often applied to patients who have a combination of chronic bronchitis and emphysema which frequently occur together (and may also include asthma). In the majority of cases, chronic bronchitis is the major cause of obstruction, but in some cases emphysema is predominant. There are many patients who
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report shortness of breath increasing in severity over several years and, on examination, are found to have a chronic cough, an overinflated chest and poor exercise tolerance. Patients may also present with a more episodic form of disease, which is a characteristic of an asthmatic component. Chronic bronchitis and emphysema often coexist in COPD, the disease is progressive and is characterised by acute exacerbations. It is not usually diagnosed until irreversible damage has occurred.

CHRONIC BRONCHITIS
Chronic bronchitis is a chronic or recurrent increase in the volume of mucus secretion sufficient to cause expectoration when this is not due to localized bronchopulmonary disease. In the definition of this disease, chronic/recurrent is further defined as a daily cough with sputum for at least 3 months of the year for at least two consecutive years. This is more common in middle to late adult life and in men more than women. Pathology of Chronic Bronchitis : The hallmark is hypertrophy of, and an increase in number of, mucous glands in the large bronchi and evidence of inflammatory changes in the small airways. Some irritative substance stimulates overactivity of the mucus-secreting glands and causes secretion of excess mucus. This mucus coats the walls of the airways and tends to clog the bronchioles. The airways obstruction is enhanced during expiration, with resulting trapping of air in the alveoli. The lungs gradually lose their elasticity as the disease progresses. They will gradually become distended permanently, which eventually may cause extensive rupture of the alveolar walls. After repeated exacerbations due to infection there is widespread damage to the bronchioles and the alveoli with fibrosis and kinking occurring as well as compensatory overdistension of the surviving alveoli. This is closely allied to and contributory to the development of emphysema. Signs and symptoms of Chronic Bronchitis : Cough : The patient will complain of a cough for many years, initially intermittent and gradually becoming continuous. The patient may also complain of bouts of coughing occasionally on lying down or in the morning. Sputum : This is mucoid and tenacious, usually becoming mucopurulent during an infective exacerbation. Wheeze : Wheezing is a symptom described by as many as 80% of patients with COPD. Wheezing is caused by the sound generated by turbulent airflow through the narrowed conducting airways and may be worse in the mornings or may be related to weather changes. Dyspnoea or shortness of breath: This occurs in patients with COPD and, together with the energy-requiring consequences of chronic infection and inflammation, leads to increased work of breathing. The patient becomes progressively more short of breath as the disease progresses. Exercise intolerance : Patients with COPD ventilate excessively and ineffectively at all work levels compared with subjects with normal lung function. This limits exercise performance.

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6. Deformity: These patients often develop a barrel chest due to hyperinflation and use of accessory muscles of respiration. The thoracic movements are gradually diminished and paradoxical indrawing in the intercostals spaces may develop. 7. Cyanosis : This is a blue coloration of the skin caused by the presence of desaturated haemoglobin due to reduced gaseous exchange. Cyanosis is also related to the development of complications, such as poor cardiac output due to ventricular failure leading to increased peripheral oxygen extraction. Cyanosis may also be due to an increase in red blood cells (polycythaemia) in response to chronic hypoxaemia. 8. Cor pulmonale : This may occur in the later stages of COPD. The impaired gas exchange in COPD caused by the disruption of ventilation and perfusion and the resulting hypoxia leads to widespread hypoxic pulmonary vasoconstriction. This leads to an increase in pulmonary vascular resistance resulting in pulmonary hypertension. This eventually leads to hypertrophy and dilatation, a condition known as cor pulmonale. The development of pulmonary oedema, which exacerbates the hypoxia and low cardiac output in patients with COPD, leads to a terminal stage of the disease. 9. Lung function : There is reduction of FEV1 and the forced vital capacity (FVC) is grossly reduced. The residual volume (RV) will be increased at the expense of the vital capacity (VC) because of air trapping and the inability of the expiratory muscles to decrease the volume of the thoracic cavity. The expiratory flow-volume curve is grossly abnormal in severe disease. 10. Blood gases : Ventilation/perfusion mismatch is inevitable in COPD and leads to a low arterial oxygen pressure (PaO2) with or without retention of carbon dioxide (CO2). As the disease becomes severe, the arterial carbon dioxide pressure (PaCO2) may rise, and there is some evidence that the sensitivity of the respiratory centre to CO2 is reduced. 11. Auscultation signs : There will be inspiratory and expiratory wheeze with added coarse crepitations. The breath sounds are vesicular with prolonged expiration. 12. X-ray signs : No characteristic abnormality is seen in the early stages of the disease. If there is significant airways obstruction there may be signs of chest over-expansion (flattening of the diaphragm) and an enlarged reterosternal airspace. EMPHYSEMA Emphysema is a condition of the lung characterised by permanent dilatation of the air spaces distal to the terminal bronchioles with destruction of the walls of these airways. Causes and predisposing factors : Congenital or primary emphysema may be caused by alpha1-antitrypsin deficiency. This is a rare inherited condition. Emphysema may be secondary to other factors, such as: - obstructive airways disease (e.g. asthma, cystic fibrosis, chronic bronchitis) - occupational lung diseases (e.g. pneumoconiosis) - compensatory to contraction of one section of the lung (e.g. fibrous collapse or removal, when the remaining lung expands to fill the space.) Types of emphysema : Centrilobular (centri-acinar) emphysema tends to affect the respiratory bronchioles with most of the alveoli remaining normal. Panlobular (panacinar) emphysema results in widespread destruction of most alveoli as well as respiratory bronchioles.
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Clinical Features of Emphysema : Progressive dyspnoea : Shortness of breath occurs initially on exertion, but as the disease progresses it will gradually occur after less and less activity and finally at rest. This disabling breathlessness is what prevents the patient from working and gradually transforms the patient into a state of severe exercise intolerance and disability. Respiratory pattern : The patient has a 'fishlike' inspiratory gasp, which is followed by prolonged, forced expiration usually against 'pursed lips'. This creates back-pressure to try to prevent airways shutdown during expiration. Owing to increased intrathoracic pressure the jugular veins fill on expiration. A 'flick' or 'bounce' of the abdominal muscles may be seen on expiration as the outward flow of air is suddenly checked by the obstruction of the airways. Cough with sputum : This will be present if the disease is associated with chronic bronchitis or if there is infection. Chest shape : The chest becomes barrel-shaped, fixed in inspiration with widening of the intercostal spaces. There may also be indrawing of the lower intercostal spaces and supraclavicular fossa on inspiration. The ribs are elevated by the accessory muscles of respiration and there is loss of thoracic mobility. Poor posture : There may be a thoracic kyphosis plus elevated and protracted shoulder girdles. Polycythaemia : This may develop if there is a prolonged decrease in PaO2 owing to the ventilation/perfusion imbalance. Cor pulmonale : This occurs in the advanced stages of the disease. Lung function : The FEV/FVC ratio is usually below 70%. Residual volume (RV) is increased and lung volume may exceed the predicted total lung capacity.

