Cystic Fibrosis Foundation Evidence-Based Guideline for the Management of CRMS/CFSPID.

A multidisciplinary committee developed evidence-based guidelines for the management of cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen-positive, inconclusive diagnosis (CRMS/CFSPID). A total of 24 patient, intervention, comparison, and outcome questions were generated based on surveys sent to people with CRMS/CFSPID and clinicians caring for these individuals, previous recommendations, and expert committee input. Four a priori working groups (genetic testing, monitoring, treatment, and psychosocial/communication issues) were used to provide structure to the committee. A systematic review of the evidence was conducted, and found numerous case series and cohort studies, but no randomized clinical trials. A total of 30 recommendations were graded using the US Preventive Services Task Force methodology. Recommendations that received ≥80% consensus among the entire committee were approved. The resulting recommendations were of moderate to low certainty for the majority of the statements because of the low quality of the evidence. Highlights of the recommendations include thorough evaluation with genetic sequencing, deletion/duplication analysis if <2 disease-causing variants were noted in newborn screening; repeat sweat testing until at least age 8 but limiting further laboratory testing, including microbiology, radiology, and pulmonary function testing; minimal use of medications, which when suggested, should lead to shared decision-making with families; and providing communication with emphasis on social determinants of health and shared decision-making to minimize barriers which may affect processing and understanding of this complex designation. Future research will be needed regarding medication use, antibiotic therapy, and the use of chest imaging for monitoring the development of lung disease.

Perceptions of the Fatigue Experience and a Breathing Awareness Meditation-Integrated mHealth App for Fatigue and Stress in Patients with Sarcoidosis.

Objective: Sarcoidosis-associated fatigue is a debilitating consequence of sarcoidosis, a multi-system inflammatory disease, and may be related to increased stress associated with sarcoidosis. Breathing awareness meditation has potential as an intervention for managing stress and fatigue for sarcoidosis patients (SPs). This project's aim was to obtain feedback from key informants to design and tailor a patient-centered Sarcoidosis Patient Resource and Companion (SPARC) mHealth App developed for SPs to manage fatigue and stress at home using breathing awareness meditation. Methods: We used a mixed-method patient/user-centered design with triangulation to understand SPs experiences of sarcoidosis-associated fatigue and stress (n = 13), and obtain feedback on the SPARC App-prototype integrating breathing awareness meditation from these SPs and health care team members (HCTMs; n = 5). Using deductive content analysis, transcribed interviews were coded for themes and subthemes. Results: We report on findings from qualitative interviews and assessment of SPs' experiences including themes describing fatigue/stress interactions. Themes indicated that SPs find fatigue to be a profoundly difficult experience affecting multiple domains of functioning for which they perceive few effective strategies to cope. SPs and HCTMs shared feedback on the SPARC App-prototype after a test session; it was reported to be user friendly and to have potential for improving fatigue/stress, and key points for tailoring the App to SPs were shared. Conclusion: Sarcoidosis-associated fatigue poses a significant burden for SPs. The SPARC App-prototype with breathing awareness meditation was acceptable and feasible to use and was well-received by SPs and HCTMs. Future iterations of the SPARC App to test the effect of breathing awareness meditation on fatigue will need to incorporate recommendations for tailoring for SPs.

Experiences of cystic fibrosis newborn screening and genetic counseling.

