Cystic Fibrosis Patients Step-Up to Better Breathing

By Michael Cantine, BSAST, RRT, CPFT, AE-C, and Paula Lomas, MAS, RN, CCRP 

Individuals with cystic fibrosis (CF) have many demands placed on them because of the great burden of their daily treatment. They may have to complete up to several hours of chest physiotherapy (CPT), high frequency chest wall oscillation (HFCWO), or a variety of airway clearance techniques (ACT) every day. In addition, they have many different nebulized therapies such as bronchodilators, dornase alpha, hypertonic saline, or inhaled antibiotics. CF patients also have to contend with oral antibiotics and pancreatic enzyme replacement therapy, not to mention all of the issues that have to do with IV antibiotics as a part of their regimen.1

Due to the abnormal function of the cystic fibrosis transmembrane conductance regulator (CFTR), individuals with CF have to cope with declining lung function due to the reduced ability to remove thick, sticky secretions. This setting in the lung produces a perfect environment for a host of organisms that create a downward spiral of infection, inflammation, mucus-plugging, and diffuse bronchiectasis. This downward spiral contributes to pulmonary exacerbation, and pulmonary exacerbation contributes to the decline of lung function in the CF patient.2

Quality Improvement

The Cystic Fibrosis Foundation (CFF) has been an advocate for the multidisciplinary team approach in the treatment of CF patients. In using this approach, they foster optimal care of the CF patient. The typical CF center team includes physicians, nurses, social workers, dieticians, pharmacists, respiratory care practitioners, and physical therapists. In addition, the CFF supports the work of scientists and researchers to develop therapies and drugs to improve the health of CF patients. Presently, there is a great deal of interest in the new medication, ivacaftor (Kalydeco, Vertex), a CFTR potentiator in individuals with the G551D mutation. Other drugs are also being developed. Currently, there are almost 30 drugs in the pipeline.3

The CFF places a great deal of importance on quality improvement activities at the CF centers it supports. One such program is the Adult Quality Improvement (AQI) Program with the Dartmouth Institute Microsystem Academy. It seeks to improve pulmonary and nutritional outcomes for CF patients. The program creates a platform for CF centers to work together to improve their overall care. One notable project that came out of the first program supported by the CFF was the REACT program. REACT, an acronym for Re-Education of Airway Clearance Techniques, is the brain child of Robert Zanni, MD, and the CF team at Monmouth Medical Center, Monmouth, NJ. Essentially, the REACT program reviews airway clearance on an annual basis and reteaches about any deficiencies that may be noted. After 2 years of the program, Monmouth’s average forced expiratory volume in the first second (FEV1) scores among its patients had improved 10%. This illustrates innovative thinking that is practical and useful. As a result, many CF centers have adopted this approach and the winner is the CF patient who has improved lung function.4

The Adult CF Center at Morristown Medical Center (MMC), Morristown, NJ, was one of 13 CF centers that participated in the AQI program in 2011–2012. The MMC team wanted to improve pulmonary outcomes. Many times when patients came into the CF center, they were surprised by the decline in their lung function. In some cases, lung function was better than what the patients had anticipated. But overall, the observation was that patients did not have a grasp of what their lung function was at any given time. They knew what their lung function was at the time of their visit to the Adult CF Center at MMC, but that was it. Clinical decisions are made based on lung function, as well as other clinical values. Noting this observation, the center’s staff asked patients if they thought a home spirometer would help them keep track of their lung function. Overwhelmingly, they reported that they would find this information useful.

Patient Commitment

In 2008, the Adult CF Center at Morristown Medical Center performed a Plan-Do-Study-Act (PDSA) project to improve pulmonary scores. In this project, patients were asked to perform one extra ACT during the course of the day. This was to illustrate the importance of ACT and its ability to not only relieve obstruction, but reduce the amount of infection and inflammation present in the airways.5 In the first quarter of 2008, 26 patients were asked to participate in the project. Sixteen patients agreed to perform an extra ACT (adherent group). Ten patients either declined or did not keep up their commitment (nonadherent group). The mean FEV1 in the adherent group improved by 21%. The FEV1 in the nonadherent group declined by 4%. From this project, the MMC Adult CF Center demonstrated the importance of ACT’s effect on pulmonary function. The results of this project were communicated to patients via the MMC CF patient newsletter.

