Patients with sickle cell disease (SCD) experience a disproportionately high use of health care resources. Several studies have examined depression and other negative mood states as risk factors for increased health care utilization; however, there have been no systematic reviews examining and summarizing this evidence in SCD. The aim of this systematic review, therefore, was to determine whether depression or depressive symptoms are associated with health care utilization among children and adults with SCD. We followed a quantitative systematic review protocol based on the Preferred Reporting Items for Systematic Reviews and Meta- Analyses guidelines and performed a literature search of records from January 1980 to April 2014 using six databases. Empirical studies were eligible if the sample was primarily composed of patients with SCD and included data on depression, mood disorder diagnosis or depressive symptoms and health care utilization. We included 12 studies involving 54 036 unique participants. The prevalence estimates for depression ranged from 2-57%. Seven studies found a significant, or marginally significant, association between depression and utilization while five did not. Patients reporting depression had an estimated 2·8 times greater relative risk of being a high utilizer, and 2·9 versus 1·8 hospitalizations per year on average compared to patients without depression. Overall, depressive symptoms are common in SCD and may increase risk for poor outcomes including health care utilization. The available studies on depression in SCD, however, are limited by small sample sizes, retrospective designs or short follow-up. This systematic review found a modest association between depression and health care utilization in SCD. Abstract source Full text

Context: Sickle cell disease (SCD) vaso-occlusive crisis (VOC) remains an important cause of acute pain in pediatrics and the most common SCD complication. Pain management recommendations in SCD include nonpharmacological interventions. Yoga is one nonpharmacological intervention that has been shown to reduce pain in some populations; however, evidence is lacking in children with VOC.   Objectives: The primary objective of this study was to compare the effect of yoga vs. an attention control on pain in children with VOC. The secondary objectives were to compare the effect of yoga vs. an attention control on anxiety, lengths of stay, and opioid use in this population.   Methods: Patients were eligible if they had a diagnosis of SCD, were 5-21 years old, were hospitalized for uncomplicated VOC, and had an admission pain score of >=7. Subjects were stratified based on disease severity and randomized to the yoga or control group.   Results: Eighty-three percent of patients approached (N = 73) enrolled on study. There were no significant differences in baseline clinical or demographic factors between groups. Compared with the control group, children randomized to yoga had a significantly greater reduction in mean pain score after one yoga session (-0.6 ± 0.96 vs. 0.0 ± 1.37; P = 0.029). There were no significant differences in anxiety, lengths of stay, or opioid use between the two groups.   Conclusion: This study provides evidence that yoga is an acceptable, feasible, and helpful intervention for hospitalized children with VOC. Future research should further examine yoga for children with SCD pain in the inpatient and outpatient settings. Abstract source Full text

OBJECTIVES: Sickle cell disease (SCD) is a chronic pain disorder in which abnormally shaped red blood cells obstruct microcirculation causing ischemia and pain. The lack of SCD responsiveness to analgesics has led many to propose that nociceptive neural systems engaged when detecting pain become sensitized, resulting in an enhancement of pain response. METHODS: SCD patients and healthy controls were exposed to a painful stimuli of varying intensity using a pressure algometer and underwent several neuroimaging tasks. RESULTS: This study identified and characterized the neural correlates of possible central sensitization in SCD. We found functional connectivity abnormalities in patients with SCD in the brain's somatosensory network, salience network, and default mode network during both an unstructured resting state and paradigm involving acute pain challenge. DISCUSSION: There was evidence for both increased and decreased connectivity which is consistent with findings in other chronic pain disorders. Preliminary evidence was found that subcortical brain regions might contribute to neurodevelopmental abnormalities in chronic pain. The results support a model in which SCD pain sensitization involves abnormally low functional integration of brain regions that make use of nociceptive information to plan movements, and hyperconnectivity of various frontal and parietal lobe regions that direct attention to or represent higher-order abstractions within circuits involved with either nocioceptive processing or detection of abnormally salient environmental stimuli. Abstract source Full text

Red blood cell (RBC) alloimmunization is a significant clinical complication of sickle cell disease (SCD). It can lead to difficulty with cross-matching for future transfusions and may sometimes trigger life-threatening delayed hemolytic transfusion reactions. We conducted a retrospective study to explore the association of clinical complications and age of RBC with alloimmunization in patients with SCD followed at a single institution from 2005 to 2012. One hundred and sixty six patients with a total of 488 RBC transfusions were evaluated. Nineteen patients (11%) developed new alloantibodies following blood transfusions during the period of review. The median age of RBC units was 20 days (interquartile range: 14-27 days). RBC antibody formation was significantly associated with the age of RBC units (P = 0.002), with a hazard ratio of 3.5 (95% CI: 1.71-7.11) for a RBC unit that was 7 days old and 9.8 (95% CI: 2.66-35.97) for a unit that was 35 days old, 28 days after the blood transfusion. No association was observed between RBC alloimmunization and acute vaso-occlusive complications. Although increased echocardiography-derived tricuspid regurgitant jet velocity (TRV) was associated with the presence of RBC alloantibodies (P = 0.02), TRV was not significantly associated with alloimmunization when adjusted for patient age and number of transfused RBC units. Our study suggests that RBC antibody formation is significantly associated with older age of RBCs at the time of transfusion. Prospective studies in patients with SCD are required to confirm this finding. Abstract source Full text

