User-perceived impact of long-term mechanical assisted cough in paediatric neurodisability.

AIM: To (1) compare the perceived benefit of long-term mechanical insufflation-exsufflation (MI-E) of children with neuromuscular disorders (NMDs) and central nervous system (CNS) disorders, including health care needs and treatment routines and (2) describe the children's health-related quality of life (HRQoL). METHOD: This cross-sectional study used a questionnaire and memory card data to assess the perceived benefit of MI-E via the Visual Analogue Scale (VAS; 10 maximum), willingness to pause treatment, level of health care needs before and after MI-E initiation, and the children's treatment routines. A DISABKIDS questionnaire assessed HRQoL (100 maximum). RESULTS: Seventy-three children using MI-E participated (42 males, median [interquartile range {IQR}] age 10 years 2 months [6 years 3 months-14 years 1 month]), 47 with NMDs (such as spinal muscular atrophy and Duchenne muscular dystrophy) and 26 with CNS disorders (such as cerebral palsy, encephalitis, neurometabolic and other diseases). The median (IQR) VAS score for the perceived benefit of MI-E therapy at stable state and respiratory tract infection were 9 (6-10) and 10 (8.5-10) respectively. Sixty-two per cent were reluctant or unwilling to pause MI-E therapy, with no NMD versus CNS disorder group difference. After MI-E initiation, fewer physician consultations and hospitalizations were reported by the group with NMDs. The MI-E routine was similar in both groups. The mean (SD) HRQoL score for 26 of 51 eligible children was 71 (16.7). INTERPRETATION: MI-E treatment was generally perceived as beneficial and performed equally in both diagnostic groups. HRQoL was in line with children with a moderate-to-severe chronic condition. WHAT THIS PAPER ADDS: Mechanical insufflation-exsufflation (MI-E) was generally perceived as beneficial by the children and parents. The reported benefit of MI-E was higher among daily than sporadic MI-E users. The MI-E treatment routine did not differ between diagnostic groups. The health-related quality of life in this neuropaediatric population was in line with that of children with other moderate-to-severe chronic conditions.

Effect of Respiratory Events on Health-Related Quality of Life in Patients Treated with Long-Term Noninvasive Ventilation.

BACKGROUND: Long-term noninvasive ventilation (NIV) can increase or maintain health-related quality of life (HRQoL) for patients with chronic hypercapnic respiratory failure (CHRF). Evidence from studies systematically assessing how NIV-specific factors influence HRQoL is limited. OBJECTIVES: The objective of this study was to describe HRQoL measured by the Severe Respiratory Insufficiency Questionnaire (SRI) in patients with CHRF treated with long-term NIV and to analyze the associations between HRQoL and hypoxemia, hypercapnia, and respiratory events such as apneas, hypopneas (AHI), and patient ventilator asynchrony (PVA) occurring during long-term NIV. METHODS: We included sixty-seven stable patients with established long-term NIV due to neuromuscular disease or thoracic cage disorders in a prospective cross-sectional study at Oslo University Hospital. Patients answered the SRI and underwent daytime arterial blood gases, nocturnal pulse oximetry, sleep polygraphy, and nocturnal transcutaneous CO2. RESULTS: The mean global SRI for 62 patients was 64.8 ± 14.5, with the highest score in SRI Social Relationships (79.5 ± 15.6). There were no differences in HRQoL between the different patient groups. Compliant patients had a significantly higher score in SRI Attendant and Sleep. Residual nocturnal hypoxemia affected both the subscale SRI "Respiratory Complaints" and SRI "Attendant Symptoms and Sleep." Persisting daytime hypercapnia, nocturnal hypoventilation, and high AHI affected the subscale SRI "Anxiety" negatively, while frequent PVA was associated with a lower score in SRI "Physical Function." CONCLUSION: In a group of patients with long-term NIV, undesired respiratory events during NIV are associated with lower HRQoL in several of the SRI subscales. We suggest designing interventional studies to confirm the possible relationship between HRQoL and respiratory events during long-term NIV.

