Evidence
Commentary on selected evidence
Commentary on selected evidence
With advice from topic experts we selected 2 studies for further commentary.
Information and support
We selected a qualitative study, Sampson et al. (2015), for a full commentary because it is a UK‑based study that emphasises the need for individualised care and psychosocial support at key transition points for patients diagnosed with idiopathic pulmonary fibrosis (IPF) and their carers.
What the guideline recommends
NICE guideline CG163 recommends that the consultant respiratory physician or interstitial lung disease specialist nurse should provide accurate and clear information (verbal and written) to people with IPF, and their families and carers. This should include information about investigations, diagnosis and management, and an interstitial lung disease specialist nurse should be available at all stages of the care pathway to provide information and support to people with IPF, and their families and carers (recommendations 1.3.1–1.3.4).
Methods
Sampson et al. (2015) conducted a qualitative study aiming to explore the perspectives of patients and their carers across the IPF spectrum to inform the development of clinical pathways and multidisciplinary service interventions.
Recruitment was carried out at 2 UK specialist interstitial lung disease clinics. Twenty seven patients (18 men and 9 women) with IPF and 21 carers (6 men and 15 women) were included. Six patients had no carer and none of the patients had been prescribed pirfenidone. A carer was defined as a person who provided emotional support and contributed most to the patient's care in the earlier stages of disease. Patients' IPF was categorised according to disease extent. An experienced qualitative researcher carried out semi-structured interviews with participants. Anonymised transcripts were analysed by the same researcher using Interpretative Phenomenological Analysis (IPA). The generated themes from the analysis were discussed and agreed by other members of the research team. The consolidated criterion for reporting qualitative research (COREQ) 32‑item checklist was used for explicit and comprehensive reporting of the study methods.
Results
Several common themes emerged from patients at different disease stages.
Communication and information
The key needs of patients and carers were structured around 3 areas: context, timing, and content and format of information. Patients and carers described a need to balance honesty and hope, while dealing with a terminal prognosis. They felt that the focus of the consultation at specialist IPF clinics was often disconnected from their experience and the impact of the disease on their everyday life. They also felt that they need to receive information at an appropriate pace triggered by changes in their health status.
Patients and carers showed a good understanding of the overall prognosis for IPF. However, patients had difficulty translating this to their own particular disease progression and to the available management and support options.
Changes in health status
Patients developed their own subjective strategies for monitoring IPF. The findings indicated that patients and carers need different information and that these needs change over time and in particular for those at the progressive stage of the disease. The findings showed that although patients might be reluctant to receive detailed information, the carers need frank information about functional deterioration in order to plan future care.
Functional activity
The risk of social isolation for patients and carers was increased along with patient's health deterioration. Because of the uncertainty about the IPF stages, most patients and carers felt unable to identify potential sources of help and support. Although deteriorating health increased the fears of unemployment and financial status in patients, it extended future emotional and domestic burden on carers.
Understanding of symptoms and medical interventions
Specific concerns were particularly specified from carers in relation to symptom monitoring regarding breathing, cough and use of oxygen. Different expectations were observed between patients and carers around oxygen use. Although carers viewed the oxygen use positively as facilitating daily living, patients often perceived it as a failure on their part. Pulmonary rehabilitation was viewed as positive intervention by patients; however, carers felt these strategies were better accepted if offered by a professional rather than by themselves. Patients and carers often compared their situation unfavourably with cancer patients whom they thought to have "help coming from every direction".
Roles and coping strategies of patients and carers
Significant differences were identified in the range of coping strategies employed by patients and carers. Acceptance and adapting to change were the main coping strategies for patients through all stages of the disease. Carers had expressed that they placed the needs of the patient above their own and provided emotional support and motivation. Carers expressed fears about managing their domestic situation in the future and living with feelings of guilt.
Strengths and limitations
Strengths
The aim of the research was clearly specified and the appropriate qualitative methodology was used. The research is a UK‑based study that addresses a topic related to NICE guideline CG163 recommendations about information and support. Improvement in psychosocial health and quality of life in patients with IPF was considered as a relevant outcome in the included studies in NICE guideline CG163. That is broadly covered in the current paper.
Limitations
The population for this study was recruited from 2 clinics in the UK, which the service delivery model and setting may have been different from other parts of the UK. In addition none of the patients included in the study received pirfenidone as a part of their treatment. That might have an effect on their experience.
