TITLE:
Outpatient Management of Sickle Cell Disease: Assessment of 13 Years Follow-Up of Pediatric Patients at the Sylvanus Olympio University Hospital
AUTHORS:
Koffi Mawuse Guedenon, Djatougbe Ayaovi Elie Akolly, Essohana Padaro, Ounoo Elom Takassi, Etsè Akpako, Balakibawi Esso, Kokou Dzata, Adama Dodji Gbadoe
KEYWORDS:
Sickle Cell Disease, Outpatient Follow-Up, Lomé
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.12 No.2,
May
20,
2022
ABSTRACT: Objective: To evaluate thirteen years of ambulatory follow-up of patients with major
forms of sickle cell disease. Methods: This was a study of 1055 records of sickle cell patients aged 6 months to 45
years followed between 2006 and 2018. Results: Six hundred and eighty-one (64.5%) homozygous SS, 283 (26.8%) SC, 86
(8.2%) SF, 4 (0.4%) S? thalassemia and 1 (0.1%) SD sickle cell patients were
followed. The majority of the patients (97.9%) were living in the capital city of Lomé and its surrounding suburbs. Most children
(67.3%) were less than 5-year-old when they were diagnosed with sickle cell disease, and
only 21.3% of cases were discovered before the first year birthday of the
patients. Osteo-articular vaso occlusive crisis (VOC) was the principal symptom
at the onset of the diagnosis, approximately 28.9% of the cases, followed by
severe anemia (19%), then hand-foot syndrome in 15.6%. The systematic screening was
performed in 5.4%. Twenty-six percent were G6PD deficient, 4 were HIV
immunocompromised, and 29.7% were transfused. Among the degenerative complications, 2.4% had
retinopathy, 1.9% osteonecrosis of the femoral head, 1.4% leg ulcers, and 0.8% cardiomyopathy.
Salmonella typhi was the main cause of infection, 51.4% of the cases. The mean
baseline hemoglobin level (n = 27) was 7.67 ± 1.64 g/dl. Four (0.4%) patients had a splenectomy. The mortality
rate was 2.4%. The primary cause of death was anemia (28%), followed by sepsis in 20% of the cases. One case
of suicide was documented as the cause of death. Conclusion: Outpatient medical
follow-up of sickle cell patients is essential in sub-Saharan
African countries where neonatal screening does not exist, and diagnosis is
often late. The improvement in quality of life observed should be studied.