Comparison of Pulmonary Function, Exercise Capacity, and Physical Activity Level Between Children With Hemophilia and Healthy Controls

Comparison of Pulmonary Function, Exercise Capacity, and Physical Activity Level Between Children With Hemophilia and Healthy Controls

Hemophilia is a rare X-linked congenital bleeding disorder characterized by deficiency of clotting factor VIII (hemophilia A) or deficiency of factor IX (hemophilia B) with complex diagnosis and management. Participation in physical activity is still limited in children with hemophilia, probably due to protective attitudes of families/patients and avoidance of activity, and possibly also as a result of chronic pain. Exercise capacity has been identified as a protective factor against joint problems in hemophilia. Aerobic fitness is associated with better pulmonary function in children. Chronic pain and decreased range of motion due to recurrent bleeding in joints and muscles in hemophilia may indirectly affect posture and respiratory mechanics, leading to impaired pulmonary function. This study aims to compare pulmonary function, exercise capacity, posture, and physical activity level between children with hemophilia and healthy controls and to investigate the relationship between these parameters.

Hemophilia group

Inclusion criteria:

• Having been diagnosed with hemophilia and being clinically stable,
• Being between the ages of 8-18,
• Being on prophylaxis treatment (a routine treatment),
• Being 125 cm or taller,
• Being inhibitor negative at the time of enrollment,
• To be able to cooperate with the tests to be performed,
• Being willing to participate in the study.

Exclusion criteria:

• Being inhibitor positive at the time of enrollment,
• Having a history of acute joint bleeding,
• Being unable to bear weight on the extremities,
• Having a history of acute intramuscular bleeding,
• Having undergone radionuclide synovectomy,
• Having a history of surgery on any of the lower extremity joints,
• Having a severe cardiovascular, orthopedic or neurological problem that may affect the assessments.

Control group

Inclusion criteria:

• Not having any known internal or chronic disease
• Being male between the ages of 8-18
• Having full range of motion in the upper extremity
• Having full range of motion in the lower extremity
• Not having any history of injury or surgery in the lower extremity
• Being 125 cm or taller
• Being willing to participate in the study

Exclusion criteria:

• Having any problem that may affect the tests
• Not having full range of motion in the upper extremity
• Having a history of injury or surgery in any of the lower extremity joints
• Having a neurological deficit
• Having pain or movement limitation in the lower extremity