Purpose: Primary immunodeficiency (PID) is a clinically, immunologically, and genetically heterogeneous group of diseases resulting from quantitative and/or qualitative deficiencies of immune system. This study aimed to evaluate adults with primary immunodeficiency (PID) within the scope of the International Classification of Functioning, Disability, and Health (ICF).
Materials and methods: We evaluated 20 adults with PID and 20 healthy adults according to the ICF items. For domain b, exercise capacity, hand-grip strength (HGS), sleep functions, fatigue perception, and body composition; for domain s, posture, and muscle mass were evaluated. Health-related quality of life, activities of daily living, and physical activity were evaluated for domain d. For domain e, supports and relationships, societal attitudes, services, and policies of health, employment, and transportation were determined as barriers.
Results: Exercise capacity, HGS, sleep function, and body composition were significantly impaired, fatigue severity from domain b increased in adult patients with PID compared to healthy controls (p < 0.05). Daily life activities and quality of life from domain d were significantly poorer in patients with PID compared to healthy controls (p < 0.05).
Conclusions: The evaluation and selecting appropriate outcomes based on ICF-framework will assist health professionals in goal-based rehabilitation plans and better improving functional capacity in adult PID.
Keywords: ICF; Primary immunodeficiency; exercise capacity; physical activity; quality of life.
Most affected components of body functions and structures are temperament and personality functions, energy and drive functions, sensations of pain, general metabolic functions, sleep functions, and exercise tolerance functions in patients with primary immunodeficiency.The adults patients with primary immunodeficiency have severe problems in carrying out daily routine, doing housework, caring for household objects, recreation and leisure, and remunerative employment categories.Rehabilitation professionals should select appropriate outcome measures to evaluate the effectiveness of rehabilitation on daily life limitations and participation in primary immunodeficiency.Rehabilitation professionals should evaluate body composition, peripheral muscle functions, exercise capacity, fatigue perception, sleep quality, postural deviations, activities of daily living, physical activity, and quality of life as outcome measures in primary immunodeficiency.