In the introduction to this installment of the “How I treat…” series in the Blood Journal, the author highlights that for patients with hemoglobinopathies, survival has significantly improved. As such, more patients face new long-term risks of their disease as well as age-related conditions.
For adults over 40 with sickle cell disease (SCD), common age-related non-sickle conditions are often associated with SCD, leading to delays in diagnosis and appropriate intervention. In their article, Drs. Swee Lay Thein and Jo Howard recommend regular comprehensive or annual reviews and provide strategies including assessment of organ function, pain history and treatment, as well as other comorbidities that could benefit from additional intervention.
Comprehensive reviews provide an opportunity for the physician and patient to discuss disease-modifying treatment options, including the use of hydroxyurea, blood transfusions, and iron chelation therapy to manage SCD.
The authors suggest a proactive approach in offering curative therapy options for eligible patients, including hematopoietic cell transplantation and clinical trials for gene therapy. They recommend offering these therapy options to patients at younger ages, prior to patients experiencing complications that could make them ineligible.
The other articles in the Blood series include:
- How
I Manage Medical Complications of Beta-thalassemia in Adults
- A discussion on organ damage and extramedullary hematopoiesis, which both affect the survival and quality of life in adults with thalassemia syndromes who are transfusion-dependent and non-dependent.
- How
we Diagnose and Treat Venous Thromboembolism in Sickle Cell Disease
- A look at venous thromboembolism in SCD and how the natural increase in thrombosis as a person ages interacts with SCD and predisposition to clotting.
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How
I Manage Hypoxia in Adults with Hemoglobinopathies and Hemolytic Disorders
- A discussion of the pulmonary consequence of hemoglobinopathy and chronic hemolysis.
Related Resources
Sickle Cell Disease Resource Center – on-demand education activities, clinical trials for SCD and more
Sickle Cell Disease Clinical Decision Aid