What CF Patients Should Know About Pulmonary Exacerbations (PExs)

Cystic fibrosis pulmonary exacerbations (CFPExs) are generally defined as a sudden decrease in lung function accompanied by a change in symptoms. Pulmonary exacerbations (PExs) are linked to worsening lung function, low quality of life, increased hospitalizations, and shorter life expectancy in people with cystic fibrosis (CF). Symptoms include coughing, mucus production, and shortness of breath. Lung infections are believed to be the most common cause.

Even though declining lung function accurately describes PExs, there is no universal definition of the condition. This means that there is no standardized treatment for PExs. Therefore, if you develop a PEx, your healthcare provider will create a unique treatment plan based on your symptoms and medical history.

In this article, you learn what CF is, what causes PExs in people with CF, treatment options, and current research on the condition.

What Is Cystic Fibrosis?

Cystic fibrosis is a condition that causes the mucus in your body to become too thick. The organs most heavily impacted by this are the lungs, intestines, sinuses, pancreas, liver, and sex organs. Repeated lung infections and breathing problems are common because the thicker mucus clogs up the lungs. Eventually, this results in lung damage.

People with CF are also at higher risk for developing diabetes, liver disease, arthritis, and osteoporosis (weak and brittle bones).

Chronic Nature of CF

CF is an inherited condition with no cure, so it is considered a chronic disease and must be managed for life with a treatment plan. Symptoms can be mild to severe and either present at birth or appearing later in life. Screening for this disease is done at birth in the United States.

Treatments have greatly improved over the years, so people with CF live well into their 40s, 50s, and beyond. However, living longer means they will be faced with managing multiple chronic conditions, such as PEx.

Facts About Pulmonary Exacerbation (PEx)

There are no standardized treatments for PExs because there is no agreed-upon definition of what it is. Generally, if lung exacerbations increase, a healthcare provider will change the CF patient’s treatment plan. But these changes will be different for every individual.

CF Exacerbations Symptoms

Signs of PEx in CF patients may include some or all of the following symptoms:

• Decrease in lung function
• Increase in coughing
• Increase in mucus and/or change in its color
• Shortness of breath
• Decrease in appetite or weight loss
• Fatigue

These symptoms may come on suddenly or be an increase in severity.

Varying Definitions Can Guide PEx Treatment

Despite there not being a universal definition for the condition, a few widely recognized signs and symptoms of PEx can help guide a diagnosis, including:

• Having at least four symptoms from a predefined list: Decreased lung function, increased mucus, increased coughing or coughing up blood, increased shortness of breath, fatigue, fever of 100.4 degrees Fahrenheit.
• When additional antibiotics are needed due to a change in at least two symptoms: Increase in mucus and/or change in color, increased cough, fatigue, weight loss, increased shortness of breath, decrease in lung function, lung changes seen through an x-ray.
• Three or more symptoms in patients older than 6 years: Decline in FEV1 (forced expiratory volume, the amount of air exhaled in a forced breath in one second)_, increased cough, new crackles, coughing up blood.
• Point system to diagnose PEx: Decreased exercise tolerance, increased cough, increased mucus, changes in the lungs on examination, decreased appetite, change in FEV1.
• One to two symptoms of the following: Fever of 100.4 degrees or more, 50% increase in cough and/or mucus, weight loss, work or school absence (three of previous seven days), upper lung infection, decrease in FEV1 of at least 10%, an increased breathing rate, elevated white blood cell count (from list two).
  

"Lung Attack" Responsible for CF Mortality

PEx, COPD (chronic obstructive pulmonary disease) exacerbations, and asthma are all considered “lung attacks.” Patients with CF that have regular PExs experience a rapid decline in lung function and have an increased risk of needing a lung transplant or death.

Even though CF treatments have greatly improved and contributed to longer life expectancy, PEx rates have not decreased in the same manner. Early detection of PEx is critical because of its link to a decline in lung function, poor quality of life, and premature death.

PEx Treatment Options

Treatment options for PEx include antibiotics, corticosteroids, and pulmonary rehabilitation. There are also special considerations for treating PEx in children. Early diagnosis and treatment help increase quality of life and survival.

