Living with Atypical Hemolytic Uremic Syndrome in the Netherlands: Patient and Family Perspective.

Autor:	Bouwmeester RN; Radboud University Medical Center, Amalia Children's Hospital, Department of Pediatric Nephrology, Nijmegen, Netherlands., Engel LJ; Radboud University Medical Center, Amalia Children's Hospital, Department of Pediatric Nephrology, Nijmegen, Netherlands., Altena W; Dutch Kidney Patients Association, Bussum, Netherlands., Renette C; Dutch Kidney Patients Association, Bussum, Netherlands., van Daelen C; Dutch Kidney Patients Association, Bussum, Netherlands.; The Netherlands Patients Federation, Utrecht, Netherlands., van Kempen E; Dutch Kidney Patients Association, Bussum, Netherlands., de Wildt R; Dutch Kidney Patients Association, Bussum, Netherlands., van de Kar NCAJ; Radboud University Medical Center, Amalia Children's Hospital, Department of Pediatric Nephrology, Nijmegen, Netherlands.
Jazyk:	angličtina
Zdroj:	Kidney international reports [Kidney Int Rep] 2024 Apr 27; Vol. 9 (7), pp. 2189-2197. Date of Electronic Publication: 2024 Apr 27 (Print Publication: 2024).
DOI:	10.1016/j.ekir.2024.04.047
Abstrakt:	Introduction: 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. Methods: 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. Results: 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. Conclusion: 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. (© 2024 International Society of Nephrology. Published by Elsevier Inc.)
Databáze:	MEDLINE
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