Common variable immunodeficiency (CVID) is a complex disease with various influences on perceived health, which correlate with different outcomes, including new morbidity and mortality. Our hypothesis was that CVID patients fall into distinct clusters of perceived health which can inform care. Ward hierarchical cluster analysis and K-means cluster analysis were performed on data of 209 CVID patients to identify subgroups regarding their self-reported physical and mental health status, assessed by the physical (PCS) and mental component scores (MCS) of the Short Form-12 (SF-12). Four clusters of CVID-patients were identified. Cluster 1 was the largest cluster, characterized by a relatively high physical and mental health status (44·0%). In contrast, cluster 2 (21·1%) included patients with low physical and mental health status. Clusters 3 and 4 were mixed groups with high mental and low physical health (15·8%) and vice versa (19·1%). Significant differences between the clusters were found for patient-reported outcomes such as work ability and health literacy, but not for CVID-associated complications such as enteropathy, interstitial lung disease, granulomatosis, lymphadenopathy and autoimmune cytopenia or laboratory parameters such as immunoglobulin levels or B cell-based classification. The results suggest different subgroups of CVID patients with contrasting individual needs which, surprisingly, did not differ in clinical or laboratory characteristics. The main finding of this study is that patients with CVID fall into four distinct clusters according to perceived health, which are largely independent of CVID complications.

Background: Patients with asplenia have a significantly increased lifelong risk of severe invasive infections, particular post-splenectomy sepsis (PSS). Clear preventive measures have been described in the literature, but previous studies found poor implementation of prevention recommendations. Aim of the study is to improve the adherence to guideline-based preventive measures and thereby reduce the incidence of PSS by a novel telephone-delivered intervention that involves both patients and their physicians. Methods: A prospective controlled, two-armed historical control group design is used to evaluate the new intervention compared to usual care. The intervention for patients includes both educational aspects and, building on the Health Action Process Approach (HAPA), intervention components that promote motivation and planning of preventive measures. For physicians the intervention is primarily information-based. The primary outcome, the adherence to preventative measures, is indicated by a study-specific 'Preventing PSS-score' (PrePSS-score), which is assessed at baseline and at 6-months follow-up. Secondary outcomes include, amongst others, patient self-efficacy and action-planning, asplenia-specific health literacy, general self-management and asplenia-specific self-management. In a process-evaluating part of the study interview-data on patients' and physicians' evaluation of the intervention will be gathered. Discussion: This trial will provide evidence about the effectiveness of the novel prevention intervention for asplenic patients. If demonstrated beneficial, the intervention manual will be made publicly available to enable implementation in practice. The experience gained within this trial may also be valuable for prevention strategies in patients with other diseases.

ObjectiveTo explore patients’ with asplenia and general practitioners’ (GPs) (1) perceptions of a novel,Health Action Process Approach(HAPA)-based, educational intervention which targets to increase adherence to post-splenectomy sepsis (PSS) prevention measures and (2) their experience in implementing prevention measures following this intervention.DesignA process evaluation conducted on average 3.5 (for patients) and 3.8 (for GPs) months after the intervention between January 2020 and April 2021 individually by means of semi-structured guideline-based telephone interviews. Data was analysed using qualitative content analysis.ParticipantsVolunteer subsample of N=25 patients with asplenia and N=8 GPs who received the intervention. Inclusion criteria were met by prior participation in the intervention (German-speaking, of full age and insured by the cooperating health insurance). Patient selection was done by purposeful selection aiming at maximum variability in terms of adherence to preventative measures prior to intervention participation. Participating GPs are a non-purposeful selected convenience sample. For reasons of data protection, no personal data was collected.ResultsThe intervention was positively evaluated and its personal relevancy for patients and for the GPs’ professional work became apparent. The intervention promoted risk awareness, intention to action, action planning and subsequently, improved adherence to preventative measures. Helpful factors for implementation among the patients were social support by relatives and GPs. Barriers to adherence identified in both groups can be divided into patient-attributed (eg, comorbidities), doctor-related (eg, lack of knowledge or support) as well as contextual factors (eg, vaccine supply constraints).ConclusionsOur findings indicate a patient and GP perceived benefit of the intervention, but still identify prevailing barriers to implementation. In a further step, a quantitative evaluation of the intervention will be conducted and recommendations for integrating the intervention in usual care will be made.Trial registration numberDRKS00015238.

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