Journal Article Annotations
2022, 4th Quarter

Sickle Cell

Annotations by Liz Prince, DO
January, 2023

Discrimination and Pain Sequentially Mediate the Association Between Racism-Based Discrimination in Healthcare Settings and Clinical Pain Among Adults With Sickle Cell Disease.
A case-control and seven-year longitudinal neurocognitive study of adults with sickle cell disease in Ghana.

- High incidence of suicidal ideation in a series of patients with sickle cell disease after hematopoietic stem cell transplantation.
- New York Times Obituary: Lloyd Newman, Teenage Chronicler of ‘Ghetto Life,’ Dies at 43.

PUBLICATION #1 — Sickle Cell

Discrimination and Pain Sequentially Mediate the Association Between Racism-Based Discrimination in Healthcare Settings and Clinical Pain Among Adults With Sickle Cell Disease.

Lakeya S McGill, Katrina R Hamilton, Janelle E Letzen, Patrick H Finan, Sophie M Lanzkron, Michael T Smith, Jennifer A Haythornthwaite, Claudia M Campbell

Abstract: J Pain. 2022 Nov 20;S1526-5900(22)00452-7. doi: 10.1016/j.jpain.2022.11.004. Online ahead of print.

Racism-based discrimination in healthcare settings has been associated with clinical pain in adults living with sickle cell disease (SCD); however, no studies have examined depressive and insomnia symptoms as mechanisms that may drive this relationship. This secondary data analysis examined associations between depressive and insomnia symptoms, racism-based discrimination, and clinical pain. Seventy-one adults with SCD (70% female, M_age = 38.79) provided baseline reports of racism-based discrimination, depressive symptoms, insomnia symptoms, and pain (severity, interference, catastrophizing), and they completed daily diaries of pain severity and interference over three months. In a sequential mediation model, baseline depressive (1^st) and insomnia symptoms (2^nd) significantly mediated the association between racism-based discrimination and baseline pain interference, average daily diary pain severity, and average daily diary pain interference. Although the mediation model with baseline pain severity as the outcome was significant, the total and direct effects were not. Results indicate that discrimination in healthcare settings contributes to depression, which may act on pain through sleep disturbance. Findings support the need for systemic and structural changes to eliminate discrimination in healthcare settings and behavioral mood and sleep interventions to reduce the impact of discrimination on clinical pain.

Annotation

The finding:
In a sample of adults with sickle cell disease (SCD), racism-based discrimination contributed to depressive symptoms (as measured by the Center for Epidemiologic Studies Depression Scale (CES-D)). Depressive symptoms may act on pain through sleep disturbance (as measured by the insomnia severity index (ISI)).

Strength and weaknesses:
The separate analysis of pain diary ratings at baseline and 3 months allowed for consideration of different relationships between mediating factors (e.g. discrimination, depression, insomnia) depending on the pain assessment method. The study focused on racism-based discrimination, but adults with SCD also face disease-based discrimination, which has also been associated with pain in prior studies. Participants in this study from a single academic hospital reported low levels of racism-based discrimination in healthcare settings which may not be generalizable as many patients do not receive treatment in institutions with comprehensive sickle cell centers.

Relevance:
Interventions targeting mood and sleep may be important for the treatment of SCD-related painexperiences. Discrimination in healthcare settings negatively impacts this patient population and interferes with adequate and appropriate treatment. The C-L psychiatrist can advocate for systems changes to minimize the discrimination experience for patients, as well as ensuring depression and insomnia are adequately assessed and managed in this patient population.

PUBLICATION #2 — Sickle Cell

A case-control and seven-year longitudinal neurocognitive study of adults with sickle cell disease in Ghana.

Mary A Ampomah, Jermon A Drake, Adote Anum, Benjamin Amponsah , Yvonne Dei-Adomakoh, Kofi Anie, Christopher C Mate-Kole, Charles R Jonassaint, Fenella J Kirkham.

