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

项目概要/摘要 在美国，有3000万人患有一种罕见的疾病，其中大约一半的人患有这种疾病。 都是孩子目前的研究集中在儿童血管异常（VA），一系列罕见的 经常被误诊和误治的先天性疾病。复杂的VA会导致疼痛，毁容， 凝血病、功能障碍和死亡，如果不治疗。因此，儿童需要VA专家的护理， 对于患有罕见疾病的儿童，初级和专科护理之间的协调至关重要， 患者得到全面、安全和有效的护理。对于生活在农村地区的家庭来说， 社区，鉴于VA专家聚集在城市儿科医疗中心。然而，护理人员 患有罕见疾病的儿童很难让他们的孩子得到他们所需要的全面护理。我们 初步数据表明，保健的常见障碍包括保健系统支离破碎， 专家，缺乏知识渊博的儿科医生，以及当地资源不足。协调的主要和 专业护理对VA儿童至关重要，因为它可以帮助降低急诊室的风险 访视、住院和再入院。因此，拟议的研究旨在确定促进者， 对VA患者进行高质量护理协调的障碍，特别是农村地区的VA患者。知之甚少 关于罕见病患者的护理协调和护理连续性方面的差距， 位置影响护理协调。因此，我们提出的研究目标是填补这一重要空白， 采用创新的三位一体研究设计，包括与儿童-儿科医生-VA专家的访谈 三合会，以获得全面了解照顾协调与VA儿童。我们将实现这一目标 通过三个目标实现目标：目标1：表征VA专家对护理促进者和障碍的观点 为了协调VA患者，我们将与VA专家进行半结构化访谈， 多学科VA中心。目的2：描述VA患者的照顾者对促进者的看法， 与VA患者的护理协调障碍，我们将采访VA儿童的照顾者谁住在 农村和非农村地区。目的3：描述儿科医生对护理促进者和障碍的看法 协调VA患者，我们将采访照顾VA患者的儿科医生， 在农村和非农村地区执业的临床医生。我们将用主题分析法来分析我们的访谈 成绩单该分析将全面概述护理协调的促进因素和障碍， 通过综合儿科医生、专科医生和 照顾者的观点。结果将揭示可用于设计未来的可修改因素 采取措施改善初级保健。

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.