Aerobic capacity in children with hemophilia.

OBJECTIVE: To determine whether aerobic capacity is normal in boys with different types of hemophilia compared with healthy peers and whether the level of aerobic capacity correlates with the amount of physical activity, joint health status, muscle strength, and anthropometrics. STUDY DESIGN: 47 patients (mean [SD] age, 12.9 [3.2] years; age range, 8.2-17.4 years) from the "Van Creveldkliniek" of the University Medical Center Utrecht, participated. Anthropometry, muscle strength, joint impairment, functional ability, and aerobic capacity were measured. The amount of energy expenditure during daily living was assessed. RESULTS: All boys were able to perform at maximal or near-maximal level on exercise tests, and none of them reported bleeds or other adverse events. Relative peak oxygen, peak heart rate, and peak working capacity were significantly lower compared with healthy control subjects. 30% had Z-scores >2 for weight. Total muscle strength was normal, and almost no joint impairment and no decrease in functional ability were found. CONCLUSION: The aerobic capacity of children with hemophilia is still lower than the normal population, whereas their overall muscle strength is comparable with healthy peers. The functional ability does not differ from healthy peers, and joint health status showed very minor impairments. A substantial proportion of Dutch children with hemophilia was overweight, without showing a reduction in the amount of self-reported physical activities.

Use of implantable venous access devices in children with severe hemophilia: benefits and burden.

BACKGROUND AND OBJECTIVES: Since venous access in small children can be difficult to obtain, implantable venous access devices (IVAD) are used to administer clotting factor in such patients with severe hemophilia. The aim of our study was to evaluate how many children in our center needed an IVAD in order to be able to start early prophylaxis, what the differences were between children who needed an IVAD and those who did not and what the complications of the IVAD were. DESIGN AND METHODS: All 70 patients with severe hemophilia born between January 1987 and October 2000 treated at our center before they were 6 years old were studied. RESULTS: An IVAD was placed in 23 children (33%). Children with an IVAD started prophylactic treatment at a mean age of 2 years (SD 1.3), those without at a mean age of 3.6 years (SD 1.6) (p< 0.001). Home treatment was feasible at a mean age of 2.8 years (SD 1.3) in children with an IVAD and at 4.5 years in those without an IVAD (SD 1.8) (p = 0.001). Infection was the most frequent complication; the mean number of infections per IVAD was 0.61. Thrombosis was more common than initially thought (15%). The infection rate in children with inhibitory antibodies was 3.1 per 1000 patient-days; in children without an inhibitor it was 0.72 per 1000 patient-days. INTERPRETATION AND CONCLUSIONS: In 33% of the children in our cohort an IVAD was needed in order to start early prophylaxis. IVAD are needed more frequently when prophylaxis is started at an early age, but have the advantage that home treatment is feasible earlier. Infection is the most common complication, particularly in children with inhibitors.