Diverse learners: learning disabilities and quality of life following mind-body and health education interventions for adults with neurofibromatosis.

PURPOSE: Neurofibromatosis (NF) is associated with low quality-of-life (QoL). Learning disabilities are prevalent among those with NF, further worsening QoL and potentially impacting benefits from mind-body and educational interventions, yet research on this population is scarce. Here, we address this gap by comparing NF patients with and without learning disabilities on QoL at baseline and QoL-related gains following two interventions. METHODS: Secondary analysis of a fully-powered RCT of a mind-body program (Relaxation Response Resiliency Program for NF; 3RP-NF) versus an educational program (Health Enhancement Program for NF; HEP-NF) among 228 adults with NF. Participants reported QoL in four domains (Physical Health, Psychological, Social Relationships, and Environmental). We compare data at baseline, post-treatment, and 12-month follow-up, controlling for intervention type. RESULTS: At baseline, individuals with NF and learning disabilities had lower Psychological (T = -3.0, p = .001) and Environmental (T = -3.8, p < .001) QoL compared to those without learning disabilities. Both programs significantly improved all QoL domains (ps < .0001-0.002) from baseline to post-treatment, regardless of learning disability status. However, those with learning disabilities exceeded the minimal clinically important difference in only one domain (Psychological QoL) compared to three domains in individuals without learning disabilities. Moreover, those with learning disabilities failed to sustain statistically significant gains in Psychological QoL at 12-months, while those without learning disabilities sustained all gains. CONCLUSION: Adults with NF and learning disabilities have lower Psychological and Environmental QoL. While interventions show promise in improving QoL regardless of learning disabilities, additional measures may bolster clinical benefit and sustainability among those with learning disabilities.

The impact of a virtual mind-body program on symptoms of depression and anxiety among international English-speaking adults with neurofibromatosis.

The neurofibromatoses (NFs) are a set of incurable genetic disorders that predispose individuals to nervous system tumors. Although many patients experience anxiety and depression, there is little research on psychosocial interventions in this population. The present study examined the effects of a mind-body intervention on depression and anxiety in adults with NF. This is a secondary analysis of the Relaxation Response Resiliency Program for NF (3RP-NF), an 8-week virtual group intervention that teaches mind-body skills (e.g., relaxation, mindfulness) to improve quality of life. Participants were randomized to 3RP-NF or the Health Enhancement Program for NF (HEP-NF) consisting of health informational sessions and discussion. We evaluated depression (PHQ-9) and anxiety (GAD-7) at posttreatment, 6 months, and 12 months. Both groups improved in depression and anxiety between baseline and posttest, 6 months, and 12 months. The 3RP-NF group showed greater improvements in depression scores from baseline to 6 months compared with HEP-NF and with lower rates of clinically significant depressive symptoms. There were no between-group differences for anxiety. Both interventions reduced distress and anxiety symptoms for individuals with NF. The 3RP-NF group may be better at sustaining these improvements. Given the rare nature of NF, group connection may facilitate reduced distress.

Minimal clinically important difference in the World Health Organization Quality of Life Brief (WHOQOL-BREF) for adults with neurofibromatosis.

PURPOSE: This study aimed to estimate minimal clinically important difference (MCID) values for the World Health Organization Quality of Life Brief version (WHOQOL-BREF) among adults with neurofibromatosis (NF). An MCID is needed to demonstrate clinical meaningfulness of interventions for NF. METHODS: We estimated MCID for the WHOQOL-BREF: the quality of life (QoL) measure recommended by the Response Evaluation in Neurofibromatosis and Schwannomatosis International Collaboration. We used data from 228 clinical trial participants with NF type 1, NF type 2-related schwannomatosis, or schwannomatosis (SCHWN) who completed 10 weeks of a virtual group mind-body program targeting resiliency or a time- and attention-matched control. Following established guidelines, we estimated MCIDs using both anchor-based and distribution-based methods for physical, psychological, social relationships, and environmental domains of the WHOQOL-BREF. RESULTS: MCID results varied across method and QoL domain. Three anchor-based methods, average change (AC), change difference (CD), and regression (REG), yielded the most consistent and comparable MCID across QoL domains. Based on these methods, we recommend ranges for each QoL domain: Physical QoL (3.9-7.3), Psychological QoL (4.7-8.1), Social QoL (2.6-5.9), and Environmental QoL (4.1-6.6). CONCLUSION: Establishing a rigorous MCID for QoL in NF is a critical step toward evaluating meaningful change in response to psychosocial interventions.

Dificultades de acceso al sistema de procesamiento linguístico en diferentes poblaciones infantiles / Difficulties of access to the language processing system in different pediatric populations

El estudio del lenguaje en niños presupone la puesta en marcha de un marco teórico que permita indagar en procesos de la evolución lingüística, con un criterio conceptual, abierto y sensible a los diferentes signos y síntomas que exhiben los niños en su desarrollo. La Psicología Cognitiva ofrece un marco de referencia que se ha considerado útil para ese objetivo. En ese contexto, considerar que la mente humana, con sede en el cerebro, está constituida por módulos o procesadores de diferente información, con aptitud de registro bastante independiente entre ellos, conectados por accesos, admite su aplicación para analizar cómo procede un sistema individual frente a los distintos estímulos lingüísticos que recibe. Con ese criterio se analizaron las producciones de poblaciones portadoras de cuadros diferentes: Neurofibromatosis 1, Síndrome de Prader – Willi, Trastorno Generalizado del Desarrollo y Trastorno Específico del Lenguaje, de manera de interpretar la modalidad particular de procesamiento de cada una, presuponiendo el hallazgo de marcadores clínicos descriptivos de las dificultades de acceso al sistema lingüístico.

The study of language in children implies the need for atheoretical framework that allows investigation of the pro-cesses of language development from a conceptual point ofview, open and sensitive to the different signs and symp-toms children present with during growth. Cognitive psy-chology provides a reference framework that is considereduseful for this aim. In this context, the human mind, loca-ted in the brain, is considered to consist of modules or pro-cessors for different kinds of information with a quite inde-pendent capacity of registration among them, connectedby accesses. This construct allows us to analyze how eachindividual system proceeds in the face of the different lin-guistic stimuli it receives. Based on this construct, langua-ge production of children with different pathologies wereanalyzed: Neurofibromatosis type 1, Prader–Willi syndro-me, pervasive developmental disorder, and specific lan-guage impairment, to evaluate the specific way of proces-sing in each of these conditions, taking into account thefinding of descriptive clinical markers of difficulties of accessto the language system.

Neurofibromatose (VIII): tratamento / Neurofibromatosis (VIII): treatment

Discutimos os diferentes tratamentos aplicados à neurofibromatose. Chamamos atençäo para o apoio psicológico que deve ser dado ao portador da síndrome e a importância da sua integraçäo na sociedade