The South Carolina cystic fibrosis (CF) newborn screening (NBS) program changed in 2019 to include CFTR genotyping for babies with top 4% immunoreactive trypsinogen, which improves sensitivity and timeliness but increases carrier detection. Carrier identification has genetic implications for the family and parents of NBS+ babies have increased emotional distress. Genetic counseling (GC) may increase parent understanding and reduce anxiety yet is not uniformly offered at CF centers. We report our early results after implementing GC for NBS+ families at the time of sweat chloride testing based on GC availability, which resulted in an unselected GC- control arm. Sixteen mothers (GC+ = 9, GC- = 7) participated in an online survey about their experience. Responses were analyzed in aggregate and for differences between GC+ and GC- groups. All-respondent sadness and anxiety increased with notification of the NBS+ result and decreased after sweat test results. Anxiety and sadness were greater in GC- compared to GC+ until after the diagnosis was resolved, though emotional differences between the groups were not statistically significant. On a scale of 0 = not at all to 10 = extremely, GC was rated very helpful (mean 9.0, range 5-10), informative (mean 8.9, range 4-10), comforting (mean 9.1, range 6-10), and minimally distracting (mean 1.8, range 0-9). All participants correctly identified that a risk for a child to have CF exists when both parents are (at least) carriers. Delivery of NBS results to respondents varied by timing, informant, and information given. The child's pediatrician notified 10 (62.5%) of the NBS+ result. Parents felt they were notified in a timely manner (68.8%), by someone knowledgeable about NBS (62.5%), the sweat test (62.5%), CF (43.8%), and genetics (43.8%) and who cared about them (81.3%). Parents felt worried (81.3%), confused (81.3%), empowered (25%), and other (sad, shocked, scared, overwhelmed, devastated, defeated). Data from this single-center study suggest benefit of GC, that families would value earlier contact with an expert, and that prompt diagnostic resolution may reduce duration of parental distress.

Design and rationale of a pilot randomized clinical trial investigating the use of a mHealth app for sarcoidosis-associated fatigue.

Fatigue is the most reported symptom in patients with sarcoidosis (SPs) and is a significant predictor of decreased quality of life that is strongly associated with stress and negative mood states. Few medications exist for treating fatigue in SPs, and outpatient physical rehabilitation programs are limited by availability and cost. Sarcoidosis in the US predominantly impacts minorities and underserved populations who are of working age and often have limited resources (e.g., financial, transportation, time off work) that may prevent them from attending in-person programs. The use of mobile health (mHealth) is emerging as a viable alternative to provide access to self-management resources to improve quality of life. The Sarcoidosis Patient Assessment and Resource Companion (SPARC) App is a sarcoidosis-specific mHealth App intended to improve fatigue and stress in SPs. It prompts SPs to conduct breathing awareness meditation (BAM) and contains educational modules aimed at improving self-efficacy. Herein we describe the design and methods of a 3-month randomized control trial comparing use of the SPARC App (10-min BAM twice daily) to standard care in 50 SPs with significant fatigue (FAS ≥22). A Fitbit® watch will provide immediate heartrate feedback after BAM sessions to objectively monitor adherence. The primary outcomes are feasibility and usability of the SPARC App (collected monthly). Secondary endpoints include preliminary efficacy at improving fatigue, stress, and quality of life. We expect the SPARC App to be a useable and feasible intervention that has potential to overcome barriers of more traditional in-person programs.

The efficacy of psychosocial interventions for cancer caregiver burden: A systematic review and meta-analysis of randomized controlled trials.

Caregivers of adults with cancer often provide prolonged demanding assistance (e.g., physical, emotional) to their loved ones, resulting in caregiver burden. This meta-analytic review examined the efficacy of psychosocial interventions in reducing caregiver burden in caregivers of adults with cancer. Randomized controlled trials (RCTs) were identified from six electronic databases and clinical trial registries. Random-effects meta-analyses were conducted for subgroups of interventions and control conditions. Overall, 90 records describing 50 RCTs showed that psychosocial interventions reduced caregiver burden compared to passive controls (e.g. wait-list) at post-intervention (g = 0.26, 95%CI [0.12, 0.40]), but not at the first follow-up (g = 0.10, 95%CI [-0.05, 0.24]). Subgroup analyses showed that compared to passive controls, therapeutic counseling and skills training interventions significantly reduced caregiver burden at post-intervention, whereas psycho-education/support interventions did not significantly reduce burden. Very few RCTs examined intervention efficacy compared to active controls (e.g., psycho-education/support). The evidence grade ranged from very low to moderate due to inconsistency and imprecision of the results. Therapeutic counseling and skills training interventions appear efficacious in improving caregiver burden at post-intervention, although these improvements attenuate over time. Rigorous trials examining intervention effects on long-term outcomes are needed to better understand the effective mechanisms to sustain reduction in caregiver burden.

Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation.

