How I treat the older adult with sickle cell disease

Abstract

With increasing survival, cumulative complications of sickle cell disease (SCD), which develop insidiously over time, are becoming more apparent and common in older patients, particularly those in their fifth decade and beyond. The older patient is also more likely to develop other age-related nonsickle conditions that interact and add to the disease morbidity. A common misconception is that any symptom in a SCD patient is attributable to their SCD and this may lead to delays in diagnosis and appropriate intervention. We recommend regular comprehensive reviews and monitoring for early signs of organ damage and a low threshold for the use of hydroxyurea and blood transfusions as preventative measures for end-organ disease. Treatable comorbidities and acute deterioration should be managed aggressively. Although the primary goal in management of the older adult with SCD is improving anemia and minimizing organ damage, the time has come for us to be more proactive in considering curative therapies previously offered to the younger patient. Curative or experimental interventions should be discussed early, before complications render the patients ineligible for these treatments.

Non‐myeloablative human leukocyte antigen‐matched related donor transplantation in sickle cell disease: outcomes from three independent centres

“…Sickle cell disease (SCD) is an inherited red‐cell disorder, characterized by life‐long manifestations of acute and chronic complications, and leading to accumulation of organ injury and early mortality 1 . For many years, hydroxycarbamide was the only disease‐modifying treatment, 2,3 and recently L‐glutamine, 4 voxelotor, 5 and crizanlizumab 6 expanded this short list of sickle‐specific treatments.…”

“A child with sickle cell disease can't live with just anyone.” A mixed methods study of socio‐behavioral influences and severity of sickle cell disease in northern Nigeria

“…Further, there could be age‐related differences in pain perception, psychological response, and health care utilization 40 . Similarly, increased transfusion rates with age could reflect the severity of anemia in older children 41 …”

Aspirin in childhood acute ischemic stroke: The evidence for treatment and efficacy testing

“…Both large‐ and small‐vessel arteriopathy occur. Similar to other arteriopathies, AIS is the more common stroke presentation in children, especially in those with moyamoya, but unlike other populations, ASA is rarely used due to hemorrhagic stroke concerns that increase with age . Strong evidence guides SCA stroke prevention, including early hydroxyurea initiation, screening by transcranial Doppler (TCD) examinations, and chronic red cell transfusions (or possibly hematopoietic stem cell transplantation) for those at high risk for primary or secondary stroke .…”

“…Similar to other arteriopathies, AIS is the more common stroke presentation in children, especially in those with moyamoya, but unlike other populations, ASA is rarely used due to hemorrhagic stroke concerns that increase with age. 48,49 Strong evidence guides SCA stroke prevention, including early hydroxyurea initiation, screening by transcranial Doppler (TCD) examinations, and chronic red cell transfusions (or possibly hematopoietic stem cell transplantation) for those at high risk for primary or secondary stroke. 50,51 One small pediatric SCA series reported a stroke recurrence risk of 0.58 per 100 patient years when receiving ASA in conjunction with chronic transfusion, with one hemorrhagic stroke occurring two years after the initial AIS.…”