Longitudinal Effects of Elexacaftor/Tezacaftor/Ivacaftor: Multidimensional Assessment of Neuropsychological Side Effects and Physical and Mental Health Outcomes in Adolescents and Adults.

BACKGROUND: Italy initiated elexacaftor/tezacaftor/ivacaftor (ETI) for people with cystic fibrosis (pwCF) in July 2021. It has led to dramatic improvements in lung function, BMI, sweat chloride, and respiratory symptoms. However, few data are available on side effects or effects on a broad range of outcomes. RESEARCH QUESTION: How does ETI affect mental health, cognitive processing, neuropsychological side effects, GI symptoms, and health-related quality of life over time? STUDY DESIGN AND METHODS: This was a prospective, "real-world" longitudinal study. Participants were recruited consecutively and evaluated at initiation (T0) and after 1 month, 3 months, and 6 months of starting treatment. Assessments included depression (nine-item Patient Health Questionnaire), anxiety (seven-item Generalized Anxiety Disorder), cognition (Symbol Digit Modalities Test), GI Symptom Tracker, and health-related quality of life (Cystic Fibrosis Questionnaire-Revised). Based on literature, an ad hoc questionnaire was developed to assess side effects: insomnia, headache, memory problems, "brain fog," and concentration problems. Following descriptive analyses, longitudinal data were analyzed by using mixed models for repeated measures, controlling for age and sex when appropriate. RESULTS: Ninety-two consecutive pwCF (female/male, 46/46; mean age, 25.4 years) participated. FEV1 increased initially and then remained stable. BMI also increased significantly from T0 to 6 months (P < .01). Depression improved from T0 to 1 month (P < .001); however, no changes in anxiety were found. Cognitive processing improved from T0 to subsequent assessments. Positive changes were reported on the GI Symptom Tracker for stools and adherence challenges, although no changes were found for abdominal pain and digestion. Side effects occurred in 10% to 29%, with no reduction over time; insomnia increased significantly across time. Female participants reported more side effects than male participants (ie, insomnia, headache, concentration problems, brain fog). INTERPRETATION: This prospective study evaluated the effects of ETI using multiple measures. Significant improvements were found in many domains; however, side effects were reported by a substantial proportion of pwCF, with no improvements over time. Female participants reported more side effects than male participants. pwCF should be followed up systematically to assess the frequency of side effects after starting this new modulator.

The Italian version of the "tool to measure parental self-efficacy-short form": psychometric properties of the measure and initial validation.

BACKGROUND AND AIM: Parental self-efficacy is recognized as a core element of the transition to parenthood process. It affects parental mental health, as well as children's psychosocial, neurodevelopmental and health outcomes since early infancy. Parents with higher parenting self-efficacy have a higher likelihood of engaging in quality parenting practices that support optimal self-regulation development in children. Our study evaluated the psychometric properties of the Italian TOPSE-Short Form (I-TOPSE-SF) questionnaire. METHODS: 673 Italian mothers (Mage=37.5; SD=5.7) of children aging 0 to 6 years (Mage=3.9; SD=1.7) were involved in this study. RESULTS: Initial results of Confirmatory Factor Analysis highlighted that the fit indices of the hypothesized 6-factor structure of the TOPSE weren't satisfactory. Acceptable internal consistencies for the total score and the six dimensions of the measure were observed. Evidence for convergent and divergent validity were provided. CONCLUSIONS: The I-TOPSE-SF could be a valuable tool to assess parental self-efficacy up to school age, but it is in need of further considerations about its measurement properties.

Comparison of mental health in individuals with primary ciliary dyskinesia, cystic fibrosis, and parent caregivers.

