Treatable Traits in Chronic Respiratory Disease: A Comprehensive Review.

Chronic respiratory diseases are major contributors to the global burden of disease. While understanding of these diseases has improved, treatment guidelines have continued to rely on severity and exacerbation-based approaches. A new personalised approach, termed the "treatable traits" approach, has been suggested to address the limitations of the existing treatment strategies. We aim to systematically review the current evidence regarding treatable traits in chronic respiratory diseases and to identify gaps in the current literature. We searched the PubMed and Embase databases and included studies on treatable traits and chronic respiratory diseases. We then extracted information on prevalence, prognostic implications, treatment options and benefits from these studies. A total of 58 papers was included for review. The traits identified were grouped into five broad themes: physiological, biochemical, psychosocial, microbiological, and comorbidity traits. Studies have shown advantages of the treatable traits paradigm in the clinical setting. However, few randomised controlled trials have been conducted. Findings from our review suggest that multidisciplinary management with therapies targeted at treatable traits has the potential to be efficacious when added to the best practices currently implemented. This paradigm has the potential to improve the holistic care of chronic respiratory diseases.

COVID-19 related concerns of people with long-term respiratory conditions: a qualitative study.

BACKGROUND: The COVID-19 pandemic is having profound psychological impacts on populations globally, with increasing levels of stress, anxiety, and depression being reported, especially in people with pre-existing medical conditions who appear to be particularly vulnerable. There are limited data on the specific concerns people have about COVID-19 and what these are based on. METHODS: The aim of this study was to identify and explore the concerns of people with long-term respiratory conditions in the UK regarding the impact of the COVID-19 pandemic and how these concerns were affecting them. We conducted a thematic analysis of free text responses to the question "What are your main concerns about getting coronavirus?", which was included in the British Lung Foundation/Asthma UK (BLF-AUK) partnership COVID-19 survey, conducted between the 1st and 8th of April 2020. This was during the 3rd week of the UK's initial 'social distancing measures' which included advice to stay at home and only go outside for specific limited reasons. RESULTS: 7039 responses were analysed, with respondents from a wide range of age groups (under 17 to over 80), gender, and all UK nations. Respondents reported having asthma (85%), COPD (9%), bronchiectasis (4%), interstitial lung disease (2%), or 'other' lung diseases (e.g. lung cancer) (1%). Four main themes were identified: (1) vulnerability to COVID-19; (2) anticipated experience of contracting COVID-19; (3) pervasive uncertainty; and (4) inadequate national response. CONCLUSIONS: The COVID-19 pandemic is having profound psychological impacts. The concerns we identified largely reflect contextual factors, as well as their subjective experience of the current situation. Hence, key approaches to reducing these concerns require changes to the reality of their situation, and are likely to include (1) helping people optimise their health, limit risk of infection, and access necessities; (2) minimising the negative experience of disease where possible, (3) providing up-to-date, accurate and consistent information, (4) improving the government and healthcare response.

Pandemic with COVID-19 and Families with Children with Chronic Respiratory Diseases.

COVID-19 pandemic has provoked a huge change in daily functioning in millions of people worldwide. The epidemiological measures in prevention of possible infection have increased the possible risks on the mental and physical health. We have conducted a survey in order to investigate the needs and challenges of families with children with chronic respiratory diseases. In this order, we have created a questionnaire with general information about the family, general information about the child with chronic respiratory disease, overall physical and mental health before and during the pandemic, needs and mental health condition of the parents/caregivers. This survey showed that this group of families of children with respiratory disorders have suffered financially in significant way and has changed the way they perform professional and educational patterns due to the pandemic. Most of the children were stable in their physical health, but their mental health has deteriorated. This is probably due to the regular contact with the medical staff, but not with the mental health professionals. This group of children has a significant reduction in their physical activity and increase the hours in front of TV screens. The further effect on the physical and mental health is to be investigated.

Respiratory patient experience of measures to reduce risk of COVID-19: findings from a descriptive cross-sectional UK wide survey.