Health-related quality of life as predictor for mortality in patients treated with long-term mechanical ventilation.

BACKGROUND: The Severe Respiratory Insufficiency (SRI) questionnaire is a specific measure of health-related quality of life (HRQoL) in patients treated with long-term mechanical ventilation (LTMV). The aim of the present study was to examine whether SRI sum scores and related subscales are associated with mortality in LTMV patients. METHODS: The study included 112 LTMV patients (non-invasive and invasive) from the Norwegian LTMV registry in Western Norway from 2008 with follow-up in August 2014. SRI data were obtained through a postal questionnaire, whereas mortality data were obtained from the Norwegian Cause of Death Registry. The SRI questionnaire contains 49 items and seven subscales added into a summary score (range 0-100) with higher scores indicating a better HRQoL. The association between the SRI score and mortality was estimated as hazard ratios (HRs) with 95% confidence intervals (95% CI) using Cox regression models and HRs were estimated per one unit change in the SRI score. RESULTS: Of the 112 participating patients in 2008, 52 (46%) had died by August 2014. The mortality rate was the highest in patients with chronic obstructive pulmonary disease (75%), followed by patients with neuromuscular disease (46%), obesity hypoventilation syndrome (31%) and chest wall disease (25%) (p < 0.001). Higher SRI sum scores in 2008 were associated with a lower mortality risk after adjustment for age, education, hours a day on LTMV, time since initiation of LTMV, disease category and comorbidity (HR 0.98, 95% CI: 0.96-0.99). In addition, SRI-Physical Functioning (HR 0.98, 95% CI: 0.96-0.99), SRI-Psychological Well-Being (HR 0.98, 95% CI: 0.97-0.99), and SRI-Social Functioning (HR 0.98, 95% CI: 0.97-0.99) remained significant risk factors for mortality after covariate adjustment. In the subgroup analyses of patient with neuromuscular diseases we found significant inverse associations between some of the SRI subscales and mortality. CONCLUSIONS: SRI score is associated with mortality in LTMV-treated patients. We propose the use of SRI in the daily clinic with repeated measurements as part of individual follow-up. Randomized clinical trials with interventions aimed to improve HRQoL in LTMV patients should consider the SRI questionnaire as the standard HRQoL measurement.

Effectiveness of nursing interventions for breathlessness in people with chronic obstructive pulmonary disease: A systematic review and meta-analysis.

AIM: To critically review and synthesize the findings of studies that evaluated the effectiveness of nursing interventions for improving breathlessness in adults with chronic obstructive pulmonary disease. BACKGROUND: Systematic reviews of nursing interventions for breathlessness in people with chronic obstructive pulmonary disease have not been specifically addressed. DESIGN: Systematic review with meta-analysis. DATA SOURCES: A systematic search of Medline, CINAHL, PsycINFO and Embase was performed for studies published between January 2000 and June 2017. REVIEW METHODS: Risk of bias, data extraction and meta-analysis were conducted using Cochrane methodology. The quality of evidence was assessed using the GRADE approach. RESULTS: Twenty papers were included. A meta-analysis of interventions performed at home, including two trials, showed a significant effect in favour of experimental groups for the symptom score of the St. George Respiratory Questionnaire compared with controls. A meta-analysis of interventions performed in clinics with home follow-up showed a significant effect in favour of experimental groups for the mastery and fatigue scores of the Chronic Respiratory Questionnaire compared with controls. In this category of intervention, an additional meta-analysis showed a significant effect in favour of experimental groups for the symptom, activity and total scores of the St. George Respiratory Questionnaire compared with controls. The quality of evidence was assessed to be very low to moderate. CONCLUSION: The results are equivocal as to whether nursing interventions performed at home and nursing interventions performed in hospital with follow-up improve breathlessness in people with chronic obstructive pulmonary disease.

Factors associated with change in health-related quality of life among individuals treated with long-term mechanical ventilation, a 6-year follow-up study.