Antibiotics

CF patients with PEx may be treated with oral, inhaled, or intravenous (IV, within a vein) antibiotics. It is estimated that 25%–35% of adults with CF have chronic airway infections with bacteria that are resistant to multiple antibiotics. A combination of antibiotics will be prescribed to prevent drug resistance. Your healthcare provider will decide which is best based on the PEx severity.

Corticosteroids

A short course of corticosteroids may be beneficial for a PEx, but research does not support this being a standard for treatment. Studies show that corticosteroids are not always effective at reducing inflammation from a PEx, especially if it is severe. Currently, there is an ongoing large clinical trial to determine the role of steroids in CF pulmonary exacerbations.

Pulmonary Rehabilitation

Pulmonary rehabilitation (PR) is an exercise program that helps improve lung function for people with chronic breathing problems. PR acts as a supplement to your treatment plan and may include the following:

• Exercise training: This will help improve endurance and muscle strength.
• Nutritional counseling: Being overweight or underweight can cause breathing difficulties.
• Disease management education: You will learn how to avoid situations that can cause PEx (such as infections) and how to best take your medications.
• Techniques to help save energy for daily tasks: You will learn how to move in specific ways that are easier on your lungs and stress management to help you stay calm.

PEx In Children

Children who have PEx can also be treated with antibiotics. However, signs, symptoms, and diagnosis look different compared to adults. For instance, the type of intervention to ease symptoms (such as antibiotics) is sometimes used to diagnose PEx in adults. But it is recommended that healthcare providers rely on symptoms alone to diagnose PEx in children.

In addition, PEx in children is typically triggered by a viral infection, but symptoms tend to be mild. Blood oxygen may still be normal. The most common symptoms include:

• Cough
• Wheeze
• Fever
• Runny nose
  

Co-Occuring Conditions

While chronic lung disease is the main complication of CF, other parts of the body can be impacted as well. This means CF patients will have a higher risk of other conditions such as diabetes, arthritis, liver disease, infertility, and intestinal issues.

Nutritional Considerations

CF can cause digestive problems such as constipation, gas, nausea, weight loss, pale stools, and malnutrition. Treatments for these issues can include:

• A diet high in protein and calories
• Pancreatic enzymes to help your body absorb fats and protein
• Supplements like vitamin K, D, E, and A
• Treatment for constipation

Lung Health and PEx Recovery

Preventing chronic PEx can be challenging, especially for CF patients. However, doing everything you can to avoid PEx will protect your lungs from long-term damage and a decline in lung function.

Good nutrition, controlling blood sugar (if you have diabetes), and getting treatment as soon as you think you may have PEx will provide an excellent foundation to safeguard lung function. Because there is no universal standard for PEx treatments, your healthcare provider will decide what treatments are right for you. With treatment, most people recover from PEx within a few weeks.

Summary

Cystic fibrosis (CF) is a condition that makes the mucus inside your body thick and sticky. Symptoms include coughing, wheezing or shortness of breath, increased mucus, and fatigue. This causes chronic lung disease and puts you at risk for pulmonary exacerbations (PEx).

Because there is no standard for diagnosis or treatment, your healthcare provider will use their professional judgment to determine if you have PEx and what treatments will help you. Antibiotics are prescribed for most PEx episodes. PEx in children can be challenging because their symptoms are usually mild, and their oxygen levels may be normal during an episode of PEx.

Other treatments for PEx may include corticosteroids or pulmonary rehabilitation (PR). PR will help you learn how to do daily tasks that will not negatively impact your breathing and trigger PEx. However, research does not support the use of corticosteroids for PEx, especially if symptoms are severe.

Chronic PEx will cause long-term damage to your lungs and cause poor lung function. Eating a CF-friendly diet (high calorie, high protein), following your treatment plan, and checking in with your doctor when you believe you may have PEx is the best prevention.

A Word From Verywell

Cystic fibrosis can cause multiple chronic illnesses at the same time. While there is no cure for CF, treatments have greatly improved over the years.

It can be extremely challenging to prevent lung infections and chronic pulmonary exacerbations. However, preventing PEx is the only way to protect your lung health and preserve lung function. Get familiar with your baseline lung function so that if anything changes, you can see your healthcare provider right away to get treatment. Eat well, exercise, and take all prescribed medicines as directed.

If you find that you are having more PExs than usual or having a new onset of PEx, consider talking to your healthcare provider about the possibility of changing your treatment plan.