OBJECTIVES: To assess the experience of people with long-term respiratory conditions regarding the impact of measures to reduce risk of COVID-19. DESIGN: Analysis of data (n=9515) from the Asthma UK and British Lung Foundation partnership COVID-19 survey collected online between 1 and 8 April 2020. SETTING: Community. PARTICIPANTS: 9515 people with self-reported long-term respiratory conditions. 81% female, age ranges from ≤17 years to 80 years and above, from all nations of the UK. Long-term respiratory conditions reported included asthma (83%), chronic obstructive pulmonary disease (10%), bronchiectasis (4%), interstitial lung disease (2%) and 'other' (<1%) (eg, lung cancer and pulmonary endometriosis). OUTCOME MEASURES: Study responses related to impacts on key elements of healthcare, as well as practical, psychological and social consequences related to the COVID-19 pandemic and social distancing measures. RESULTS: 45% reported disruptions to care, including cancellations of appointments, investigations, pulmonary rehabilitation, treatment and monitoring. Other practical impacts such as difficulty accessing healthcare services for other issues and getting basic necessities such as food were also common. 36% did not use online prescriptions, and 54% had not accessed online inhaler technique videos. Psychosocial impacts including anxiety, loneliness and concerns about personal health and family were prevalent. 81% reported engaging in physical activity. Among the 11% who were smokers, 48% reported they were planning to quit smoking because of COVID-19. CONCLUSIONS: COVID-19 and related social distancing measures are having profound impacts on people with chronic respiratory conditions. Urgent adaptation and signposting of services is required to mitigate the negative health consequences of the COVID-19 response for this group.

Validity and reliability of the Turkish version of the Multidimensional Dyspnea Profile in outpatients with respiratory disease

Background/aim: Dyspnea is the subjective feeling of breathing discomfort, which is a significant problem for patients with heart and respiratory disease and also an important determinant of exercise tolerance, quality of life, and mortality in various diseases. Most of the scales are not enough to investigate the multidimensional effects of dyspnea; therefore, the Multidimensional Dyspnea Profile (MDP) was developed and validated in many languages. This study aimed to translate and culturally adapt the MDP into Turkish and investigate the psychometric properties of this adapted version in outpatients with respiratory disease. Materials and methods: The MDP was translated and culturally adapted into Turkish following published guidelines. A total of 170 outpatients with respiratory disease were included to assess psychometric properties. The factorial structure was investigated using a principal component analysis. Two situations were used in this study evaluating dyspnea in activity-related and resting conditions. We formulated 17 hypotheses for each MDP domain (in total 68) to assess construct validity, and correlations were investigated between the MDP and measures of body mass index, pulmonary function test, other dyspnea assessments, anxiety, depression, and health-related quality of life. To investigate the test-retest reliability, the MDP was administered again after 1-h and 1 week Results: Internal consistency of the MDP was excellent (Cronbach's alpha coefficients ranged from 0.89 to 0.93). The exploratory factor analysis revealed 2 components explaining a 70% and 76% variance. Overall, 64 of the 68 predetermined hypotheses (94%) were confirmed to test construct validity. The MDP showed excellent test-retest reliability for a 1-hperiod (intraclass correlation coefficient values ranged from 0.98 to 0.99). However, test-retest reliability decreased moderate-to-high after 1 week (0.53­0.80). Conclusion: The MDP was successfully translated and culturally adapted into Turkish and this version showed good psychometric properties including the factorial structure, internal consistency, test-retest reliability, and construct validity to assess multidimensional aspects of dyspnea.

Digital habits of PR service-users: Implications for home-based interventions during the COVID-19 pandemic.

Remote models of pulmonary rehabilitation (PR) are vital with suspension of face-to-face activity during the COVID-19 pandemic. We surveyed digital access and behaviours and PR delivery preferences of current PR service users. There was significant heterogeneity in access to and confidence in using the Internet with 31% having never previously accessed the Internet, 48% confident using the Internet and 29% reporting no interest in accessing any component of PR through a Web-based app. These data have implications for the remote delivery of PR during the COVID-19 pandemic and raise questions about the current readiness of service users to adopt Web-based delivered models of PR.