Tens of thousands of patients with advanced lung diseases may be eligible to be considered as potential candidates for lung transplant around the world each year. The timing of referral, evaluation, determination of candidacy, and listing of candidates continues to pose challenges and even ethical dilemmas. To address these challenges, the International Society for Heart and Lung Transplantation appointed an international group of members to review the literature, to consider recent advances in the management of advanced lung diseases, and to update prior consensus documents on the selection of lung transplant candidates. The purpose of this updated consensus document is to assist providers throughout the world who are caring for patients with pulmonary disease to identify potential candidates for lung transplant, to optimize the timing of the referral of these patients to lung transplant centers, and to provide transplant centers with a framework for evaluating and selecting candidates. In addition to addressing general considerations and providing disease specific recommendations for referral and listing, this updated consensus document includes an ethical framework, a recognition of the variability in acceptance of risk between transplant centers, and establishes a system to account for how a combination of risk factors may be taken into consideration in candidate selection for lung transplantation.

Cystic fibrosis foundation consensus statements for the care of cystic fibrosis lung transplant recipients.

Cystic fibrosis (CF) is the indication for transplantation in approximately 15% of recipients worldwide, and Cystic Fibrosis Lung Transplant Recipients (CFLTRs) have excellent long-term outcomes. Yet, CFLTRs have unique comorbidities that require specialized care. The objective of this document is to provide recommendations to CF and lung transplant clinicians for the management of perioperative and underlying comorbidities of CFLTRs and the impact of transplantation on these comorbidities. The Cystic Fibrosis Foundation (CFF) organized a multidisciplinary committee to develop CF Lung Transplant Clinical Care Recommendations. Three workgroups were formed to develop focused questions. Following a literature search, consensus recommendations were developed by the committee members based on literature review, committee experience and iterative revisions, and in response to public comment. The committee formulated 32 recommendation statements in the topics related to infectious disease, endocrine, gastroenterology, pharmacology, mental health and family planning. Broadly, the committee recommends close coordination of care between the lung transplant team, the cystic fibrosis care center, and specialists in other disciplines with experience in the care of CF and lung transplant recipients. These consensus statements will help lung transplant providers care for CFLTRs in order to improve post-transplant outcomes in this population.

Facilitators and barriers to utilization of medications for opioid use disorder in primary care in South Carolina.

OBJECTIVE: Utilization of medications for opioid use disorder (MOUD) has not been widely adopted by primary care providers. This study sought to identify interprofessional barriers and facilitators for use of MOUD (specifically naltrexone and buprenorphine) among current and future primary care providers in a southeastern academic center in South Carolina. METHOD: Faculty, residents, and students within family medicine, internal medicine, and a physician assistant program participated in focus group interviews, and completed a brief survey. Survey data were analyzed quantitatively, and focus group transcripts were analyzed using a deductive qualitative content analysis, based upon the theory of planned behavior. RESULTS: Seven groups (N = 46) completed focus group interviews and surveys. Survey results indicated that general attitudes towards MOUD were positive and did not differ significantly among groups. Subjective norms around prescribing and controllability (i.e., beliefs about whether prescribing was up to them) differed between specialties and between level of training groups. Focus group themes highlighted attitudes about MOUD (e.g., "opens the flood gates" to patients with addiction) and perceived facilitators and barriers of using MOUD in primary care settings. Participants felt that although MOUD in primary care would improve access and reduce stigma for patients, prescribing requires improved provider education and an integrated system of care. CONCLUSIONS: The results of this study provide an argument for tailoring education to specifically address the barriers primary care prescribers perceive. Results promote the utilization of active, hands-on learning approaches, to ultimately promote uptake of MOUD prescribing in the primary care setting in South Carolina.

Promoting emotional wellness in children with cystic fibrosis, Part I: Child and family resilience.

Attention should be given to individual and family well-being from a child's first interaction with the medical team and continuing throughout development, especially for families who experience chronic illnesses, such as cystic fibrosis (CF). While much attention has been given to the mental health of people with CF 12 years and older, this paper explores various areas for CF teams to assess and provide additional resources during the first 12 years of a child's life to promote child and family wellness. In this paper, we discuss parental mental health, social determinants of health, adherence/self-care, nutrition, attention to family lifestyle factors, engagement with school and peers, and modulator therapy for this age group of people with CF. This is the first of two companion papers which examines emotional wellness of children during the early years. The second paper examines mental health assessment and intervention for children under 12. Both encourage teams to strive to promote optimal child and family emotional health and wellness, emphasizing holistic health promotion and prevention, early identification, and intervention.