Abstract: Br J Haematol. 2022 Nov;199(3):411-426. doi: 10.1111/bjh.18386. Epub 2022 Aug 26.

Ageing in sickle cell disease (SCD) is associated with a myriad of end-organ complications, including cerebrovascular damage and cognitive impairment (CI). Although CI is very common in SCD, little is known about cognitive functioning and how it changes with age. This study examines cognitive patterns of 63 adults with SCD and 60 non-SCD, age- and education-matched controls in Ghana. Of those adults with SCD, 34 completed the neuropsychological battery at baseline and again seven years later. In cross-sectional data, adults with SCD performed worse than controls in all cognitive test domains (p < 0.01 for all). The seven-year follow-up data showed that the group exhibited a significant decline in visuospatial abilities (ranging from Cohen’s d = 1.40 to 2.38), and to a lesser extent, in processing speed and executive functioning. Exploratory analyses showed a significant time-by-education interaction, indicating that education may be protective from decline in cognitive performance. These findings have implications for clinical practice. Early neuropsychological surveillance coupled with early assessment and remedial programmes will provide avenues for enhancing the quality of life of adults living with SCD in Ghana.

Annotation

The finding:
Baseline cognitive test scores were lower for adults with sickle cell disease (SCD) than the comparison group. At seven year follow up, adults with SCD demonstrated significant decline in cognitive testing scores. However, patients with higher education had limited or no significant decrease in performance over seven-year period.

Strength and weaknesses:
The non-SCD comparison group was not followed longitudinally, so there are only baseline comparisons. The seven-year longitudinal follow up is a strength, as existing literature on adults with SCD is typically shorter. However, the long follow up period led to 42% attrition, with almost 15% of the baseline SCD cohort dying during the study period.

Relevance:
SCD is associated with progressive cognitive decline. Unrecognized neurocognitive dysfunction may lead providers to have unrealistic expectations as patients receiving complicated medical instructions may lack the executive function to follow providers’ instructions.

PUBLICATION #3 — Sickle Cell

High incidence of suicidal ideation in a series of patients with sickle cell disease after hematopoietic stem cell transplantation.

Adrienne D Mishkin, Stephanie G Cheung, Alison Hoffman, Elizabeth J Leimbach, Simon Dosovitz, Markus Mapara.

Abstract: Blood Adv. 2022 Oct 11;6(19):5542-5545. doi: 10.1182/bloodadvances.2021006752.

Annotation

In the one-year post-hematopoietic stem cell transplantation (HCT) 43% (n=6) of young adult patients with SCD experienced suicidal ideation. All 6 had a history of major depressive disorder (MDD) and/or suicidal ideation (SI)prior to HCT. This was a small study, with only 14 patients included.

Nevertheless, it is a good reminder that C-L psychiatrists should be aware of the strong association of depression with sickle cell disease, especially in patients undergoing curative therapies. These treatments are life-altering, and the process is stressful for an already vulnerable population. Appropriate psychiatric screening, monitoring, and support is needed for young adult patients with SCD undergoing HCT.

PUBLICATION #4 — Sickle Cell

New York Times Obituary: Lloyd Newman, Teenage Chronicler of ‘Ghetto Life,’ Dies at 43.

Roberts.

Abstract: Front Oncol. 2022 Sep 16;12:956923. doi: 10.3389/fonc.2022.956923. eCollection 2022.

Annotation (unstructured):

This obituary describes the life and accomplishments of a man with sickle cell disease. Mr. Newman’s life story also highlights challenges associated with this illness: the loss of siblings to the genetic disease, likely cognitive decline, and his tragically early death at the age of 43. As discussed by Ampomah et al, declining brain health and cognitive impairment are major complications of sickle cell disease. McGill at all discuss how discrimination, particularly in health care settings, negatively impact patients. Despite the development of disease modifying therapies, life expectancy for patients with SCD remains reduced by an estimated 20 years.