Preferences for Predictive Model Characteristics among People Living with Chronic Lung Disease: A Discrete Choice Experiment.

Background. Patients may find clinical prediction models more useful if those models accounted for preferences for false-positive and false-negative predictive errors and for other model characteristics. Methods. We conducted a discrete choice experiment to compare preferences for characteristics of a hypothetical mortality prediction model among community-dwelling patients with chronic lung disease recruited from 3 clinics in Philadelphia. This design was chosen to allow us to quantify "exchange rates" between different characteristics of a prediction model. We provided previously validated educational modules to explain model attributes of sensitivity, specificity, confidence intervals (CI), and time horizons. Patients reported their interest in using prediction models themselves or having their physicians use them. Patients then chose between 2 hypothetical prediction models each containing varying levels of the 4 attributes across 12 tasks. Results. We completed interviews with 200 patients, among whom 95% correctly chose a strictly dominant model in an internal validity check. Patients' interest in predictive information was high for use by themselves (n = 169, 85%) and by their physicians (n = 184, 92%). Interest in maximizing sensitivity and specificity were similar (0.88 percentage points of specificity equivalent to 1 point of sensitivity, 95% CI 0.72 to 1.05). Patients were willing to accept a reduction of 6.10 months (95% CI 3.66 to 8.54) in the predictive time horizon for a 1% increase in specificity. Discussion. Patients with chronic lung disease can articulate their preferences for the characteristics of hypothetical mortality prediction models and are highly interested in using such models as part of their care. Just as clinical care should become more patient centered, so should the characteristics of predictive models used to guide that care.

Palliative care for chronic respiratory disease: integrated care in outpatient settings.

Chronic respiratory diseases are progressive and often life-limiting illnesses. Patients experience debilitating and troubling symptoms that impact on their quality of life. Despite this, there is under-recognition of patients who may be entering the final year of their life and require palliative care services. The Royal Wolverhampton NHS Trust in partnership with Compton Care has established chronic respiratory disease multidisciplinary team meetings and a combined respiratory and palliative care outpatient clinic to address these issues. This article presents the impact of this service, now in to its fourth year, of delivering palliative care services to patients with chronic respiratory disease.

Behavioral predictors of subsequent respiratory illness signs in dogs admitted to an animal shelter.

Individual variability is evident in behavior and physiology of animals. Determining whether behavior at intake may predict subsequent illness in the animal shelter may influence the management of dogs housed at animal shelters and reduce overall disease. While normally associated with mild disease and low mortality rates, respiratory disease nevertheless poses significant challenges to the management of dogs in the stressful environment of animal shelters due to its highly infectious nature. Therefore, the aim of the study was to explore whether behavior at intake can predict subsequent occurrence and progression of upper respiratory disease in dogs at animal shelters. In a correlational study, 84 dogs were assessed throughout their stay at a city animal shelter. The dogs were subjected to a behavioral assessment, 1 min in-kennel behavioral observations across two observation periods, and the collection of urinary cortisol:creatinine (C:C) ratio. The occurrence and progression of upper respiratory disease was monitored through repeated clinical exams (rectal temperature and the occurrence of nasal and ocular discharge, and presence of coughing and sneezing). A basic PLS Path regression model revealed that time in the shelter (estimate = .53, p < .001), and sociability (estimate = .24, p < .001) and curiosity scores (estimate = .09, p = .026) were associated with increased illness. Activity and anxiety scores, however, were not associated with illness. Urinary C:C, taken on the first full day, did not predict subsequent illness when accounting for time. Limitations included attrition of dogs, a small percentage receiving vaccinations, and continuous and non-systematic rotation of dogs in the kennels. Understanding if behavior can predict subsequent illness may improve shelter management practices, and in turn, result in improved live-release outcomes.

Air pollution, respiratory illness and behavioral adaptation: Evidence from South Korea.

