Lungs

In people with Alpha-1, the Alpha-1 protein is mutated and gets stuck in the liver which can cause liver problems. But because the protein is stuck in the liver, it cannot get to the lungs to protect them, which can cause lung problems.

Lung disease symptoms typically occur in adults. These symptoms include shortness of breath, coughing, bronchiectasis, and frequent chest colds. Bronchiectasis is an illness that causes the tubes leading to the lungs to be permanently widened and leads to infections.

People with alpha-1 antitrypsin deficiency usually develop the first signs and symptoms of lung disease between ages 20 and 50. The earliest symptoms are shortness of breath following mild activity, reduced ability to exercise, and wheezing. Other signs and symptoms can include unintentional weight loss, recurring respiratory infections, fatigue, and rapid heartbeat upon standing. Affected individuals often develop emphysemaObstructive airway disease in which the walls of the alveoli (air sacs) are damaged or destroyed. More, which is a lung disease caused by damage to the small air sacs in the lungs (alveoliAny of the many tiny air sacs of the lungs which allow for rapid gaseous exchange. More). Characteristic features of emphysemaObstructive airway disease in which the walls of the alveoli (air sacs) are damaged or destroyed. More include difficulty breathing, a hacking cough, and a barrel-shaped chest. Smoking or exposure to tobacco smoke accelerates the appearance of emphysemaObstructive airway disease in which the walls of the alveoli (air sacs) are damaged or destroyed. More symptoms and damage to the lungs.

Resources

• Understanding Lung Disease – AlphaNet
• Understanding Pulmonary Function Testing; PFTs, Blood Gases and Oximetry – AlphaNet
• Understanding Pulmonary Exacerbations – AlphaNet
• Individual Health Management Plan; A long-term guide for Alphas with Lung Disease – AlphaNet
• Medications for Alpha-1 Lung Disease – AlphaNet
• Oxygen Therapy – AlphaNet

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