Sickle cell disease (SCD) is one of the most common inherited blood disorders in Saudi Arabia and its severity is widely variable even among patients sharing the same HBB haplotype. And since there are no data in Saudi Arabia about the life span of SCD patients or pattern of complications in elderly patients. we have studied the severity of SCD among patients older than 50 years of age. Patients with SCD (HbSS or HbSB0) who are followed in King Khalid University hospital in Riyadh and older than 50 years of age were enrolled. Each patient underwent a detailed medical history and physical examination in addition to reviewing medical charts to obtain specific information. Laboratory workups performed during steady state, and data analysis cut off was March 30, 2014. We identified five patients only from our SCD cohort (n=261). HbF level is higher among older patients compared to younger patients (P=0.001). It is clear that the number of patients in our study decline pass the age of 35 years, which could be an indication of higher rate of mortality among Saudi SCD patients as they approached their 40s. There was no difference in the clinical complications pattern in older patients compared to younger patients, however, HbF level was clearly higher among older patients. We identified only few patients ≥ 50 years (n=5), which might be an indirect indicator of the age of death among Saudi SCD patients as none of our patients is older than 60 years of age. Number of elderly patients with SCD per single institution is very small to draw any specific conclusion. Larger multi-institutional study is needed to determine the mortality pattern in SCD and define ameliorating factors that are associated with prolonged survival.
 

Download Full Text - PDF