Hematology
Sickle Cell Disease
Improving the Daily Lives and Functional Outcomes of Patients With Sickle Cell Disease
Overview
Patients with sickle cell disease (SCD) may experience debilitating complications such as silent cerebral infarcts (SCIs), which can impair cognitive functioning and compromise quality of life (QoL). Optimizing functional outcomes involves working in partnership with patients with SCD to overcome barriers and to prevent complications that arise from the disease.
Expert Commentary
Michael Rutledge DeBaun, MD, MPH
|
|
“Acknowledging that some individuals with SCD, particularly those with SCI, may not be able to process appropriate complex directions and instructions can help health care providers and family members understand the patient’s needs.”
Patients with SCD are at risk for a number of disease complications that can directly affect their QoL and their ability to function optimally. By the time individuals with SCD reach 18 years of age, nearly 40% will have SCIs and associated cognitive impairment, which can lead to challenges with working memory and executive function. Even in the absence of SCI, patients with SCD are at risk of cognitive impairment that may worsen with age. Educational attainment is crucial and cognitive impairment in the pediatric population can be very detrimental, preventing children with SCD from attaining their highest academic achievement and potentially having a negative impact on their future. Adults with cognitive impairment may be unable to engage in activities that are necessary for them to achieve their goals; for example, they have a higher rate of unemployment. This places them at risk for mental health morbidities such as depression, which can also adversely impact their QoL.
Given the high prevalence of SCIs in adults (approximately 50%), recognition of cognitive impairment across the life span is extremely important. Patients and health care providers must develop a partnership to reach common health wellness objectives. Understanding the patient’s needs and including them in the discussion about disease management are crucial talking points.
Even mild cognitive impairments can have far-reaching ramifications in decision making about therapeutic options. Consider an adult with SCD and impaired executive function whose health care provider has given them a complex algorithm for SCD pain management, along with prescriptions for numerous daily and “as-needed” medications. The patient might be labeled as nonadherent; however, the presence of executive impairment may impede the patient’s ability to follow instructions and adhere to their treatment regimen. Acknowledging that some individuals with SCD, particularly those with SCI, may not be able to process appropriate complex directions and instructions can help health care providers and family members understand the patient’s needs. Being aware of the high prevalence of cognitive impairment in children and adults with SCD is critical to provide the optimal medical care and support system for the patient and their family.
References
Bernaudin F, Verlhac S, Arnaud C, et al. Chronic and acute anemia and extracranial internal carotid stenosis are risk factors for silent cerebral infarcts in sickle cell anemia. Blood. 2015;125(10):1653-1661.
Farrell AT, Panepinto J, Carroll CP, et al. End points for sickle cell disease clinical trials: patient-reported outcomes, pain, and the brain. Blood Adv. 2019;3(23):3982-4001.
Hasan SP, Hashmi S, Alhassen M, Lawson W, Castro O. Depression in sickle cell disease. J Natl Med Assoc. 2003;95(7):533-537.
Kassim AA, Pruthi S, Day M, et al. Silent cerebral infarcts and cerebral aneurysms are prevalent in adults with sickle cell anemia. Blood. 2016;127(16):2038-2040.
Osunkwo I, Andemariam B, Inusa BPD, et al. Impact of sickle cell disease symptoms on patients' daily lives: interim results from the International Sickle Cell World Assessment Survey (SWAY). Blood. 2019;134(suppl 1):2297.
Prussien KV, Jordan LC, DeBaun MR, Compas BE. Cognitive function in sickle cell disease across domains, cerebral infarct status, and the lifespan: a meta-analysis. J Pediatr Psychol. 2019;44(8):948-958.
Sanger M, Jordan L, Pruthi S, et al. Cognitive deficits are associated with unemployment in adults with sickle cell anemia. J Clin Exp Neuropsychol. 2016;38(6):661-671.
