Social support is associated with fewer reported symptoms and decreased treatment burden in adults with cystic fibrosis.

BACKGROUND: Although social support has been linked to a variety of health outcomes in those with and without chronic illness, this construct has rarely been studied in adults with cystic fibrosis (CF), who may face a unique set of clinical and psychosocial barriers. The current study explored the relationships between social support, mental health, physical health, treatment activity, and disease-specific quality of life in a sample of adults with CF. METHODS: Participants in the study included 250 adults with CF who took part in a larger longitudinal study. Participants were administered a battery of measures including a social support evaluation (Interpersonal Support Evaluation List, ISEL), a health assessment (Memorial Symptom Assessment Scale, MSAS), a health-related quality of life measure (Cystic Fibrosis Questionnaire - Revised, CFQ-R), and treatment activity questionnaires (Tool for Adherence Behaviour Screening, TABS and other surveys). RESULTS: Linear regression analyses indicated that greater social support was associated with fewer self-reported mental and physical health symptoms, digestive symptoms, and eating disturbances over time. Social support also was associated with elevated emotional, social, and role functioning as well as vitality and improved body image. Those who reported more support perceived less treatment burden and better overall perceptions of their health. CONCLUSIONS: Social support has been shown to be associated with health outcomes in other chronic illnesses, and the same is true for adults with CF. This study fills gaps in the literature by examining outcomes of social support in this emerging adult population and providing a potential area for intervention.

Research use of leftover newborn bloodspots: attitudes of Canadian geneticists regarding storage and informed consent requirements.

PURPOSE: Leftover newborn spots can provide a powerful research tool as a population-wide DNA bank. Some provinces/states store them for more than 20 years; however, parents are usually not informed of the retention of leftover newborn spots. To examine the opinions of Canadian geneticists regarding permission for leftover newborn spots storage for research purposes and the associated risks, a web-based survey was distributed to all members of the Canadian College of Medical Geneticists with a valid e-mail address (n = 209) and completed by 78 respondents (37%). RESULTS: The majority of respondents (73%) favored opt-out notification for retention of samples that would be held for longer than 2 years. For research on multifactorial conditions using leftover newborn spots originally banked without parental permission, geneticists favored different types of permission depending on the level of identifiable information attached to samples. Thirty-eight percent were concerned that information pamphlets that state that leftover newborn spots will be stored and may be "a source of DNA for research" would lead to a decreased participation in newborn screening. Twenty-eight percent believed that group stigma or family anxiety was likely to result from using nonidentified leftover newborn spots to study multifactorial conditions. CONCLUSION: The concerns of this knowledgeable cohort supports the critical importance of public engagement about both the potential risks and societal benefits associated with the use of leftover newborn spots in research as policy for leftover newborn spots is developed.

Reasons for participation in pain research: can they indicate a lack of informed consent?

OBJECTIVE: To ascertain the self-reported reasons for participation in the clinical research of chronic low back pain and to evaluate those reasons in the context of informed consent and the concept of therapeutic misconception. This is the belief that research participation is equivalent to clinical care. DESIGN: Qualitative descriptive study with semistructured interviews. SETTING: Phone interviews with subjects with chronic low back pain after they completed a double-blind controlled trial. PARTICIPANTS: Fifty-two of 60 (86%) randomized controlled trial completers. RESULTS: Seventy-seven percent had more than one reason for study participation, including the following: to contribute to research; to seek relief of pain (both short- and long-term); to try a different drug; monetary remuneration; and to have their pain taken seriously. An initial altruistic reason for participation was often followed later in the interview by reasons of personal benefit. In most cases, the single question, "why did you participate?" was insufficient to reveal these multiple reasons. "Personal benefit" had many individual meanings, framed in the context of an illness narrative of coping with chronic pain. Despite reasons of personal benefit, subjects were still able to make the distinction between research and clinical treatment. CONCLUSIONS: Assessing the adequacy of informed consent requires a thorough understanding of how subjects viewed a study and their reasons for participation. Quantitative-based surveys may not capture the complexities of reasons for study participation. Reasons of personal benefit, seemingly contradictory reasons for participation, or overriding desire for relief may all affect the quality of informed consent. Yet, these issues may not automatically signal the presence of TM.

High treatment burden in adults with cystic fibrosis: challenges to disease self-management.

