Craniofacial microsomia: Accelerating Research and Education (CARE)

颅面微小症：加速研究和教育 (CARE)

• 批准号: 10793329
• 负责人: Carrie Lyn Heike
• 金额: $ 7.52万
• 依托单位: SEATTLE CHILDREN'S HOSPITAL
• 依托单位国家: 美国
• 财政年份: 2020
• 资助国家: 美国
• 起止时间: 2020-05-01 至 2025-02-28
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/10793329
• 关键词: Acceleration; Address; Adolescent; Advocacy; Advocate; Affect; Amendment; Area; Breathing; Caregivers; Cephalic; Child; Clinical; Clinical Data; Clinical Research; Code; Collaborations; Communities; Complex; Country; Data; Databases; Development; Diagnosis; Distress; Ear; Education; Evaluation; Face; Foundations; Future; General Population; Goals; Growth; Health; Health Personnel; Health Professional; Health Status; Healthcare; Hearing; Hearing Aids; Heart; Holistic Health; Individual; International; International Classification of Diseases; Intervention; Interview; Investigation; Jaw; Kidney; Knowledge; Live Birth; Mandible; Medical; Mental Health; Methodology; Methods; Nomenclature; Outcome Measure; Participant; Pathway interactions; Patient Outcomes Assessments; Patients; Population; Psychosocial Assessment and Care; Registries; Research; Research Project Grants; Screening procedure; Social support; Standardization; Structure; Survey Methodology; Techniques; Testing; Update; Variant; Vertebral column; Vulnerable Populations; care burden; care outcomes; cleft lip and palate; cost; craniofacial; craniofacial microsomia; design; distraction; experience; health care model; improved; informant; malformation; microtia; neurodevelopment; nutrition; optimism; orofacial cleft; psychologic; psychological distress; reconstruction; social health determinants; young adult

Project Summary Craniofacial microsomia (CFM) is a complex congenital condition that affects approximately 1:3,500 to 1:5,600 live births. Clinical features associated with CFM include underdevelopment of the facial structures, most commonly affecting the ear (e.g. microtia), and jaw (e.g. mandibular hypoplasia). Nearly half of individuals with CFM also have extra-cranial malformations, such as heart, kidney, or spine anomalies. Individuals with CFM have complex healthcare needs and often require a lifetime of staged and (ideally) coordinated evaluations and interventions to optimize growth and nutrition, hearing, neurodevelopment, breathing, and minimize the impact of extra-cranial malformations. Recent advances in techniques for hearing amplification, jaw distraction, and ear reconstruction have the potential to improve aspects of health important to individuals with CFM. However, we have large gaps in knowledge about holistic healthcare outcomes, burden of healthcare, cost, and the psychologic needs of individuals with CFM and/or their caregivers. Individuals with CFM have rarely been included in qualitative studies to explore their unique healthcare experiences. This study proposes to establish an international community of individuals affected by CFM, healthcare providers, and advocacy leaders to facilitate future research in CFM. Multi-perspective data pertaining to the psychological health and healthcare experiences of individuals with CFM and their caregivers across the treatment pathway will be collected. The study team proposes a mixed-methods multi-informant and international study. This methodologically rigorous approach will be used to achieve the following goals: validate a psychological conceptual framework for CFM through interviews with 160 individuals with CFM and/or their caregivers (Aim 1); identify predictors of psychological distress in an international population of 800 individuals affected by CFM (Aim 2); evaluate the extent to which current healthcare provisions address the areas of psychological concern identified in Aims 1 and 2 through interviews with over 60 healthcare professionals and patient advocates (Aim 3); and establish a registry for participants with CFM and/or their caregivers for future research (Aim 4). This project will provide a comprehensive approach for assessing psychological health and healthcare experiences of individuals with CFM and their caregivers. Future studies will focus on investigations of specific interventions to optimize psychologic health in this vulnerable population.

项目摘要 颅面微粒细胞（CFM）是一种复杂的先天性疾病，影响约1：3,500至1：5,5,600 活产。与CFM相关的临床特征包括面部结构的发育不足，大多数 通常影响耳朵（例如Microtia）和下巴（例如下颌骨发育不全）。近一半的人 CFM还具有颅外畸形，例如心脏，肾脏或脊柱异常。患有CFM的人 有复杂的医疗保健需求，通常需要一生的上演和（理想情况下）协调的评估 和干预措施以优化生长和营养，听力，神经发育，呼吸，并最大程度地减少 颅外畸形的影响。听力放大的技术的最新进展，下巴 分心和耳朵重建有可能改善对患有患者重要的健康方面 CFM。但是，我们在整体医疗保健成果，医疗保健负担的知识方面有很大的差距， 成本，以及CFM和/或其护理人员的人的心理需求。 CFM的人有 很少被包括在定性研究中，以探索其独特的医疗体验。这项研究提出了 建立一个由CFM，医疗保健提供者和倡导影响的个人的国际社会 领导者促进CFM的未来研究。与心理健康有关的多观点数据和 CFM患者及其护理人员在治疗途径中的医疗体验将是 集。研究团队提出了混合方法多信息和国际研究。这 方法严格的方法将用于实现以下目标：验证心理 CFM的概念框架通过对160名CFM和/或其护理人员的人进行访谈（AIM 1）;确定由CFM影响的800个人的国际人群中心理困扰的预测因素 （目标2）;评估当前医疗保健条款解决心理关注领域的程度 通过与60多名医疗保健专业人员和患者拥护者的访谈在目标1和2中确定（目标 3）;并为CFM和/或其护理人员的参与者建立注册表以进行未来研究（AIM 4）。这 项目将提供一种评估心理健康和医疗体验的全面方法 CFM及其护理人员的人。未来的研究将集中于对特定干预措施的调查 在这个脆弱人群中优化心理健康。