To the Editor:

Bronchiectasis is receiving increased awareness from clinicians, researchers and stakeholders. However, despite the development of international and national guidelines in bronchiectasis, clinical practice does not necessarily follow quality standards and clinical recommendations. The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) together with the European Lung Foundation (ELF) coordinate patient-initiated activities designed to facilitate bronchiectasis awareness and care [1].

We aimed to investigate the experiences of people with bronchiectasis in receiving their diagnosis and care. An online survey was developed by EMBARC and the ELF bronchiectasis patient advisory group. The survey consisted of 67 items, translated into nine languages, and included items for demographics, diagnosis and testing, and care and treatment experiences. The survey can be viewed here: www.europeanlunginfo.org/bronchiectasis/news/tell-us-about-your-journey-to-diagnosis-and-treatment.

The survey was accessible for completion from September 2020 to March 2021. Participation in the survey was anonymous and voluntary, and no ethical approval was required. Statistical analyses were performed using IBM SPSS Statistics 28.0 (IBM, New York, NY, USA). Inferential statistics were applied to investigate the relationship between responses for diagnosis and treatment, and self-reported clinical and demographic characteristics, such as sputum volume, dyspnoea, body mass index (BMI) and age. To assess disease severity through the patient survey, items of the Bronchiectasis Severity Index (BSI) [2] were included. This patient-derived modified severity score did not include radiology (expected to be unknown by most patients) and age (since this severity score was tested as an outcome measure). Association between categorical variables was assessed using the Chi-square test. Trend among binomial proportions of a dose–response relationship was evaluated using the p for trend.

The patient survey received 765 submissions with 760 valid respondents from 40 countries. Most respondents were from the UK (n=542, 71.3%), Germany (n=49, 6.4%), USA (n=25, 3.3%) and Canada (n=21, 2.8%). The respondents’ demographic and clinical characteristics are presented in table 1. Patient-derived severity scores were available for 324 (42.6%) respondents; the item that had the fewest responses was lung function (n=367, 48.3%). The range of the patient-derived severity score was 0 (low disease severity) to 16 (high disease severity), with a median (interquartile range) of 4 (2–7).

Demographics and clinical characteristics of the respondents to the patient survey, and availability of airway clearance management and pulmonary rehabilitation (n=760)

193 respondents (25.4%) reported having had symptoms for 10 years or longer and 114 respondents (15.0%) had symptoms for 5–10 years before being diagnosed with bronchiectasis. 195 respondents (25.7%) reported having been misdiagnosed with some other disease, such as asthma (n=96), chronic bronchitis (n=27), COPD (n=18) or other. A symptom onset to diagnosis interval of 10 years or more was associated with bacterial infection: 84 of 193 (43%) respondents with an interval of 10 years or longer reported having bacterial infection, compared to 164 of 489 (33%) of those with shorter interval (p=0.015). Hospitalisations, lung function and patient-derived severity scores were not associated with symptom to diagnosis time.

Care was mainly provided by a general practitioner (n=179, 23.6%), or a respiratory specialist who does not specialise in bronchiectasis (n=148, 19.5%), with 158 respondents (20.8%) receiving care at a bronchiectasis referral centre. The diagnostic test items of the survey were based on the European Respiratory Society (ERS) guidelines [3]. A minority of respondents answered that they had been referred to tests “that measure the immune system” (n=323/646, 42.5%), and diagnostic tests for cystic fibrosis (n=153/617, 20.1%) and primary ciliary dyskinesia (n=75/467, 9.9.%). Only 258 respondents (33.9%, total replies to item n=628) had sputum cultures sent for analysis during a clinically stable stage of their disease, while 348 of 636 respondents (45.8%) had sputum cultures during exacerbations at least once since diagnosis.

During an exacerbation, approximately half of the respondents (n=382, 50.2%) replied that they found it difficult or very difficult to access a primary care physician with a good understanding of chest infections. Respondents also commonly reported that getting a sputum culture performed when an infection had started was difficult or very difficult (n=269, 35.4%) and they experienced delays in receiving the results of sputum cultures (n=225, 29.6%).