INTRODUCTION: Individuals with chronic respiratory diseases and caregivers are at higher risk for depression and anxiety. Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are both rare genetic diseases, characterized by recurrent respiratory infections. This study compared depression and anxiety in people with PCD (pwPCD) and CF (pwCF), and caregivers, using the screening tools recommended in the CF guidelines. METHODS: Patient Health Questionnaire (PHQ-9) and Generalized Anxiety Disorder (GAD-7) were administered to a PCD and CF sample. Given that PCD is extremely rare, they were matched on age and sex to pwCF at a 1:2 ratio. Similar procedures were performed with parents. RESULTS: A total of 63 patients and 129 caregivers participated: 21 pwPCD and 42 pwCF (ages 12-34 years) plus 43 caregivers of pwPCD and 86 caregivers of pwCF. A high percentage of patients scored above the cut-off for depression (PCD: 33%; CF: 43%) and anxiety (PCD and CF both: 43%), mostly mild. Similarly, a high percentage of caregivers scored above the cut-off for depression (PCD: 42-54%; CF: 45-46%) and anxiety (PCD: 47-54%; CF: 39-56%). Suicidal ideation was endorsed by 9.5% of pwPCD, 20% of mothers and 10% of fathers and 5% of pwCF, 3% of mothers, but no fathers. CONCLUSION: A large percentage of patients and caregivers reported elevated psychological distress and suicidal ideation. Addressing psychological symptoms is critical given they are associated with poor adherence, missed clinic visits, increased inflammation and worse quality of life. Mental health screening and treatment should be integrated into PCD care.

The Efficacy of Parent Training Interventions with Parents of Children with Developmental Disabilities.

Parenting children with developmental disabilities (DD) can be generally characterized by a considerable psychological burden. The effects on parental and familial psychological well-being and, consequently, on children's developmental outcomes should not be underestimated, especially in early childhood. The current review aims to advance our understanding of the key factors (e.g., formats, sample characteristics, research design) that characterize parent training interventions, and that could be related to their outcomes, to guide researchers and clinical practitioners to develop and provide efficient programs. Studies were identified via an Internet search from three electronic databases, following PRIMSA guidelines. Studies published until November 2021 were taken into account. The initial search yielded a total of 2475 studies. Among them, 101 studies were fully reviewed. Finally, ten of the studies, which met all the inclusion criteria, formed the basis for this review. Participants' characteristics, main features of the interventions (i.e., study design, structure, and contents), outcome variables and treatment efficacy were deeply examined and discussed. Key factors of parent training interventions with parents of children affected by DD are enlightened, to guide researchers and clinicians in the design and implementation of tailored specific programs, aimed to sustain parenting and foster children's developmental outcomes, from early stages of life.

Stress and mental health in adolescents and young adults with cystic fibrosis 1 year after the COVID pandemic: Findings from an Italian sample.

BACKGROUND: The spread of COVID-19 was associated with increased stress and new mental health concerns for people with cystic fibrosis (pwCF), already at increased risk for depression and anxiety. This study assessed stress and mental health in adolescents and young adults with CF 1 year from when the pandemic began. METHODS: Sixty-six pwCF (mean age = 24; range 14-36) completed a new measure of the impact of COVID-19 (COVID-19 Exposure and Family Impact Scale-Adolescence and Young Adult; CEFIS-AYA); the Patient Health Questionnaire-9, and the Generalized Anxiety Disorder-7. The Italian translation of the CEFIS-AYA was performed. RESULTS: On the CEFIS-AYA, the mean Exposure score was 5.2 (SD = 2.6) out of 28. The mean Impact score was 1.8 (SD = 0.7; negative valence > 2.5). Individuals were more sedentary and undertaking less exercise. Average stress rating was 5.9 (SD = 2), indicating moderate stress. No significant differences were found between those who did (N = 12) and who did not have a COVID infection (N = 54). A high percentage of participants scored above the clinical cut-off for depression (45%) and anxiety (41%), with a low proportion reporting moderate-severe symptomatology. CONCLUSION: After 1 year, the pandemic was having a less significant impact on patients' daily lives. Sedentary activity and reduced exercise were common. Despite expectations that this group was particularly vulnerable, depression and anxiety scores were similar to the rates described for this population before the pandemic. Overall, these results suggested that pwCF are highly resilient and nearly 1 year after the onset of COVID-19, have returned to similar daily activities and emotional health.

