Becoming a Rare Disease Parent: An Interpretative Phenomenological Analysis of Parent-Caregivers' Postpartum Experiences.

Rare diseases constitute a group of conditions that are individually rare, but in aggregate impact between 3 and 6% of the world population. Many of these conditions present during infancy and involve substantial caregiving responsibilities, often assessed via quantitative measurements. However, few qualitative analyses examine lived experiences of parent-caregivers during the early period of their child's life. The purpose of this study was to examine the meaning that rare disease parent-caregivers apply to the postpartum year using data collected from a semi-structured interview exploring significant experiences over the course of their affected child's life. We utilized an interpretative phenomenological analysis (IPA) approach to analyze 22 interview transcripts from caregivers to children with several inherited metabolic and mitochondrial disorders, as well as an undiagnosed disease. Our analysis yielded three superordinate themes: Reckoning With the Parent-Caregiver Role, Familial Transition, and Adaptation and Adjustment. Subordinate themes expanded upon these concepts and included distinctions between the parent and caregiving identity, communal coping and shifting of family dynamics, as well as meaning applied to child milestones, anticipatory grief, and parental perception of a new normal. Exploration of these themes in relation to existing literature, as well as future research directions for qualitative research on rare disease caregivers, is discussed. Overall, this work contributes to a growing body of literature exploring the parental experience of rare disease across several condition contexts.

Stress, Coping, and Physical Health in Caregiving.

Informal caregivers experience a great deal of stress due to care-related duties and responsibilities. Caregiving stress has the ability to impact caregivers' physical health, but has been largely understudied in caregivers of children with a chronic illness. In this study, we examine the associations of stress to both caregiver self-rated health and biomarkers of the hypothalamic-pituitary-adrenal (HPA) axis and immune systems (arginine vasopressin, c-reactive protein, tumor necrosis factor alpha). We also examine whether coping style (proactive, avoidant, support coping) buffers the links of stress to health across two different stressor contexts: caregiving for a child with a rare or undiagnosed disease (n = 101) and caregiving for a typically developing child (n = 69). Results indicated perceived stress was linked to worse self-rated health, however, stress was only linked to biological markers of health for caregivers of typically developing children. Results also suggest that coping style may moderate some of the links of stress to health, as proactive coping was linked to lower arginine vasopressin. However, models also suggested the role of coping style may differ based on caregiving context, as support coping was linked to better health only for caregivers of typically developing children, and more proactive coping overall was observed in the rare disease context. Future research should continue to examine how stress and coping interact within different caregiving contexts to protect caregiver health and well-being.

Links of daily shared appraisal and collaboration to support, mood, and self-care in type 2 diabetes.

Research has increasingly recognized the links of communal coping-a shared appraisal of a stressor and collaborative action to manage it-to positive adjustment outcomes in chronic illness. However, past literature rarely examines if these two components have unique links to relationship and health outcomes, if one component is more strongly linked than the other component to these outcomes, or if the two components interact to influence outcomes. Additionally, the impact of shared appraisal and collaboration may depend on the source-the patient or the romantic partner. In a study of 200 patients with newly diagnosed type 2 diabetes and their spouses, daily reports of shared appraisal and collaboration, mood, support interactions, and patient self-care were collected over 14 consecutive days. Multi-level modeling showed that both patient and spouse reports of shared appraisal and collaboration were linked independently to support interactions and better mood for patients and spouses, while collaboration alone was linked to self-care. Further, collaboration was generally more strongly linked to behavioral outcomes-support and self-care-than shared appraisal, while links to mood were similar for shared appraisal and collaboration. Shared appraisal and collaboration also interacted such that shared appraisal was particularly beneficial for those who reported low collaboration. Finally, results suggested one's own reports of shared appraisal and collaboration were more strongly linked to outcomes than partner reports. Overall, these findings demonstrate unique impacts of daily shared appraisal and collaboration on adjustment to chronic illness for patients and spouses.

