#2877 The use of demoralization scale in patients with Fabry disease: preliminary data
Bibliographic record
Abstract
Abstract Background and Aims Fabry disease (FD) is a genetic X-linked, multisystemic, progressive lysosomal storage leading to a range of debilitating symptoms and psychiatric complications. Due to the stressful nature of diagnosis, psychosocial challenges, and adjustment needs, FD patients may face an increased risk of demoralization (DEM). DEM is a syndrome distinct from depression, characterized by a loss of meaning and purpose, with feelings of hopelessness and helplessness. It is considered a maladaptive coping response, often arising in chronic medical illness and stressful situations, such as the suffering associated with a diagnosis. Currently, no studies are available on demoralization within the FD population. Our study aimed to investigate the prevalence of demoralization in FD patients and, secondly, to explore its association with other psychological and physical symptoms. Method Adult patients (age >18 yrs) diagnosed with Fabry disease were enrolled. The exclusion criterion was the absence of cognitive disorders (mini-mental state examination ≥ 24). Each patient filled in four self-report instruments: Demoralization scale (DS-IT), Edmonton Symptom Assessment System (ESAS), generalized anxiety disorder (GAD), and experience in close relationships (ECR-S) (Table 1). Statistical analysis was performed using the SPSS-30 package. Continuous variables were reported as mean ± standard deviation. Categorical variables were shown as frequencies (%). Results Eight patients (12.5% male) were included with a mean age of 49.2 ± 15.8. Six patients had a classical FD phenotype. Of the participants, 50.0%, 37.5%, and 12.5% reported high, moderate, and low DEM levels, respectively. The average anxious attachment scores were highest in the high-DEM group (25.0 ± 10.9) compared to moderate/low-DEM group (16.0 ± 4.0). Similarly, the high-DEM group reported higher average physical (ESAS-PHYS:16.5 ± 15.8) and psychological (ESAS-PSY:9.5 ± 9.1) distress sub-scores higher than moderate/low-DEM group (ESAS-PHYS 6.75 ± 8.84 and ESAS-PSY, 2.5 ± 2.6). Notably, higher fatigue (4.0 ± 4.1) and depression (4.5 ± 3.7) ESAS sub-scores were found in the high-DEM group. No differences were found in the GAD-7 scores and avoidance attachment scores across the three DEM groups. Conclusion This preliminary data suggests that 50% of FD patients experienced high levels of demoralization, which appears to be associated with increased symptom burden (both psychological and physical) and an anxious attachment style.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".