Promoting emotional wellness in children with CF, part II: Mental health assessment and intervention.

This is the second of two companion papers that examine the emotional wellness of children with cystic fibrosis (CF) during the early years of life, defined here as the period between birth and age 12. Both papers promote optimal mental health and well-being, with an emphasis on early identification and intervention. The first paper explores child and family resilience. Here, we discuss strategies for pediatric CF teams to provide routine, systematic mental health assessment, anticipatory guidance, brief intervention, and triage to evidence-based treatment when needed, while addressing barriers to accessing care. Many mental health conditions emerge before the age of 12, with the potential for lifelong effects on individuals, their families, and society. Living with a chronic illness such as CF can further increase the risk of mental health concerns and, in a bidirectional manner, their consequences for the quality of life, sustaining daily care, and health outcomes. There has been a significant focus in recent years on the mental health and wellness of adolescents and adults with CF, but less attention to specifics of depression and anxiety in younger children, or to other common pediatric comorbidities including trauma, developmental disorders such as attention-deficit/hyperactivity disorder or autism spectrum disorder, and oppositional behavior. Given the availability of psychometrically sound screening instruments and effective interventions, routinely addressing the mental health of children with CF and their families is feasible to integrate within multidisciplinary CF care, allowing for a personalized approach respecting individual needs, values, and goals.

Pain rehabilitation's dual power: Treatment for chronic pain and prevention of opioid-related risks.

The purpose of this article is to provide a data-driven exploration of an interdisciplinary pain rehabilitation program (PRP) as a viable option for addressing the dual crises of chronic pain and opioid use. Psychologists are crucial providers in the PRP, in both intervention and leadership roles. There is well-established literature supporting pain rehabilitation as an effective treatment for chronic pain and functioning, but there are few studies examining the effects of pain rehabilitation on opioid misuse risk. We evaluated data from 60 patients with diverse chronic pain conditions who completed an interdisciplinary PRP to evaluate changes in pain, functioning (self-report and objective physical measure), psychological symptoms, and health-related quality of life. To evaluate the effect of pain rehabilitation on opioid-related risks, we examined opioid use and opioid misuse behaviors (measured by the Current Opioid Misuse Measure; COMM) pre- and posttreatment. Results demonstrated statistically significant improvements in all outcomes, with medium effect sizes for pain severity and large effect sizes for functioning, psychological symptoms, and emotional quality of life. Fifty-eight percent of patients were on opioid medications at entry compared with 15% at discharge. Among patients who entered on opioids, mean COMM scores were significantly reduced from above the cutoff for misuse risk (M = 13.57) to below the cutoff (M = 5.86). Overall, this study provided strong support for pain rehabilitation as an effective treatment for chronic pain and related suffering, while also providing a prevention-based opportunity for reducing opioid-related risk. (PsycInfo Database Record (c) 2020 APA, all rights reserved).

Implementation, feasibility, and acceptability of quality of life therapy to improve positive emotions among patients with implantable cardioverter defibrillators.

Implantable cardioverter defibrillators (ICDs) save lives, but often induce significant psychological distress among patients. Positive psychological constructs are associated with improved outcomes among cardiac patients. In this NHLBI-funded randomized controlled trial, one aim was to evaluate the feasibility and acceptability of a positive psychology intervention (Quality of Life Therapy; QOLT, n = 11), compared to a Heart Healthy Education (HHE) control (n = 10), among ICD patients. A majority of participants across groups attended all 12 sessions (71%) and completed homework assignments (80%). Agreement on participant engagement and interventionist protocol adherence were high, with no differences between groups (ps > 0.20). A greater proportion of QOLT participants rated their sessions was "very" helpful compared to HHE participants (63% vs. 10%, p = 0.19). These initial data support the feasibility and acceptability of QOLT. A larger-scale trial using positive psychology interventions among ICD patients is indicated to determine potential mechanisms underlying the relationship between positive psychological constructs and cardiovascular health.

