What lifestyle modifications should I advise patients with Alpha-1 Antitrypsin Deficiency to adopt to improve their health outcomes?

For patients with Alpha-1 Antitrypsin Deficiency (AATD), who often develop Chronic Obstructive Pulmonary Disease (COPD), lifestyle modifications are crucial for improving health outcomes ¹,². You should advise patients on the following:

• Smoking Cessation and Avoidance of Passive Smoke: It is vital to offer treatment and support to help patients stop smoking, and advise them to avoid exposure to passive smoke ¹,². Continued smoking or exposure to passive smoke increases the risk of exacerbations ¹.
• Physical Activity and Pulmonary Rehabilitation: Encourage regular physical activity ¹,². Offer pulmonary rehabilitation to appropriate patients who are functionally disabled by their condition ¹,². This multidisciplinary programme includes physical training, disease education, and nutritional, psychological, and behavioural interventions ¹. Lack of physical activity is a risk factor for exacerbations ¹.
• Nutritional Advice: Calculate the patient's Body Mass Index (BMI) ¹. If their BMI is abnormal (high or low) or changing over time (e.g., more than 3 kg change in older people), refer them for dietetic advice ¹,². For those with a low BMI, nutritional supplements may be given to increase calorific intake, alongside encouragement to exercise ¹. Nutrition should also be part of pulmonary rehabilitation programmes ¹,².
• Vaccinations: Offer pneumococcal vaccination and an annual flu vaccination to all patients ¹,².
• Self-Management Strategies: Develop an individualised self-management plan in collaboration with the patient and their family or carers ¹,². This plan should include information on COPD symptoms, managing breathlessness, appropriate use of inhaled therapies, and early recognition and management of exacerbations ¹,².
• Addressing Psychological Well-being: Be alert for anxiety and depression, particularly in patients with severe breathlessness or those who have had recent hospital admissions for exacerbations ¹. Consider referral to psychological services if anxiety or depression related to COPD is identified ¹,².
• Sputum Clearance Techniques: If patients have excessive sputum, they should be taught how to use positive expiratory pressure devices and active cycle of breathing techniques, often by a physiotherapist ¹,².