Case Report on Multiple Pathologies in an Adolescent Sickle Cell Disease Patient in Ghana

Abstract

Sickle cell Disease is the most common genetic disorder in children living in sub-Saharan Africa with a disease burden estimated between 2-4% in malaria endemic regions. The pathologies commonly affect multiple organs and present with other co-morbidities that complicate the disease process, resulting in diagnostic and management challenges. This case study presents a scenario of a sickle cell patient with multiple long term complications and Deep Vein Thrombosis (DVT) as a co-morbid condition. He did not benefit from early diagnosis and preventive management therapies. This 12-year-old known SCD patient presented with 2 months history of inability to walk, weight loss and pain in both legs to a tertiary hospital in Ghana. On examination he was wasted (Weight for Height z-score <-3SD) and pale. He had palpable axillary and inguinal lymph nodes. His right leg was swollen, warm, erythematous and tender. He had a gibbus deformity of the lower thoracic spine. Investigations revealed Hemoglobin 7.1g/dl, WBC of 13.51 X 109/dl with Neutrophils (65.3%). ESR-117, INR-1.05, Prothrombin Time-14.3secs and blood sugar-6.5mmol/L. Radiological findings revealed features suggestive of sickle cell disease with Thoracolumbar spondylodiscitis, likely pyogenic. Bilateral Avascular Necrosis of the femoral head, Septic arthritis of the right hip and left shoulder joint, Osteomyelitis of the left 8th and 9th ribs. Doppler Ultrasound of the lower limb revealed Right Popliteal and Posterior Tibial Vein Thrombosis with Pyomyositis. The patient was treated with Enoxaparin, Warfarin and IV antibiotics (Clindamycin and Ciprofloxacin). Follow ups with a repeat Doppler finding revealed a completely resolved thrombus with no evidence of DVT and normal INR and PT. Attention therefore needs to be paid to a multi-disciplinary preparedness and response approach to the management of SCD patients, as presented in this case report, to improve the quality of life, mitigate disability and reduce mortality.