Sickle cell disease in the older adult.

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.

Low Oxygen Affinity Variant Haemoglobin in an elderly woman presenting with low oxygen saturation.

An asymptomatic 81-year-old woman was referred by her general practitioner regarding a pulse-oximetry oxygen saturation (SpO2) of 74%. An arterial blood gas analysis (ABG) on air showed PaO2 12.9 kPa, oxygen saturation 80%, with normal pH, PaCO2, methaemoglobin and carboxyhaemoglobin levels. After a normal chest x-ray, tinzaparin was administered empirically for possible occult pulmonary embolus. This diagnosis was subsequently excluded with an unremarkable computed tomography pulmonary angiogram (CTPA). She was further investigated as an out patient. DNA globin-gene analysis identified a variant haemoglobin revealed to be haemoglobin Saint Mande (HbSM). Following reassurance regarding the benign nature of her condition, she has remained well.