Good Care and Long-Term Follow-up in Young Hemophiliacs

Introduction: Hemophilia is a hereditary bleeding disorder that presents with primarily hemarthrosis and hematoma. The most common and frequent complication, especially in severe hemophilia, where factor VIII or IX is not administered prophylactically, is the hemophilic arthropathy that develops as a result of recurrent bleeding. In this study, the relationship between arthropathy development and timing of the prohylaxis and orthopedic interventions in hemophiliacs was presented.

Materials and Methods: The data of 99 haemophilia (HA) and hemophilia B (HB) patients under the age of 25 years at our center were reviewed retrospectively. Age, type of disease, factor level, follow-up duration, prophylaxis dose and frequency, annual bleeding rate (ABR), target joints, arthropathic joints, applied radioactive synovectomy and orthopedic interventions were recorded.

Results: Seventy-six HA and 23 HB were included. The median age was 14 (range 1-25) years old. Ten of them (10.1%) were mild, 15 (15.2%) were moderate and 74 (74.7%) were severe. Severe hemophiliacs were most of the patients in both types. Eighty-nine (89.9%) patients including 17 of mild-to-moderate type with target joint and / or surgical intervention were under prophylaxis and 10 (10.1%) were receiving on-demand therapy. The annual bleeding rate was 1.38. Twelve (12%) patients (8 HA, 4 HB) with degenerative hemophilic arthropathy had a mean age of 22 years old and a mean age of application was 12.3 years, a mean annual bleeding rate was 1.54 and a mean follow up and prophylaxis time was 9.5 years. Six patients (4 HA, 2 HB) with median age 21.8 years (range: 18-25) had 9 major orthopedic surgeries (arthrodesis (2), radial head resection (2), TEP, contracture releasing, acyloplasty, deformity correction, arthroscopic sinovectomy) and 22 patients with median age of 17.8 years had 29 minor orthopedic interventions (radiosynovectomy (28), closed reduction to femur fracture). Orthopedic interventions were performed at an advanced age, often who met with prophylaxis too late.

Discussion: In our study, it has been shown that who had prophylaxis at an early age and prophylaxis for a longer period had less arthropathies which is the most important complication and additionally less arthroplasties. The fact that, the prevalence of permanent musclosceletal deformity was 59% before year 2000 (historical data) which reduced to 12% nowadays shows the sensitivity of hemophilia care in our center. Early and regular prophylaxis remains the most current treatment modality for the prevention of hemophilia complications.