The 2011–2012 AQI project used home spirometers to keep track of lung function, and ACT as the first-line response to increasing exacerbation symptoms. The funds used to purchase the spirometers were provided by the Atlantic Health Foundation as a part of their Mini-Grant program. The decision to use ACT as the first-line response was based on the observations in the PDSA project in 2008. The patients were given the PIKO 6 spirometer from nSpire Health Inc, a daily self-assessment of exacerbation score (based on the Akron Children’s Hospital exacerbation score), and a daily log. The patients were instructed to measure their FEV1 once per day, do a self-assessment of exacerbation, and record the results in the daily log. If they noted a decline in lung function, with an increase in exacerbation, they were asked to step-up their ACT activities. The patients noted on their logs: the numerical value of their exacerbation, their FEV1, the trend of the values, their response to the information (if they needed to step-up ACT), and, if they did step-up ACT activities, which ACT they used.

Forty-four patients were recruited for this project. The program was entitled “Step Up to Better Breathing” (SUBB). Although the program was a part of the Adult CF Center’s AQI activities, the center decided to include patients 15 to 18 years old from the pediatric center because they would be transitioning to the adult center relatively soon. Ten patients came from this age group, and 34 patients were adults 18 years old and older. The patients participated in the program by recording their daily lung function and exacerbation scores at home. This time period began with their program enrollment visit and ended with their next follow-up visit, which was usually within 2 to 3 months. At the beginning of the project, the patients were asked to assess their exacerbation level, measure cystic fibrosis quality of life score (CFQ-R), and determine their FEV1 values. The center measured CFQ-R (which is a standard measurement of quality of life in CF), because there was a concern that using the spirometer daily may increase treatment burden for the patients.

The study assessed the mean value of change in CFQ-R, change in pulmonary exacerbation score, and change in FEV1. The mean of the CFQ-R score before the study was 73.3 and the CFQ-R post-study was 72.0. There did not appear to be a significant effect in quality of life. The mean FEV1 pre-study was 2.39 liters and it was 2.44 liters post-study. While this was an improvement, it did not appear to be a significant improvement. The pre-study exacerbation score was 2.66 and post-study it was 1.87. This represented a 30% reduction in the exacerbation score. At the end of the study, the patients were given a post-study questionnaire. The questionnaire shed some light into the experience of the patients while they were involved in the study.

The questionnaire was in the form of statements, and the responses were in the form of the Likert Scale: strongly disagree, disagree, neither agree or disagree, agree, and strongly agree. For the statement, “I feel the SUBB study helped me to understand my disease better,” 87% of the respondents agreed or strongly agreed. For the statement, “I understand and I am able to identify CF pulmonary exacerbation better since I participated in the SUBB study,” again, 87% of the respondents agreed or strongly agreed, but in this statement, no one disagreed or strongly disagreed. The statement evaluating treatment burden was: “I feel that the use of the PIKO 6 increased my treatment burden.” This statement had a wider range of responses, 57% of the respondents strongly disagreed or disagreed and 29% agreed or strongly agreed. For the response to the statement “I plan to continue to use my PIKO 6 spirometer as a tool to manage my disease,” 79% agreed or strongly agreed. The responses to the questionnaire demonstrated that the patients learned from their experience. They understood pulmonary exacerbation better and how it affects them, they learned to manage their disease better, and they could see directly how stepping up ACT can improve lung function.

Other CF centers have used spirometers in their studies with various degrees of success. Johns Hopkins performed a study and made the observation, “Home monitoring detected exacerbations an average of 16 days before patients contacted their care center because of symptoms.”6 At the 2011 North American Cystic Fibrosis Conference held in Anaheim, Calif, two posters analyzed the use of home spirometry in CF. Goss et al showed that “early intervention in pulmonary exacerbation using home symptom monitoring and home spirometry is feasible with reasonable compliance.”7 In addition, Gutierrez et al demonstrated that those patients who were historically more adherent took advantage of the information provided by home spirometry.8

The Morristown experience of home spirometry revealed some additional benefits, including:

  • Greater patient information provided to the center.
  • Better clinical management of the patient at home.
  • Greater awareness of lung function between center visits.
  • Better education about pulmonary exacerbation for the patients.
  • The patients are better able to self-manage their disease at home.

Although there was not an immediate overall improvement in FEV1 in this group of patients, it takes a great amount of time to see changes in this value. The MMC Adult CF team continually looks at ways to improve the care and outcome measures of CF patients. The team is currently looking at ways to improve nutritional outcomes. The QI team at MMC not only includes CF center team members but patient members as well. The patient members provide valuable first-hand experience and insights into the quality of care at a CF center and how it may be improved. ■

 Michael Cantine, BSAST, RRT, CPFT, AE-C, is lead respiratory care practitioner for the diagnostics team, as well as the Adult Cystic Fibrosis Center Respiratory Resource, and Paula Lomas, MAS, RN, CCRP, is nurse coordinator for the Adult Cystic Fibrosis Center, Atlantic Health, Morristown Medical Center, Morristown, NJ. For further information, contact


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