INTRODUCTION: Sickle cell disease is a public health problem. The WHO has recommended that global management be implemented to reduce mortality and morbidity. Since no comprehensive care programme for bone and joint complications exists, the Caribbean Sickle Cell Disease Center added orthopaedic consultation to screen for and monitor these complications in 1992. HYPOTHESIS: Comprehensive medical and surgical care of patients with sickle cell disease will reduce the complications and disability associated with this disease. POPULATIONS AND METHODS: Two populations were compared to evaluate the impact of comprehensive disease management on the occurrence of avascular necrosis (AVN) of the femoral head (femoral head AVN). The case-control series, [E-1994], included 115 patients (58 SS and 57 S) without orthopaedic monitoring and was evaluated retrospectively. The other patient series, [E-2008], included 215 patients (94 SS and 121 SC) with systematic orthopaedic care and was followed prospectively. Age, gender, duration of follow-up, haemoglobin levels, genotype, pain before treatment, associated humerus AVN and leg ulcers were analysed. RESULTS: Femoral head AVN occurred in young adult patients (35.3 ± 4 years for [E-1994] and 29 ± 3.4 years for [E-2008]). Only elevated haemoglobin levels were associated with the occurrence of femoral head AVN, which suggests that increased blood viscosity contributes to the condition ([E-1994], P<0.0001; [E-2008], P=0.001). Treatment in [E-2008] patients reduced the number of femoral head AVN cases from 36.5% in [E-1994] to 14.4% in [E-2008] (P<0.0001). DISCUSSION: The prevention and management of femoral head AVN must include medical treatment of the disease to reduce the occurrence of painful vaso-occlusive crises, which are known to trigger femoral head AVN. The effectiveness of this programme hinged on identifying risk factors and using simple approaches (hydration, pain medication, rest and crutches) to manage painful joint crises before femoral head AVN appeared. These approaches could be implemented in disadvantaged countries where sickle cell disease is prevalent. CONCLUSION: By knowing the risk factors, symptomatic patients who are at risk for femoral head AVN can be identified and additional evaluations can be performed early on in cases of hip pain.
INTRODUCTION: Sickle cell disease is a public health problem. The WHO has recommended that global management be implemented to reduce mortality and morbidity. Since no comprehensive care programme for bone and joint complications exists, the Caribbean Sickle Cell Disease Center added orthopaedic consultation to screen for and monitor these complications in 1992. HYPOTHESIS: Comprehensive medical and surgical care of patients with sickle cell disease will reduce the complications and disability associated with this disease. POPULATIONS AND METHODS: Two populations were compared to evaluate the impact of comprehensive disease management on the occurrence of avascular necrosis (AVN) of the femoral head (femoral head AVN). The case-control series, [E-1994], included 115 patients (58 SS and 57 S) without orthopaedic monitoring and was evaluated retrospectively. The other patient series, [E-2008], included 215 patients (94 SS and 121 SC) with systematic orthopaedic care and was followed prospectively. Age, gender, duration of follow-up, haemoglobin levels, genotype, pain before treatment, associated humerus AVN and leg ulcers were analysed. RESULTS:Femoral head AVN occurred in young adult patients (35.3 ± 4 years for [E-1994] and 29 ± 3.4 years for [E-2008]). Only elevated haemoglobin levels were associated with the occurrence of femoral head AVN, which suggests that increased blood viscosity contributes to the condition ([E-1994], P<0.0001; [E-2008], P=0.001). Treatment in [E-2008] patients reduced the number of femoral head AVN cases from 36.5% in [E-1994] to 14.4% in [E-2008] (P<0.0001). DISCUSSION: The prevention and management of femoral head AVN must include medical treatment of the disease to reduce the occurrence of painful vaso-occlusive crises, which are known to trigger femoral head AVN. The effectiveness of this programme hinged on identifying risk factors and using simple approaches (hydration, pain medication, rest and crutches) to manage painful joint crises before femoral head AVN appeared. These approaches could be implemented in disadvantaged countries where sickle cell disease is prevalent. CONCLUSION: By knowing the risk factors, symptomatic patients who are at risk for femoral head AVN can be identified and additional evaluations can be performed early on in cases of hip pain.
Authors: Jin Han; Santosh L Saraf; Xu Zhang; Michel Gowhari; Robert E Molokie; Joharah Hassan; Chaher Alhandalous; Shivi Jain; Jewel Younge; Taimur Abbasi; Roberto F Machado; Victor R Gordeuk Journal: Am J Hematol Date: 2016-08-08 Impact factor: 10.047