Adjustment Difficulties of Adolescents With Sickle Cell Disease

TOPIC: The psychosocialfactors that affect adolescents with sickle cell disease (SCD). PURPOSE: To explore whether specific psychosocial factors can provide clues to the future adjustment of this population. SOURCES: Ovid Web, Medline, Psychinfo, and CINAHL databases for the years 1997 to 2001. CONCLUSIONS: Promoting effective psychosocial functioning is as important as managing the medical aspects of SCD, yet this is an area of care that is commonly overlooked. Nurses, therefore, have an opportunity to have a significant impact on the lives of adolescents with SCD if they intervene in ways to promote both biological and psychosocial adjustment.     

Information-Seeking Coping Behaviors During Painful Procedures in African-American Children with Sickle Cell Disease

This study examined the frequency of information-seeking coping behaviors in 37 African-American children (ages 5-17 years) with sickle cell disease during venipuncture. The relationships between coping behaviors and child- and parent-reported pain and observational distress were also assessed. The majority of children attended to the procedure, but did not seek information via questions. This pattern of coping was only partially effective at reducing distress and had no relation to pain. This pattern of coping is discussed within the context of cultural factors that may be important in understanding responses to procedural pain in pediatric sickle cell disease.     

Red Blood Cell Alloimmunization in Transfused Patients With Sickle Cell Disease in Sub-Saharan Africa; a Systematic Review and Meta-Analysis

Sickle cell disease (SCD) is the most common monogenic disorder in sub-Saharan Africa (SSA). Blood transfusion to increase the oxygen carrying capacity of blood is vital in the management of many patients with SCD. However, red blood cell (RBC) alloimmunization is a major challenge to transfusions in these patients. Commonly in SSA, pretransfusion tests only involve ABO D grouping and compatibility without RBC antibody testing. Data on the frequency of RBC alloimmunization in patients with SCD in SSA are limited. We performed a systematic review and meta-analysis on available data on alloimmunization in transfused patients with SCD to determine the published prevalence of RBC alloimmunization in SCD patients in SSA. Six databases were systematically searched to identify relevant studies, without year or language restrictions. In all, 249 articles were identified and 15 met our selection criteria. The overall proportion of alloimmunization was 7.4 (95% confidence interval: 5.1-10.0) per 100 transfused patients. Antibodies against E, D, C, and K antigens accounted for almost half of antibody specificities, and antibodies to low- and high-frequency antigens were also common and represented almost 30% (20% to low-frequency antigens and 9% to high-frequency antigens) of specificities. Heterogeneity between studies was moderate, and meta-analysis found region of Africa as the major contributor to the heterogeneity. We also observed inconsistencies across studies in reporting of factors that may influence alloimmunization. This review provides an overview of the extent of the alloimmunization problem in SSA and provides a baseline against which to compare the effect of any interventions to reduce the alloimmunization risk.     

Pain Management Issues as Part of the Comprehensive Care of Patients with Sickle Cell Disease

BackgroundVaso-occlusive pain crisis is one of the primary complications of sickle cell disease (SCD) and is responsible for the majority of hospital visits in patients with SCD. Stints of severe pain can last for hours to days and are difficult to treat and manage, often resulting in drastically reduced quality of life.PurposeOur purpose is to provide an overview of pain management issues in SCD populations.MethodsWe explored literature using PubMed and Embase for the etiology and management of pain in SCD. Databases were searched employing the following terms: sickle cell, pain pathways, pain perception, pharmacological therapies, psychological therapies, physical therapies and genetics.ResultsPain in SCD can vary from acute to chronic (persistent) or mixed and understanding of the underlying mechanisms is important for proper pain management. Currently, there are many means of managing pain in children with SCD, which involve pharmacological and non-pharmacological approaches. A combination of psychotherapy and pain medications can be used for treatment of pain and other psychosocial co-morbidities in complex persistent pain.ConclusionsProviding more appropriate medication and optimal dosage based on individual's genomic variations is the future of medicine, and this will allow the physicians to hone in on optimal pain management in patients with SCD.     

Improving Transition Readiness in Young Adults With Sickle Cell Disease

Transition of young adults with sickle cell disease is essential. This project examined the effectiveness of an educational intervention at a pediatric hematology practice to improve transition readiness in young adults with sickle cell disease. Interventions used were 2 Stepping Up videos and 5 questions from “Incorporating Health Care Transition Services Into Preventive Care for Adolescents and Young Adult.” Data was collected and results were analyzed preintervention, immediately postintervention, and at return office visit. Non–high school graduates showed greater improvement in total and subscale mean scores after the intervention at the return office visit.     