The LiberAction Project: Implementation of a Pediatric Liberation Bundle to Screen Delirium, Reduce Benzodiazepine Sedation, and Provide Early Mobilization in a Human Resource-Limited Pediatric Intensive Care Unit.

Background: Delirium, bed immobilization, and heavy sedation are among the major contributors of pediatric post-intensive care syndrome. Recently, the Society of Critical Care Medicine has proposed the implementation of daily interventions to minimize the incidence of these morbidities and optimize children functional outcomes and quality of life. Unfortunately, these interventions require important clinical and economical efforts which prevent their use in many pediatric intensive care units (PICU). Aim: First, to evaluate the feasibility and safety of a PICU bundle implementation prioritizing delirium screening and treatment, early mobilization (<72 h from PICU admission) and benzodiazepine-limited sedation in a human resource-limited PICU. Second, to evaluate the incidence of delirium and describe the early mobilization practices and sedative drugs used during the pre- and post-implementation periods. Third, to describe the barriers and adverse events encountered during early mobilization. Methods: This observational study was structured in a pre- (15th November 2019-30th June 2020) and post-implementation period (1st July 2020-31st December 2020). All patients admitted in PICU for more than 72 h during the pre and post-implementation period were included in the study. Patients were excluded if early mobilization was contraindicated. During the pre-implementation period, a rehabilitation program including delirium screening and treatment, early mobilization and benzodiazepine-sparing sedation guidelines was developed and all PICU staff trained. During the post-implementation period, delirium screening with the Connell Assessment of Pediatric Delirium scale was implemented at bedside. Early mobilization was performed using a structured tiered protocol and a new sedation protocol, limiting the use of benzodiazepine, was adopted. Results: Two hundred and twenty-five children were enrolled in the study, 137 in the pre-implementation period and 88 in the post-implementation period. Adherence to delirium screening, benzodiazepine-limited sedation and early mobilization was 90.9, 81.1, and 70.4%, respectively. Incidence of delirium was 23% in the post-implementation period. The median cumulative dose of benzodiazepines corrected for the total number of sedation days (mg/kg/sedation days) was significantly lower in the post-implementation period compared with the pre-implementation period: [0.83 (IQR: 0.53-1.31) vs. 0.74 (IQR: 0.55-1.16), p = 0.0001]. The median cumulative doses of fentanyl, remifentanil, and morphine corrected for the total number of sedation days were lower in the post-implementation period, but these differences were not significant. The median number of mobilizations per patient and the duration of each mobilization significantly increased in the post-implementation period [3.00 (IQR: 2.0-4.0) vs. 7.00 (IQR: 3.0-12.0); p = 0.004 and 4 min (IQR: 3.50-4.50) vs. 5.50 min (IQR: 5.25-6.5); p < 0.0001, respectively]. Barriers to early mobilization were: disease severity and bed rest orders (55%), lack of physicians' order (20%), lack of human resources (20%), and lack of adequate devices for patient mobilization (5%). No adverse events related to early mobilization were reported in both periods. Duration of mechanical ventilation and PICU length of stay was significantly lower in the post-implementation period as well as the occurrence of iatrogenic withdrawal syndrome. Conclusion: This study showed that the implementation of a PICU liberation bundle prioritizing delirium screening and treatment, benzodiazepine-limited sedation and early mobilization was feasible and safe even in a human resource-limited PICU. Further pediatric studies are needed to evaluate the clinical impact of delirium, benzodiazepine-limited sedation and early mobilization protocols on patients' long-term functional outcomes and on hospital finances.

