End organ damage in sickle cell disease, a tertiary care hospital experience in Pakistan

Background

Sickle cell disease (SCD) is characterized by progressive end-organ damage (EOD) with significant morbidity and mortality. Certain regions of Pakistan have reported a high prevalence of SCD. Despite this, no organized data for clinical information on SCD is available. The study aimed to evaluate EOD and sickle-related complications in a tertiary care hospital.

Methods

The secondary aim was to associate these complications with various HbS genotypes. This was a descriptive study from a single institute in Southern Pakistan. All patients admitted to the hospital till 2022 were evaluated for demographics, clinical, and laboratory details. EOD and mortality were determined for various HbS genotypes.

Results

Forty-one unique patients (25 males and 16 females) were evaluated. The mean (±SD) age was 27(±15.4) years at their last follow-up. Hb S/beta thalassemia (n = 18 or 43.9 %) and homozygous Hb SS (n = 14 or 34.1 %) were identified as the most common sickle cell disorders. The management of anemia (n = 69) and pain crisis (n = 98) were the two most frequent events for hospital admissions. Overall, 33 of 41 patients (80.5 %) had 64 end-organ complications, including acute chest syndrome (n = 19), sickle cell nephropathy (n = 19), acute vascular necroses (n = 15), gallstones (n = 10), and overt stroke (n = 1). Two of 41 patients (4.8 %) died because of multiorgan failure.

Conclusions

Anemia, pain crisis, pulmonary complications, and bone necrosis were the main complications in patients with SCD requiring hospital admissions. A national registry and neonatal screening for SCD are needed to elucidate the disease's burden, enabling health policymakers to plan proper preventive and therapeutic strategies.