Department of Pediatrics, Children's Mercy Kansas City, Kansas City, Missouri, USA.
Department of Pediatrics, University of Missouri-Kansas City School of Medicine, Kansas City, Missouri, USA.
Mol Genet Genomic Med. 2022 Nov;10(11):e2057. doi: 10.1002/mgg3.2057. Epub 2022 Sep 24.
Ehlers-Danlos syndrome (EDS) represents a family of heritable connective tissue disorders with overlapping phenotypic features, frequently including joint hypermobility, tissue fragility, and skin hyperextensibility. Comorbid symptoms are common for patients with EDS and include multiple body systems marked by neurologic, cardiovascular, gastrointestinal, musculoskeletal issues, chronic pain, headaches, and anxiety and depression. The many comorbidities lead to high disease burden, which requires greater healthcare utilization.
This survey of families examines healthcare utilization, of adults and minors, through evaluation of subspecialty care appointments across many healthcare systems in one region.
There were 155 adults and 83 minors with a diagnosis of EDS with a total of 693 unique visits across 27 different specialties at over 20 different hospitals or clinics in the surveyed area. Cardiology, neurology, and gastroenterology were the most utilized subspecialties for adults, while rheumatology, cardiology, and neurology were most utilized by minors. Many respondents (67%) reported their medical care needs are not being met, and 87% reported interest in a multidisciplinary clinic for EDS with the most interest in pain management, physical and occupational therapy, and rheumatology.
Understanding healthcare utilization and needs of those with EDS can provide the foundation for improved care for those with EDS through a coordinated multidisciplinary care model.
埃勒斯-当洛斯综合征(EDS)是一组遗传性结缔组织疾病,具有重叠的表型特征,常包括关节过度活动、组织脆弱和皮肤过度伸展。EDS 患者常伴有合并症,涉及多个身体系统,包括神经系统、心血管系统、胃肠道系统、肌肉骨骼系统问题、慢性疼痛、头痛以及焦虑和抑郁。这些众多的合并症导致了高疾病负担,需要更多的医疗保健利用。
本项针对家庭的调查通过评估一个地区多个医疗系统的专科护理预约,来研究成年人和未成年人的医疗保健利用情况。
在调查区域的 20 多家不同的医院或诊所中,共有 27 个不同专科的 693 次就诊,涉及 155 名成年人和 83 名未成年人,他们均被诊断为 EDS。对于成年人而言,心内科、神经内科和消化内科是利用率最高的专科,而对于未成年人而言,则是风湿科、心内科和神经内科。许多受访者(67%)表示他们的医疗需求未得到满足,87%的受访者对 EDS 多学科诊所感兴趣,他们最感兴趣的领域是疼痛管理、物理和职业治疗以及风湿病学。
了解 EDS 患者的医疗保健利用情况和需求,可以为通过协调的多学科护理模式为 EDS 患者提供更好的护理提供基础。