Bronchiectasis

Bronchiectasis

Bronchiectasis is a lung disease, which is characterized by the permanent damage and widening 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 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. 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