A semi-standardized music therapy intervention for girls with Rett syndrome: A descriptive essay

Rett syndrome (RTT) is a severe neurodevelopmental disorder resulting in a wide range of functional impairments and therefore greatly impacts the lives of both patients and their families. While genetic and medical aspects have been studied for several decades, rehabilitation intervention research is still in its infancy. In this study, the investigating researchers have presented a rehabilitative framework by using music therapy for girls with RTT. This model is founded upon the use of music therapy in light of Stern’s proposal of subjective experience and affect attunement; it also refers to Rosenbaum’s family-centered rehabilitation medicine perspective. This study both describes the theory behind this intervention and presents a newly developed outcome measure. This novel tool may have future clinical and research applications. Music therapy for patients with RTT has not been well researched yet, and as a result, is not universally recommended. However this study’s findings suggest that music therapy is an important component of multidisciplinary therapy. Further collaborative research should be encouraged in order to study and implement the use of music therapy in the treatment of severe disabilities. Projects such as the Enablin+program with the support from the European Commission constitute fundamental tools in promoting integrative medical research and international networks.

Co-development of a transitions in care bundle for patient transitions from the intensive care unit: a mixed-methods analysis of a stakeholder consensus meeting

Background Intensive care unit (ICU) patients undergoing transitions in care are at increased risk of adverse events and gaps in medical care. We evaluated existing patient- and family-centered transitions in care tools and identified facilitators, barriers, and implementation considerations for the application of a transitions in care bundle in critically ill adults (i.e., a collection of evidence-based patient- and family-centred tools to improve outcomes during and after transitions from the intensive care unit [ICU] to hospital ward or community). Methods We conducted a concurrent mixed methods (quan + QUAL) study, including stakeholders with experience in ICU transitions in care (i.e., patient/family partners, researchers, decision-makers, providers, and other knowledge-users). First, participants scored existing transitions in care tools using the modified Appraisal of Guidelines, Research and Evaluation (AGREE-II) framework. Transitions in care tools were discussed by stakeholders and either accepted, accepted with modifications, or rejected if consensus was achieved (≥70% agreement). We summarized quantitative results using frequencies and medians. Second, we conducted a qualitative analysis of participant discussions using grounded theory principles to elicit factors influencing AGREE-II scores, and to identify barriers, facilitators, and implementation considerations for the application of a transitions in care bundle. Results Twenty-nine stakeholders attended. Of 18 transitions in care tools evaluated, seven (39%) tools were accepted with modifications, one (6%) tool was rejected, and consensus was not reached for ten (55%) tools. Qualitative analysis found that participants’ AGREE-II rankings were influenced by: 1) language (e.g., inclusive, balance of jargon and lay language); 2) if the tool was comprehensive (i.e., could stand alone); 3) if the tool could be individualized for each patient; 4) impact to clinical workflow; and 5) how the tool was presented (e.g., brochure, video). Participants discussed implementation considerations for a patient- and family-centered transitions in care bundle: 1) delivery (e.g., tool format and timing); 2) continuity (e.g., follow-up after ICU discharge); and 3) continuous evaluation and improvement (e.g., frequency of tool use). Participants discussed existing facilitators (e.g., collaboration and co-design) and barriers (e.g., health system capacity) that would impact application of a transitions in care bundle. Conclusions Findings will inform future research to develop a transitions in care bundle for transitions from the ICU, co-designed with patients, families, providers, researchers, decision-makers, and knowledge-users.

Individualized Family-Centered Developmental Care for Infants With Congenital Heart Disease in the Intensive Care Unit

Background Individualized family-centered developmental care (IFDC) is considered the standard of care for premature/medically fragile newborns and their families in intensive care units (ICUs). Such care for infants with congenital heart disease (CHD) varies. Objective The Consortium for Congenital Cardiac Care– Measurement of Nursing Practice (C4-MNP) was surveyed to determine the state of IFDC for infants younger than 6 months with CHD in ICUs. Methods An electronic survey was disseminated to 1 nurse at each participating center. The survey included questions on IFDC-related nursing practice, organized in 4 sections: demographics, nursing practice, interdisciplinary practice, and parent support. Data were summarized by using descriptive statistics. Differences in IFDC practices and IFDC-related education were assessed, and practices were compared across 3 clinical scenarios of varying infant acuity by using the χ2 test. Results The response rate was 66% (25 centers). Most respondents (72%) did not have IFDC guidelines; 63% incorporated IFDC interventions and 67% documented IFDC practices. Only 29% reported that their ICU had a neurodevelopmental team. Significant differences were reported across the 3 clinical scenarios for 11 of 14 IFDC practices. Skin-to-skin holding was provided least often across all levels of acuity. Nurse education related to IFDC was associated with more use of IFDC (P < .05). Conclusion Practices related to IFDC vary among ICUs. Opportunities exist to develop IFDC guidelines for infants with CHD to inform clinical practice and nurse education. Next steps include convening a C4-MNP group to develop guidelines and implement IFDC initiatives for collaborative evaluation.

Family-Centered Telehealth Supporting Motor Skills and Activity in Individuals With Rett Syndrome

Rett syndrome is a rare genetically caused condition associated with severe disability and impaired motor functions. Local therapists typically see small numbers of affected individuals, and this limits their capacity to gain experience. Telehealth is being used increasingly to counter poor access to rehabilitation services. Moreover, there is a need to develop management plans that support individuals with Rett syndrome over their lifespan. Three projects in which telehealth support was provided by therapists experienced in Rett syndrome and supported by available local resources are presented in this chapter. The three projects responded to locally identified needs in a cost-efficient way and empowered those working with people with Rett syndrome to maintain and improve their clients' physical function and activity. This chapter will discuss the conceptual underpinnings of delivering a service using a telehealth approach and describe the results and the strategies implemented in the projects mentioned above.

Family-Centered Intervention for Deaf and Hard of Hearing Multilingual Learners

This chapter explores principles of family-centered listening and spoken language (LSL) intervention, research, and best practices for children who are d/Deaf or hard of hearing (DHH) using multiple spoken languages and their families. Children with any degree/type of hearing loss who are in environments where multiple languages are spoken are referred to as deaf multilingual learners (DMLs). The language landscape for these children is varied. Some DMLs acquire a first language (L1) at home and are exposed to subsequent spoken languages in school or community settings; others are born into families where multiple languages are spoken from the beginning. While the chapter focuses on a framework of family-centered intervention applied to language development for DMLs whose families have selected LSL outcomes, the principles discussed broadly apply to DMLs using varied language(s) or modality(ies). Through analysis of best practices for interventionists and case studies, readers will understand bi/multilingual spoken language development for children who are DHH.

Using Family-Centered Practices to Increase Language Access for Multilingual Deaf or Hard of Hearing Children

Children who are deaf or hard of hearing may experience language deprivation in the early years that impacts long-term communication and educational outcomes. Fortunately, family engagement in the early childhood years has been shown to increase outcomes for young learners, and the standards for early childhood family engagement align with best practices for teaching children who are deaf or hard of hearing from multilingual families. Best practices for early childhood education, deaf or hard of hearing education, and multilingual education all place the family at the forefront with a strong belief that family is the first and best teacher for their child. The purpose of the chapter is to present the alignment of family centered practices across early childhood, deaf or hard of hearing, and multilingual education literature and present family centered collaboration strategies to increase early childhood language access for young multilingual children who are deaf or hard of hearing.