Chronic Pain Does Not Impact Baseline Circulating Cytokine Levels in Adults with Sickle Cell Disease

Background: The management of acute and chronic pain for individuals living with sickle cell disease (SCD) is a clinical challenge. This reflects the paucity of clinical SCD pain research and limited understanding of the complex biological differences between acute and chronic pain. These issues collectively create barriers to effective, targeted interventions. Optimal pain management requires interdisciplinary care. Objective: These evidence-based guidelines developed by the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in pain management decisions for children and adults with SCD. Methods: ASH formed a multidisciplinary panel, including 2 patient representatives, that was thoroughly vetted to minimize bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic reviews. Clinical questions and outcomes were prioritized according to importance for clinicians and patients. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations, which were subject to public comment. Results: The panel reached consensus on 18 recommendations specific to acute and chronic pain. The recommendations reflect a broad pain management approach, encompassing pharmacological and nonpharmacological interventions and analgesic delivery. Conclusions: Because of low-certainty evidence and closely balanced benefits and harms, most recommendations are conditional. Patient preferences should drive clinical decisions. Policymaking, including that by payers, will require substantial debate and input from stakeholders. Randomized controlled trials and comparative-effectiveness studies are needed for chronic opioid therapy, nonopioid therapies, and nonpharmacological interventions.

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Outcome of Transitioning Pediatric Patients with Sickle Cell Disease to Adult Programs.

Blood ◽

10.1182/blood.v104.11.3743.3743 ◽

2004 ◽

Vol 104 (11) ◽

pp. 3743-3743 ◽

Cited By ~ 3

Author(s):

Samir K. Ballas ◽

Carlton Dampier

Keyword(s):

Chronic Pain ◽

Young Adults ◽

Pain Management ◽

Sickle Cell Disease ◽

Medical Care ◽

Sickle Cell ◽

Adolescents And Young Adults ◽

Cell Disease ◽

Adult Care ◽

Female Ratio

The transition of medical care of patients with sickle cell disease (SCD) from pediatric to adult providers represents a milestone in their lives. Major concerns among adolescents and young adults about transition include taking responsibility for self, making own decisions, cost of medical care, fear of suboptimal pain management, and reluctance to leave known providers. In this study we present our experience in the process of transition to adult care and its outcome over the last ten years. Adolescents and young adults were given information about the nature of medical care provided by adult internists and hematologists. The sickle cell programs available in the city were described. Moreover, site visits to the hospitals where adult care was to be provided were arranged. During these visits, adolescents and young adults had the chance to meet the hematologist and other potential providers and ask questions, visit the emergency room, the clinic, and the sickle day unit if applicable. Patients were empowered to choose the program to which they wished to be transitioned. During the last 10 years, 90 adolescents and young adults (See Table) with SCD (Sickle Cell Anemia [SS], Hemoglobin SC Disease, and Sickle Thalassemia [ST]) were transitioned to the adult sickle cell program of Thomas Jefferson University. Age of transition varied between 18 and 25 years. Eighteen patients (20%) died. Age at death was 24.9 ± 2.95 years and the male/female ratio was 10:8. Complications of sickle cell disease after transition included leg ulcers, stroke, avascular necrosis, anxiety, depression, and priapism. Nineteen patients (10 males, 9 females) were employed. Twenty-nine (32%) patients developed chronic pain syndrome and its sequelae. Many patients failed to achieve their childhood goals. The data show that a significant number of patients die within 10 years after transition. The quality of life of survivors is suboptimal and drifts into issues of chronic pain management in the adult environment. Identifying these issues may provide predictors that identify children at risk to have undesirable outcomes after transition. Aggressive management and refining the process of transition should improve the outcome after transition. Distribution of the Transitioned Patients SS SC ST Total Male 31 8 4 43 Female 34 8 5 47 Total 65 16 9 90

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The Complex Relationship Between Sickle Cell Disease and Depression.

