scholarly journals How I treat acute and persistent sickle cell pain

2020 ◽  
Vol 12 (1) ◽  
pp. e2020064 ◽  
Author(s):  
Samir Ballas

Sickle pain is the hallmark of sickle cell disease (SCD). It could be acute, persistent/relapsing, chronic or neuropathic. Although there is a general consensus that pain is a major manifestation of SCD there is a controversy as to the types of pain and their interrelationship between acute, chronic, relapsing, persistent, etc. This report first reviews the general approach to the management of acute vaso-occlusive crisis (VOC) pain including education, counseling, pharmacotherapy, non-pharmacotherapy, and fluid therapy. This is followed by the presentation of five patients that represent typical issues that are commonly encountered in the management of patients with SCD. These issues are: individualized treatment of pain, bilaterality of pain, use of illicit drugs, tolerance to opioids, opioid induced hyperalgesia and withdrawal syndrome. The clinical aspects and management of each of these issues are described. Moreover, such complications as tolerance and withdrawal may persist after discharge and may be mistaken as chronic pain rather than resolving, persistent or relapsing pain.

Author(s):  
Madison Irwin ◽  
William Gunther ◽  
Patricia Keefer ◽  
D'Anna Saul ◽  
Sharon Singh ◽  
...  

PEDIATRICS ◽  
1971 ◽  
Vol 48 (4) ◽  
pp. 629-635
Author(s):  
Howard A. Pearson ◽  
Louis K. Diamond

This brief review, being limited in scope to the recognition and management of the life-threatening and painful crises in infants and children with sickle-cell disease, has not even touched on the intriguing mystery of the molecular basis for the sickling phenomenon–how one amino-acid substitution (gene controlled) in the beta chain sequence of 146 amino acids can cause such serious disruption in form and function; or how this mutation occurred in the first place and why it has persisted in contrast to the rapid disappearance of many other deleterious mutants. Nor has there been even mention of the many milder symptoms, signs, and complications due to the presence of Hb. S., either in the homozygous (disease-producing) state or heterozygous form when found in combination with other hereditary hemoglobin defects. The accumulated knowledge about this mutant gene, its biochemical effects, and geographic distribution is enormous. From a fundamental scientific standpoint, sickle cell disease is one of the best understood of human afflictions. However, from a practical point of view treatment of the patient himself is often only symptomatic and palliative. Nevertheless, prompt and effective therapy of the myriad manifestations of sickle cell disease can effectively reduce morbidity and mortality. The pediatrician who cares for black children in his practice should be familiar with the cardinal diagnostic and clinical aspects of sickle cell disease and its crises.


2020 ◽  
Vol 4 (12) ◽  
pp. 2656-2701 ◽  
Author(s):  
Amanda M. Brandow ◽  
C. Patrick Carroll ◽  
Susan Creary ◽  
Ronisha Edwards-Elliott ◽  
Jeffrey Glassberg ◽  
...  

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.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3743-3743 ◽  
Author(s):  
Samir K. Ballas ◽  
Carlton Dampier

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


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2585-2585 ◽  
Author(s):  
Soheir S. Adam ◽  
Charlene Flahiff ◽  
Mary R Abrams ◽  
Marilyn J. Telen ◽  
Laura M. De Castro

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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