Intervention Programs to Promote the Quality of Caregiver-Child Interactions in Childcare: A Systematic Literature Review.

Sensitive caregiver-child interactions appear fundamental throughout childhood, supporting infants' wellbeing and development not only in a familial context but in professional caregiving as well. The main aim of this review was to examine the existing literature about Early Childhood Education Context (ECEC) intervention studies dedicated to caregiver-child interaction, fostering children's socioemotional developmental pathways. Studies published between January 2007 and July 2021 were identified in four electronic databases following PRIMSA guidelines. The initial search yielded a total of 342 records. Among them, 48 studies were fully reviewed. Finally, 18 of them met all inclusion criteria and formed the basis for this review. Main factors characterizing implemented programs were recorded (e.g., intervention and sample characteristics, dimensions of the teacher-child interaction targeted by the intervention, outcome variables, main results) in order to frame key elements of ECE intervention programs. Our review points to a range of fundamental issues that should consider to enhance ECEC interventions' efficacy, supporting children's socioemotional development and caregiver-child interaction. Reflections and considerations for future research are provided.

Psychological interventions during COVID pandemic: Telehealth for individuals with cystic fibrosis and caregivers.

INTRODUCTION: Coronavirus disease 2019 (COVID-19) emerged in China, leading to worldwide morbidity and mortality, including depression and anxiety. As the pandemic spread throughout Italy, mental health concerns increased for people with cystic fibrosis (pwCF), who are at greater risk. The aim was to pilot a Telehealth Psychological Support Intervention for pwCF and caregivers to reduce stress, depression, and anxiety during the lockdown in Italy in March 2020. METHODS: This intervention utilized cognitive behavioral skills (e.g., cognitive reframing). Participants included 16 pwCF and 14 parents, who completed four individual telehealth sessions with a psychologist. Stress ratings, Patient Health Questionnaire and General Anxiety Disorder, PHQ-8 and GAD-7, were completed, in addition to Feasibility and Satisfaction ratings. RESULTS: Ratings of stress significantly decreased from pre- to post-testing for pwCF (paired t(14) = -4.06, p < .01) and parents (paired t = -5.2, p < .001). A large percentage of both groups scored in the clinical range for depression and anxiety at baseline (pwCF: depression/anxiety = 71%; parents: depression = 57%; anxiety = 79%); a large proportion (20%-40%) reported moderate to severe symptomatology. Significant reductions in depression for pwCF were found (pre: M = 8.0 to post: M = 4.7; paired t(14) = 2.8, p < .05) but not anxiety (pre: M = 6.9 to post: M = 5.6, t(14) = 1.2, p = NS-non-significant). Parental depression decreased for parents (pre: M = 6.4 to post: M = 5.1, t(14) = -2.5, p < .05), but not anxiety (pre: M = 8.1 to post: M = 7.9, t(14) = -0.2, p = NS). Feasibility and Satisfaction were positive. CONCLUSION: This telehealth intervention yielded reductions in stress and depression for participants. Anxiety did not significantly decrease, possibly because COVID was ongoing. This feasible, satisfactory intervention was effective for improving mental health.

Predictive Factors of Toddlers' Sleep and Parental Stress.

BACKGROUND: Although most infants consolidate their sleep habits during the first year of life, for many children, sleep is described as disrupted during toddlerhood. Along with individual child variables such as temperamental characteristics, parenting behaviors play a key role in determining children's sleep-wake patterns. The aims of the current study were to evaluate the relationship among toddlers' sleep quality, emotion regulation, bedtime routines, parental bedtime involvement, parental perceived social support and stress, and to integrate a novel combination of the aforementioned dimensions into predictive models of toddlers' sleep quality and parental stress. METHODS: One hundred and sixty parents with 2-3-year-old children filled out the following self-report questionnaires: the Parent-Child Sleep Interaction Scale; the Emotion Regulation Checklist; the Social Provisions Scale; and an ad-hoc questionnaire to assess parental involvement in everyday and bedtime care for children. Three multiple regression analyses were conducted by regressing maternal and paternal parenting stress and infant's quality sleep onto the independent variables described above. RESULTS: Toddlers' emotion regulation and parental psychosocial functioning were related to parental stress. Toddlers' night awakenings and the time required by toddlers to fall asleep were related to parental distress. CONCLUSIONS: The findings evidenced the bidirectional associations among the studied variables, highlighting the protective role of social support in reducing parenting stress and of paternal bedtime involvement in improving toddlers' sleep quality.