Psychological Characteristics and Pain Frequency Are Associated With Experimental Pain Sensitivity in Pediatric Patients With Sickle Cell Disease

Sickle cell disease (SCD) is associated with episodes of severe vaso-occlusive pain beginning in infancy with a subset of patients with SCD transitioning to chronic pain. Response to experimental pain using quantitative sensory testing in these patients suggests altered pain processing. The objectives of this study were to characterize sensitivity to multiple modalities of experimental pain stimuli and to interrogate the relationship of psychological covariates, clinical pain burden, and pain-related outcomes to experimental pain sensitivity in children with SCD compared with healthy individuals of similar age and sex. Cross-sectional assessments of psychological characteristics were performed, and quantitative sensory testing methods were used to measure experimental pain sensitivity in children age 8 to 21 years. Anxiety, depressive symptoms, catastrophizing, and somatization were found to be associated with increased sensitivity to experimental pain stimuli. Increased frequency of painful episodes in SCD was associated with decreased sensitivity to heat pain and decreased mechanical temporal summation. These data suggest that careful consideration be given to psychological factors, age, sex, and clinical burden of pain when studying response to experimental pain in SCD.

Emergency Department Management of Acute Pain Episodes in Sickle Cell Disease

Objectives To characterize the initial management of patients with sickle cell disease and an acute pain episode, to compare these practices with the American Pain Society Guideline for the Management of Acute and Chronic Pain in Sickle-Cell Disease in the emergency department, and to identify factors associated with a delay in receiving an initial analgesic.
Methods This was a multicenter retrospective design. Consecutive patients with an emergency department visit in 2004 for an acute pain episode related to sickle cell disease were included. Exclusion criteria included age younger than 18 years. A structured medical record review was used to abstract data, including the following outcome variables: analgesic agent and dose, route, and time to administration of initial analgesic. Additional variables included demographics, triage level, intravenous access, and study site. Mann–Whitney U test or Kruskal–Wallis test and multivariate regression were used to identify differences in time to receiving an initial analgesic between groups.
Results There were 612 patient visits, with 159 unique patients. Median time to administration of an initial analgesic was 90 minutes (25th to 75th interquartile range, 54–159 minutes). During 87% of visits, patients received the recommended agent (morphine or hydromorphone); 92% received the recommended dose, and 55% received the drug by the recommended route (intravenously or subcutaneously). Longer times to administration occurred in female patients (mean difference, 21 minutes; 95% confidence interval = 7 to 36 minutes; p = 0.003) and patients assigned triage level 3, 4, or 5 versus 1 or 2 (mean difference, 45 minutes; 95% confidence interval = 29 to 61 minutes; p = 0.00). Patients from study sites 1 and 2 also experienced longer delays.
Conclusions Patients with an acute painful episode related to sickle cell disease experienced significant delays to administration of an initial analgesic.     

Advances in the Treatment of Sickle Cell Disease

Sickle cell disease (SCD) is a monogenic disorder that afflicts approximately 100,000 Americans and millions of people worldwide. It is characterized by hemolytic anemia, vaso-occlusive crises, relentless end-organ injury, and premature death. Currently, red blood cell transfusion and hydroxyurea are the major disease-modifying therapies available for SCD. Hematopoetic stem cell transplant is curative, but barriers to treatment are substantial and include a lack of suitable donors, immunologic transplant rejection, long-term adverse effects, prognostic uncertainty, and poor end-organ function, which is especially problematic for older patients. Gene therapy to correct the β^s point mutation is under investigation as another curative modality. Deeper insights into the pathophysiology of SCD have led to the development of novel agents that target cellular adhesion, inflammation, oxidant injury, platelets and/or coagulation, vascular tone, and hemoglobin polymerization. These agents are in preclinical and clinical trials. One such agent, L-glutamine, decreases red blood cell oxidant injury and is recently US Food and Drug Administration approved to prevent acute pain episodes of SCD in patients 5 years of age or older. The purpose of this review is to describe the currently established therapies, barriers to curative therapies, and novel therapeutic agents that can target sickle cell hemoglobin polymerization and/or its downstream sequelae. A PubMed search was conducted for articles published up to May 15, 2018, using the search terms sickle cell disease, novel treatments, hematopoietic stem cell transplantation, and gene therapy. Studies cited include case series, retrospective studies, prospective clinical trials, meta-analyses, online abstracts, and original reviews.