Sickle cell disease in the older adult

Abstract

Sickle cell disease (SCD) is an inherited haemoglobin disorder, associated with recurrent painful episodes, ongoing haemolytic anaemia and progressive multi-organ damage. Until the early 1990s, survival beyond the fourth decade for a patient with SCD was considered unusual and prompted case reports. Nowadays, in countries with developed health care systems, more than 90 percent of newborns with SCD survive into adulthood. Nevertheless, their life expectancy is still shortened by more than two decades compared to the general population. With an increasing life expectancy, SCD has now evolved into a debilitating disorder with substantial morbidity resulting from ongoing sickle cell vasculopathy and multi-organ damage. Limited data on health care issues of older adults with SCD poses multiple challenges to patients, their families and health care providers. In this review, we will address and discuss acute and chronic complications of SCD with a special focus on the older adult.

Keywords: Sickle cell disease; multi-organ damage; older adults; vasculopathy.

Fig. 1

Life expectancy of SCD. Improvement in survival of patients with SCD. Reproduced from Pleasants (Nature 2014) with permission.

Fig. 2

Important clinical milestones in SCD.

Fig. 3

Pathophysiology and complications of SCD.