Journal Article Annotations
2023, 4th Quarter
Annotations by Lauren Fields, MD and Elizabeth Prince, DO
This qualitative study of 36 focus groups and 70 individual interviews with people with sickle cell disease (SCD) and stakeholders in caregiver roles in three African countries found that SCD-related stigma exists across socio-ecological levels (individual, interpersonal, organizational, community, public policy). Identified contributors to SCD-related stigma included self-blame, discrimination based on medical complications of SCD, and cultural beliefs that associate SCD with supernatural elements such as witchcraft. Primary manifestations of stigma included social discrimination (e.g., employers reluctant to hire individuals with SCD), explicit bias even among healthcare workers, pity, and social isolation (e.g., “othered” by peers in school). Proposed strategies to mitigate stigma fell into four categories: increasing SCD advocacy to improve awareness, stakeholder education to positively influence community perception, counselling for patients on the psychosocial impacts of SCD, and better clinical management of SCD to improve outcomes.
Strength and weaknesses:
This article highlights the lived experience of individuals with SCD in Cameroon, Ghana, and Tanzania and provides a lens through which to appreciate the widespread impact that cultural context has on the perception of medical illnesses. However, demographic information was not consistently obtained for all participants, which makes it difficult to assess demographic impact in this sample.
Patients with sickle cell disease often encounter stigma across socio-ecological contexts on a global scale, not just in more racially heterogenous countries such as the United States. Not only should we be mindful of the ways in which self-perception and cultural dynamics may impact our patients with SCD, we as C-L psychiatrists should also be aware of the stigma and bias within the healthcare settings in which we practice and the potential impact on care delivery.
Studies of psychiatric comorbidities in SCD have largely focused on depression, with limited data suggesting prevalence of 24-30% based primarily on screening tools. Data describing anxiety disorders is comparatively less prominent with wide variation in prevalence of anxiety symptoms, although anxiety was consistently found to be comorbid with depression. Neurocognitive impairment is common in adult patients but understudied compared to pediatric populations. Regarding pain and opioid use, patients with SCD have both acute and chronic pain however high-dose chronic opioid use is uncommon. Additional factors that contribute significantly to quality of life include sleep and adherence to care. Particularly understudied areas include mood disorders other than depression, personality disorders, psychotic disorders, and suicidality.
Strength and weaknesses:
This review provides a comprehensive overview of available literature, as well as concrete tools to inform patient care such as a schema of potential etiologies of non-adherence. However, this was a narrative review rather than a systematic review. More systematic review of the literature was limited by the quality of available research studies, many of which involve screening instruments or patient-reported outcomes rather than diagnostic instruments.
Awareness of potential psychiatric comorbidities in patients with SCD can aid C-L psychiatrists in managing patients with SCD and educating patients and other healthcare providers. The dearth of quality studies in these areas should additionally motivate continued research efforts to better characterize the prevalence of psychiatric conditions in this patient population in order to more accurately inform clinical care.
The US Food and Drug Administration approved the first gene therapies to treat patients with sickle cell disease in December. This commentary from sickle cell disease experts calls for the development of a long term medical home for individuals exposed to curative therapies for sickle cell disease (gene therapy and stem cell transplant). Ongoing follow up is needed to address health risks associated with these therapies, as well as with permanent sickle cell disease complications (including cognitive impairments and psychiatric disorders). CL psychiatrists may encounter patients before, during, or after receiving curative therapies. Available research suggests increased risk of psychiatric symptoms for these patients. Therefore, ensuring access to psychiatric care is important. The piece concludes with a poignant call for care; “Cure without care jeopardises the freedom promised by successful haematopoietic cell therapies and risks exacerbating, rather than alleviating, the sickle cell disease care crisis.”