AIM: To test feasibility of a guided audio-visual relaxation intervention protocol for reducing stress and pain in adults with sickle cell disease. BACKGROUND: Sickle cell pain is inadequately controlled using opioids, necessitating further intervention such as guided relaxation to reduce stress and pain. DESIGN: Attention-control, randomized clinical feasibility pilot study with repeated measures. METHODS: Randomized to guided relaxation or control groups, all patients recruited between 2013-2014 during clinical visits, completed stress and pain measures via a Galaxy Internet-enabled Android tablet at the Baseline visit (pre/post intervention), 2-week posttest visit and also daily at home between the two visits. Experimental group patients were asked to use a guided relaxation intervention at the Baseline visit and at least once daily for 2 weeks. Control group patients engaged in a recorded sickle cell discussion at the Baseline visit. Data were analysed using linear regression with bootstrapping. RESULTS: At baseline, 27/28 of consented patients completed the study protocol. Group comparison showed that guided relaxation significantly reduced current stress and pain. At the 2-week posttest, 24/27 of patients completed the study, all of whom reported liking the study. Patients completed tablet-based measures on 71% of study days (69% in control group, 72% in experiment group). At the 2-week posttest, the experimental group had significantly lower composite pain index scores, but the two groups did not differ significantly on stress intensity. CONCLUSION: This study protocol appears feasible. The tablet-based guided relaxation intervention shows promise for reducing sickle cell pain and warrants a larger efficacy trial. Abstract source Full text

BACKGROUND: Sickle cell anemia is an inherited blood disorder that is characterized by painful vaso-occlusive crises, for which there are few treatment options. Platelets mediate intercellular adhesion and thrombosis during vaso-occlusion in sickle cell anemia, which suggests a role for antiplatelet agents in modifying disease events. METHODS: Children and adolescents 2 through 17 years of age with sickle cell anemia were randomly assigned to receive oral prasugrel or placebo for 9 to 24 months. The primary end point was the rate of vaso-occlusive crisis, a composite of painful crisis or acute chest syndrome. The secondary end points were the rate of sickle cell-related pain and the intensity of pain, which were assessed daily with the use of pain diaries. RESULTS: A total of 341 patients underwent randomization at 51 sites in 13 countries across the Americas, Europe, Asia, and Africa. The rate of vaso-occlusive crisis events per person-year was 2.30 in the prasugrel group and 2.77 in the placebo group (rate ratio, 0.83; 95% confidence interval, 0.66 to 1.05; P=0.12). There were no significant differences between the groups in the secondary end points of diary-reported events. The safety end points, including the frequency of bleeding events requiring medical intervention, of hemorrhagic and nonhemorrhagic adverse events that occurred while patients were taking prasugrel or placebo, and of discontinuations due to prasugrel or placebo, did not differ significantly between the groups. CONCLUSIONS: Among children and adolescents with sickle cell anemia, the rate of vaso-occlusive crisis was not significantly lower among those who received prasugrel than among those who received placebo. There were no significant between-group differences in the safety findings. (Funded by Daiichi Sankyo and Eli Lilly; ClinicalTrials.gov number, NCT01794000.). Abstract source Full text

Magnesium, a vasodilator, anti-inflammatory, and pain reliever, could alter the pathophysiology of sickle cell pain crises. We hypothesized that intravenous magnesium would shorten length of stay, decrease opioid use, and improve health-related quality of life (HRQL) for pediatric patients hospitalized with sickle cell pain crises. The Magnesium for Children in Crisis (MAGiC) study was a randomized, double-blind, placebo-controlled trial of intravenous magnesium vs normal saline placebo conducted at 8 sites within the Pediatric Emergency Care Applied Research Network (PECARN). Children 4 to 21 years old with hemoglobin SS or Sβ(0) thalassemia requiring hospitalization for pain were eligible. Children received 40 mg/kg of magnesium or placebo every 8 hours for up to 6 doses plus standard therapy. The primary outcome was length of stay in hours from the time of first study drug infusion, compared using a Van Elteren test. Secondary outcomes included opioid use and HRQL. Of 208 children enrolled, 204 received the study drug (101 magnesium, 103 placebo). Between-group demographics and prerandomization treatment were similar. The median interquartile range (IQR) length of stay was 56.0 (27.0-109.0) hours for magnesium vs 47.0 (24.0-99.0) hours for placebo (P = .24). Magnesium patients received 1.46 mg/kg morphine equivalents vs 1.28 mg/kg for placebo (P = .12). Changes in HRQL before discharge and 1 week after discharge were similar (P > .05 for all comparisons). The addition of intravenous magnesium did not shorten length of stay, reduce opioid use, or improve quality of life in children hospitalized for sickle cell pain crisis. This trial was registered at www.clinicaltrials.gov as #NCT01197417. Abstract source Full text Full text (PMC)

