Journal of sickle cell disease最新文献

Objectives: Readiness for discharge for a SCD vaso-occlusive crisis is dictated by factors far beyond pain control, including physical function/activity. We therefore designed and tested a functional status-based pain assessment questionnaire in SCD patients hospitalized with vaso-occlusive crises.

Methods: Sickle cell disease patients on a preselected nursing unit rated 10 draft Functional status-Based Pain Assessment items of activities of daily living on a five-point Likert scale (0-5) from "very easy" to "very difficult" daily on each day of their admission until discharge, at approximately the same time. Concurrently, they reported Numeric Rating Scale (0-10) pain intensity. For validation, we used exploratory factor analysis, confirmatory factor analysis, and item response theory analysis.

Results and discussion: We analyzed 503 observations from 175 admissions of 88 patients. Half were female, the mean age was 32.1 ± 11.8 years, and the mean length of stay was 7.1 ± 6.9 days. The mean Numeric Rating Scale (6.8 ± 1.9) was inversely correlated with the mean Functional Status-based Pain Assessment (0-50) score (27 ± 8.0, r = -0.4342, P < .0001). Functional Status-based Pain Assessment item means ranged from 2.1 to 3.3. Cronbach's alpha was 0.91. Exploratory factor analysis showed that all Functional Status-based Pain Assessment items loaded on a single factor. Confirmatory factor analysis found adequate convergent and discriminant validity and showed strong fit of the model to the data. Item response theory analysis showed item discrimination ranging from 0.56 to 4.1, while difficulty ranged from -2.8 to 7.5.

Conclusion: The Functional Status-based Pain Assessment shows strong correlation with daily Numeric Rating Scale, is multidimensional, and demonstrates strong construct validity. It may improve assessment of SCD vaso-occlusive crisis pain and may enhance vaso-occlusive crisis discharge discussions.

High-risk SCD may be managed with chronic red blood cell exchange transfusions. We examined the prevalence of sleep-associated hypoxemia (≥5 minutes at SpO₂ ≤88%) or nocturnal Hb desaturation (NHD) in chronically transfused adults with SCD. Of 41 identified participants, 15 (36.6%) had tested positive for NHD at some point prior to enrollment. The median duration of desaturation (SpO₂ ≤88%) in those that tested positive was 88.3 minutes (IQR 27.9-226.0 minutes). Participants with and without NHD were compared using non-parametric statistical tests. Compared to participants without NHD, those with NHD had higher absolute reticulocyte counts (P = .018) and white blood counts (P = .05) and tended to be older. They had more anemia (P = .11) and higher lactate dehydrogenase levels (P = .072). Older age at initiation of chronic red blood cell transfusions associated the strongest with a longer duration of NHD (ρ = 0.4253, P = .0067), while prior history of cerebrovascular events associated with a shorter duration of NHD (P = .0315). Our results demonstrate that NHD is common in adults being treated with red blood cell exchange for high-risk SCD and associates with laboratory evidence of increased disease activity. Increased awareness of this complication and appropriate screening may provide an additional simple, low-cost, and physiologically relevant treatment intervention, that is oxygen therapy.

Objectives: Religiosity and spirituality often play a role in managing chronic diseases. Sickle cell disease (SCD) is a genetic chronic disease associated with lifelong complications, but there is limited analysis of the potential impact of religiosity and spirituality on SCD. This study aims to analyze the association between constructs of religiosity and spirituality with health outcomes (disease severity, depression, and pain) in an SCD population (n = 275).

Methods: Data from the INSIGHTS Study (NCT02156102) were used for this analysis. The relationships between religious/spirituality measures (religious attendance, self-reported spirituality, religious saliency, coping, and support) and SCD outcomes (disease severity, depression, and pain scores) were analyzed through bivariate correlations and multivariable models. Models adjusted for different dimensions of religiosity and spirituality separately (Model 1) and all measures simultaneously (Model 2).

Results: In the bivariate analyses, we found a positive association between disease severity and spirituality (P < .05) and an inverse relationship between positive religious coping and depression (P = .01). In Model 1, which adjusted for individual measures separately, there was an association between SCD severity and spirituality (β = 0.07, P < .001) as well as with religious attendance (β = 0.02, P < .01). However, in the models that simultaneously adjusted for all the measures (Model 2), religious attendance was no longer associated with SCD severity (β = 0.006, P > .05), while the association with spirituality remained significant (β = 0.05, P < .05).

Conclusions: The observed associations between spirituality and disease severity among patients with SCD suggest that individuals with severe disease may beneficially use spirituality to cope with complications. Future research should rigorously evaluate this hypothesis.