Shared Responsibility and Network Collaboration in Caregiving.

Communal coping may benefit caregivers, but most communal coping research focuses on dyads. Using an egocentric network design, we examine caregivers' we-talk-a linguistic marker of shared responsibility-and caregiver reports of 1) network member involvement in collaborative care roles and 2) met/unmet expectations across typically developing and rare disease contexts. We-talk was linked to involvement in direct care and support, but links of we-talk to decision-making varied based on network member closeness; we-talk was linked to meeting expectations for decision-making only. There were no differences across context, suggesting shared responsibility is linked to collaborative roles across caregiving contexts.

The impact of caregiving for children with chronic conditions on the HPA axis: A scoping review.

Caregiving has been robustly linked to caregiver health through the dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis in the context of caregiving for an adult with a chronic illness. However, little research examines the physiological impact of caregiving for a child with a chronic illness despite high burden and unique stressors. In this review, we explore the links of caregiving for a child with a congenital, chromosomal, or genetic disorder to the regulation or dysregulation of the HPA axis. A search was conducted in PubMed, Embase, and the Web of Science and 15 studies met inclusion criteria. Overall, there were inconsistent links of caregiving to HPA axis functioning, perhaps due to the heterogeneity across disease contexts, study designs, and biomarker measurement. Future research should standardize measurement and study designs, increase participant diversity, and examine moderators of the links of caregiving to the HPA axis.

Life under stay-at-home orders: a panel study of change in social interaction and emotional wellbeing among older Americans during COVID-19 pandemic.

BACKGROUND: Recent research has shown the mental health consequence of social distancing during the COVID-19 pandemic, but longitudinal data are relatively scarce. It is unclear whether the pattern of isolation and elevated stress seen at the beginning of the pandemic persists over time. This study evaluates change in social interaction over six months and its impact on emotional wellbeing among older adults. METHODS: We drew data from a panel study with six repeated assessments of social interaction and emotional wellbeing conducted monthly May through October 2020. The sample included a total of 380 White, Black and Hispanic participants aged 50 and over, of whom 33% had low income, who residing in fourteen U.S. states with active stay-at-home orders in May 2020. The analysis examined how change in living arrangement, in-person interaction outside the household, quality of relationship with family and friends, and perceived social support affected trajectories of isolation stress, COVID worry and sadness. RESULTS: While their living arrangements (Odds Ratio [OR] = 0.95, 95% Confidence Interval [CI] = 0.87, 1.03) and relationship quality (OR = 0.94, 95% CI = 0.82, 1.01) remained stable, older adults experienced fluctuations in perceived social support (linear Slope b = -1.42, s.e. = 0.16, p < .001, quadratic slope b = 0.50, s.e. = 0.08, p < .001, cubic slope b = -0.04, s.e. = 0.01, p < .001) and increases in in-person conversations outside the household (OR = 1.19, 95% CI = 1.09, 1.29). Living with a spouse/partner stabilized isolation stress (change in linear slope b = 1.16, s.e. = 0.48, p < .05, in quadratic slope b = -0.62, s.e. = 0.26, p < .05, and in cubic slope = 0.09, s.e. = 0.04, p < .05) and COVID worry (change in quadratic slope b = -0.66, s.e. = 0.32, p < .05 and in cubic slope = 0.09, s.e. = 0.04, p < .05) over time. Individuals with better relationship quality with friends had decreased sadness over time (OR = 0.90, 95% CI = 0.82, 0.99). Changes in social support were associated with greater fluctuations in isolation stress and COVID worry. CONCLUSIONS: During the pandemic, social interactions are protective and lack of stability in feeling supported makes older adults vulnerable to stress. Efforts should focus on (re)building and maintaining companionship and support to mitigate the pandemic's negative impact.

Links of we-talk to caregiver social network systems and health.