Sickle cell disease (SCD), an inherited red blood cell disorder, is characterized by anemia, end-organ damage, unpredictable episodes of pain, and early mortality. Emergency department (ED) visits and hospitalizations are frequent, leading to increased burden on patients and increased health care costs. This study assessed the effects of a multidisciplinary care team intervention on acute care utilization among adults with SCD. The multidisciplinary care team intervention included monthly team meetings and development of individualized care plans. Individualized care plans included targeted pain management plans for management of uncomplicated pain crisis. Following implementation of the multidisciplinary care team intervention, a significant decrease in ED utilization was identified among those individuals with a history of high ED utilization. Findings highlight the potential strength of multidisciplinary interventions and suggest that targeting interventions toward high-utilizing subpopulations may offer the greatest impact. Abstract source Full text

BACKGROUND: Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines (medicine derived from plants in their original state) encompass many of the plant remedies from traditional healers which the populations most affected would encounter. There has been little systematic appraisal of their benefits. This is an update of a Cochrane Review first published in 2010 and updated in 2013. OBJECTIVES: To assess the benefits and risks of phytomedicines in people with sickle cell disease of all types, of any age, in any setting. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, the International Standard Randomised Controlled Trial Number Register (ISRCTN) and the Allied and Complimentary Medicine Database (AMED).Dates of most recent searches: Haemoglobinopathies Trials Register: 13 October 2014; ISRCTN: 17 January 2015; AMED: 20 January 2015. SELECTION CRITERIA: Randomised or quasi-randomised trials with participants of all ages with sickle cell disease, in all settings, comparing the administration of phytomedicines, by any mode to placebo or conventional treatment, including blood transfusion and hydroxyurea. DATA COLLECTION AND ANALYSIS: Both authors independently assessed trial quality and extracted data. MAIN RESULTS: Two trials (182 participants) and two phytomedicines Niprisan(®) (also known as Nicosan(®)) and Ciklavit(®) were included. The Phase IIB (pivotal) trial suggests that Niprisan(®) was effective in reducing episodes of severe painful sickle cell disease crisis over a six-month period. It did not affect the risk of severe complications or the level of anaemia. No serious adverse effects were reported. The single trial of Cajanus cajan (Ciklavit(®)) reported a possible benefit to individuals with painful crises, and a possible adverse effect (non-significant) on the level of anaemia. AUTHORS' CONCLUSIONS: While Niprisan(®) appeared to be safe and effective in reducing severe painful crises over a six-month follow-up period, further trials are required to assess its role in the management of people with sickle cell disease and the results of its multicentre trials are awaited. Currently no conclusions can be made regarding the efficacy of Ciklavit(®). Based on the published results for Niprisan(®) and in view of the limitations in data collection and analysis of both trials, phytomedicines may have a potential beneficial effect in reducing painful crises in sickle cell disease. This needs to be further validated in future trials. More trials are required on the safety and efficacy of phytomedicines used in managing sickle cell disease. Abstract source Full text

Cocaine: is a potent stimulant. It can cause your heart to race, and it can make you feel nervous and agitated. It can increase heart rate, breathing rate, and blood pressure. Street names include coke, snow, nose candy, flake blow, big C, lady, and snow birds.Cocaine increases the risk of major organ damage. It can cause stroke and even death in people with sickle cell disease. Crack: is a solid (rock) form of cocaine that is smoked, which is known as freebasing. It has the same effects as cocaine in the body. It makes the blood vessels smaller and decreases blood flow throughout the body. This can increase blood pressure, body temperature, and heart rate. It also has the risks associated with smoking, including shortness of breath, chest pains, and lung damage. It is highly addictive. Often, harmful chemicals are added to crack to create the rock form. In addition to the above street drugs, using prescription drugs without your doctor’s advice can lead to addiction, severe damage to your body, and even death. We understand that teens and young adults might feel more pressure from friends to drink, smoke, or take street drugs (drugs not prescribed by a doctor and obtained illegally). As a person with sickle cell disease, you need to know the extra health problems that alcohol, tobacco, and street drugs can cause in your body. We hope this fact sheet will help you make smart choices that will keep you as healthy as possible. Alcohol, tobacco, and street drugs are harmful to everyone but especially to those with sickle cell disease. They can cause addiction and permanent damage to vital body organs. They can also disrupt your life in many ways. Drinking, smoking, or the use of street drugs can lead to severe health problems for people with sickle cell disease (SCD). Abstract source