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Cystic fibrosis care

Cystic fibrosis care

People with CF have chronic persistent infection and inflammation of their airways that periodically worsens, causing episodes of airway obstruction and increased symptoms that we call pulmonary exacerbations. It is critical to recognize these exacerbations and properly treat them with antibiotics and other therapies in order to prevent lung damage. Obstruction of the air passages can be measured through lung function testing. The most important measure of lung function is forced expiratory volume in one second (FEV1), which correlates with quality of life and life expectancy. 

How we are improving cystic fibrosis care

When the CHoR at VCU CF Care Center team recognized in 2012 that the average FEV1 of our patients was well below the national average, we initiated a program to improve FEV1 by ensuring that pulmonary exacerbations were promptly recognized and treated. The team developed tools and strategies to assist in their recognition and an algorithm to guide their treatment and follow-up to ensure success of treatment. Patient trend reports and the goals of the visit were discussed during pre-clinic meetings, and shared with families, who were carefully integrated into the process. 

Why it matters to our patients

We know from the CF Foundation Patient Registry, which tracks the experience of all patients in the United States cared for at accredited CF care centers, that

  • Pulmonary exacerbations are the most important cause of lung damage in people with CF
  • Recovery from pulmonary exacerbations is more likely if they are treated with antibiotics
  • Despite their best efforts, CF care providers do not consistently diagnose and treat pulmonary exacerbations
  • CF centers that are best at keeping their patients lung function (FEV1) high are more consistent in their treatment of pulmonary exacerbations.

By closely monitoring lung function, following consistent treatment algorithms and verifying treatment success, we aimed to improve the lung function, quality of life, and life expectancy of people with CF.

How we measure

Adherence by the team to the algorithm improved over time; it was never 100%, because the intention of the algorithm was to provide guidance in general, but not dictate all care decisions. 

collection of graphs

Average lung function of patients at our care center increased substantially as a result. In particular, the gap in FEV1 between adolescents 13-18 years of age and younger children 6-12 narrowed considerably as adolescence is typically a time of rapid decline in lung function. 

Rolling 12 month mean of the best ppFEV1 measured for each patient 6-18 years attending the CHOR at VCU CF center graph

Overall, the average FEV1 of our patients rose from the lowest decile among US CF care centers in 2012 to the highest decile in the years 2015-2017.

average FEV graphs

What you can do as a caregiver

To keep your child healthy, have him or her follow the regular prescribed treatment routine that includes airway clearance therapy, medication, a fitness plan, and nutritional therapies. Make sure the CF Care Team knows about any new symptoms or drops in lung function, and expect that something will be done to treat this and that a follow up visit to ensure that the treatment works is very important.

For more information, go to https://www-ncbi-nlm-nih-gov.proxy.library.vcu.edu/pubmed/30017327

Or see

Schechter MS, Schmidt HJ, Williams R, Norton R, Taylor D, Molzhon A. Impact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis. J Cyst Fibros. 2018 Jul 14. pii: S1569-1993(18)30628-3. doi: 10.1016/j.jcf.2018.06.003.


We're here to help with any quality-related questions. Please contact:

Michelande Ridoré, MS 
Quality Manager
Michelande.Ridore@vcuhealth.org