Caring for a child, particularly one with a rare disease, presents a challenging set of stressors that can impact entire family networks. Given this shared impact, caregivers can engage in communal coping to address the caregiving process, defined as the perception of caregiving as shared and collaborative behaviors to address it. In this study, we examined one common measure of communal coping-first person plural pronouns or "we-talk"-in caregivers of either (a) children with rare or undiagnosed diseases or (b) typically developing children. We sought to examine how we-talk is linked to (a) caregiver health and well-being and (b) social network involvement in caregiving. Caregivers (n = 311) participated in (a) survey questionnaires (b) a network enumeration interview and (c) a semistructured interview of caregiving. Results indicated we-talk was unrelated to stress or self-rated health, but was linked to more benefit-finding; greater individual-focused language (I-talk) was also linked to worse self-rated health. Additionally, we-talk was unrelated to malfeasant behavior (e.g., overly critical), but was linked to less nonfeasant behavior (e.g., underinvolvement), more uplift behavior (e.g., helping with caregiving), and more health-related communication with network members. Further, these findings did not differ across caregiving context. This work highlights the role of communal coping for caregivers managing general parenting stress as well as the stress associated with caring for a child with complex medical needs. Future research should continue to examine how interpersonal coping processes operate within social networks to impact health and well-being for all involved. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Observed dyadic collaboration among couples coping with type 1 diabetes.

Previous research linking collaboration to relationship and health outcomes has relied almost exclusively on individual self-report and failed to take into consideration the dyadic nature of collaboration. We enrolled couples (n = 193) in which one person had Type 1 diabetes (52% female; 91% non-Hispanic white) into the study and asked them to discuss a diabetes-related issue of high concern for 8 min. These videotaped interactions were coded for dyadic collaboration. Mood was measured before and after the discussion. After the discussion, patients and partners reported support provided and received during the discussion. Results showed that observed dyadic collaboration was related to improvements in mood and greater support exchanges for both patients and partners when self-reported collaboration was statistically controlled. Gender moderated the effects on partners, such that benefits of dyadic collaboration were stronger for women than men. Future research may benefit from including collaborative elements into couple-focused interventions. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

A multimethod approach to measuring communal coping in adults with type 1 diabetes.

OBJECTIVE: Communal coping with a chronic illness has been associated with better health outcomes and includes two components: an individual's appraisal of the illness as shared and collaborative strategies to manage the illness. Although multiple methods have been used to assess these constructs, there is limited understanding of whether these methods tap similar components of communal coping. The study goals were to assess how individuals diagnosed with type 1 diabetes communally cope with their romantic partner using multiple methods to (a) distinguish between the two components of communal coping and (b) examine links of both components to health outcomes and test whether interactions between the two are linked to health outcomes. METHOD: Individuals with type 1 diabetes (n = 199, 52% female, 90% non-Hispanic white) completed self-report, diary, observational, and open-ended interviews to measure communal coping. Psychological well-being, diabetes distress, and diabetes health outcomes were assessed. RESULTS: A confirmatory factor analysis supported our hypothesis that communal coping is reflected by 2 distinct components: shared appraisal and collaborative coping. There were no direct effects of either shared appraisal or collaboration to outcomes, however, the interaction between shared appraisal and collaboration was linked to diabetes distress, self-care, and self-efficacy. Specifically, collaboration was linked to worse outcomes at low shared appraisal but not high shared appraisal. CONCLUSIONS: These findings support the two components of communal coping and suggest that collaboration can be detrimental for health among those who do not view an illness as shared. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Shared illness appraisal and self-efficacy among adults with type 1 diabetes.

We examined the interaction between shared illness appraisal and self-efficacy among couples in which one partner was diagnosed with type 1 diabetes (n = 199). We hypothesized that the relation between self-efficacy and health would be weakened under conditions of shared rather than individual appraisal. Multiple regression analyses demonstrated that partner shared illness appraisal interacted with the self-efficacy of the person with type 1 diabetes to predict overall psychological distress and daily diabetes stressors in the predicted direction. Plots of the interactions suggest that partner appraisal of diabetes as shared buffers individuals with lower levels of self-efficacy from poorer health.

