Bronchiectasis is a lung disease, which is characterized by the widening and permanent damage of one or more of the large connecting bronchi (airways). Infections cause a change in the muscular and elastic components of the bronchial wall, which then become distorted and enlarged. This enlargement can be uniform or irregular. It begins a vicious cycle in which the airways slowly lose their ability to clear mucus. As the mucus builds up, serious lung infections can then occur, which can cause more damage to the bronchi. This results in the vicious cycle of bronchial damage, bronchial dilation, inability to clear secretions, reoccurring infection and more bronchial damage. As this continues over time, the airways become chronically inflamed, more stretched out, scarred and easily collapsed, resulting in airflow obstruction. In advanced cases, this can affect how much oxygen reaches the body's organs, leading to other serious illnesses involving damage to those critical organs.

There are two basic types of bronchiectasis:

      Congenital bronchiectasis is present at birth, usually only affects infants and children, and is the result of developmental arrest of the bronchial tree in the fetus.

      Acquired bronchiectasis occurs in adults and older children and is the more common form. Some of the more common causes are:

• Chronic long diseases such as:
       • Cystic Fibrosis
              Note: CF is considered the major lung disease contributing up to 50% of bronchiectasis cases
       • Tuberculosis
       • Allergic Aspergillosis
       • Young Syndrome, a disease very similar to CF and may be a genetic variant
       • Kartagener's Syndrome, a rare inherited disease that combines the loss of ability to clear mucus and chronic sinusitis
       • Alpha1-antitrypsin deficiency

• Severe lung infections such as repeated episodes of pneumonia, fungal infections, whooping cough, and other
  disorders that affect the cilia (the small hair like structures that line the airways and help clear out mucus

• Immunodeficiency disorders such as HIV

• Blockages of the airways
       • Growths and tumors
       • Inhaled substances (a peanut, a small toy, etc.)
       • Mucus plugs

• Impaired ability to swallow, causing food or saliva to enter the lungs

• Severe heartburn (gastroesophageal reflux disease) (GERD) allowing stomach contents to enter the lungs

• Drug abuse (especially heroin)

• In a small number of case, inhaling toxic substances that injure the bronchi, such as noxious fumes, gases, smoke
  (including tobacco smoke), and injurious dust (silica, coal dust, glass dust) can lead to bronchiectasis.  Chlorine gas,
  sulfur dioxide and ammonia are among the more suspected noxious fume agents.
             
              NOTE: There have been some reports that some service personnel who have seen duty in Iraq were exposed
                         to large concentrations of ammonia and have developed bronchiectasis.

The symptoms for bronchiectasis fall into two broad categories.

      The most common symptoms in early stages of bronchiectasis are:

• Daily cough, over months or years

• Daily production of large amounts of mucus, or phlegm (flem)

• Repeated lung infections

• Shortness of breath
       • Wheezing
       • Chest pain
       • Breath odor 

      The more serious longer term symptoms and conditions occurring in more advanced stages of bronchiectasis in addition to the above may include:

• Coughing up blood or bloody mucus

• Coughing that worsened by lying on one side

• Clubbing of fingers

• Change in skin color
        • Bluish indicating oxygen deficiency
        • Paleness

• Weight loss

• Fatigue

• Sinus drainage

• Collapsed lung

• Heart failure

• Brain abscesses

Diagnosing bronchiectasis:
     This involves a series of tests designed to identify underlying causes of any symptoms and to determine the amount of damage to the lungs. Since the symptoms for bronchiectasis are very similar to other conditions such as chronic asthma and chronic bronchitis, a major part of the diagnosis involves eliminating the possible other conditions. The most common tests are:

• Chest X-ray, which can show infections and scarring

• CT Scan, considered a defining test for bronchiectasis, can show how much damage is done to the airways as
  well as the exact location of the damage

• Pulmonary Function Tests (PFT), including:
       • Spirometry
       • Lung volume measurements
       • Diffusion capacity (DLCO) which measure how well the lungs take in and exhale air, and how efficiently
         they transfer oxygen
       • Blood Tests

• Complete blood count (CBC)

• Arterial Blood Gas (ABG)
  • Sputum cultures can show if you have bacteria, fungi, or tuberculosis
  • Sweat tests (A patch test for Cystic Fibrosis)
  • Bronchoscopy

• A bronchoscope is a long narrow, flexible tube with a light on the end which is inserted through your nose or
  mouth into your airways, and provides a video image of the airways. It also allows your doctor to see possible
  blockages as well as collecting samples of mucus.

There are three classifications of bronchiectasis which describe the severity of the condition:

• Cylindrical - most common and refers to the slight widening of the respiratory passages. This type can be reversed
  and may be seen after acute bronchitis

• Varicose - bronchial walls have both extended and collapsed portions

• Cystic - most severe and involves irreversible ballooning of the bronchi

Treating and managing bronchiectasis.
     Early diagnosis and treatment plans are designed primarily to slow the progression of the disease, and to prevent additional damage to the lungs.

• Treatment plans encompass several categories
       • Treatment of underlying conditions
       • Treatment of respiratory infections early and aggressively
       • Medications
              Antibiotics
              Bronchodilators
              Corticosteroids
              Mucus thinners
              Expectorants
              Nasal washes
       • Mucus removal
       • Prevention of future complications
       • Pulmonary rehab
       • In advanced instances
              Oxygen therapy
              Surgery to remove portions of the lungs.
              Single and double lung transplant

The prognosis for people with bronchiectasis is quite varied and dependent on a number of factors, including:

• How early a comprehensive treatment plan was implemented

• How well subsequent infections are controlled

• The prevention of relapses is critical to a patient’s longevity. Relapses of bronchiectasis can be controlled with
  antibiotics, chest physiotherapy, inhaled bronchodilators, proper hydration, and good nutrition.

• The effects of other systemic diseases which impact the effectiveness of treatments – Among the more common
  diseases are:
       • Chronic bronchitis
       • Emphysema
       • Pulmonary hypertension
       • Cor pulmonale

Last modified: February 10, 2013

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