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What is Alpha-1?

Alpha-1 is a hereditary and progressive condition that affects your lungs

  • Alpha-1 is the common name for the condition known as alpha-1 antitrypsin (A1AT) deficiency1
  • Alpha-1 is hereditary, which means it can be passed on from parents to their children through DNA, or genes2
  • Over 90% of patients living with Alpha-1 have not been properly diagnosed3
  • Patients are often given an initial diagnosis of asthma or smoking-related chronic obstructive pulmonary disease (COPD).
    COPD includes emphysema and chronic bronchitis3,4
  • The first signs and symptoms of Alpha-1 may include lung disease, wheezing, shortness of breath, reduced tolerance for exercise or exertion, and fatigue1
  • Alpha-1 is also a progressive disease, which means it can worsen over time and cause more damage to your lung function if left undiagnosed and untreated1
  • When you have Alpha-1, your lungs may weaken over time, placing you at higher risk for developing lung diseases such as emphysema and COPD2
boy with airplane

How does A1AT work in your body?

A1AT helps maintain balance in your lungs5

  • A1AT is a protein that your body makes in your liver
  • Although A1AT is made in the liver, its main job is to keep certain enzymes from damaging fragile lung tissue
  • One of these enzymes (neutrophil elastase) is released when bacteria or irritants invade the lungs
  • Although neutrophil elastase destroys the bacteria and irritants, it can continue to attack anything around it, including healthy lung tissue
  • The job of A1AT is to bind neutrophil elastase and prevent it from damaging healthy lung tissue
  • People with Alpha-1 don’t have enough A1AT protein to protect the lungs, which can lead to progressive lung damage and emphysema

How A1AT protein works in your body

A1AT helps protect your lungs against outside pollutants

  • The main function of A1AT is to protect the lungs from inflammation caused by infection and inhaled irritants such as tobacco smoke2

Low levels of A1AT cause Alpha-1

  • Alpha-1 occurs when there is a low level or insufficient amount of A1AT protein in the blood. When this happens, there is not enough A1AT traveling to your lungs. The low level of A1AT in the blood occurs because misfolded A1AT cannot be released from the liver at a normal rate. This leads to a buildup of A1AT in the liver that can cause liver disease2
healthy lung
healthy lung

A1AT coats lungs, protecting them from neutrophil elastase.

Alpha-1 antitrypsin is made in the liver and released into the bloodstream where it travels to the lungs.

Neutrophil elastase is a powerful enzyme produced by white blood cells that helps break down harmful bacteria in the lung. Unchecked, neutrophil elastase can cause damage to lung tissue.

Lungs lack A1AT coating, leaving them open to damage by neutrophil elastase.

A1AT Misfolded A1AT trapped in the liver, causing liver damage

Neutrophil elastase Uninhibited, causing progressive lung damage

Healthy
A1AT Deficiency
healthy lung
Healthy Lung
Deficient Lung

Healthy Lungs vs Alpha-1 Lungs

When you have low levels of A1AT, the risk of lung damage increases

In this image, the squares inside the lungs represent the A1AT protein that normally binds neutrophil elastase, which is represented by the light blue circles.

Should you be tested for Alpha-1?

Professional healthcare organizations such as the American Thoracic Society and the Medical and Scientific Advisory Committee of the Alpha-1 Foundation recommend Alpha-1 testing for everyone with the following risk factors6:

  • A family history of Alpha-1*
  • All individuals with COPD regardless of age or ethnicity
  • Unexplained chronic liver disease
  • Unexplained thickening of the walls of the lungs due to chronic inflammation and infection (bronchiectasis)
  • The skin disease known as necrotizing panniculitis

*Family testing of first-degree relatives is currently the most efficient way to detect Alpha-1.

How Alpha-1 affects the lungs

Watch this video to see how Alpha-1 affects the lungs

Quick questions to ask yourself and your doctor about Alpha-1

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Take the Alpha-1 risk quiz
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Question 2

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Results

YOUR SCORE:

You have a LOW score. Continue to follow your doctor's instructions regarding any symptoms you may be experiencing. If your symptoms linger or worsen, you may want to consider getting tested for Alpha‑1. Print your assessment result and keep it on hand for future reference.

You have a MEDIUM score. Talk with your doctor about this evaluation to discuss the need for testing for Alpha‑1. Print this results page and bring it with you to your doctor's office for discussion.

REMEMBER: Not all genetically predisposed individuals will develop clinically significant symptoms. Your doctor can conduct tests to determine if you have Alpha‑1.

You have a HIGH score. Talk with your doctor about this assessment to discuss the need for testing yourself and your family members for Alpha‑1. Remember to print your assessment result and bring it with you to your doctor’s office.

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