Primary Care Needs for Patients with Vascular Anomalies: Improving Continuity of Care and Care Coordination in Rare Diseases

项目摘要/摘要 美国有3000万人患有一种罕见的疾病，其中约一半受到影响 都是孩子。目前的研究重点是患有血管畸形(VAS)的儿童，这是一种罕见的疾病 经常被误诊和误治的先天性疾病。复杂的VAS会导致疼痛，毁容， 如果不治疗，会出现凝血障碍、功能障碍和死亡。因此，儿童需要退伍军人管理局专家的照顾，并且 对于患有罕见疾病的儿童来说，初级护理和专科护理之间的协调势在必行，以确保 患者得到全面、安全、有效的护理。对于生活在农村的家庭来说，情况尤其如此 社区，因为退伍军人管理局的专家聚集在城市的儿科医疗中心。然而，照顾者 许多患有罕见疾病的儿童难以获得他们所需的全面护理。我们的 初步数据表明，常见的护理障碍包括零散的护理系统、距离 专家，缺乏知识渊博的儿科医生，以及当地资源不足。协调小学和 特殊护理对于患有VAS的儿童是必不可少的，因为它可以帮助降低急诊室的风险 探视、住院和再入院。因此，拟议的研究旨在确定促进者和 阻碍VAS患者高质量护理协调的障碍，特别是农村地区的患者。鲜为人知 关于罕见疾病患者的护理协调和连续性方面的差距，或地理位置 位置影响护理协调。因此，我们提议的研究的目标是通过以下方式填补这一重要空白 使用创新的三元研究设计，包括对照顾者-儿科医生-VA专家的采访 三合会，以全面了解照顾协调的儿童与视障人士。我们将实现这一目标 通过三个目标实现目标：目标1：描述退伍军人管理局专家对促进者和护理障碍的看法 对于VAS患者的协调，我们将对在以下地点执业的VA专家进行半结构化访谈 多学科退伍军人管理局中心。目的2：了解退伍军人病患者的照顾者对促进者和 VAS患者护理协调的障碍，我们将采访VAS儿童的照顾者 农村和非农村地区。目标3：描述儿科医生对促进者和护理障碍的看法 协调VAS患者，我们将采访护理VAS患者的儿科医生，抽样 在农村和非农村地区执业的临床医生。我们将使用主题分析来分析我们的采访 成绩单。该分析将全面概述护理协调的促进者和障碍， 通过综合儿科医生、专科医生和非农村环境，比较农村和非农村背景 看护人的观点。结果将揭示可以用来设计未来的可修改因素 改善初级保健的干预措施。

PROJECT SUMMARY/ABSTRACT Thirty million people in the United States live with a rare disorder, and approximately half of those affected are children. The current study focuses on children with vascular anomalies (VAs), a spectrum of rare congenital disorders that are often misdiagnosed and mistreated. Complex VAs can result in pain, disfigurement, coagulopathy, dysfunction, and death if left untreated. Therefore, children require the care of VA specialists, and coordination between primary and specialty care is imperative for children with rare diseases to ensure patients receive comprehensive, safe, and effective care. This is especially true for families living in rural communities, given that VA specialists are clustered at urban pediatric medical centers. However, caregivers of children with rare diseases struggle to get their children the comprehensive care they need. Our preliminary data suggests that common barriers to care include a fragmented system of care, distance to specialists, lack of knowledgeable pediatricians, and inadequate local resources. Coordinated primary and specialty care is essential for children with VAs because it can help reduce the risk of emergency room visits, hospitalizations, and readmissions. Therefore, the proposed study aims to identify facilitators and barriers to high-quality care coordination for patients with VAs, especially those in rural areas. Little is known about gaps in care coordination and continuity of care for patients with rare diseases or the ways that geographic location influences care coordination. Therefore, the goal of our proposed study is to fill this important gap by using an innovative triadic research design that involves interviews with caregiver-pediatrician-VA specialist triads to gain a comprehensive understanding of care coordination for children with VAs. We will achieve this goal through three aims: Aim 1: To characterize VA specialists’ perspectives on facilitators and barriers to care coordination for patients with VAs, we will conduct semi-structured interviews with VA specialists practicing at multidisciplinary VA centers. Aim 2: To characterize caregivers of VA patients’ perspectives on facilitators and barriers to care coordination for patients with VAs, we will interview caregivers of children with VAs who live in rural and non-rural areas. Aim 3: To characterize pediatricians’ perspectives on facilitators and barriers to care coordination for patients with VAs, we will interview pediatricians who care for patients with VAs, sampling clinicians who practice in rural and non-rural areas. We will use thematic analysis to analyze our interview transcripts. The analysis will provide a comprehensive overview of facilitators and barriers to care coordination, with a comparison of rural and non-rural contexts, through a synthesis of pediatricians’, specialists’, and caregivers’ perspectives. The results will reveal modifiable factors that can be leveraged to design future interventions to improve primary care.