Examination : The percussion note will be normal or hyper-resonant due to air trapping. Auscultation will reveal decreased breath sounds and prolonged expiration. The chest radiograph shows low flat diaphragms and hyperinflation. Prognosis of Emphysema The patients become progressively more disabled, with death ultimately occurring from respiratory failure. Complications of the disease are pneumothorax due to rupture of an emphysematous bullae, and congestive cardiac failure.

COMBINED CHRONIC BRONCHITIS AND EMPHYSEMA

Within the spectrum of COPD, two extremes of clinical presentation are recognized : type A and type B. At one time these were classified either as 'pink puffers' (type A) or 'blue bloaters' (type B) to correlate with the relative amounts of emphysema and chronic bronchitis respectively. Blue bloaters Patients with this syndrome often show the following symptoms: obesity comparatively mild dyspnoea copious sputum which may become infected low PaO2 and high PaCO2 because they tend to hypoventilate central cyanosis with cor pulmonale. peripheral oedema
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 an increased residual volume but normal total lung capacity. Pink puffers : Patients with this syndrome often show the following symptoms: an anxious expression general thinness severe breathlessness little or no sputum production relatively normal PaO2and PaCO2 due to hyperventilation early on in the disease central cyanosis and the development of cor pulmonale in the later stages of the disease generally no peripheral oedema until the late stages of the disease an increased total lung capacity due to hyperventilation.

MEDICAL TREATMENT OF COPD

Principles of Treatment 1 Decrease the bronchial irritation to a minimum 2 Control infections : The patient should have a supply of antibiotics at home and receive a vaccination against influenza each winter. The main affecting organisms are Streptococcus pneumoniae and Haemophilus influenzae, which are usually sensitive to amoxycillin or trimethoprim. 3 Control bronchospasm : bronchospasm is not a prominent feature of this disease. 4 Control/decrease the amount of sputum : Patients with chronic bronchitis may present with excessive bronchial secretions and are usually able to eliminate this by themselves. However, during an episode when secretions may become difficult to eliminate, physiotherapy techniques including humidification, positioning and manual techniques may aid expectoration and reduce airflow obstruction in the short term. 5 Oxygen therapy : Oxygen must be prescribed and should be given with great care, especially if a normal pH indicates a chronic compensated respiratory acidosis. Controlled oxygen may be given via a Ventimask (or equivalent) with careful monitoring of blood gas levels. 6 Long-term oxygen therapy (LTOT) : As respiratory function deteriorates, the level of oxygen in the blood falls leading to an increase in pulmonary hypoxic vasoconstriction and a deterioration in cardiac function. Medications Drugs used in the treatment of respiratory disease broadly fall into two categories: relievers and preventers. The relievers are used to reduce bronchospasm and include the beta2 agonists (which may be short- or long-acting), the anticholinergics and the xanthenes derivatives. The preventers may be used to prevent bronchial hyper-reactivity and reduce bronchial mucosal inflammatory reactions - they include the corticosteroids.