Subjective Happiness and Compassion Are Enough to Increase Teachers' Work Engagement?

The present quantitative multi-trait cross-sectional study aims to gain a better understanding of the network of relationship between subjective happiness, compassion, levels of work engagement, and proactive strategies (self- and co-regulation) in a sample of teachers. Participants were 187 full-time in-service teachers (89% female; age M = 48.5; SD = 7.88) from Rome, Italy. We hypothesized that subjective happiness and compassion of early childhood teachers would be related with work engagement in such a way that subjective happiness would promote the engagement of teachers. In a similar fashion, we theorized that subjective happiness would be positively related to self- and co-regulation strategies and that proactive strategies would be in turn associated to work engagement. As expected, the results revealed that subjective happiness and compassion showed effects on work engagement and that this association among constructs was mediated by the role of proactive strategies (ß = 0.22, p < 0.001; ß = 0.37, p < 0.001, respectively). Proactive strategies also have a significant direct effect on work engagement (ß = 0.56, p < 0.001). The study's findings suggest the importance of investing in the quality of the working environment.

Maternal Alexithymia and Attachment Style: Which Relationship with Their Children's Headache Features and Psychological Profile?

INTRODUCTION: A growing body of literature has shown an association between somatic symptoms and insecure "attachment style." In a recent study, we found a relationship between migraine severity, ambivalent attachment style, and psychological symptoms in children/adolescents. There is evidence that caregivers' attachment styles and their way of management/expression of emotions can influence children's psychological profile and pain expression. To date, data dealing with headache are scarce. Our aim was to study the role of maternal alexithymia and attachment style on their children's migraine severity, attachment style, and psychological profile. MATERIALS AND METHODS: We enrolled 84 consecutive patients suffering from migraine without aura (female: 45, male: 39; mean age 11.8 ± 2.4 years). According to headache frequency, children/adolescents were divided into two groups: (1) high frequency (patients reporting from weekly to daily attacks), and (2) low frequency (patients having ≤3 episodes per month). We divided headache attacks intensity into two groups (mild and severe pain). SAFA "Anxiety," "Depression," and "Somatization" scales were used to explore children's psychological profile. To evaluate attachment style, the semi-projective test SAT for patients and ASQ Questionnaire for mothers were employed. Maternal alexithymia traits were assessed by TAS-20. RESULTS: We found a significant higher score in maternal alexithymia levels in children classified as "ambivalent," compared to those classified as "avoiding" (Total scale: p = 0.011). A positive correlation has been identified between mother's TAS-20 Total score and the children's SAFA-A Total score (p = 0.026). In particular, positive correlations were found between maternal alexithymia and children's "Separation anxiety" (p = 0.009) and "School anxiety" (p = 0.015) subscales. Maternal "Externally-oriented thinking" subscale correlated with children's school anxiety (p = 0.050). Moreover, we found a correlation between TAS-20 Total score and SAFA-D "Feeling of guilt" subscale (p = 0.014). Our data showed no relationship between TAS-20 and ASQ questionnaires and children's migraine intensity and frequency. CONCLUSION: Maternal alexithymia and attachment style have no impact on children's migraine severity. However, our results suggest that, although maternal alexithymic traits have no causative roles on children's migraine severity, they show a relationship with patients' attachment style and psychological symptoms, which in turn may impact on migraine severity.