Diabetes stress and health: Is aging a strength or a vulnerability?

The purpose of this work was to examine (1) relations of diabetes stress to psychological well-being and health, (2) links of age to such outcomes and (3) the extent to which age moderated relations from diabetes stress to outcomes. These aims were addressed in a diverse community sample of 207 individuals recently diagnosed with type 2 diabetes, employing survey and daily diary methods. Participants reported age, diabetes distress, and psychological distress at baseline and 6 months later. Glycemic control also was assessed. Participants completed a 14-day daily diary protocol in which they reported daily diabetes stressors, mood, and self-care. Both cross-sectional and longitudinal results showed diabetes distress was associated with poorer outcomes. Daily diary data showed that individuals who reported more daily diabetes stressors reported poorer outcomes. Older age was linked to less psychological distress, but was unrelated to daily diabetes stressors. Older age attenuated relations of diabetes distress to outcomes.

Patient and partner illness appraisals and health among adults with type 1 diabetes.

In a study of 199 couples in which one person had type 1 diabetes, we examined how patient appraisal of the diabetes as shared versus individual was associated with collaborative, supportive and unsupportive behavior and whether patient shared illness appraisal was most beneficial for health when it occurred in the context of supportive behavior. We assessed illness appraisal among patients with type 1 diabetes and their partners and had patients complete relationship and health measures. Results showed partners were more likely than patients to hold shared illness appraisals. Patients' shared appraisals were associated with more collaborative and instrumental support, more emotional support, less protective buffering, and more overprotective behavior. When patients and partners were consistent in their shared appraisals, support was highest. Regression analysis showed collaborative and instrumental support, as well as emotional support, was related to better psychological and physical health when patients held shared compared to individual illness appraisals.

Daily Communal Coping in Couples With Type 2 Diabetes: Links to Mood and Self-Care.

Background: Adjusting to the challenges of a chronic illness does not affect patients alone but also influences social network members-most notably spouses. One interpersonal framework of coping with a chronic illness is communal coping, described as when a problem is appraised as joint and the couple collaborates to manage the problem. Purpose: We sought to determine whether daily communal coping was linked to daily mood and self-care behavior and examined one potential mechanism that may explain these links: perceived emotional responsiveness. Methods: Patients who had been diagnosed with diabetes less than 5 years ago and their spouses (n = 123) completed a daily diary questionnaire that assessed communal coping and mood for 14 consecutive days. The patients also reported daily self-care behaviors. We used multilevel modeling to examine the links of communal coping to patient and spouse mood and patient self-care. Because both patients and spouses reported their mood, the actor-partner interdependence model (APIM) was employed to examine mood. Results: Multilevel APIM showed that actor communal coping was associated with lower depressed mood, higher happy mood, and lower angry mood and partner communal coping was linked to higher happy mood. Patient communal coping was related to better dietary and medication adherence, and spouse communal coping was linked to better medication adherence. Perceived emotional responsiveness partially mediated the relations of communal coping to mood but not to self-care behaviors. Conclusions: Communal coping on a daily basis may help both patients and spouses adjust psychologically to the illness as well as enhance patient self-care behaviors.

Communal Coping and Adjustment to Chronic Illness: Theory Update and Evidence.

We present a theory of communal coping that describes an optimal pathway to patient adjustment among couples in which one person faces a chronic illness. Communal coping consists of a shared illness appraisal (i.e., person perceives illness as a joint rather than individual problem) and collaboration with a partner to manage the illness. We present a model of the communal coping process that links patient and partner shared illness appraisals to collaboration and a set of supportive interactions that might be reframed as collaboration in the presence of shared illness appraisals. We then outline a model that identifies potential antecedents of communal coping and mechanisms that link communal coping to patient illness adjustment (i.e., enhanced psychological well-being, improved health behaviors, better physical health) and partner psychological well-being. We review the empirical evidence for this model and conclude by identifying several moderator variables, noting potential limitations, and outlining future research directions.

