Optimizing the management of chronic pain in sickle cell disease

Osunkwo, Ifeyinwa; O'Connor, Hazel F; Saah, Elna

doi:10.1182/hematology.2020000143

Cited by 21 publications

(23 citation statements)

References 57 publications

(58 reference statements)

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“…Relatively little research has been done evaluating NP in the setting of VOC, but our study supports the finding by Sigalla et al., which establishes an NP component to VOC in pediatric SCD patients 21 . Not only are patients in this group more likely to have NP during acute pain events, they are also more likely to be treated with opioids, which can trigger mechanisms of opioid hyperalgesia, opioid tolerance, and central sensitization 23–25 . These mechanistic sequelae can contribute to further enhancement of NP pathophysiology 1,11,21,24,26,27 .…”

Section: Discussionsupporting

confidence: 87%

“…21 Not only are patients in this group more likely to have NP during acute pain events, they are also more likely to be treated with opioids, which can trigger mechanisms of opioid hyperalgesia, opioid tolerance, and central sensitization. [23][24][25] These mechanistic sequelae can contribute to further enhancement of NP pathophysiology. 1,11,21,24,26,27 When NP was evaluated by qualitative sensory testing in 24 children and adolescents at baseline and again during admission for pain, increased cold and mechanical pain sensitivity were found during pain crisis.…”

Section: Discussionmentioning

confidence: 99%

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Prevalence of neuropathic pain in adolescents with sickle cell disease: A single‐center experience

Cregan

Puri

Kang

et al. 2022

Pediatric Blood & Cancer

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Background Neuropathic pain (NP) has been previously explored in adolescents with sickle cell disease (SCD). This study aims to describe the prevalence of NP in adolescents with SCD at a single institution and to explore associated risk factors. Procedure We used the painDETECT questionnaire, one of the few pain phenotyping questionnaires validated for adolescents. We also evaluated the relationships between painDETECT scores and frequency of acute care visits and admissions for pain in the previous 12 months, and age, respectively. Patients 12–18 years old were surveyed from June to July 2019. A retrospective approach was used to answer the remaining research questions. Results Eighty‐one and seven surveys were completed in the outpatient and inpatient settings, respectively. PainDETECT scores suggestive of NP were more prevalent in inpatient surveys than in outpatient surveys. The difference between the mean painDETECT scores of each group was significant when using a general linear mixed model. Most inpatients surveyed had ≥3 pain events in the previous 12 months. Further, older age and increased number of pain events in the previous 12 months were independently associated with higher painDETECT scores. Conclusions Overall, in our opinion, NP is not being evaluated for and treated sufficiently in pediatric SCD, especially in the setting of inpatient acute vaso‐occlusive crisis. Age and number of acute pain events/admissions in the previous 12 months can be used to identify patients likely to be at risk for NP. It is important to continue to identify NP and develop NP‐targeting treatment plans.

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Section: Discussionsupporting

confidence: 87%

Section: Discussionmentioning

confidence: 99%

Prevalence of neuropathic pain in adolescents with sickle cell disease: A single‐center experience

Cregan

Puri

Kang

et al. 2022

Pediatric Blood & Cancer

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“…Although vaso-occlusive episodes (VOE) were included as an acute event in the final list of disease attributes, it is only one dimension of pain [ 52 ]. Chronic pain, which includes neuropathic pain and pain of other etiologies, can vary in intensity and frequency over time but is a near universal feature of SCD that significantly impacts health-related quality of life (HrQoL), medical and non-medical resource use [ 52 , 53 ]. To reflect this, we will model chronic pain separately from other chronic disorders.…”

Section: Resultsmentioning

confidence: 99%

Development of a conceptual model for evaluating new non-curative and curative therapies for sickle cell disease

