Is Bronchiectasis A Terminal Illness?

A terminal disease is one that cannot be cured at the time but can be managed conservatively, to relieve troubling symptoms, and prolong life.

Bronchiectasis is a chronic respiratory disease that could cause some changes to your life, and overall affect the quality of your life. It is terminal, as it currently has no cure, but can be managed conservatively, as in, palliative care, to prolong and improve quality of life.

What is a bronchiectasis? 

Bronchiectasis is a big scary word that seems quite difficult to understand, but simply put it is a lung disease where the airway or breathing pathway becomes wider than it should be.¹

This, in normal terms, should mean good news, cause this means more air right?

Unfortunately, that’s not the case. Rather, that abnormal widening causes excess accumulation of mucus in the airway, and also causes the airway to be more exposed, and vulnerable to infections.

For more details on how bronchiectasis affects the lungs, check out the national heart, lungs and blood institute (NHLBI) website.¹

Causes of bronchiectasis

In this lung disease, the part of the airway affected is the bronchial tube called the bronchus. The abnormal widening of the bronchial tube is due to damage to the bronchus.

The exact cause of bronchiectasis is not known, but some factors have been incriminated.

This damage to the bronchus could occur as a complication of inherited diseases (for example; cystic fibrosis) or could be acquired.

Bronchiectasis could be caused by the following:

1. Previous lung infections example; Pneumonia, Tuberculosis, etc
2. Previous respiratory allergies example; Allergic bronchopulmonary Aspergillosis- which is an allergic reaction to a fungus (Aspergillus) when inhaled.
3. Severe Asthma
4. Cystic fibrosis - Cystic fibrosis is a hereditary disease caused by a genetic disorder that leads to clogging of the lungs with mucus. Cystic fibrosis can cause repeated infections which can damage the bronchus and lead to bronchiectasis.
5. Aspiration - This is a medical term that describes an abnormal process where stomach content makes its way into the respiratory tract, rather than down the intestine. The stomach acid can corrode the bronchus and lung tissue, leading to bronchiectasis
6. Immunodeficiency - This is a term used to describe the state of an individual whose immune system is low. This can be seen in people living with HIV/AIDS, cancer patients, and other chronic conditions.
7. Ciliary abnormalities -  Cilia are hair-like protrusions along your airway which help trap foreign bodies and push them out. In cases like pulmonary ciliary dyskinesia etc, the ciliary action is suboptimal, and thus exposes the airway to infections and compromise.
8. Chronic obstructive pulmonary disease - Chronic obstructive pulmonary disease is a big group of several progressive lung diseases, two of which include; emphysema and chronic bronchitis. Chronic obstructive pulmonary disease makes it harder to breathe, and in the long run, may cause the patient to develop bronchiectasis.
9. Connective tissue diseases example; Rheumatoid arthritis.

Rheumatoid arthritis is a chronic autoimmune condition that leads to pain, stiffness, and swelling of joints.

 Other connective tissue diseases implicated as a causating factor for bronchiectasis include; Ulcerative colitis, Crohn’s disease, and Sjörgen’s disease.³

Symptoms of bronchiectasis

Bronchiectasis is a chronic disease and the symptoms tend to surface after a period of months to years. Once the symptoms surface, they tend to worsen or progress over time.

The symptoms that could be seen with bronchiectasis include:

1. Chronic cough
2. Shortness of breath
3. Production of yellowish or blood-stained sputum during the following bouts of cough
4. Recurrent lung infections
5. Wheezing
6. Chest pain
7. Weight loss
8. Fatigue
9. A pale or blue skin colour

Risk factors of bronchiectasis

Certain risk factors have been implicated in the development of bronchiectasis.

These risk factors include:

1. Exposure to toxic fumes and gas can cause irritation and inflammation, which in turn damages the lung tissues, and in the long run, increase the risk of developing bronchiectasis
2. Asthmatic patients run a risk of developing bronchiectasis
3. Previous childhood respiratory infections example; whooping cough, tuberculosis
4. Immunodeficiency disorders, for example; complement deficiency, HIV/AIDS patients, cancer patients, etc.
5. Cystic fibrosis
6. Connective tissue diseases, for example; Rheumatoid arthritis
7. Chronic pulmonary aspiration, which can damage the airway
8. Disorders of cilia function, for example; primary ciliary dyskinesia
9. Allergic bronchopulmonary aspergillosis

Treatment

Treatment of bronchiectasis is mainly aimed at relieving symptoms, improving quality of life, and preventing death from the disease.⁴

Can bronchiectasis be cured?

