Sheehan's syndrome and sickle cell disease: the story of Natasha

Sheehan's syndrome (SS) is one of the pituitary disorders caused by severe blood loss during childbirth leading to necrosis of the pituitary gland. Diagnosis is made following severe haemorrhage, failure to produce milk and failure to menstruate. Rare in countries with good obstetric care, SS is still frequent in those countries with poor healthcare services. The majority of papers published on SS do not mention psychological or neuropsychological sequelae. Of those that do, mood disorders are sometimes reported and occasionally cognitive problems are noted, typically attention, memory or executive deficits. We report on Natasha, a British woman of Caribbean ancestry with sickle cell disease (SCD), diagnosed at age 33 years with SS following the birth of her second child. We know of no reports of a person with both SCD and SS. Natasha has been seen regularly for neuropsychological assessment and treatment for over two years. There has been a slight increase in her cognitive functioning but she remains with Balint's syndrome, so is unable to read. Treatment for this has been partially successful. Natasha presented with psychotic type symptoms when first seen but these have improved to a large extent. We believe that these symptoms are consistent with SS but the severe cognitive problems are more likely to be due to the strokes she suffered at the time of the haemorrhage and, possibly, to hypoxic damage caused by very low blood pressure.