Patient and Provider Perspectives on Communication About Body Image With Adolescents and Young Adults With Cystic Fibrosis.

Objective: This mixed-methods study examined perspectives of adolescents and young adults (AYAs) with cystic fibrosis (CF) and health care providers on body image communication. Methods: Interviews and questionnaires were completed by 20 AYAs and 28 providers. Results: Although 85% of patients reported they had never had a body image conversation with a health care provider, 74% of providers reported discussing this topic with patients. Patients and providers described body image as an important issue, which should be discussed comfortably and supportively. However, patients often preferred to discuss body image as a distinct topic, separate from physical health, whereas providers preferred integrating body image conversations within weight- and health-based discussions. Conclusions: Body image is an important topic for AYAs with CF that often goes unaddressed or addressed in ways that are less preferred by patients. Providers should reduce barriers to effective communication about this important topic, particularly through increased awareness of AYA preferences.

Positive Psychotherapy to Improve Autonomic Function and Mood in ICD Patients (PAM-ICD): Rationale and Design of an RCT Currently Underway.

BACKGROUND: Improving mental and physical health of patients with implantable cardioverter defibrillators (ICD) is critical because this group is at high risk for ventricular arrhythmias and sudden death and depressed or anxious cardiovascular disease (CVD) patients appear to be at even higher risk for mortality compared to nondepressed or nonanxious CVD patients. Further, autonomic dysfunction is present in these patients, and negative emotions and arrhythmias form a downward spiral further worsening mood, well-being, and cardiovascular health. Much research demonstrates that positive emotion is related to health benefits, improved physiology, and increased survival. METHODS AND RESULTS: This is a two-arm randomized controlled trial aiming to recruit 60 adult ICD patients comparing 12 individually delivered, weekly sessions of: (1) a positive emotion-focused cognitive-behavioral therapy (Quality of Life Therapy [QOLT]), and (2) Heart Healthy Education. Autonomic functioning, heart rhythm indices, and psychosocial health are measured at baseline, 3 months, and 9 months. The first goal is feasibility and acceptability, with the primary outcome being arrhythmic event frequency data. CONCLUSION: This study is designed to test whether QOLT produces changes in mood, quality of life/well-being, autonomic function, and arrhythmic and ICD therapy event rates. This feasibility trial is a foundational step for the next trial of QOLT to help determine whether a 3-month QOLT trial can reduce arrhythmias occurrences among ICD patients, and examine a mechanism of autonomic functioning. This study may help to develop and implement new medical or psychological therapies for ICD patients.

Professionals' reported provision and recommendation of psychosocial interventions for youth with autism spectrum disorder.

Children and adolescents with autism spectrum disorder (ASD) receive intervention services from multiple professionals across disciplines. Little is known about services for youth with ASD in community settings. The purpose of this study was to provide data on professionals' self-reported practices across different classes of psychosocial interventions for youth with ASD. A multidisciplinary (medicine/nursing, education, occupational/physical therapy, psychology, social work, and speech-language pathology/audiology) sample (N=709; 86% female; 86% White) of professionals who endorsed providing clinical services to youth with ASD was recruited through convenience sampling (listservs, etc.) and stratified random sampling (online provider listings). Professionals completed a survey on intervention practices with youth with ASD, specifically on their own provision of, as well as their recommendation/referral of, psychosocial interventions (focused intervention practices [FIPs], comprehensive treatment models [CTMs], and other interventions). Hierarchical multiple regression models showed discipline differences in self-reported provision and recommendation of evidence-based FIPs; training variables and unfamiliarity with FIPs predicted rates of providing and recommending. FIPs were reportedly provided and recommended at higher rates than CTMs. Descriptive data are presented on professionals' reported practice of other psychosocial interventions (e.g., cognitive-behavioral therapy). This study highlights the usefulness of examining not only provision of services but also recommendation/referral practices: professionals are important sources of information for families. Implications of the results are discussed in terms of the importance of disseminating intervention information to professionals and the need for consensus on terminology used to classify interventions and on criteria used to evaluate intervention efficacy.