Air pollution is closely associated with the development of respiratory illness. Behavioral adaptations of people to air pollution may influence its impact, yet this has not been investigated in the literature. Our hypothesis is that people experience and learn the underlying air quality to decide their adaptation, and they have a stronger incentive to behaviorally adapt to the air quality as it deteriorates. We tested our hypothesis on a sample of approximately 25,700 individuals from South Korea from 2002 to 2013 that contained information on daily doctor's visits due to respiratory disease. We matched individuals to the mean of the past seven-day concentration of the particulate matter of size between 2.5 and 10 micrometers (PM10) in their county of residence. We examined whether people living in counties with greater air pollution suffer less from respiratory disease when the concentration increases. For the analysis, we separated counties into quintiles based on their mean seven-day PM10, and regressed the binary indicator of a daily doctor's visit with a resulting diagnosis of respiratory disease on the seven-day PM10 concentration of the county of residence interacted with the quintile dummies. The key findings are that a 1-standard-deviation increase in the seven-day PM10 concentration in the two lowest quintiles is associated with an increase of 0.054 percentage points in the likelihood of a doctor's visit with a resulting diagnosis of respiratory disease, which is about 40% larger than the effect in higher quintiles, and the size of 1-standard-deviation gradually increases from 0.037 percentage points in the third quintile to 0.040 percentage points in the fifth quintile. The smaller increase in the likelihood of respiratory disease in more polluted locations can be explained by the behavioral adaptation to the environment, but the effectiveness of the adaptation seems limited among the highly polluted locations.

Evaluating the extremely elderly at a pulmonary function clinic for the diagnosis of respiratory disease: frequency and technical quality of spirometry.

OBJECTIVE: To determine the frequency of spirometry in elderly people, by age group, at a pulmonary function clinic, to assess the quality of spirometry in the extremely elderly, and to determine whether chronological age influences the quality of spirometry. METHODS: This was a cross-sectional retrospective study evaluating information (spirometry findings and respiratory questionnaire results) obtained from the database of a pulmonary function clinic in the city of Aracaju, Brazil, for the period from January of 2012 to April of 2017. In the sample as a whole, we determined the total number of spirometry tests performed, and the frequency of the tests in individuals ≥ 60 years of age, ≥ 65 years of age, and by decade of age, from age 60 onward. In the extremely elderly, we evaluated the quality of spirometry using criteria of acceptability and reproducibility, as well as examining the variables that can influence that quality, such a cognitive deficit. RESULTS: The sample comprised a total of 4,126 spirometry tests. Of those, 961 (23.30%), 864 (20.94%), 102 (2.47%), and 26 (0.63%) were performed in individuals ≥ 60, ≥ 65, ≥ 86, and ≥ 90 years of age (defined as extreme old age), respectively. In the extremely elderly, the criteria for acceptability and reproducibility were met in 88% and 60% of the spirometry tests (95% CI: 75.26-100.00 and 40.80-79.20), respectively. The cognitive deficit had a negative effect on acceptability and reproducibility (p ≤ 0.015 and p ≤ 0.007, respectively). CONCLUSIONS: A significant number of elderly individuals undergo spirometry, especially at ≥ 85 years of age, and the majority of such individuals are able to perform the test in a satisfactory manner, despite their advanced age. However, a cognitive deficit could have a negative effect on the quality of spirometry.

Health-related Quality of Life of Macedonian Families Experiencing Cystic Fibrosis in Pediatric Practice.

BACKGROUND: Health-related quality of life (HRQoL) is a parameter that is examined in the area of clinical effectiveness. Like other chronic health conditions, paediatric cystic fibrosis (CF) impacts not only children but also their families. AIM: The present study investigates for the first time the HRQoL of children and parents in the Republic of North Macedonia. MATERIALS AND METHODS: The survey included 22 children (6 to 13 years of age) and their parents and 7 parents of children under 6 years of age by using the CFQ Revised and questions for current medical treatment. RESULTS: Children (6-13 years) reported the highest score for the digestive condition (84.85), while the lowest score was given for social activity (59.74). The highest score for digestive condition was also obtained from the parents of children from 6-13 years and under age of 6. The parents of children (6-13 years) reported the lowest score (60.56) for treatment burden activity, while the lowest score (50.0) for eating condition was obtained from the parents of children under 6 years. CONCLUSION: Nationality and gender have no significant impact on the HRQoL parameters. The highest scores for the digestive condition, respiratory function and physical condition are in a positive correlation with the fact that enzyme, antibiotic and physical therapy are given as a standard medical care. The lowest scores of the social aspect of the CF patients indicate the need for including a psychological support and support of social workers as a part of the standard medical care of these patients.

