Journal Article Annotations
2024, 1st Quarter

Sickle Cell

Annotations by Lauren Fields, MD, Elizabeth Prince, DO
April, 2024

Psychiatry referral and appointment attendance in a clinic for young adults with sickle cell disease.
Biopsychosocial Factors Associated With Pain and Pain-Related Outcomes in Adults and Children With Sickle Cell Disease: A Multivariable Analysis of the GRNDaD Multicenter Registry.

PUBLICATION #1 — Sickle Cell

Psychiatry referral and appointment attendance in a clinic for young adults with sickle cell disease.

Elizabeth J Prince, C Patrick Carroll, Lydia H Pecker.

Abstract: Pediatr Blood Cancer. 2024 Apr;71(4):e30860. doi: 10.1002/pbc.30860. Epub 2024 Jan 10.

Mental illness is a common sickle cell disease (SCD) comorbidity. This observational study evaluated psychiatry appointment attendance among 137 young adults with SCD. In their first year of adult SCD care, 43% of subjects were referred to psychiatry. Referral was associated with chronic transfusion therapy. Twenty-four percent of subjects attended a psychiatry appointment; attendance was associated with the appointment being scheduled within 6 weeks of referral and no subject characteristics. Ninety-one percent of subjects attending psychiatry appointments had a psychiatric disorder. Among young adults with SCD, psychiatric morbidity is high. Psychiatric services are, therefore, essential for this patient population.

Annotation

The finding:
This is an observational study of 137 young adults with sickle cell disease (SCD) (aged 18-30) within an adult sickle cell center. Forty-three percent of individuals were referred to psychiatry within their first year in adult care and a little over half of those patients attended a psychiatry appointment within a year of referral. Patient factors that were associated with referrals or trended toward association, respectively, included chronic transfusion therapy and history of stroke. Most psychiatry referrals occurred during the first hematologist visit and appointment attendance was associated with scheduling within 6 weeks of referral. Almost all patients assessed in a psychiatry visit had a clinically diagnosed psychiatric disorder, mostly depression.

Strength and weaknesses:
This study contributes to our understanding of potential facilitators of and barriers to engagement in mental health care for young adult patients with sickle cell disease, and adds to the limited literature regarding prevalence of psychiatric diagnoses in this population. This study, however, is a single-site study of a center with embedded psychiatrists, which is not common. As such, this study may not be representative of most adult sickle cell disease treatment centers. In addition, prevalence of psychiatric diagnoses was possibly underestimated, as not all individuals included in the sample were evaluated

Relevance:
Psychiatric comorbidities are common among patients with SCD. Particularly for patients at a point of transition between pediatric and adult care settings, ensuring access to mental health care is crucial. Better understanding of barriers to mental health care, such as prolonged time to scheduled appointment, can lead to improved measures to help facilitate referrals and engagement.

PUBLICATION #2 — Sickle Cell

Biopsychosocial Factors Associated With Pain and Pain-Related Outcomes in Adults and Children With Sickle Cell Disease: A Multivariable Analysis of the GRNDaD Multicenter Registry.

Martha O Kenney, Samuel Wilson, Nirmish Shah, Andrey Bortsov, Wally R Smith, Jane Little, Sophie Lanzkron, Julie Kanter, Susan Padrino, Amma Owusu-Ansah, Alice Cohen, Payal Desai, Deepa Manwani, Sana Saif Ur Rehman, Ward Hagar, Francis Keefe.

Abstract: J Pain. 2024 Jan;25(1):153-164. doi: 10.1016/j.jpain.2023.07.029. Epub 2023 Aug 5.

Pain is the primary symptomatic manifestation of sickle cell disease (SCD), an inherited hemoglobinopathy. The characteristics that influence pain experiences and outcomes in SCD are not fully understood. The primary objective of this study was to use multivariable modeling to examine associations of biopsychosocial variables with a disease-specific measure of pain interference known as pain impact. We conducted a secondary analysis of data from the Global Research Network for Data and Discovery national SCD registry. A total of 657 children and adults with SCD were included in the analysis. This sample was 60% female with a median age of 34 (interquartile range 26-42 years) and a chronic pain prevalence of 64%. The model accounted for 58% of the variance in pain impact. Low social (P < .001) and emotional (P < .001) functioning, increasing age (P = .004), low income (P < .001), and high acute painful episodes (P = .007) were most strongly associated with high pain impact in our multivariable model. Additionally, multivariable modeling of pain severity and physical function in 2 comparable samples of registry participants revealed that increasing age and low social functioning were also strongly associated with higher pain severity and low physical functioning. Overall, the results suggest that social and emotional functioning are more strongly associated with pain impact in individuals with SCD than previously studied biological modifiers such as SCD genotype, hemoglobin, and percentage fetal hemoglobin. Future research using longitudinally collected data is needed to confirm these findings.

Annotation

The finding:
This study is a secondary analysis of data from the Global Research Network for Data and Discovery (GRNDaD) national sickle cell registry, including 745 patients who were 8 years old or older and completed at least one pain-related survey of interest. Over 60% of patients who completed the pain impact assessment reported chronic pain. Increased pain severity was associated with pain variability, age, social functioning, and number of pain locations. Biological markers of disease severity such as SCD genotype and percentage of fetal hemoglobin did not demonstrate significant associations with pain impact, pain severity, or physical function.

Strength and weaknesses:
This study includes a larger number of patients from multiple SCD treatment centers via a national data registry with a wide range of biological, psychological, and sociodemographic variables and their associations with pain outcomes. Nearly half of the sample had zero hospitalizations per enrolment year – aligning with prior studies and suggesting a representative sample of patients with SCD. Limitations include that social and emotional functioning were measured with a SCD-specific quality of life measure, rather than a more standard psychological symptom screening tool such as the Patient Health Questionnaire. Additionally, the study design does not allow for assessment of causality in the relationships between variables, and all measures included were based on self-report or proxy report which are vulnerable to recall bias.

Relevance:
This paper moves the literature forward toward a goal of more nuanced understanding of predictors of adverse pain outcomes in individuals with sickle cell disease to inform better clinical care. This study demonstrates the need for continued research beyond biological factors to improve insight into psychosocial contributors to pain outcomes.