The mother as hunter: significant reduction in foraging costs through enhancements of predation in maternal rats.

In previous laboratory investigations, we have identified enhanced cognition and reduced stress in parous rats, which are likely adaptations in mothers needing to efficiently exploit resources to maintain, protect and provision their immature offspring. Here, in a series of seven behavioral tests on rats, we examined a natural interface between cognition and resource gathering: predation. Experiment 1 compared predatory behavior (toward crickets) in age-matched nulliparous mothers (NULLs) and postpartum lactating mothers (LACTs), revealing a highly significant enhancement of predation in LACT females (mean = -65s in LACTs, vs. -270s in NULLs). Experiment 2 examined the possibility that LACTs, given their increased metabolic rate, were hungrier, and thus more motivated to hunt; doubling the length of time of food deprivation in NULLs did not decrease their predatory latencies. Experiments 3-5, which examined sensory regulation of the effect, indicated that olfaction (anosmia), audition (blockade with white noise), and somatosensation (trimming the vibrissae) appear to play little role in the behavioral enhancement observed in the LACTs; Experiment 6 examined the possibility that visual augmentations may facilitate the improvements in predation; testing LACTs in a 0-lux environment eliminated the behavioral advantage (increasing their latencies from -65s to -212s), which suggests that temporary augmentation to the visual system may be important, and with hormone-neural alterations therein a likely candidate for further study. In contrast, testing NULLS in the 0-lux environment had the opposite effect, reducing their latency to catch the cricket (from -270s to -200s). Finally, Experiment 7 examined the development of predatory behavior in Early-pregnant (PREG), Mid-PREG, and Late-PREG females. Here, we observed a significant enhancement of predation in Mid-PREG and Late-PREG females--at a time when maternity-associated bodily changes would be expected to diminish predation ability--relative to NULLs. Therefore, as with the increasing reports of enhancements to the maternal brain, it is apparent that meaningful behavioral adaptations occur that likewise promote the survival of the mother and her infants at a crucial stage of their lives.

Disparities in the Clinical Encounter: Virginia's African American Children with Special Health Care Needs.

This study analyzed Virginia data from the most recent National Survey of Children with Special Health Care Needs. Logistic regression models were run for six Maternal and Child Health Bureau core outcomes and included demographics, child characteristics, health care providers, and health care access variables as predictors. Race/ethnicity disparities were judged to be present if the race/ethnicity variable was a significant predictor in the final model. Examining the components of disparate outcomes, African American children were found to be less likely than their white counterparts to have a usual source for sick and preventive care and to have a personal doctor or nurse. Their parents were less likely to say that doctors spent enough time, listened carefully, were sensitive to values and customs, and made them feel like a partner. These findings emphasize the need to examine health care disparities at a state level in order to guide efforts at remediation.

Reproductive experience and the response of female Sprague-Dawley rats to fear and stress.

The present work examines the relationship between reproductive experience (comprising breeding, parturition, and lactation) and the behavioral and hormonal processes of fear and stress in the female laboratory rat. Previous research has indicated that reproductive experience functions to decrease the female's stress response in potentially harmful environments, thereby providing her with numerous survival benefits, including decreased fearfulness, increased aggression, and refined hunting skills. This study was designed to determine how nulliparous (no reproductive experience), primiparous (1 reproductive experience) and multiparous (at least 2 reproductive experiences) rats respond to a Pavlovian paradigm of learned fear, involving the pairing of a neutral stimulus (conditioned stimulus) with an aversive stimulus (unconditioned stimulus). We report evidence that reproductive experience is linked with fear-response and anxiety-like behaviors. Our findings indicate that reproductive experience has an additive effect: primiparous mothers showed a different response to the paradigm of conditioned fear not only compared with those of nulliparous rats as well as multiparous mothers. Assessing the complex interconnections among the behavioral and physiologic measures recorded in this study, multidimensional scaling confirmed a clear separation among the 3 groups of rats in terms of the behavioral and physiologic responses to the experimental paradigm, supporting the conclusion that reproductive experience influences the maternal mind.