Most respondents (n=626 of 672 responses to item, 82.4%) reported having cough productive of sputum. However, only 473 of 672 respondents (62.2%) had been advised to perform airway clearance techniques, and 469 respondents (61.7%) were referred to a specialist for instructions.

A minority of respondents (n=149, 19.6%) had been referred to pulmonary rehabilitation (PR) and only 90 of 187 respondents who reported modified Medical Research Council (mMRC) dyspnoea scores ≥2 attended PR, despite the recommendation for a PR referral according to the clinical guidelines [3, 4]. Attending PR was associated with a higher mMRC dyspnoea score (p<0.001), older age (p<0.001) and higher disease severity (p=0.01). Sputum volume, sex, BMI and numbers of hospitalisations and exacerbations were not associated with attending PR.

Our results indicate that receiving a diagnosis of bronchiectasis typically takes years, often more than a decade, after symptom onset, which suggests diagnostic delays [3–6]. A long interval of symptoms is associated with bacterial infection. Moreover, implementation of the recommendations is often limited, and patients experience difficulties in accessing care. A social-media-based study reported similar findings [7] and a recent paper using EMBARC data from 16 723 patients with bronchiectasis also showed that access to specialist respiratory physiotherapy was low throughout Europe [8]. Patients reported that they often did not get a sputum sample tested, which makes the guideline implementation impossible. Patients also struggled to get a general practitioner to manage exacerbations, suggesting a need for education of primary care physicians. This is particularly relevant to the UK due to the demographics of our respondents.

Most patients with bronchiectasis reported that they are productive of sputum, but airway clearance is still underutilised. Despite that, most patients who had been advised to perform airway clearance had received education for this. This is in line with recent evidence that the most common reason for not performing airway clearance is the “clinician decision that it was not needed” [8]. Few recommendations for airway clearance require further investigation, so appropriate support is implemented within clinical services. PR is a comprehensive intervention that can improve exercise capacity, dyspnoea and quality of life in bronchiectasis [9].

Using surveys has some inherent limitations. Recall bias in the patient responses cannot be excluded, particularly for the items that inquired about experiences occurring over an extending duration or many years ago. For instance, time of diagnosis was over a decade ago for many respondents, but it is unlikely that the replies would have a large enough error to affect our findings due to our suggested categories. Assessing disease severity was limited in our study, as we were unable to collect measures such as radiology data, and most patients could not report their lung function. Future studies ideally should incorporate clinical data alongside patient surveys to correlate findings with disease severity. Moreover, as the survey partially overlapped with the COVID-19 pandemic, it might introduce bias in healthcare accessibility. However, given that most items addressed long-term issues, this potential bias would likely be minimal.

Our survey was conducted via an open link and ensured anonymity to encourage participation. Consequently, we cannot evaluate the risk of non-response or response bias. It is probable that self-selection and voluntary participation attracted patients who are deeply involved in medical management and those with a higher socioeconomic status. Moreover, we were unable to calculate the survey response rate, so caution should be exercised regarding the generalisability of our findings.

Our patient respondents were 82% female, which contrasts with previously reported patient demographics, as bronchiectasis registries report about 60% females [10]. It is possible that these findings relate to the tendency of women to answer questionnaires [11]. The online survey format may have also caused a bias towards patients with accessibility and familiarity with web-based questionnaires [12]. Nevertheless, our results highlight some novel findings based on patients’ experiences in accessing treatment, such as the low airway clearance and PR implementation. Formulating a disease severity score based solely on patients’ responses was feasible and although this requires further investigation, the association with treatments such as attending PR suggests acceptable validity.

In summary, the survey suggests a gap between bronchiectasis care recommendations and clinical practice. Our results highlight a need for improving education of primary care providers regarding bronchiectasis management, and improving accessibility to airway clearance management and PR.