Adherence of Subjects With Cystic Fibrosis to Their Home Program: A Systematic Review

Abstract

BACKGROUND: The management of cystic fibrosis (CF) includes adherence to a home management program (airway clearance, medication, nutritional advice, and exercise). This has led to an increase in life expectancy, although the benefits depend greatly on a patient's level of adherence to daily treatments at home. To date, no systematic review has established adherence rates to all World Health Organization guidelines in the home setting; hence, this review was undertaken. METHODS: The review comprised 3 phases. A methodological assessment of databases (Embase, CINAHL, PsychINFO, PEDro, PubMed, Cochrane Central Register of Controlled Trials) identified potentially relevant papers. These papers were screened for inclusion criteria by 2 independent reviewers, data were extracted, and the internal validity was rated using a valid and reliable scale. Results were categorized into 4 themes: medication, nutrition, airway clearance techniques, and exercise. RESULTS: The search generated a total of 26 papers, 24 of which were rated as being poor quality. Adherence to a treatment program for CF patients is generally low (from 22% for nutritional guidelines to 130% for oral antibiotics), and it varies greatly depending on the type of treatment and the method of assessment employed (objective tool vs self-reported questionnaires). CONCLUSIONS: Consensus on how to measure adherence is lacking, and the quality of studies addressing adherence in this population is generally poor. Overall, studies using self-reported measures yielded higher adherence scores than those that used objective measures, suggesting that current efforts to improve methods of adherence are appropriate. The prevalence of non-adherence remains unclear due to these limitations.

A weekend/weekday comparison of adherence to daily treatment regimens in adults with cystic fibrosis

“…In line with previous research, the results of the current research indicate that adherence differs between treatment types (Abbott et al, 1994;O'Donohoe et al, 2014). However, in the current research adherence was highest for pancreatic enzymes and vitamins, whereas Abbott et al (1994) reported that adherence was highest for pancreatic enzymes and exercise.…”

“…Adherence rates vary across studies, due to the variety of different methodologies used and the limitations of these methodologies (Kettler et al, 2002). As self-reported measures often inflate rates of adherence, there is a need to investigate methodologies that are accurate and reliable O'Donohoe & Fullen, 2014). Electronic data collection methods, for instance nebulisers which directly record adherence to nebulised treatment (Daniels et al, 2011), are the "gold standard", but this technology cannot currently be used to assess adherence to tablet treatments and chest physiotherapy.…”

Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis

“…The changing role of parents, siblings and friends during transition are also important to consider. Approaches for optimal transition are detailed in the ECFS standards of care and are well described in important disease-specific and generic guidelines [8,21,22,[25][26][27].…”

“…Adolescence is a vulnerable period due to physiological, developmental and psychosocial changes. This stage of life is frequently associated with poor treatment adherence and decline in respiratory function and nutritional status [24][25][26]. Transition works best when there is close integration between paediatric and adult centres and the process directly involves the adolescents with CF and their families in the process [27].…”

Effectiveness of a Home-Based Active Video Game Programme in Young Cystic Fibrosis Patients

“…In general, poor levels of exercise adherence have been reported for this population and are caused by the long-term and arduous nature of the therapeutic regimens [13]; in another study, however, evidence of good adherence (57-88%) was found over other therapies [14]. Young patients with CF experience difficulty adhering to exercise routines when the activity is tedious or disliked [15].…”