Impact on guideline
This multicentre, mixed-methods study assessed the care needs of patients with IPF at 4 stages of the disease through semi-structured interviews with patients and paired carers. The study suggests that patients diagnosed with IPF have a clear understanding of their prognosis but little understanding of how their disease will progress and how it will be managed. The evidence indicates that support needed for patients and carers at key transition points. This is in line with NICE guideline CG163 that recommends 'an interstitial lung disease specialist nurse should be available at all stages of the care pathway to provide information and support to people with IPF and their families and carers with the person's consent' (recommendation 1.3.3). Current recommendations promote a supportive approach coordinated by a multidisciplinary team with appropriate skills (recommendations 1.3.1–1.3.4).
NICE has also produced guidance on the components of good patient experience in adult NHS services.
Management – disease-modifying pharmacological interventions
We selected Sun et al. (2016) for a full commentary because it addresses pharmacological management of IPF in relation to guidance on use of oral N‑acetylcysteine.
What the guideline recommends
NICE guideline CG163 recommends 'advise the person that oral N‑acetylcysteine is used for managing IPF, but its benefits are uncertain' (recommendation 1.5.13).
Methods
The systematic review included studies that compared N‑acetylcysteine-treatment group with a control group that reported specified outcomes. Searches only performed on the limited databases (PubMed, EMBASE, Cochrane Central and Google Scholar). No clear search period was specified.
Methodological quality of the included studies was varied and most were of low quality.
Results
Findings from meta-analysis of 5 trials, with a total of 564 patients, reported no beneficial effect of N‑acetylcysteine on changes in forced vital capacity (standard mean difference [SMD]=0.07, 95% confidence interval [CI] 0.13 to 0.27, p=0.52), changes in predicted carbon monoxide diffusing capacity (SMD=0.12, 95% CI 0.06 to 0.30, p=0.18), rates of adverse events (odds ratio [OR]=4.50; 95% CI 0.19 to 106.41, p=0.35), or death rates (OR=1.79, 95% CI 0.3 to 5.12, p=0.28) between the N‑acetylcysteine group and the control group. N‑acetylcysteine compared with placebo was found to have a significant effect only on decreases in percentage of predicted vital capacity (SMD=0.37, 95% CI 0.13 to 0.62, p=0.003) and decline in 6‑minute walking test (SMD=0.25; 95% CI 0.02 to 0.48, p=0.04).
Strengths and limitations
Strengths
The systematic review targeted the exact population and outcomes reported in NICE guideline CG163. All‑cause and IPF‑related mortality was 1 of the critical outcomes outlined by the guideline that was reported in the study. The study also reported other relevant outcomes including changes in forced vital capacity, changes in predicted carbon monoxide diffusing capacity, and rates of adverse outcomes.
Limitations
No exclusion criteria were specified. Searches were only performed on the limited databases (PubMed, EMBASE, Cochrane Central and Google Scholar). No clear search period was specified. Additional searches were not carried out on the bibliographic databases.
All included studies in the systematic review had risk of biases and generally were assessed as low quality. No sensitivity analysis was performed based on the quality of the studies. It is unclear how and by whom the quality assessment of the studies was performed. The systematic review targeted the exact population and outcomes reported in NICE guideline CG163. However, the stage of the disease was not reported and considered in the analysis of the data.
Studies were heterogeneous in terms of population and interventions. There were variations in dosage regimens of N‑acetylcysteine in the included studies. There were also variations between the control groups in the included studies. Two of the 5 included studies had no therapy in the control group, whereas 2 had placebo and 1 used bromhexine hydrochloride in the control group. These variations were not taken into account in data analyses in the form of stratified and subgroup analyses, which undermines the validity of the findings, although the random-effects model was used in pooling results of heterogeneous studies.
Impact on guideline
This systematic review evaluated the efficacy of N‑acetylcysteine, compared with control, for treating IPF. Findings showed no beneficial effect of N‑acetylcysteine on changes in forced vital capacity, changes in predicted carbon monoxide diffusing capacity, rates of adverse events, or death. N‑acetylcysteine was found to have a significant effect only on decreases in percentage of predicted vital capacity and decline in 6‑minute walking test distance.
NICE guideline CG163 recommends 'advise the person that oral N‑acetylcysteine is used for managing IPF, but its benefits are uncertain' (recommendation 1.5.13). The recommendation already acknowledges uncertainty about the benefits of this drug, and no new safety concerns have been raised about its use. Therefore the findings from this study do not impact on current recommendations.
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