Pulmonary Complications of Cystic Fibrosis

Introduction

Cystic fibrosis can result in a number of complications that involves the lungs such as a cycle of pulmonary infection, inflammation, bronchiectasis (chronic lung condition that causes widening and scarring of the airways that causes difficulty in moving air in and out of the lungs), reduced mucociliary clearance, etc.

What Is Cystic Fibrosis?

Cystic fibrosis (CF) is a genetic disease in which thick, sticky mucus builds up on the organs such as the lungs and pancreas which results in clogging the airways and making it difficult to breathe. This mucus in the pancreas makes it difficult to digest food. In children and babies, it becomes difficult to absorb enough nutrients from food. In long-term cases of cystic fibrosis (chronic CF) and progressive CF, the liver, intestine, and sex organs are also affected.

CF is a genetic disease that is caused by a mutation (change) in a gene called CFTR (cystic fibrosis transmembrane conductance regulator). This gene is responsible for the flow of salt and fluids in and out of the cells. If the CFTR gene does not function properly a sticky mucus is built up on the organs of the body.

To develop CF two faulty genes one, from each parent, are inherited by people having this disease. If only one copy is inherited it indicates that the person can be a carrier of the disease and there are chances that the disease can be transferred to the next generation.

What Are the Symptoms of Cystic Fibrosis?

The symptoms associated with cystic fibrosis are:

• Frequent wheezing.

• Difficulty in breathing.

• Frequent lung infection (pneumonia, bronchitis etc).

• Nagging cough.

• Trouble while breathing.

• Loose or oily stools.

• Failure to thrive (inability to gain weight while having a good appetite).

• Slow growth.

Which Are the Bacterias Responsible for Pulmonary Complications of Cystic Fibrosis?

Pulmonary complications or respiratory complications are the most common complications of cystic fibrosis. The normal flow of salt and water is interfered with by the mutation caused by the CTPR gene which causes mucus to build up on the organs. This mucus provides an environment for bacteria and viruses to get developed. This causes infection in the lungs and sinuses mostly. The most common bacteria and viruses that are responsible for pulmonary complications are:

• Staphylococcus aureus.

• Haemophilus influenzae.

• Pseudomonas aeruginosa.

• Methicillin-resistant Staphylococcus aureus (MRSA).

• Mycobacteria (Mycobacterium abscessus).

What Are the Pulmonary Complications Associated With Cystic Fibrosis?

Some of the complications commonly associated with cystic fibrosis are:

• Lung Infection: The environment of the lungs is wet which makes it easy for bacteria and viruses to grow. Bacteria and viruses are removed normally while breathing. But in people with CF, the process of removal gets delayed or sometimes stops and these germs get accumulated and start to grow and multiply there. In response to this, the body’s immune system gets activated and causes inflammation. This inflammation causes the lining of the lungs to swell.

Infection weakens the immune system which leads to more frequent lung infections. Over time this leads to further complications.

• Bronchiectasis: In this condition, the lungs get damaged by infection and inflammation. It occurs when the bronchi(walls of the airways) which carry the air in and out of the body get thickened and widened and become flabby and scarred. Most cases of CF result in bronchiectasis. Infections make the lungs damaged leading to greater susceptibility to infection and damaged lungs. Over time, this prevents getting enough oxygen to the vital organs, which causes strain on the heart and lungs and leads to respiratory failure and heart failure.

• Endobronchial Infections: Hyperconcentrated respiratory secretions can lead to chronic airway obstruction and acute and chronic infection.

• Sinusitis: The 4 pairs of air-filled spaces in the head called sinus cavity. When these spaces get accumulated with mucus the area gets infected. This is called sinusitis or sinus infection. Sinusitis is a common condition seen in people with CF. Common symptoms include breathing through the mouth, cold, cough, headache, stuffy nose, pain in the face, reduced sense of hearing, etc.

• Pneumothorax: Pneumothorax is a condition in which a collection of air is seen outside the lung between the lung and chest wall. This condition results in shortness of breath and pain. This is caused due to the side effects of the lung infections common to CF.

How Are Pulmonary Complications in People With Cystic Fibrosis Diagnosed?

• Chest X-rays: Chest x-rays can be done to confirm the presence of cystic fibrosis.

• Sinus X-rays: Sinus x-rays can be done in people who show certain symptoms of sinusitis along with other tests.

• Lung Function Test: The most common lung function done is by an instrument called a spirometer. Air is filled with full capacity and is exhaled in the mouthpiece of the spirometer.

• Sputum Culture: A sample from the sputum (spit) is obtained and tested for the bacteria. Certain bacteria such as Pseudomonas, are usually found in people having CF.

How Are Pulmonary Complications in People With Cystic Fibrosis Treated?

The treatment for pulmonary complications in people with cystic fibrosis varies as per the involved organ and from person to person. These include the following medications or a combination of them:

• Antibiotics: They help in reducing the infection caused by the bacteria.

• Bronchodilators: They help people who experience shortness of breath.

• CFTR Modulators: They do not attack infection but help the body in improving the function of the mutated gene.

• Mucus Thinners: To help mucus remove from the body.

• Anti-Inflammatory Medications: To reduce the inflammation which makes breathing difficult

Conclusion

Pulmonary complications are usually seen in people suffering from cystic fibrosis. Cystic fibrosis is a condition in which sticky mucus gets accumulated in the organs. It is a genetic condition caused due to improper functioning of the gene called CFTR. This mucus causes insufficient air to reach the vital organs. Pulmonary complications of CF include sinusitis, lung infections, pneumothorax, breathing difficulties, respiratory failure, etc. It can be treated with antibiotics, bronchodilators, mucus thinners, and anti-inflammatory medications, and may require lifelong management.