Blood ◽

10.1182/blood.v114.22.2585.2585 ◽

2009 ◽

Vol 114 (22) ◽

pp. 2585-2585 ◽

Cited By ~ 1

Author(s):

Soheir S. Adam ◽

Charlene Flahiff ◽

Mary R Abrams ◽

Marilyn J. Telen ◽

Laura M. De Castro

Keyword(s):

Chronic Pain ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Chronically Ill ◽

Neurocognitive Function ◽

Clinical Depression ◽

Current Therapy ◽

Cell Disease ◽

Severity Scores ◽

Prevalence Of Depression

Abstract Abstract 2585 Poster Board II-561 A high prevalence of depression has been described in both chronic diseases and diseases associated with chronic pain. Sickle cell disease (SCD) is a congenital and lifelong complex hematological illness in which both acute and chronic pain are described as hallmarks. Depression prevalence has also been reported as high in SCD. Clinical depression may lead to decreased compliance with prescribed medical treatments, and thus deteriorating function and health status. Furthermore, depressed patients report more frequent painful episodes. Pain and depression can also both have negative effects on health-related quality of life (QoL) measures in a chronically ill population. Methods: We performed an analytic epidemiologic prospective study to determine the prevalence of depression (as measured by the Beck Depression Inventory [BDI]) in 70 adult SCD patients at baseline (no pain episodes within 30 days) who receive their SCD disease-related care primarily from our clinic. We also assessed the association between the prevalence of depression, QoL, and severity scores measuring end organ damage as previously described (Afenyi-Annan et al. 2008). To measure mental and physical QoL domains, patients were administered the SF36 QoL scale. A short computerized test, “CNS Vital Signs,” was used to assess patients' neurocognitive function. Pain diaries were used to determine the use of short-term and long-term narcotics. Results: The sample included 38 females and 32 males, ages 19 – 76 years (mean 36). Mean severity score was 1.61 (SD 1.1; range 0–4). Nineteen patients (27%) had clinical depression by BDI. Nine of them (47%) were classified as severe. The gender ratio was 2:1 F:M. Patients with depression were significantly older (mean age 39.8 vs. 35.0 yrs, p<0.05). The ratio of hemoglobin SS versus other sickle-related hemoglobinopathies was 1:1 in those with depression versus 2:1 in those without depression. Severity scores were not statistically different between those with or without depression (1.8 ±1.1 vs. 1.5±1.2). Nineteen of 51 patients (37%) without depression had a prior history of central nervous system events, while only 4 of 19 (21%) with depression did; this difference was not statistically significant. Similarly, no statistical difference in neurocognitive function was noted between patients with and without depression, when tested for the following 5 domains: memory, psychomotor speed, reaction time, complex attention, and cognitive flexibility. Both the physical and mental domains of the QoL testing showed significantly lower scores for those patients with depression when compared with those without depression (p=0.007 and p<0.0001, respectively). For the mental domain, there was also a statistically significant inverse correlation between the level of depression and domain score. Lastly, neither current therapy with long-acting narcotics or antidepressive medications showed association with the presence of depression. Summary: We conclude that there is likely a complex and thus far poorly understood interaction between multiple SCD cofactors and the presence of depression in this patient population. QoL rather than disease severity is the measure most strongly related to depression in our study. Disclosures: No relevant conflicts of interest to declare.

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High dose vitamin D therapy for chronic pain in children and adolescents with sickle cell disease: results of a randomized double blind pilot study

British Journal of Haematology ◽

10.1111/bjh.12019 ◽

2012 ◽

Vol 159 (2) ◽

pp. 211-215 ◽

Cited By ~ 46

Author(s):

Ifeyinwa Osunkwo ◽

Thomas R. Ziegler ◽

Jessica Alvarez ◽

Courtney McCracken ◽

Korin Cherry ◽

...

Keyword(s):

Vitamin D ◽

Chronic Pain ◽

Pilot Study ◽

Sickle Cell Disease ◽

Children And Adolescents ◽

Sickle Cell ◽

High Dose ◽

Cell Disease ◽

Double Blind ◽

High Dose Vitamin

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Can Heterogeneity of Chronic Sickle-Cell Disease Pain Be Explained by Genomics? A Literature Review

Biological Research For Nursing ◽

10.1177/1099800409337154 ◽

2009 ◽

Vol 11 (1) ◽

pp. 81-97 ◽

Cited By ~ 7

Author(s):

Maxine A. Adegbola

Keyword(s):

