Effects of a primary palliative care intervention on quality of life and mental health in cystic fibrosis.

BACKGROUND: Despite the significant impact of chronic symptoms on quality of life with cystic fibrosis (CF), the role of palliative care in management of this disease is not well defined. The coping, goal assessment, and relief from evolving CF symptoms (CF-CARES) model is a primary palliative care intervention designed to provide chronic symptom management at all stages of the disease. The goal of this pilot study was to estimate the effectiveness of the CF-CARES intervention on improving chronic symptoms and quality of life for people living with CF. METHODS: A structured assessment was used to guide referral to supportive services intended to address burdensome symptoms. Follow-up assessments were performed approximately 3 and 6 months later. Longitudinal regression analyses of changes in symptoms and quality of life were performed for all participants regardless of utilization of supportive services. Subgroup analyses were performed for subjects participating in mental health and alternative health services. RESULTS: Forty-one subjects completed assessment and referral processes. The mean number of CF-associated symptoms decreased over time, as did respiratory symptom-related distress and depressive symptoms. Subjects utilizing alternative health services reported less psychological distress at follow-up. Among subjects with severe disease, mental health, and quality of life improved, especially for those using mental health services. CONCLUSIONS: The CF-CARES model resulted in significant mental health and quality-of-life benefits, suggesting the value of integrating symptom management interventions into routine CF care. Moreover, mental health services can play a key role in CF-specific primary palliative care, especially for those with advanced disease.

Advance Care Planning Experiences and Preferences among People with Cystic Fibrosis.

BACKGROUND: Advance care planning (ACP) is recommended for people with cystic fibrosis (CF), yet guidance for optimal implementation is lacking. OBJECTIVE: To assess ACP-related thoughts, comfort level, and preferences among people with CF to guide evidence-based routine implementation of ACP in the CF clinic. DESIGN: A cross-sectional survey assessed ACP-related experiences and preferences. SUBJECTS: Thirty-eight adolescents and adults with CF from an urban CF center. RESULTS: Few subjects reported talking to their CF team about ACP care preferences (5%) or completing advance directives detailing desired medical treatments (11%). However, most participants worried about living with advanced disease (84%) and felt comfortable discussing ACP preferences with CF providers (92%). Subjects largely preferred that ACP conversations occur when they are generally healthy, in the outpatient setting, and with any familiar CF team member. Disease severity was not associated with frequency of worry about living with advanced disease, comfort level with ACP discussions, or ACP setting preferences. CONCLUSIONS: People with CF worry about advanced disease and feel comfortable discussing ACP, but need more guidance to understand and document ACP choices. CF patient experiences and preferences support implementation of an early, active approach to ACP for people with CF.

The CF-CARES primary palliative care model: A CF-specific structured assessment of symptoms, distress, and coping.

BACKGROUND: Current palliative care tools do not address distressing chronic symptoms that are most relevant to cystic fibrosis. METHODS: A CF-specific structured assessment based on a primary palliative care framework was administered to 41 adolescents and adults with CF. Descriptive and correlational analyses were conducted. RESULTS: Patients reported numerous physical and psychological symptoms (mean of 10 per patient), with psychological symptoms rated as more distressing. Anxiety (34%) and depression (44%) were prevalent and correlated with distress attributable to physical symptoms and difficulty with CF self-management, but did not correlate with disease severity. CONCLUSIONS: Individuals with CF, regardless of disease severity, face challenges managing symptom burden. Frequently reported symptoms are not consistently associated with distress, suggesting the importance of individualized evaluation. The CF-CARES (Coping, goal Assessment, and Relief from Evolving CF Symptoms) primary palliative care assessment model provides a framework for patients experiencing chronic symptoms to explore interventional options with their clinicians.

Psychophysiological Associations with Gastrointestinal Symptomatology in Autism Spectrum Disorder.

Autism spectrum disorder (ASD) is often accompanied by gastrointestinal disturbances, which also may impact behavior. Alterations in autonomic nervous system functioning are also frequently observed in ASD. The relationship between these findings in ASD is not known. We examined the relationship between gastrointestinal symptomatology, examining upper and lower gastrointestinal tract symptomatology separately, and autonomic nervous system functioning, as assessed by heart rate variability and skin conductance level, in a sample of 120 individuals with ASD. Relationships with co-occurring medical and psychiatric symptoms were also examined. While the number of participants with significant upper gastrointestinal tract problems was small in this sample, 42.5% of participants met criteria for functional constipation, a disorder of the lower gastrointestinal tract. Heart rate variability, a measure of parasympathetic modulation of cardiac activity, was found to be positively associated with lower gastrointestinal tract symptomatology at baseline. This relationship was particularly strong for participants with co-occurring diagnoses of anxiety disorder and for those with a history of regressive ASD or loss of previously acquired skills. These findings suggest that autonomic function and gastrointestinal problems are intertwined in children with ASD; although it is not possible to assess causality in this data set. Future work should examine the impact of treatment of gastrointestinal problems on autonomic function and anxiety, as well as the impact of anxiety treatment on gastrointestinal problems. Clinicians should be aware that gastrointestinal problems, anxiety, and autonomic dysfunction may cluster in children with ASD and should be addressed in a multidisciplinary treatment plan. Autism Res 2017, 10: 276-288. © 2016 International Society for Autism Research, Wiley Periodicals, Inc.