Short communication: Behavioral attitude scores associated with bovine respiratory disease identified using calf lung ultrasound and clinical respiratory scoring.

The objective of this study was to determine whether calves exhibit differences in behavioral attitude when diagnosed with their first bovine respiratory disease (BRD) event and whether fever (≥39°C) at the time of BRD diagnosis affected attitude. Preweaned dairy calves (n = 280; 21 ± 6 d) were examined twice weekly until weaning using a clinical respiratory score (CRS; CRS+: 2 respiratory categories with scores of 2 or greater; CRS-: 1 respiratory category with a score of 2 or greater or all respiratory categories scoring less than 2), lung ultrasound, and attitude score (normal = bright, alert, responsive; depressed = dull but responds to stimulation, slow to stand, or reluctant to lie down). Bovine respiratory disease was categorized as subclinical BRD (SBRD; CRS- and lung consolidation ≥1 cm2; n = 164) or clinical BRD (CBRD; CRS+, with or without lung consolidation; n = 79). Calves without BRD (NOBRD; n = 37) remained CRS- with lung consolidation <1 cm2 for the study. Depressed attitudes were found in 23, 6, and 0% of CBRD, SBRD, and NOBRD calves, respectively. In calves with CBRD, the odds of having a depressed attitude were 5.2 (95% confidence interval, confidence interval: 1.1-23.7) and 4.5 (95% confidence interval: 2.0-10.4) times higher compared with the odds of NOBRD and SBRD calves having a depressed attitude, respectively. The odds of having a depressed attitude did not differ between SBRD and NOBRD calves. Fever was associated with the odds of having a depressed attitude score, whereby calves with a fever had 6.2 (95% confidence interval: 2.8-14) times higher odds of having a depressed attitude score compared with calves without a fever. Sensitivity and specificity of the attitude score for identifying CBRD were 23% (95% confidence interval: 14-33) and 95% (95% confidence interval: 82-99), respectively. Producers should be cautious when using this attitude score as the primary means of detecting calves affected by BRD.

Chronic Respiratory Disease Questionnaire (CRQ-SAS): Analysis of psychometric properties. / Cuestionario de enfermedad respiratoria crónica (CRQ-SAS): análisis de las propiedades psicométricas.

Introduction: The Chronic Respiratory Disease Questionnaire Self-Administered Standardized (CRQ-SAS) format is used to assess health-related quality of life in different languages and adult populations, but it has not been validated in adolescents. This study analyzes the psychometric properties of the CRQ-SAS in a sample of adolescent patients with chronic respiratory disease and correlates them to anxiety and depression. Method: In relation to the CRQ-SAS psychometric properties, exploratory and confirmatory factor analyses were done to assess the instrument's reliability and validity. Correlations and multiple linear regressions with the Hospital Anxiety and Depression Scale were done to assess the relation with anxiety and depression. The mean difference was estimated based on sociodemographic outcome measures. Results: The CRQ-SAS was administered to 280 children and adolescents with chronic respiratory disease aged 9-18 years (mean=12.02), with a similar male-female distribution. The original 4-factor structure was maintained; 3 items were removed from the original scale and a new 17-item version was obtained. This showed adequate psychometric properties and discriminant validity. The dyspnea and emotional functioning domains better predicted anxiety and depression. Lastly, scales were obtained for the interpretation of health-related quality of life scores. Conclusions: This questionnaire, which has been previously used in the adult population, may be an adequate instrument to assess health-related quality of life in adolescent patients with chronic respiratory disease.

