PSYCHOLOGICAL FEATURES OF THE SUBJECTIVE PERCEPTION OF THE QUALITY OF LIFE OF PATIENTS WITH HEPATOCEREBRAL DYSTROPHY

Wilson's disease (WD) is a rare autosomal recessive disorder of copper metabolism. The clinical features associated with WD are highly varied. However, subtypes generally reflect neurological, hepatic, and psychiatric symptoms. Cognitive dysfunction is often reported, and patients suffer from additional depressive symptoms. The goal of our work was detection of psychological features of the subjective perception of the quality of life of patients with Wilson's disease Material and methods: Psychodiagnostic examination was performed for 33 patients with WD. All patients were presented as General group (GG), trembling form (TF), trembling-rigid form (TRF), rigid-arrhythmic-hyperkinetic form (RAHF), extrapyramidal-cortical form (ECF) and abdominal form (AF). To determine the characteristics of the subjective perception of quality of life (QOL) by patients with WD and the effectiveness of their social functioning in general, a method for assessing the integrative indicator of quality of life according to recommendations J. Mezzich. Quality of Life Index is a concise instrument for comprehensive, culture-informed, and self-rated assessment of health-related quality of life. It is composed of 10 items (from physical well-being to global perception of quality of life). Each item is rated on a 10-point scale. The subjective perception was eliminated with general perception, physical well-being, psychological well-being, self-care and independence in actions, working capacity, interpersonal interaction, socio-emotional support, civil and service support, personal realization, spiritual realization. Detection of degree of voluntary-regulatory, cognitive, speech, motor and emotional disorders were performed also for all patients.

The results of the study showed that a decrease in the assessments of the general perception of QOL (β=-0.64, p=0.00) and the nature of interpersonal interaction (β=-0.56, p=0.01) in patients with WD is associated with an increase in the level of depression. Satisfaction with their physical well-being in them is associated with the level of depression (β=-0.55, p=0.00) and movement disorders (β=-0.30, p=0.03). Subjective perception of psychological well-being in these patients is associated with the degree of anxiety (β=-0.48, p=0.00), depression (β=0.55, p=0.00) and the level of voluntary regulation disorders (β=-0.65, p=0.00). Satisfaction with the level of self-care and independence in actions is associated with the degree of anxiety (β=-0.50, p=0.00), rigidity (β=-0.50, p=0.00) and movement disorders (β=-0.47, p=0.00). Subjective assessments of patients' ability to work are associated with the level of movement disorders (β=-0.81, p=0.00) and depression (β=-0.34, p=0.04). Satisfaction with socio-emotional support is associated with rigidity (β=-0.87, p=0.00) and anxiety (β=-0.43, p=0.00). Subjective perception of personal fulfillment in these patients is associated with the degree of voluntary-regulatory disorders (β=-0.61, p=0.00) and the level of anxiety (β=-0.43, p=0.00). Patients' satisfaction with their spiritual fulfillment is combined with rigidity of behavioral reactions (β=-0.75, p=0.00). Thus, the features of the subjective perception of QOL in patients with WD are primarily associated with the characteristics of their emotional sphere, namely, with the level of expression of anxiety-depressive symptoms.

The level of subjective perception of quality of life of patients with hepatocerebral dystrophy is associated with a number of clinical and psychological factors, such as the form of the disease, its duration, and the severity of motor and emotional disorders. Thus, the highest assessments of almost all QOL parameters are noted in patients with the abdominal form of WD. Patients with extrapyramidal-cortical and rigid-arrhythmohyperkinetic forms assess their level of physical and psychological well-being significantly lower than other patients.