PDF(Pubmed)
Abstract：
UNASSIGNED: Atypical hemolytic uremic syndrome (aHUS) poses a significant health challenge due to its rarity and severity within the spectrum of thrombotic microangiopathy. Despite efforts to optimize and personalize health care for patients with aHUS, understanding the individual experiences, needs, and desires of patients with aHUS and their relatives remains limited.
UNASSIGNED: Here, we present a nationwide, exploratory, qualitative interview study with a direct content analysis approach. In-depth interviews and a 6-week evaluation were audio-recorded and conducted using a semistructured topic guide, based on the Institute for Positive Health (IPH) model.
UNASSIGNED: Analysis of 10 interviews involving 6 patients with aHUS and 13 relatives revealed the prevalence of long-term disease symptoms in adult patients, notably fatigue, which significantly impacted daily functioning. Moreover, the resilience demonstrated by patients and their relatives was noteworthy; however, the acute phase of aHUS and the unpredictable nature of disease recurrence could profoundly affect mental well-being. The emotional toll of aHUS is pervasive, with feelings of fear, guilt, and trauma persisting across disease phases in both patients and relatives. Challenges in medical care, including delays in diagnosis and the need for personalized and uniform protocols, were highlighted. Support was deemed crucial, indicating the necessity for enhancements in the accessibility to comprehensible disease information and psychological counseling. Finally, complexities surrounding genetic testing and carriership were discussed.
UNASSIGNED: This study underscores the profound, enduring, and multifaced impact of aHUS. The insights gleaned from the experiences and needs of patients with aHUS and their relatives could lay the foundation for development and implementation of more personalized innovations in aHUS health care.
UNASSIGNED: Here, we present a nationwide, exploratory, qualitative interview study with a direct content analysis approach. In-depth interviews and a 6-week evaluation were audio-recorded and conducted using a semistructured topic guide, based on the Institute for Positive Health (IPH) model.
UNASSIGNED: Analysis of 10 interviews involving 6 patients with aHUS and 13 relatives revealed the prevalence of long-term disease symptoms in adult patients, notably fatigue, which significantly impacted daily functioning. Moreover, the resilience demonstrated by patients and their relatives was noteworthy; however, the acute phase of aHUS and the unpredictable nature of disease recurrence could profoundly affect mental well-being. The emotional toll of aHUS is pervasive, with feelings of fear, guilt, and trauma persisting across disease phases in both patients and relatives. Challenges in medical care, including delays in diagnosis and the need for personalized and uniform protocols, were highlighted. Support was deemed crucial, indicating the necessity for enhancements in the accessibility to comprehensible disease information and psychological counseling. Finally, complexities surrounding genetic testing and carriership were discussed.
UNASSIGNED: This study underscores the profound, enduring, and multifaced impact of aHUS. The insights gleaned from the experiences and needs of patients with aHUS and their relatives could lay the foundation for development and implementation of more personalized innovations in aHUS health care.
摘要：
■非典型溶血性尿毒综合征(aHUS)由于其在血栓性微血管病中的罕见性和严重程度，对健康构成重大挑战。尽管努力为aHUS患者优化和个性化医疗保健，了解个人经历，需要,并且aHUS患者及其亲属的愿望仍然有限。
■这里，我们提出了一个全国性的，探索性,采用直接内容分析法的定性访谈研究。深入访谈和为期6周的评估是音频记录的，并使用半结构化的主题指南进行。基于积极健康研究所(IPH)模型。
■对涉及6名aHUS患者和13名亲属的10次访谈的分析揭示了成人患者中长期疾病症状的患病率，特别是疲劳,严重影响日常运作。此外，患者及其亲属表现出的韧性值得注意；然而，aHUS的急性期和疾病复发的不可预测性质可能会严重影响心理健康。aHUS的情感代价无处不在，带着恐惧的感觉，内疚，内疚和创伤持续在患者和亲属的疾病阶段。医疗保健方面的挑战，包括诊断的延迟以及对个性化和统一协议的需求，被突出显示。支持被认为是至关重要的，表明有必要增强可理解的疾病信息和心理咨询的可及性。最后,围绕基因检测和运输的复杂性进行了讨论。
■这项研究强调了深刻的，持久,以及aHUS的多重影响。从aHUS患者及其亲属的经验和需求中获得的见解可以为aHUS医疗保健中更个性化创新的开发和实施奠定基础。
■这里，我们提出了一个全国性的，探索性,采用直接内容分析法的定性访谈研究。深入访谈和为期6周的评估是音频记录的，并使用半结构化的主题指南进行。基于积极健康研究所(IPH)模型。
■对涉及6名aHUS患者和13名亲属的10次访谈的分析揭示了成人患者中长期疾病症状的患病率，特别是疲劳,严重影响日常运作。此外，患者及其亲属表现出的韧性值得注意；然而，aHUS的急性期和疾病复发的不可预测性质可能会严重影响心理健康。aHUS的情感代价无处不在，带着恐惧的感觉，内疚，内疚和创伤持续在患者和亲属的疾病阶段。医疗保健方面的挑战，包括诊断的延迟以及对个性化和统一协议的需求，被突出显示。支持被认为是至关重要的，表明有必要增强可理解的疾病信息和心理咨询的可及性。最后,围绕基因检测和运输的复杂性进行了讨论。
■这项研究强调了深刻的，持久,以及aHUS的多重影响。从aHUS患者及其亲属的经验和需求中获得的见解可以为aHUS医疗保健中更个性化创新的开发和实施奠定基础。