Introducción. El Cuestionario de Enfermedad Respiratoria Crónica Autoadminsitrado (CRQ-SAS) es un instrumento utilizado para evaluar calidad de vida relacionada con la salud en diferentes idiomas y poblaciones adultas, aunque no en adolescentes. Este estudio analiza las propiedades psicométricas del CRQ-SAS en una muestra de pacientes adolescentes con enfermedad respiratoria crónica y las relaciona con la clínica ansioso-depresiva. Método. Para analizar propiedades psicométricas del CRQ-SAS, se realizaron análisis factoriales exploratorios y confirmatorios, para estudiar la fiabilidad y validez de la escala. Para evaluar las relaciones con la clínica ansioso-depresiva, se realizaron correlaciones y regresiones lineales múltiples con la Escala de Ansiedad y Depresión Hospitalaria. Se calcularon diferencias de medias en función de variables sociodemográficas. Resultados. El CRQ-SAS fue administrado en 280 niños y adolescentes con enfermedad respiratoria crónica de edades comprendidas entre 9 y 18 años (Media= 12,02) con una distribución similar entre varones y mujeres. Se mantuvo la estructura original de cuatro factores, se eliminaron 3 ítems de la escala original, y se obtuvo una nueva versión de 17 ítems. Esta mostró adecuadas propiedades psicométricas y de validez discriminante. La disnea y la función emocional fueron las dimensiones que mejor predijeron la clínica ansioso-depresiva. Por último, se obtuvieron baremos para la interpretación de las puntuaciones en la calidad de vida relacionada con la salud. Conclusiones. Este cuestionario, utilizado anteriormente en población adulta, puede ser un adecuado instrumento para evaluar calidad de vida relacionada con la salud en pacientes adolescentes con enfermedad respiratoria crónica.

The roles of dispositional coping style and social support in helping people with respiratory disease cope with a breathlessness crisis.

AIM: To explore the role of coping moderators in self-management of breathlessness crises by people with advanced respiratory disease. DESIGN: A secondary analysis of semi-structured interview data. METHODS: Interviews with patients who had advanced respiratory disease, chronic breathlessness and at least one experience where they considered presenting to Emergency but self-managed instead (a "near miss"). Participants were recruited from New South Wales, Queensland, Victoria, South Australia or Tasmania. Eligible caregivers were those who contributed to Emergency-related decision-making. Interviews were coded inductively and then deductively against the coping moderators social support and dispositional coping style, defined by the Transactional Model of Stress and Coping. RESULTS: Interviews were conducted between October 2015 - April 2016 with 20 patients and three caregivers. Social networks offered emotional and practical support but also had potential for conflict with patients' 'hardy' coping style. Patient hardiness (characterized by a sense of 'commitment' and 'challenge') promoted a proactive approach to self-management but made some patients less willing to accept support. Information-seeking tendencies varied between patients and were sometimes shared with caregivers. An optimistic coping style appeared to be less equivocally beneficial. CONCLUSION: This study shows that social support and coping style may influence how people self-manage through their breathlessness crises and identified ways coping moderators can facilitate or hinder effective self-management. IMPACT: This study confers insights into how social-support and coping style can be supported and optimized to facilitate breathlessness self-management. Acknowledging coping moderator interactions is beneficial for developing resources and strategies that recognise patient mastery.

Psychiatric Collaborative Care for Patients With Respiratory Disease.

