Managing and Treating Cystic Fibrosis Exacerbations

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 a hospital stay followed by more care at home.

Frequency of exacerbations

Some people with cystic fibrosis (CF) remain relatively healthy and only experience an exacerbation every few years. Those with more damaged lungs may develop exacerbations two to four times a year.

People with regularly scheduled hospital stays aimed at controlling their lung infections sometimes call these hospitalizations a CF tune-up or CF spa.

All exacerbations should be treated quickly and aggressively to help keep the lungs as healthy as possible. Exacerbations can be dangerous because up to 25% of people with CF do not recover their baseline lung function within 3 months of treatment.¹

Signs of an exacerbation

There is no set definition for when a lung infection has become an exacerbation. It may vary widely from person to person. The general signs of an exacerbation are:

• Increased cough
• Change in the sputum’s (lung mucus) color or quantity
• Decreased appetite or weight
• Decline in FEV1 numbers
• Increased wheezing or crackling
• Difficulty exercising, feeling more tired than usual
• Blood streaked mucus (hemoptysis)
• Fever
• Repeated lung infections²

Pregnant women who have CF and those with CF-related diabetes should be monitored closely for early signs of exacerbations.²

Treating cystic fibrosis exacerbations

Treatment for an exacerbation requires hospitalization, often for as long as two weeks. Some people with CF may be able to spend a few days in the hospital and then continue their treatments at home.

The type of medicines given for an exacerbation depend on the results of blood tests and sputum cultures, which will tell the medical team which specific germs are causing worsening symptoms.

Exacerbation treatments include:

• Round-the-clock antibiotics often delivered through a peripherally inserted central catheter, or PICC line
• More frequent airway clearance techniques, sometimes concentrated on certain areas of the lungs
• Other drugs such as mucus thinners and corticosteroids
• Intensive nutrition, sometimes delivered through a (enteral) feeding tube
• Supplemental oxygen or supported ventilation by need, depending on severity of exacerbations
• As much exercise as possible³

This intensive combination of therapies are designed to drive back the infections and inflammation that damage the lungs and improve lung function as much as possible.

Managing a hospital stay

Long hospital stays can be challenging. According to the CF Foundation’s 2017 Patient Data Registry, people in the U.S. with CF averaged 28.9 days a year receiving exacerbation treatments. On average, 18.3 of those days were in the hospital and 10.6 were home IV treatments. Adults are more likely to complete some of their treatment at home.³

Parents of school children with CF should coordinate lessons and homework during their child’s hospital stay so that the exacerbation does not interfere too much with school. For both children and adults with CF, it is important to arrange for visitors who will cheer them up by providing a break to the monotony of hospital routines. Consider bringing news of family and friends or going for a walk.

Preventing exacerbations

Most people with CF cannot avoid exacerbations completely, but being diligent about daily treatments, daily drug and enzyme regimes, exercise and good nutrition can help reduce the chances of a lung flare-up.¹

To prevent exacerbations, people with CF will often be given what are called prophylactic antibiotics, meaning these drugs are given to prevent an infection from taking hold in the first place or from getting worse. Other recommendations include:

• Infection prevention and control techniques, such as frequent hand washing
• Disinfecting equipment and surfaces
• Keeping a six-foot distance from others with CF and anyone with a cold, flu, or infection.
• Getting all recommended immunizations and flu shots¹

Preventing exacerbations is vital to improve or maintain lung function and improve quality of life.