et al. 2022

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Background Sickle cell disease (SCD) is a clinically heterogeneous disease with many acute and chronic complications driven by ongoing vaso-occlusion and hemolysis. It causes a disproportionate burden on Black and Hispanic communities. Our objective was to follow the SMDM/ISPOR Task Force recommendations for good practices and create a conceptual model of the progression of SCD under current clinical practice to inform cost-effectiveness analyses (CEA) of promising curative therapies in the pipeline over a lifetime horizon. Methods We used consultations with experts, providers, and patients to identify acute events and chronic conditions in the conceptual model. We compared our model structure to previous CEA models of interventions for SCD, assessed the prevalence of the identified disease attributes in Medicaid and Medicare claims databases, and identified relevant outcomes following the 2nd Panel in CEA. We determined an appropriate modeling technique and relevant data sources for parameterizing the model. Results The conceptual model structure included four dimensions of disease: chronic pain, acute events, chronic conditions, and treatment complications, spanning 26 disease attributes with significant impacts on health-related quality of life and resource. We modeled chronic pain separately to reflect its importance to patients and interaction with all other disease attributes. We identified additional data sources for health state utilities and non-medical costs and benefits of SCD. We will use a microsimulation model with age- and sex-specific transitions between health states predicted by patient demographic characteristics and disease history. Conclusion Developing the model structure through an explicit process of model conceptualization can increase the transparency and accuracy of results. We will populate the conceptual model with the data sources described and evaluate the cost-effectiveness of curative therapies.

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“…Pain is the hallmark of SCD, and it accounts for most of the emergency room visits in patients living with the disease [ 21 ]. Those living with SCD describe their pain as unimaginable, agonizing, and sometimes impossible to describe [ 22 ]. Pain in SCD, acute or chronic, reduces the quality of life in several ways and is not limited to altered mood, irritability, depression, anxiety, and sleep disturbance [ 23 ].…”

Section: Discussionmentioning

confidence: 99%

Health-Related Quality of Life Among Patients With Sickle Cell Disease in an Adult Hematology Clinic in a Tertiary Hospital in Lagos, Nigeria

Amaeshi¹,

Kalejaiye²,

Ogamba³

et al. 2022

Cureus

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BackgroundSickle cell disease (SCD) is a genetic disease of public health concern. Improved quality healthcare has increased the life expectancy of these patients; however, they also face an increased frequency of vasoocclusive crises and other SCD complications. These complications affect their quality of life, an area of care, which healthcare providers often overlook. We sought to determine the health-related quality of life among patients living with sickle cell disease in Lagos, Nigeria. Materials and methodsWe conducted a cross-sectional study of 198 patients with sickle cell disease who attended the adult sickle cell clinic at a tertiary hospital in Lagos, Nigeria, during the period from October 1, 2018, to February 28, 2019. A self-administered questionnaire was used to obtain the clinical and socio-demographic characteristics of the patients and the 35-item Short-Form Health Survey (SF-36) questionnaire was used to determine their health-related quality of life (HRQoL). Determinants of HRQoL were established using bivariate and multivariate regression analysis. ResultsThe mean age of the 198 patients who participated in the study was 28.4±9.1 years, mean steady-state hemoglobin was 8.2 ± 1.3 g/dl, and 85 (42.9%) patients had a monthly income of 150 USD or less. In the previous year, 65 (32.1 %) and 33 (16.6%) patients, respectively, suffered one to two episodes (s) of acute bone pain crises and acute chest syndrome, and 43 (24.7%) had blood transfusion. Using the scoring system for SF-36 provided by RAND Health, role limitation due to physical health had the lowest median score of 50 (interquartile range {IQR}: 0-100). On bivariate analysis, bone pain crisis was associated with statistically significant low scores across all the 8 HRQoL domains of the SF36 questionnaire. Other variables, including having received blood transfusion, recent hospitalization, acute chest syndrome, lower level of income, and younger age, were also associated with significantly low scores. On regression analysis, bone pain crisis, level of income, and acute chest syndrome were found to be independent determinants of quality of life in the patients. ConclusionSickle cell disease has a negative impact on the health-related quality of life of those affected. The presence of bone pain crisis is an important predictor of health-related quality of life in sickle cell disease patients. To improve patient outcomes, healthcare providers should take a holistic approach in evaluating and managing this disease, taking into cognizance how the complications and the financial burden of this disease impact the quality of life of affected patients.

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Optimizing the management of chronic pain in sickle cell disease

Cited by 21 publications

References 57 publications

Prevalence of neuropathic pain in adolescents with sickle cell disease: A single‐center experience

Prevalence of neuropathic pain in adolescents with sickle cell disease: A single‐center experience

Development of a conceptual model for evaluating new non-curative and curative therapies for sickle cell disease

Health-Related Quality of Life Among Patients With Sickle Cell Disease in an Adult Hematology Clinic in a Tertiary Hospital in Lagos, Nigeria

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