Lung Function in Cystic Fibrosis Patients

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Each year we care for more than 300 children and young adults with cystic fibrosis, a life-threatening genetic disease that affects the lungs. Our Cystic Fibrosis Center is one of the largest treatment centers accredited by the Cystic Fibrosis Foundation in the nation, and we strive to provide the highest-quality care available anywhere.

One of the ways we measure our success in treating cystic fibrosis is the overall lung function of our patients. Beginning at around age six, children with cystic fibrosis regularly undergo a test that involves taking a deep breath and blowing as hard as they can into a measuring device for one second. The results of the test — a measure known as forced expiratory volume in one second (FEV1) — are reported as a percentage of what would normally be expected (based on factors such as a patient’s age, gender and size).

The higher the percentage, the better the patient's lung function. By looking at the average lung function across all of our cystic fibrosis patients, we can assess how effective our treatment is.

How are we doing?

We receive annual data from the Cystic Fibrosis Foundation's national patient registry, which collects information on the lung function of approximately 20,000 children and adolescents with cystic fibrosis who receive treatment at the 115 hospitals and care centers accredited by the foundation. Our goal is to meet or exceed the average lung function (as measured by FEV1) of the 10 accredited institutions with the highest overall patient FEV1 scores. We also compare ourselves to the registry as a whole.

Our historical performance shows that the lung function of our patients has been consistently higher than the national average in all three age groups covered by the registry:

Average Patient Lung Function (FEV1)

Ages 6-12

Ages 13-17

Ages 18-30

(Last updated: March 2, 2015. All charts show most recent available data.)

We fall below the benchmark set by the top 10 care centers in the Cystic Fibrosis Foundation registry. This is a sign that the care we provide our cystic fibrosis patients can be improved, and we're working to make that happen.

What are we doing to improve?

Our team of doctors and nurses reviews all of our patients on a regular basis to make sure they're receiving care that follows the Cystic Fibrosis Foundation's clinical guidelines. We also ask our cystic fibrosis patients to visit us frequently — at least four times a year — so we can monitor their lung function, medications, nutrition and overall health.

How do we compare to other hospitals?

Hospitals and care centers accredited by the Cystic Fibrosis Foundation report lung function data for their patients to the foundation's registry. As noted above, our patients' lung function is above the national average. You can find more detail and compare our results with other hospitals and care centers by looking at the Care Center Data section of the Cystic Fibrosis Foundation's website. (Please note that free registration is required to access the data.)

For more information on our cystic fibrosis care, visit our Cystic Fibrosis Center.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

Boston Children's Hospital 300 Longwood Avenue, Boston, MA 02115 617-355-6000 | 800-355-7944

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