BACKGROUND: More aggressive management of cystic fibrosis (CF), along with the use of new therapies, has led to increasing survival. Thus, the recommended daily treatment regimens for most CF adults are complex and time consuming. METHODS: In the Project on Adult Care in CF (PAC-CF), an ongoing longitudinal study of CF adults, we assessed self-reported daily treatment activities and perceived treatment burden as measured by the CF Questionnaire-Revised (CFQ-R), a disease-specific quality of life measure. RESULTS: Among the 204 respondents, the median number of daily therapies reported was 7 (IQR 5-9) and the mean reported time spent on treatment activities was 108 minutes per day (SD 58 min). Respondents reported a median of 3 inhaled and 3 oral therapies on the day prior to the survey. Only 49% reported performing airway clearance (ACT) on that day. There were no differences in the number of medications or the time to complete therapies based on gender, age or FEV1. The mean CFQ-R treatment burden domain score was 52.3 (SD 22.1), with no significant differences in the treatment burden based on age or FEV1. In a multivariable model controlling for age, gender, and FEV1, using 2 or more nebulized medications and performing ACT for >or=30 min were significantly associated with increased treatment burden. CONCLUSION: The level of daily treatment activity is high for CF adults regardless of age or disease severity. Increasing number of nebulized therapies and increased ACT time, but not gender, age, or pulmonary function, are associated with higher perceived treatment burden. Efforts to assess the effects of high treatment burden on outcomes such as quality of life are warranted.

Self-reported physical and psychological symptom burden in adults with cystic fibrosis.

Symptom burden is a key component of health-related quality of life in patients with cystic fibrosis (CF). To examine symptom prevalence and characteristics of adults with CF, we administered the Memorial Symptom Assessment Scale (MSAS), a previously validated measure of symptom burden, to CF patients enrolled in the Project on Adult Care in CF. The mean age of the 303 respondents (response rate 91%) was 32.8 years (range, 19-64); 58% were female, and their mean baseline pulmonary function (FEV(1) % predicted) was 69% (SD 28%). The median number of symptoms reported was 10, and there was no difference in the number of symptoms reported based on age, gender, or FEV(1). The most prevalent symptoms were cough (94%), shortness of breath (77%), and lack of energy (77%). Lack of energy and irritability caused the highest level of distress. MSAS symptom subscales were only moderately correlated with symptom status domains from existing CF health-related quality of life measures. Factor analysis led to the development of three distinct MSAS CF-symptom subscales, each with high internal validity. These findings show that adults with CF have a high symptom burden, particularly with respiratory and psychological symptoms, and that the new MSAS CF-specific subscales are a reliable measure of symptom distress in the CF population.

Chronic ventilator need in the community: a 2005 pediatric census of Massachusetts.

OBJECTIVES: The purpose of this study was to describe the population of children with chronic mechanical ventilation in Massachusetts and their patterns of medical care. PATIENTS AND METHODS: Investigators surveyed all of the Massachusetts home ventilator clinics, pediatric pulmonary services, hospital-based pediatric services for special health care needs, insurers, home care vendors, nursing agencies, the Massachusetts Department of Public Health, selected individual providers, and rehabilitation and long-term care facilities providing services to children with chronic respiratory support needs. Support was defined as daily use of noninvasive, negative-pressure, or invasive/transtracheal ventilators. Subsequent matching of demographic data, including date of birth, zip code, and gender supported maximal census yield without duplications. Geographic information systems were used to create distribution maps and estimate distances between children with chronic mechanical ventilator needs and key resources. RESULTS: A total of 197 children were identified as requiring chronic mechanical respiratory support in Massachusetts in 2005, which was a nearly threefold increase in this population in the 15-year interval since the last census. Congenital or perinatal-acquired neurologic or neuromuscular disorders constituted the majority of primary diagnoses (n = 107 [54%]). Chronic lung disease attributed to prematurity represented only 7% of the sample. CONCLUSIONS: Children receiving chronic mechanical respiratory support are a growing population. The shift in underlying diagnoses from pulmonary disease to neurogenic respiratory insufficiency has implications for hospital and community-based providers from all disciplines in extending services to the home setting. Barriers encountered when performing this study, however, reflect an overall lack of coordination among the many individuals and agencies involved in their care. Coordinated and centralized care efforts require a clear and managed flow of information; census reports such as this one are only the beginning. Direct needs assessments and quality-of-life surveys from families are needed to design and implement programmatic changes and advocacy efforts.