Psychiatric disorders are common in patients with advanced respiratory diseases, including COPD and asthma. These comorbid illnesses are often associated with poor compliance with medical treatment, increased disability, heightened health-care utilization, and premature mortality. Seeking to improve patient outcomes, improve patient satisfaction, and decrease the cost of care has led to the creation of alternative care and reimbursement models. One of the most mature of these models is the collaborative care model (CoCM). This model is team-based care; team members being the primary care provider, a care manager, and a psychiatric care provider. Studies have shown improved outcomes, improved patient satisfaction, and decreased cost when this model has been used to care for patients with general medical illness and psychiatric comorbidities. The primary care provider really drives the care, identifying the comorbidities and enlisting the patient's participation with care. Care managers could include nurses, social workers, or psychologists. Their responsibilities include monitoring symptoms, brief behavioral interventions, and other activities including case review with the psychiatric care provider. The psychiatric care provider is not expected to be on-site but will review cases with the care manager, who will communicate recommendations back to the primary care provider. Those services could be billed for under other Current Procedural Terminology (CPT) codes. As of January 1, 2018, report CoCM services using CPT codes 99492, 99493, and 99494 have been utilized for psychiatric collaborative care, in this new model to provide mental health services to patients with chronic medical conditions such as advanced respiratory diseases. They are endorsed by the Centers for Medicare and Medicaid Services, these new CPT codes support CoCM services and replace the 2017 codes G0502, G0503, and G0504 for Behavioral Health Integration. This article provides guidance on CoCM for patients with advanced respiratory disease and the new CPT codes for reimbursement of these services.

Introducing a model for emotional distress in respiratory disease: A systematic review and synthesis of symptom management models.

AIM: To undertake a theoretical systematic review to develop a conceptual model of illness-related emotional distress in the context of symptom management in chronic respiratory disease. DESIGN: We performed a systematic search to identify conceptual models. DATA SOURCES: Electronic databases MEDLINE, CINAHL, EMBASE and PsycINFO were searched and papers included from inception of the search term until June 2017. REVIEW METHODS: The review was conducted following Pound and Campbell's and Turner's theory synthesis. Conceptual models were appraised using Kaplan's criteria. Models were excluded if they referred to a specific condition and/or lacked clarity. RESULTS: This synthesis, which includes five models and additional evidence, yielded a new conceptual model describing the processes of regulation and symptom self-management in chronic respiratory disease. Identified sources of illness-related emotional distress are new or increased symptoms, additional treatment, new restrictions in performance of daily life roles and increased unpredictability. People goals and self-efficacy were identified as further drivers of symptom self-management. The regulation process is embedded in contextual factors. CONCLUSION: Theory synthesis provided transparent guidance in developing a model to understand of the factors driving self-management decisions. Therefore, the model has the potential to guide development of interventions that support symptom self-management in chronic respiratory disease. IMPACT: This newly presented conceptual model of illness-related emotional distress provides an understanding of the factors that drive self-management decisions when peoples experience new or increased symptoms. Such understanding is critical for nursing practice to developing appropriate interventions, especially in support of people decision-making.

Incorporating the patient perspective in the study of rare bone disease: insights from the osteogenesis imperfecta community.

There is limited research which examines health concerns of individuals with osteogenesis imperfecta (OI). Discussion groups with leaders of the adult OI community identified a broad range of medical priorities beyond fractures and brittle bones. Our work underscores the need to include patient-reported outcomes in rare bone disease research. INTRODUCTION: Osteogenesis imperfecta (OI) is a rare genetic disorder affecting collagen protein leading to brittle bones and a number of other medical complications. To date, there is limited research which examines the life-long process of aging with this rare disease, much less the perspective of individuals with OI. METHODS: In order to explore and prioritize health concerns that adults with OI feel have been inadequately addressed in health care and research, investigators held discussions with leaders from the global adult OI community. The meetings were held in August 2017 at the 13th International Conference on OI in Oslo, Norway as part of the preconference seminar "Patient Participation in OI Research". Investigators were part of the Brittle Bone Disease Consortium (BBDC), a multicenter research program devoted to the study of OI, and their focus was on patient-reported outcomes (PRO). RESULTS: Participants noted that while fractures and brittle bones are the most common feature of OI, a number of body systems are under-studied in this disorder. They particularly emphasized breathing, hearing, and the effects of aging as primary concerns that researchers and physicians may not fully understand or address. Other areas included pain, gastrointestinal problems, mental health, nutrition, menopause/pregnancy, and basilar invagination. Participants also emphasized that they must be informed of study results. They underscored that outcome measures incorporated into future drug trials must look beyond fractures and consider the whole patient. CONCLUSIONS: This work will help guide the incorporation of PROs into the next phase of the BBDC Natural History Study of OI and underscores the importance of including PROs in the study of rare diseases.