View clinical trials related to Anemia, Sickle Cell.
Filter by:The goal of the study is to understand how best to help parents of young children with sickle cell disease and their clinicians have a shared discussion about hydroxyurea (one that takes into account medical evidence and parent values and preferences). The study will compare two methods to help clinicians facilitate this-a clinician pocket guide and a clinician hydroxyurea shared decision making toolkit-in a group of parents of children ages 0-5 with sickle cell disease. The investigators hope that both methods lead to parents reaching a high-quality, well-informed decision. In addition, the team hopes to demonstrate that parents who experience a shared decision will have lower anxiety and decisional uncertainty. The researchers also expect these parents to be more likely to choose hydroxyurea and that their children will have less pain, fewer hospitalizations, better developmental outcomes, and higher quality of life. The project team hopes to show that the toolkit method is easy for clinicians to use and gives parents the support needed to make an informed decision.
Children with sickle cell disease (SCD) are living longer with the advent of medical advances such as prophylactic penicillin, chronic transfusion, and hydroxyurea. Despite greater longevity in SCD, the period following the transition from pediatric to adult care is critical; youth aged 18-30 years are at high risk for mortality and have high rates of healthcare utilization, leading to high healthcare costs. As such, health care transition (HCT) programs have been created to prepare patients for adult-centered care and subsequently, improve health outcomes. However, very few programs have been evaluated for effectiveness in achieving optimal health outcomes in SCD. This paucity of program evaluation is attributed to a lack of identifiable predictors and outcomes. Researchers at St. Jude Children's Research Hospital want to identify factors and patterns of successful HCT. This information will be used to develop approaches to best evaluate HCT interventions and identify areas of improvement of HCT programming. PRIMARY OBJECTIVE: Describe hospital utilization, treatment adherence, and health-related quality of life in a cohort of patients with sickle cell disease (SCD) who will transfer to adult care during the study period. SECONDARY OBJECTIVE: Examine the associations between various factors and health care transition (HCT) outcomes.
Investigators hypothesize that administration of ketamine for pain relief in sickle cell patients with vaso-occlusive crisis early on will lead to a more rapid improvement in pain score and less narcotic requirement.
This is a prospective pilot study of matched-related donor allogeneic stem cell transplantation in adults with severe sickle cell disease using a matched-sibling PBSC graft with a non-myeloablative conditioning regimen (Alemtuzumab).
Sickle cell disease (SCD) is a genetic disease characterized by abnormal hemoglobin, the main constituent of red blood cells. People with SCD have nutritional deficiencies, and vitamin D deficiency is one of the most common. Symptoms of vitamin D deficiency are similar to those of SCD and include chronic pain and bone complications. Correcting vitamin D nutrition of children with SCD represents a treatment that will improve their health. A single oral high-dose of vitamin D3 will be given to SCD children during one of their follow-up visits at the SCD clinic of CHU Sainte-Justine, Montreal, Canada. This mode of administration was chosen to ensure a better adherence to the treatment. The investigators will determine whether this dose is safe and its administration feasible in clinic. The impact of this dose on blood vitamin D and calcium, urinary calcium, growth, inflammation, bone health, pain and quality of life will also be assessed. This study intends to propose a new intervention to improve the nutrition of children with this disease.
Hyperbaric oxygen therapy in acute sickle cell pain crisis. The purpose of this study is to explore if hyperbaric oxygen therapy would decrease hospital length of stay and pain associated with acute sickle cell pain crisis. Eligibility criteria include both female and males age 19 years or older with sickle cell who are in an acute pain crisis. Exclusions include pregnancy and a sickle cell crisis complicated by any acute significant concomitant factors/conditions (i.e., acute chest syndrome, acute myocardial infarction/stroke). Interventions would be 1-3 hyperbaric oxygen sessions depending on response to the therapy. Each treatment session will be approximately two hours in length. Evaluation would be through patients' self assessment via the visual analog scale for pain level before and after treatments as well as tracking length of stay in the hospital.
Bearers of the sickle cell allele (S) are currently eligible for blood donations in Belgium. As blood donors are not tested for this allele, their heterozygous status is unknown. However, guidelines recommend to transfuse sickle cell patients with blood that is negative for the 'S' hemoglobin. To the investigator's knowledge, no study has been conducted to evaluate the impact of transfusion with blood originating from heterozygous donors on the transfusion performance and the improvement of clinical status of the sickle cell disease patients.
Sickle cell patients have a high prevalence of alloimmunization. This high rate of alloimmunization can be partially explained by the existence of an antigenic difference between the predominantly Caucasian donor population and the sickle cell patients of African origin. Genetic and environmental risk factors have also been described. The main risk factors that have been shown in retrospective or cross-sectional studies are some HLA alleles, the age of the patient, the number of leukocyte-depleted erythrocyte concentrates (CED) transfused, the number of transfusion episodes, the age of the CEDs, the existence of an inflammatory event at the time of transfusion and the presence of anti-erythrocyte autoantibodies.There is also evidence of an impaired TH response but the underlying immunological mechanism is not fully understood. The aim of this study is to study the prevalence and the risk factors for anti-erythrocyte alloimmunization and to try to understand the immunological mechanisms.
Sickle cell patients have a high prevalence of alloimmunization. This high rate of alloimmunization can be partially explained by the existence of an antigenic difference between the predominantly Caucasian donor population and the sickle cell patients of African origin. Genetic and environmental risk factors have also been described. The main risk factors that have been shown in retrospective or cross-sectional studies are some HLA alleles, the age of the patient, the number of leukocyte-depleted erythrocyte concentrates (CED) transfused, the number of transfusion episodes, the age of the CEDs, the existence of an inflammatory event at the time of transfusion and the presence of anti-erythrocyte autoantibodies.There is also evidence of an impaired TH response but the underlying immunological mechanism is not fully understood. The aim of this study is to study the prevalence and the risk factors for anti-erythrocyte alloimmunization in pediatric and adult patients with Sickle Cell Disease (with a SS genotype) who are being followed at Queen Fabiola University Children's Hospital (HUDERF) and at the CHU Brugmann Hospital. The identification of risk factors would allow the investigators to improve, or at least adapt, their transfusion policy to certain clinical or immuno-haematological situations.
Study of IMR-687 in adult participants with sickle cell anemia (SCA) (homozygous HbSS or sickle-β0 thalassemia).