Thursday, December 16, 2010

Everything you need to know about Bronchiectasis

It seems that about twice a year we have a patient admitted to the hospital with bronchiectasis. They tend to have a presentation similar to other patients with chronic respiratory diseases, yet the cause of their illness is unique.

According to nationaljewishhealth.com, the disease in characteristic of air passages that are chronically inflamed, dilated, and prone to infections that effect the entire airway from the nasal passages all the way down to the alveoli. Where exactly in the air passage the infection occurs is dependent on the infecting agent.

Thus, these patients are prone to getting both pneumonia, ear infections and sinus infections. Of course sinus infections, over time, can lead to other nasal complications such as nasal polyps and deviated nasal septums.

I found this article called, "Bronchiectasis Is" which notes a little of the history of bronchiectasis. Rene Laennec, the man who invented the stethescope, "used his creation to first discover bronchiectasis in 1819. The disease was researched in greater detail by Dr. William Osler in the late 1800s..."

Like asthma, it is kind of a mysterious disease, a conundrum of sorts, as why a person develops it is unknown, although the gene has been isolated and research is ongoing. While asthma wisdom has been ongoing since 1903 when epinepherine was invented, bronchiectasis research and wisdom was generally started in the 1930s when cystic fibrosis was recognized as a disease and research was started to learn more about it.

Inexplicably, some people get bronchiectasis by itself, and it is the main pulmonary complication of those with cystic fibrosis.

The disease is indicative of thick secretions which make the patient susceptible to getting pneumonia, and these infections (including those of the upper airway) tend to make the disease worse over time. NJH also notes that these infections are more likely in patients with impaired drainage, which would include those with increased number of goblet cells that secrete thick, tenacious secretions (as in CF) that are hard to cough up and therefore create a climate in the lungs ideal for certain bacteria.

So, what causes bronchiectasis? Ironically, while this is a disease indicative of chronic respiratory infections, it's believed to be caused due to damage caused by respiratory infections. Otherwise, it's one of the components of the disease cystic fibrosis (which you can read about here) and occasionally even COPD who are also susceptible to reapeated respiratory infections.

Specific ailments that might cause this disease include:

  1. Viral infections (measles, adenovirus, influenza),

  2. Bacterial infections (Pseudomonas aeruginosa, Staphylococcus aureus, Klebsiella),

  3. Mycobacterial infections (tuberculosis, Mycobacterium avium complex) and

  4. Fungal infections (histoplasmosis).

  5. Antibody deficiencies (e.g., common variable immunodeficiency) (immune disorder)

  6. White blood cell dysfunctions (e.g., chronic granulomatous disease). (immune disorder)

  7. Diseases that effect the cilia (Impaired drainage and high risk of infection by diseases such as primary ciliary dyskinesia, Kartagener's syndrome and Young's syndrome)

  8. Post inflammatory pneumonitis

  9. Gastrointestinal Reflux (GERD) due to long term lung exposure to stomach acids damaging the linings of the lungs

  10. Autoimmune and Connective tissue diseases (Rheumatoid arthritis, Sjogren's syndrome and Wegener's granulomatosis)

  11. Cystic Fibrosis (Due to impaired drainage and high infection rate)

  12. Tumor that obstructs the airway and causes impaired drainage

  13. COPD due to thick secretions that create a good environment for bacteria

  14. Alpha-1 Antitrypsin Deficiency (genetic COPD)

  15. Other
Another interesting thing that might cause bronchiectasis is post inflammatory pneumonitis which is generally caused by aspiration (food to enter the lungs) due to an impaired ability to swallow (dysphagia). Another cause of this is GERD, which is when gastric return up the esophagus and enter the lungs due to an impaired or relaxed esophageal sphincter.

Gastric material may damage the airways in such a way that it results in chronic inflammation that leads to asthma or bronchiectasis.

Symptoms include:
  1. Chronic cough

  2. Productive cough (thick secretions)

  3. Hemoptysis (bloody sputum)

  4. Smelly sputum (due to infection)
  5. Colored sputum (due to infection)
  6. Dyspnea (shortness of breath)

  7. Wheezing

  8. Weight loss

  9. Fatigue
Early warning signs of bronchiectasis exacerbation:
  1. Increased shortness of breath

  2. New onset or worsening cough

  3. Increased secretions

  4. worsening quality of life (inability to perform normal things, such as walking, brushing teeth, etc.)

  5. Heart Failure (pedal edema, high blood pressure, etc.)
Diagnosis is made by:

  1. Medical history

  2. CT

  3. Pulmonary Function Testing

  4. Testing for underlying disease such as cystic fibrosis or other based on patient or family history
Early treatment is essential to prevent worsening of the condition. According to "What Are The Treatment Options For Bronchiectasis (Bronchiectasia)?" , from medicalnewstoday.com (April 17, 2010), treatment included:

1. Controlling infections: This includes use of antibiotics as soon as the patient notices the symptoms. Sometimes antibiotics are given long term, and sometimes the patient (especially the CF patient) will even take IV antibiotics at home.

2. Bronchodilators: As the pulmonary complications often result in bronchospasm (asthma-like symptoms), medicines such as Ventolin and Xopenex are generaly a regular part of therapy.

3. Hydration: Drinking lots of water is a good way to prevent the mucus from getting too thick. This makes it easier to expectorate secretions.

4. Corticosteroids: Medicines like Flovent, Pulmicort, Azmanex, and even Advair and Symbicort, are often prescribed. This helps to treat chronic inflammation that is usually present in the lungs.

5. Mucolytics: Medicine such as Mucomyst and Pulmizyne are often used to help thin secretions.

6. Hypertonic aerosols: Inhaling solutions that have more salt that that of which is in cells lining your airways can help thin secretions.

7. Postural drainage and chest percussion : Also referred to as Chest Physiotherapy (CPT). The vibrations caused by cupping your hands and pounding on their chests is believed to help "pound" secretions from their lungs so they can cough it up. Likewise, by performing chest percussion while having the patient lie in certain positions allows gravity to move secretions up.

8. Bronchoscopy: Thick secretions can often cause mucus plugs which block air from getting to the alveoli and the arterioles. When this occurs, a scope of the lungs can help to remove the obstruction.

9. Surgery: This will remove part of the lungs where the mucus plug occurs. This may also be done to remove lungs damaged by constant infections, especially if the lung damage causes the patient to cough up lots of bloody secretions. This is very rare.

10. Embolization: A catheter is inserted into the airway to inject a substance that stops the lungs from bleeding.

11. Oxygen therapy: As the disease progresses it may cause less oxygen from getting from the lungs to the blood (hypoxemia) and tissues (hypoxia). When this occurs supplemental oxygen may be necessary.

12. Lung transplantation: Usually only done with those with advanced bronchiectasis who also have cystic fibrosis. This also presents with a lot of complications of its own.

13. Treat co-existing conditions: Any condition that leads to bronchiectasis must be treated as well, such as CF, GERD, COPD, etc.

Prognosis for patients with bronchiectasis depends on the progression and treatment of the co-existing condition, yet it also depends on early diagnosis and swift treatment.

Medicalnewstoday.com likewise notes that "Patients with co-existing conditions generally have worse outcomes. Examples of co-existing conditions include chronic bronchitis, emphysema. Individuals with complications, such as pulmonary hypertension or cor pulmonale tend to have worse outcomes."

Prognosis (outlook) may also be based on compliance of the patient. Those who take their meds as prescribed, knows the early signs and symptoms and seek speedy help, have the greatest chance of slowing progression of the disease.

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