Adjusting to Chronic Health Conditions.

Research on adjustment to chronic disease is critical in today's world, in which people are living longer lives, but lives are increasingly likely to be characterized by one or more chronic illnesses. Chronic illnesses may deteriorate, enter remission, or fluctuate, but their defining characteristic is that they persist. In this review, we first examine the effects of chronic disease on one's sense of self. Then we review categories of factors that influence how one adjusts to chronic illness, with particular emphasis on the impact of these factors on functional status and psychosocial adjustment. We begin with contextual factors, including demographic variables such as sex and race, as well as illness dimensions such as stigma and illness identity. We then examine a set of dispositional factors that influence chronic illness adjustment, organizing these into resilience and vulnerability factors. Resilience factors include cognitive adaptation indicators, personality variables, and benefit-finding. Vulnerability factors include a pessimistic attributional style, negative gender-related traits, and rumination. We then turn to social environmental variables, including both supportive and unsupportive interactions. Finally, we review chronic illness adjustment within the context of dyadic coping. We conclude by examining potential interactions among these classes of variables and outlining a set of directions for future research.

cAMP-responsive element modulator α (CREMα) trans-represses the transmembrane glycoprotein CD8 and contributes to the generation of CD3+CD4-CD8- T cells in health and disease.

T cell receptor-αß(+) CD3(+)CD4(-)CD8(-) "double-negative" T cells are expanded in the peripheral blood of patients with systemic lupus erythematosus and autoimmune lymphoproliferative syndrome. In both disorders, double-negative T cells infiltrate tissues, induce immunoglobulin production, and secrete proinflammatory cytokines. Double-negative T cells derive from CD8(+) T cells through down-regulation of CD8 surface co-receptors. However, the molecular mechanisms orchestrating this process remain unclear. Here, we demonstrate that the transcription factor cAMP-responsive element modulator α (CREMα), which is expressed at increased levels in T cells from systemic lupus erythematosus patients, contributes to transcriptional silencing of CD8A and CD8B. We provide the first evidence that CREMα trans-represses a regulatory element 5' of the CD8B gene. Therefore, CREMα represents a promising candidate in the search for biomarkers and treatment options in diseases in which double-negative T cells contribute to the pathogenesis.

Estrogen upregulates cyclic AMP response element modulator α expression and downregulates interleukin-2 production by human T lymphocytes.

Systemic lupus erythematosus (SLE) is an autoimmune disease with a complex multifactorial pathogenesis. T lymphocytes play a critical role in disease pathogenesis and display abnormal gene expression and poor interleukin (IL)-2 production. We previously showed that the expression of the transcriptional repressor cyclic AMP response element modulator α (CREMα) is increased in SLE T cells and contributes to reduced IL-2 production. Although estrogen is implicated in the onset and exacerbation of SLE, the precise nature of molecular events regulated by estrogen in immune cell function is not well understood. Here, we asked whether estrogen regulates the expression of CREMα in human T lymphocytes. We show that exposure of human T cells to 17-ß-estradiol leads to a dose-dependent increase in CREMα mRNA expression, and this increase appears to be mediated through the estrogen receptors α and ß. We show that the increased expression of CREMα is due to increased transcriptional activity of the CREM promoter and is mediated by increased expression and binding of the Sp1 transcriptional activator. We further show that estrogen treatment leads to a dose-dependent decrease in IL-2 mRNA and cytokine production by T cells. Finally, the effect of ß-estradiol on CREMα is observed more frequently in T cells from women than from men. We conclude that estrogen can modulate the expression of CREMα and lead to IL-2 suppression in human T lymphocytes, thus revealing a molecular link between hormones and the immune system in SLE.