Cystic Fibrosis/Bronchiectasis

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Overview

Although better insights into the natural course of cystic fibrosis (CF) have led to treatment approaches that have improved pulmonary health and increased the life expectancy of individuals with this disorder, lung disease remains the main cause of morbidity and mortality in patients with CF. Evidence suggests that airway epithelial defects in ions-water transport lead to dehydrated mucus, impaired mucus clearance, and mucus adhesion to airway surfaces. An increase in mucin secretion is also suggested by the formation of endobronchial mucus plaques and plugs, which become the main sites of air flow obstruction, infection, and inflammation conducing to early small airways disease followed by the development of bronchiectasis. The lung involvement is usually progressive with intermittent exacerbations. Aggressive management and advances in treatment delay, but, do not prevent progression of lung disease. Respiratory failure ensues and is the major cause of death. The lung parenchyma is virtually untouched for much of the course of the disease. This review focuses on the lung involvement in cystic fibrosis and summarizes new developments on the diagnostic approach of CF and pathogenesis of related lung disease. Current therapeutic modalities, novel therapies targeting the basic genetic defect, and lung transplantation are also reviewed.


Symptoms

Coughing that results in a lot of mucus.

Coughing up mucus that has blood in it (known as hemoptysis)

Chest pain or tightness because it is harder to breathe.

Wheezing or making whistling noises when breathing.

Clubbing of nails.

Loss of weight.

Flare-ups that usually include: Fatigue.

Causes

Bronchiectasis may be caused by cystic fibrosis (CF), a genetic condition that results in long-lasting lung infections and reduced ability to breathe. CF gets worse over time.


For non-CF bronchiectasis, the cause is not always known. This is called idiopathic bronchiectasis. However, in other cases, causes include:


Past severe infection that has damaged the lung

Genetic diseases like primary ciliary dyskinesia or alpha-1 antitrypsin deficiency

Immune system conditions that make it difficult to fight off infections

Aspirating (breathing in) things like fluids, stomach acid, or foods into the lungs

Allergic bronchopulmonary aspergillosis, an allergy to a particular type of fungus

Other conditions like rheumatoid arthritis, Crohn’s disease, and Sjogren’s syndrome

Obstructed airways (airways blocked by something like a tumor or an inhaled object)

Risk factors

People who have conditions that damage the lungs or increase the risk of lung infections are at risk of bronchiectasis. Examples of such conditions include:


Cystic fibrosis, which causes almost half of the cases of bronchiectasis in the United States

Immunodeficiency disorders, such as common variable immunodeficiency and, less often, HIV and AIDS

Allergic bronchopulmonary aspergillosis, which is an allergic reaction to a fungus called aspergillus that causes swelling in the airways

Disorders that affect cilia function, such as primary ciliary dyskinesia, can cause bronchiectasis. Cilia are small, hair-like structures that line your airways. They help clear mucus (a slimy substance) out of your airways.

Chronic (long-term) pulmonary aspiration, which can inflame the airways

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Complications

Cystic fibrosis (CF) lung disease is characterized by the development of progressive bronchiectasis and impaired lung function with severe airflow obstruction. CF patients suffer from shortened life expectancy, primarily driven by respiratory failure. The mechanism by which CF lung disease develops is the result of an interplay of multiple intrinsic and extrinsic factors including genotype, abnormalities in mucus composition and movement, chronic inflammation, and chronic airway infection. Although all CF patients are at increased risk for pulmonary complications including hemoptysis, pneumothorax, pulmonary hypertension, and chronic hypoxic and hypercapnic respiratory failure, the risk of developing these complications increases with progression of lung disease. The focus of this article is to summarize the pathophysiology, epidemiology, and management of these key pulmonary complications.

Prevention

he following steps can help prevent bronchiectasis: Make sure you and your children have had the vaccines for measles and whooping cough. Get annual flu vaccinations and advised pneumonia vaccinations. Stay away from harmful substances that can damage your lungs, such as smoke, gases, and fumes.

Quit smoking and avoid secondhand smoke.

Maintain a healthy diet, low in sodium, added sugars, saturated fats and refined grains.

Stay hydrated, drinking plenty of water to help prevent mucus build-up.