AIMS: To examine changes and explanatory variables for changes in health-related quality of life in patients treated with long-term mechanical ventilation over a 6-year period. BACKGROUND: Long-term mechanical ventilation is a treatment for individuals with chronic hypercapnic respiratory failure, primarily caused by neuromuscular diseases, obesity hypoventilation syndrome, chronic obstructive pulmonary and restrictive thoracic diseases. Studies on long-term outcome on health-related quality of life and factors influencing it are lacking. DESIGN: Prospective cohort study. METHODS: Data were collected from the Norwegian Long-Term-Mechanical-Ventilation Registry and from patient-reported questionnaire in 2008 and 2014. Health-related quality of life was measured by the Severe Respiratory Insufficiency questionnaire, containing 49 items and seven subdomains. Linear mixed effects models were used to measure changes and identify factors for changes. RESULTS: After 6 years, 60 patients were still participating, out of 127 at baseline. Health-related quality of life improved significantly in the total score and in four subdomains of the questionnaire. Satisfaction with training in long-term mechanical ventilation was an explanatory variable for improved 'psychological well-being' and follow-up for improvement of 'anxiety'. Side effects of the treatment like facial soreness were associated with the total score. High age and high forced vital capacity were related to lower 'physical function' and improved 'social functioning', respectively. CONCLUSION: Long-term mechanical ventilation over 6 years improved health-related quality of life in most patients. Patient training, follow-up and reduction of side effects, largely delivered by trained nurses, contribute to achieve the main goal of the treatment-improved health-related quality of life.

The Norwegian version of the Severe Respiratory Insufficiency Questionnaire.

The aims of this study were to translate and adapt the Severe Respiratory Insufficiency (SRI) questionnaire into Norwegians and to test its reliability and validity.Data were collected from a cross-sectional survey and were linked to the Norwegian Registry of patients receiving long-term mechanical ventilation (LTMV). Of 193 potential participants, 127 responded to the SRI questionnaire. Reliability as measured with Cronbach's α varied between 0.68 and 0.88 for the subscales and was 0.94 for SRI-sum score. Construct validity was obtained with high correlations between subscales in SF-36 and SRI. The SRI questionnaire discriminated well between universally accepted clinical differences among categories of patients receiving LTMV by significant dissimilarities in SRI-sum score and SRI subscales. The Norwegian version of SRI has well-documented psychometric properties regarding reliability and validity. It might be used in clinical practice and in international studies for assessing health-related quality of life in patients receiving LTMV.

Patient-reported fatigue in patients with rheumatoid arthritis who commence biologic therapy: a longitudinal study.

AIMS AND OBJECTIVES: To examine changes in patient-reported fatigue, over a twelve month period, in rheumatoid arthritis patients who commence biologic treatment, and to identify possible predictors for such changes. BACKGROUND: Fatigue is a burdensome symptom for patients with rheumatoid arthritis. Despite biologics being effective in reducing disease activity, patients still report fatigue. DESIGN: A longitudinal observational study. METHODS: A total of 48 patients were enrolled in the study. Fatigue was measured by the Fatigue Severity Scale. Independent samples T-tests were used to test gender differences, and paired samples T-tests were used to measure differences between repeated measures. Bivariate and multiple regression analyses were used to examine potential predictors for changes in fatigue, such as age, sex, Disease Activity Score 28, pain and physical and emotional well-being. RESULTS: Forty-seven patients completed the study. From baseline to 12-month follow-up, fatigue decreased significantly in both women and men. Analyses of predictors were performed step-wise, and the final model included sex and physical well-being. The results from this final step showed that female sex was the only significant predictor for changes in fatigue. CONCLUSION: Patients commencing biologic therapy reported a significant reduction in fatigue. Female sex was a significant predictor of changes in fatigue. RELEVANCE TO CLINICAL PRACTICE: Despite improvements in pharmacological treatment, patients with rheumatoid arthritis still report fatigue. This is a multifaceted health problem encompassing personal and emotional factors in addition to the clinical factors directly connected to the disease.