No, bronchiectasis cannot be cured. As earlier buttressed, bronchiectasis is a terminal disease, and hence cannot be cured. As earlier stated, bronchiectasis is a terminal disease, and hence cannot be cured. It can rather be managed by palliative care.

Medications

Some medications are given, which help to ease breathing, and also stop further progression of the disease. They include:

• Antibiotics to treat underlying lung infections, and also prevent new infections
• Bronchodilators; help relax the muscles of the airway, and ease breathing
• Expectorants and mucus thinners, help reduce mucus in the airway 
• Inhaled corticosteroids help breathing, especially in patients with co-existing asthma

In addition, hydration is of great value in therapy. Taking enough fluids helps thin out mucus, preventing them from blocking the airway. Water is best recommended for hydration.

Vaccines

Vaccines are useful in preventing some lung infections that could lead to bronchiectasis. According to NHS,⁶ the vaccine seen to prevent several respiratory tract infections ranging from whooping cough to pneumonia is the pneumococcal conjugate vaccine (PCV).⁶

Surgery 

Surgery is only considered in cases that are unresponsive to other treatment modalities, and if the damage is confined to a small area on the bronchus. Surgery is not recommended in cases of widespread damage to the bronchus.

Oxygen therapy and chest physical therapy

As earlier stated, bronchiectasis patients suffer difficulty breathing, and in severe bronchiectasis, oxygen administration will be vital for survival.

Chest Physiotherapy: This is a modality where several breathing techniques are used to aid in coughing up mucus from the airway, and also clearing up the lungs. Various methods are used, some of which include; Active Cycle Breathing (ACB) and Forced Expiration Technique (FET).

This physiotherapy is performed by a respiratory therapist, however, a family member can be trained to perform them for the patient. Read more on chest physiotherapy in bronchiectasis at the NHS website.⁷

Things that can help relieve the symptoms of bronchiectasis

• Hydration: Taking enough fluids helps thin out mucus, preventing them from blocking the airway. Water is best recommended for hydration.
• Being consistent with your hospital appointments, and taking the drugs prescribed by your doctor
• Quit smoking and/or vaping

Outlook 

Is bronchiectasis fatal?

Yes, bronchiectasis is a serious condition, that can lead to death if not properly managed. The good news however is, that if the condition is properly managed and monitored by a physician, the life expectancy for such patients becomes the same as the life expectancy of a healthy person without the disease.

Complications

Common complications of bronchiectasis include:

• Coughing up blood (Haemoptysis)
• Anemia due to blood loss
• Recurrent pneumonia requiring hospitalization
• Formation of empyema (Collection of pus in the lungs due to infections)
• Lung abscess
• Pneumothorax (air in the thoracic cavity)
• Respiratory distress with decreased lung function
• Respiratory failure
• Pulmonary hypertension
• Lung cancer
• Death

Living with bronchiectasis

Living with bronchiectasis can be stressful and emotionally draining, and thus requires self-affirmation, and also a solid support system. 

Regularity to hospital checkups and compliance with prescribed drugs are the keys to living a great life, even with the disease.

Some lifestyle modifications would be required to prevent exacerbations, some of which include quitting smoking, and also avoiding environments or workplaces where toxic fumes are released.

Prevention

Acquired bronchiectasis can be prevented by the following means:

• Adequate vaccination with the pneumococcal conjugate vaccine
• Avoid smoking
• Avoid environments or workplaces that deal with toxic fumes and gas.
• Present to the hospital for adequate treatment of any respiratory tract infection

Summary

Bronchiectasis is a terminal disease which, as frightening as it looks, is not a death sentence. 

Yes, there is no definite cure, but it's however managed conservatively to relieve troubling symptoms which may include; chronic cough, shortness of breath, chest pain, wheezing etc.

You however, don’t need to be confined to the hospital, as long as you comply with your doctor’s appointment, and medications if any. Some of these medications include; antibiotics, expectorants etc, with the aim of relieving troubling symptoms and preventing progression of the disease.

In some severe cases, surgery may be required.

As with any disease, bronchiectasis can be very stressful and scary, and I'd encourage the reader to reach out to their family, friends and medical professionals for support when they are ready to do so.