Associations between cytokines, endocrine stress response, and gastrointestinal symptoms in autism spectrum disorder.

Many children and adolescents with autism spectrum disorder (ASD) have significant gastrointestinal (GI) symptoms, but the etiology is currently unknown. Some individuals with ASD show altered reactivity to stress and altered immune markers relative to typically-developing individuals, particularly stress-responsive cytokines including tumor necrosis factor alpha (TNF-α) and interleukin 6 (IL-6). Acute and chronic stress is associated with the onset and exacerbation of GI symptoms in those without ASD. The present study examined whether GI symptoms in ASD were associated with increases in cortisol, a stress-associated endocrine marker, and TNF-α and IL-6 in response to stress. As hypothesized, a greater amount of lower GI tract symptoms were significantly associated with post-stress cortisol concentration. The relationship between cortisol response to stress and GI functioning was greater for children who had a history of regressive autism. Exploratory analyses revealed significant correlations between cortisol response, intelligence, and inappropriate speech. In contrast, symptoms of the lower GI tract were not associated with levels of TNF-α or IL-6. Significant correlations were found, however, between TNF-α and IL-6 and irritability, socialization, and intelligence. These findings suggest that individuals with ASD and symptoms of the lower GI tract may have an increased response to stress, but this effect is not associated with concomitant changes in TNF-α and IL-6. The relationship between cortisol stress response and lower GI tract symptoms in children with regressive autism, as well as the relationships between cortisol, IL-6, and intelligence in ASD, warrant further investigation.

Development and evaluation of a palliative care curriculum for cystic fibrosis healthcare providers.

BACKGROUND: Primary palliative care refers to basic skills that all healthcare providers can employ to improve quality of life for patients at any stage of disease. Training in these core skills is not commonly provided to clinicians caring for cystic fibrosis (CF) patients. The objective of this study was to assess change in comfort with core skills among care team members after participation in CF-specific palliative care training focused on management of burdensome symptoms and difficult conversations. METHODS: A qualitative needs assessment was performed to inform the development of an 18-hour curriculum tailored to the chronicity and complexity of CF care. A 32-question pre- and post-course survey assessed CF provider comfort with the targeted palliative care skills in 5 domains using a 5-point Likert scale (1=very uncomfortable, 3=neutral, 5=very comfortable). RESULTS: Among course participants (n=16), mean overall comfort score increased by 0.9, from 3 (neutral) to 3.9 (comfortable) (p<0.001). Mean comfort level increased significantly (range 0.8 to 1.4) in each skill domain: use of supportive care resources, pain management, non-pain symptom management, communication, and psychosocial skills. CONCLUSIONS: CF-specific palliative care training was well received by participants and significantly improved self-assessed comfort with core skills.

Secondary Psychopathy, but not Primary Psychopathy, is Associated with Risky Decision-Making in Noninstitutionalized Young Adults.

Although risky decision-making has been posited to contribute to the maladaptive behavior of individuals with psychopathic tendencies, the performance of psychopathic groups on a common task of risky decision-making, the Iowa Gambling Task (IGT; Bechara, Damasio, Damasio, & Anderson, 1994), has been equivocal. Different aspects of psychopathy (personality traits, antisocial deviance) and/or moderating variables may help to explain these inconsistent findings. In a sample of college students (N = 129, age 18 to 27), we examined the relationship between primary and secondary psychopathic features and IGT performance. A measure of impulsivity was included to investigate its potential as a moderator. In a joint model including main effects and interactions between primary psychopathy, secondary psychopathy and impulsivity, only secondary psychopathy was significantly related to risky IGT performance, and this effect was not moderated by the other variables. This finding supports the growing literature suggesting that secondary psychopathy is a better predictor of decision-making problems than the primary psychopathic personality traits of lack of empathy and remorselessness.

A method to estimate treatment efficacy among latent subgroups of a randomized clinical trial.

Subgroup analysis arises in clinical trials research when we wish to estimate a treatment effect on a specific subgroup of the population distinguished by baseline characteristics. Many trial designs induce latent subgroups such that subgroup membership is observable in one arm of the trial and unidentified in the other. This occurs, for example, in oncology trials when a biopsy or dissection is performed only on subjects randomized to active treatment. We discuss a general framework to estimate a biological treatment effect on the latent subgroup of interest when the survival outcome is right-censored and can be appropriately modelled as a parametric function of covariate effects. Our framework builds on the application of instrumental variables methods to all-or-none treatment noncompliance. We derive a computational method to estimate model parameters via the EM algorithm and provide guidance on its implementation in standard software packages. The research is illustrated through an analysis of a seminal melanoma trial that proposed a new standard of care for the disease and involved a biopsy that is available only on patients in the treatment arm.