It may be time for clinicians to rethink how they manage sickle cell disease (SCD) in patients aged 40 years and older. Traditionally, the primary approach to managing older adults with SCD has focused on improving anemia and minimizing organ damage. However, researchers now suggest being more proactive. In an article published in Blood, researchers urged clinicians to discuss curative therapies, which are typically only offered to younger patients, with older patients before complications render these patients ineligible.1
The authors contend that older patients with SCD have many unmet needs. Many symptoms in older patients with SCD may be mistakenly attributed to their SCD, possibly contributing to delays in appropriate diagnoses and interventions. In addition, current management is largely based on expert consensus, but evidence-based guidelines are lacking for most complications.
The authors recommend regular comprehensive reviews and monitoring for early signs of organ damage, as well as a low threshold for the use of hydroxyurea and blood transfusions as preventative measures for end organ disease. They also suggest that treatable comorbidities and acute deterioration should be managed aggressively.
Based on survival data, the authors noted that there is room for improvement. Life expectancy of patients with SCD is still approximately 20 to 30 years shorter than the general population, as SCD-related complications are associated with multiple chronic organ comorbidities and nearly all organ systems can be affected. Current data suggest the main causes of death in adults with SCD include infection, acute chest syndrome, stroke, renal failure, and pulmonary hypertension.
The Roles of Regular Comprehensive Review and Disease-Modifying Therapy
The researchers presented 4 clinical vignettes that highlight some of the issues faced by older patients with SCD as well as the therapies available for managing this patient population.
The first and second clinical vignettes described patients with HbSS. The patient in the first vignette demonstrated pronounced impairment of renal functioning. The patient in the second vignette reported experiencing severe pain and was ultimately found to suffer from sleep apnea. In both vignettes, the patient’s symptoms were identified through regular comprehensive review. The study authors emphasized the importance of conducting a thorough annual review that includes a multisystem clinical evaluation, routine blood and urine testing, identification of any SCD-related complications, and assessment of the frequency and severity of acute complications.
The third and fourth clinical vignettes highlighted the role of disease-modifying therapy in older adult patients. The patient in the third vignette had a fully matched HbAA sibling and underwent non-myeloablative sibling donor hematopoietic stem cell transplantation (HSCT) at age 48. The patient in the fourth vignette was 40 years old and had numerous complications including recurrent lower extremity ulcers, osteomyelitis, multiple episodes of acute chest syndrome, and multiorgan system failure. Both patients briefly underwent hydroxyurea therapy, but the therapy was discontinued due to a lack of clinical response.