Clinical Presentation & Protocol
Patient Usually Complains Of
Patient presents with progressive dyspnea on exertion, chronic productive cough, and wheezing. History significant for early-onset emphysema, non-smoker or minimal smoking history, and family history of AAT deficiency or liver disease. Symptoms are refractory to standard bronchodilator therapy.
Clinical Examination Findings
Physical exam reveals tachypnea, use of accessory muscles, and prolonged expiratory phase. Chest auscultation demonstrates diminished breath sounds globally, particularly at the lung bases. Percussion reveals hyper-resonance. Signs of right-sided heart failure (cor pulmonale) including jugular venous distension and peripheral edema may be present.
Treatment Protocol
Management includes intravenous alpha-1 antitrypsin augmentation therapy (weekly infusions), smoking cessation counseling, and pulmonary rehabilitation. Pharmacotherapy includes long-acting bronchodilators (LABA/LAMA) and inhaled corticosteroids if indicated. Supplemental oxygen therapy for resting or exertional hypoxemia. Evaluation for lung volume reduction surgery or lung transplantation in severe cases.