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Abstract: PUB236

Desires and Needs of Dutch Atypical Hemolytic Uremic Syndrome Patients and Relatives

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Bouwmeester, Romy N., Radboud UMC, Nijmegen, Netherlands
  • Van De Kar, Nicole, Radboud UMC, Nijmegen, Netherlands

Group or Team Name

  • In collaboration with the Dutch Kidney Patient Association
Background

Atypical hemolytic uremic syndrome (aHUS) is a rare and severe form of a thrombotic microangiopathy (TMA). Our knowledge in the biochemical and genetic mechanisms of aHUS is continuously increasing, resulting in the development of targeted treatments such as eculizumab. The improved outcome perspectives and switch to a restrictive eculizumab treatment strategy could be considered life changing for aHUS patients. However, little is known on individual experiences, needs and desires of atypical HUS patients and their relatives.

Methods

This is a nationwide, exploratory, qualitative interview study with a direct content analysis approach. In-depth interviews and a six-week evaluation were audio-recorded and semi-structured. A topic guide included the Institute for Positive Health (IPH) model, which contains six domains of health on a subjective scale.

Results

Thirteen interviews with five aHUS patients and fourteen relatives were conducted after which data saturation was obtained. Long-term disease symptoms (e.g. fatigue) are present in the majority of patients and negatively influence bodily and daily functioning. The resilience of both patients and relatives is remarkable. However, despite a high rate of acceptation, among others the (potentially traumatical) acute phase of aHUS and the unpredictable possibility of disease recurrence have a lasting impact on mental well-being. Support is essential yet to be improved by increasing the accessibility of psychological support in aHUS healthcare and the availability of more comprehensible information.

Conclusion

A new era should begin, as we strive to further optimize health care for aHUS patients while focusing on the person and not only the disease. These insights in the actual needs and desires of Dutch aHUS patients and their relatives will help optimize and personalize aHUS health care. In addition, this study can be an example of a more personalized approach in both research and health care of other rare kidney diseases.