Structuring and standardizing outcome measures using the ICF framework in adults with primary immunodeficiency

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).

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.

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).

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.