What Are Cystic Fibrosis Exacerbations?

Lung infections are the most common and easily recognized complication of cystic fibrosis (CF). It is the repeated lung infections that most often lead to poor health and death in people with CF.

Why do people with cystic fibrosis get lung infections?

Cystic fibrosis is caused by a mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This mutation prevents the body from properly moving salt and water in and out of the lungs and other organs. This creates a salt imbalance that creates thick, sticky mucus that builds up in the lungs where germs settle in and grow, creating an infection.

The body sends out white blood cells to attack the germs, which causes inflammation. The inflammation spurs the body to create even more mucus, which then blocks the airways, making it hard to breathe. This cycle of infection and inflammation causes lung damage, which makes it even harder to breathe.

What are common types of lung infections?

Any organism or germ that causes disease, such as a virus, bacteria, or fungus, can cause a lung infection. The bacteria and viruses that most often cause lung infections in people with CF include:

• Staphylococcus aureus (staph)
• Haemophilus influenza (flu)
• Pseudomonas aeruginosa
• Methicillin-resistant staphylococcus aureus (MRSA)
• Aspergillus
• Burkholderia cepacian
• Mycobacteria (Mycobacterium abscessus and Mycobacterium avium-intracellulare)
• Achromobacter (Alcaligenes or Acinetobacter) xylosoxidans
• Stenotrophomonas maltophilia1^1,2,4

These germs tend to lead quickly to exacerbations, or serious illness in people with CF.

How is a lung infection diagnosed?

A test called a sputum culture is used to understand which germ is causing the lung infection. Sputum is another name for the mucus from the lungs. The sputum is placed in a dish and the problem organisms are identified. Sometimes more than one bacterium or virus may need to be treated even if it is not yet causing a decline in lung function.

Knowing exactly which germs are causing the lung infection allows the doctor to prescribe antibiotics and other medicines that kill that specific bacteria, virus or fungus.³

Antibiotic resistance

Because people with CF battle these infections so often, they tend to be at risk for antibiotic resistance, meaning these vital drugs stop working to fight the infections.

What is an exacerbation?

When a lung infection gets severe, it is called an exacerbation or flare-up. Some people think of exacerbations as a “lung attack” much like a heart attack is a severe problem with the heart. Exacerbations usually require hospitalization, sometimes in intensive care. Round-the-clock antibiotics, airway clearance, and enteral nutrition help the person with CF fight the infection.

All lung infections and exacerbations should be treated quickly to keep the lungs as healthy and strong as possible.³

Reducing the risk of lung infections

People with CF and their loved ones can take many steps to reduce their chances of getting a lung infection.

• Wash hands often
• Wear a mask or staying at least 6 feet away from others who have a cough or cold
• Stay at least 6 feet away when around another person with CF
• Cover your cough
• Clean and disinfect your nebulizer
• Clean and disinfect any surfaces you and others touch often such as toys, doorknobs, phones or computer keyboards
• Don’t share personal items that come in contact with saliva and other body fluids such as straws, utensils, toothbrushes or nebulizers
• Avoid dust and dirt
• Stay current on all vaccinations⁵