The problem

Cystic fibrosis is the most common, life-limiting, recessive genetic condition in Caucasian populations.1 Respiratory disease accounts for 90% of mortality.1 Internationally recommended models of CF care include a multidisciplinary team (MDT) and annual review (AR).2–4 Our centre implemented an AR in 2013 after a 2011 peer review highlighted lack of AR as concerning. Furthermore, 2010 national data demonstrated our centre was one of the lowest performing in terms of lung function.5

The AR was introduced to improve patient outcomes, partly by encouraging standardisation of care, including prescription of inhaled mucolytic agents dornase alfa (DA) and hypertonic saline (HS). Both agents reduce morbidity and pulmonary exacerbations in CF, and DA is associated with improved lung function.6 7 Despite evidence for its efficacy, DA was used significantly less in Australia compared with the USA in 2012.8

The aim

Given DA and HS are recommended as long-term therapy for all patients with CF ≥6 years,9 10 we used increase in mucolytic prescription as our marker of improvement and utility of AR.

The AR is a MDT assessment conducted by physiotherapists, nurses, dieticians, psychologists and doctors over weeks and months prior to patients’ birthdays (time of the AR). The findings are entered into a report (online supplementary appendix 1) together with recent investigations (spirometry, imaging and so on). Reports are reviewed at weekly MDT meetings and consensus recommendations are made, which are then discussed with the patient and family.

Making a case for change

A case for change was identified by the CF MDT and a Quality Improvement Working Group formed by representatives from each discipline. All members collaborated in creating a standardised AR template (online supplementary appendix 1). Families were educated about the rationale and details of the process.

AR processes require significant resources to run effectively and sourcing funding was an obstacle. Funding was ultimately obtained from the Department of Health, after demonstration that lack of AR contravened 2008 CF Standards of Care.2 This funding allowed for creation of a new position, the CF Fellow, an integral member of the team.

Improvements seen

We evaluated prescription of inhaled mucolytic agents in a retrospective cohort review of all patients with CF, before and after introduction of the AR; July 2011–June 2012 (2012 cohort) and July 2014–June 2015 (2015 cohort). Baseline characteristics were similar (table 1).

Mucolytic use was overall higher in 2015 than 2012; 78.4% versus 41.7%, respectively (P<0.01, risk difference (RD) 0.37, CI 0.28 to 0.45).

There was particularly increased DA use for patients >6 years (82.9% vs 34.8%, P<0.01, RD 0.48, CI 0.39 to 0.57) (figure 1).

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Figure 1

The use of dornase alfa (DA), 2015 versus 2012, by age.6 Dark blue <6 years. Light blue ≥6 years.

HS use was also higher overall in 2015 than 2012 (24.8% vs 16.2%, P=0.02), particularly for those ≥6 years (figure 2).

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Figure 2

The use of hypertonic saline (HS), 2015 versus 2012, by age.7 Dark red <6 years. Light red ≥6 years.

There was increased use of DA in the presence of comorbidities and markers of disease severity (table 2).

2015 DA use for all ages was 74%, higher than the 41.6% reported in the most recent CF Registry data from 2014.11 For children >6 years, DA use was comparable with the USA (82.9% vs 86%, respectively).9 We did not expect improvements in forced expiratory volume in one second (FEV₁) to be demonstrable in the short time between cohorts. Lung function was similar: normal or mildly impaired (FEV₁≥70%), 90% (2015) versus 89% (2012) and moderate or severely impaired (FEV₁ <70%), 10% (2015) versus 10.5% (2012). This is comparable with CF Registry data: 90.3% FEV₁ ≥ 70% and 9.7% FEV₁ <70%.

Of patients with lung function recorded in both 2012 and 2015 (n=139), 28.9% had better lung function in 2015— a surprising finding given expected decline in FEV₁ over time. The mean decline in FEV₁ over 3 years between 2012 and 2015 was 4.2%; 5.6% for adolescents and 1.9% for children<12 years, consistent with expected trajectories.12 For those on DA in 2012, the decline was 4% versus 4.3% for those not on DA.

ARs have been shown to increase investigations and MDT assessments,4 without affecting medical interventions.13 Our evaluation importantly showed improvement in a medical intervention.

Learning and next steps

There were several secrets to success. Most notable was involvement of the whole CF team in the AR design and implementation. By providing education to families, we gained consensus about the need for an AR and garnered enthusiasm.

In hindsight, it would have been beneficial to incorporate patient satisfaction and quality of life assessments into the intervention evaluation. It would have also been valuable to measure patient-reported adherence. Looking forward, we hope to evaluate these measures and incorporate patient preference into the model of care.

It would be beneficial to evaluate long-term success of the AR by evaluating health outcomes, including lung function, over a longer time period.