Chronic Pain ◽

Sickle Cell Disease ◽

Literature Review ◽

Sickle Cell ◽

Care Delivery ◽

Association Studies ◽

Single Gene ◽

Susceptibility Genes ◽

Genomic Research ◽

Cell Disease

This literature review explores the potential of genomics to explain, or at least contribute to the discussion about, heterogeneity in chronic pain in sickle-cell disease (SCD). Background: Adults with SCD, a single-gene disorder, are living longer than in years past, yet report being burdened by chronic pain. With only a few studies on chronic pain in this population, the epidemiology is unclear. However, research in the area of pain genetics continues to advance since the conclusion of the Human Genome Project. Two pain susceptibility genes, catechol-O-methyltransferase (COMT) and cytochrome P450, have, to date, been discovered that can increase individual susceptibility to the development of chronic pain. Method: A search was conducted in PubMed, CINAHL, and EBSCO using the terms ``sickle cell,'' ``chronic pain,'' ``polymorphism,'' ``genetics,'' ``pain genetics,'' ``human,'' ``adult,'' ``association studies,'' and ``pain susceptibility genes'' to search for articles published between 1970 and 2008. Findings: Chronic pain generally is more prevalent and severe than previously reported, and individuals with SCD report daily pain. The genomic era has made it possible for scientists to identify pain susceptibility genes that contribute to variability in the interindividual experience of chronic pain. Conclusion: Nurses are well positioned to generate and translate genomic research, thus improving care delivery. Such research may lead to the identification of polymorphisms associated with pain sensitivity in individuals with SCD.

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Average Chronic Pain Medication Needed in Adult Patient with Sickle Cell Disease and Its Relation to Vitamin D Level

Blood ◽

10.1182/blood.v128.22.4857.4857 ◽

2016 ◽

Vol 128 (22) ◽

pp. 4857-4857

Author(s):

Samip Master ◽

Shajadi Patan ◽

Shashank Cingam ◽

Runhua Shi ◽

Richard Preston Mansour

Keyword(s):

Vitamin D ◽

Chronic Pain ◽

Standard Deviation ◽

Sickle Cell Disease ◽

Adult Patient ◽

Retrospective Analysis ◽

Sickle Cell ◽

Pain Medication ◽

Cell Disease ◽

Vitamin D Levels

Abstract Introduction: The chronic pain in sickle cell disease (SCD) arises from chronic bone damage as a consequence of bone marrow infraction during vaso-occulsive events. There are certain barriers to adequate pain management in adult patients with SCD, namely, limited knowledge among the clinicians, inadequate assessment, concerns about addiction, and biases against opioid use. We did retrospective analysis to investigate the average pain medication needed by adult patient with SCD. We also did analysis to see if there was a relation between plasma vitamin D level and amount of pain medication needed. Methods: We take care of approximately 300 active adult SCD at Hematology clinic at our institute. We did a retrospective analysis of 458 adult patients with SCD seen at our clinic between 2001 and 2016. We collected data on type of SCD, plasma 25 -hydroxyvitamin d level and amount of opioid pain medication in mg. To get uniform units of opiates, we converted all the different opiates into morphine. Results: The average morphine dose in a 24 hours period needed to manage chronic pain in an adult patient with SS type was 84 mg with standard deviation of 72, for SC type was 60 mg with standard deviation of 72 and for sickle beta thal type was 72 mg with standard deviation of 71. There were 4 patients with SS with hereditary persistence of hemoglobin F and average opiate dose in them was 84 mg. We obtained vitamin d level on 223 patients and out of them, 47 had vitamin d level of <4.2 ng/ml (lowest level reportable by our lab). We also found negative correlation between amount of pain medication and vitamin D level. The spearman correlation coefficient was -0.2 and p value was <0.01. Conclusion: Because we were unable to find any previous reports of the correlation of vitamin D levels and opiate use in an adult population with SCD, we believe that this is the first study to report this correlation. It also provides a rough estimate regarding average amount of opiates that an adult patient with SCD needs. This correlation between Vitamin D levels and opiate requirement supports our current practice of screening all patients for Vitamin D deficiency using 25hydroxy vitamin D levels and treating all patients who are found to be deficient. We do not know if this Vitamin D replacement will reduce pain, bone health or the amount of opiate medication needed in the future. Disclosures No relevant conflicts of interest to declare.

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