Perioperative Management of Sickle Cell Disease.
Ontology highlight
ABSTRACT:
SUBMITTER: Adjepong KO
PROVIDER: S-EPMC5937979 | biostudies-literature |
REPOSITORIES: biostudies-literature
Similar Datasets
Project description:Sickle cell disease (SCD) afflicts millions of people worldwide but is referred to as an orphan disease in the United States. Over the past several decades, there has been an increasing understanding of the pathophysiology of SCD and its complications. While most individuals with SCD in resource-rich countries survive into adulthood, the life expectancy of patients with SCD remains substantially shorter than for the general African-American population. SCD can be cured using hematopoietic stem cell transplantation and possibly gene therapy, but these treatment approaches are not available to most patients, the majority of whom reside in low- and middle-income countries. Until relatively recently, only one drug, hydroxyurea, was approved by the US Food and Drug Administration to ameliorate disease severity. Multiple other drugs (L-glutamine, crizanlizumab, and voxelotor) have recently been approved for the treatment of SCD, with several others at various stages of clinical testing. The availability of multiple agents to treat SCD raises questions related to the choice of appropriate drug therapy, combination of multiple agents, and affordability of recently approved products. The enthusiasm for new drug development provides opportunities to involve patients in low- and middle-income nations in the testing of potentially disease-modifying therapies and has the potential to contribute to capacity building in these environments. Demonstration that these agents, alone or in combination, can prevent or decrease end-organ damage would provide additional evidence for the role of drug therapies in improving outcomes in SCD.
| S-EPMC7388199 | biostudies-literature
Project description:Red cell transfusion represents one of the cornerstones of the chronic management of sickle cell disease, as well as its acute complications. Automated red cell exchange can rapidly lower the number of circulating sickle erythrocytes, without causing iron overload. Here, we describe our experience, having offered this intervention since 2011. A transient reduction in the platelet count by 61% was observed after the procedure. This was not associated with any haemorrhagic complications. Despite exposure to large volumes of blood, the alloimmunisation rate was only 0.027/100 units of red cells. The absence of any iron loading was confirmed by serial Ferriscans, performed over a number of years. However, patients with advanced chronic kidney disease showed evidence of iron loading due to reduced innate haemopoiesis and were subsequently switched to simple transfusions. A total of 59% of patients were on regular automated red cell exchange with a history of recurrent painful crises. A total of 77% responded clinically, as evidenced by at least a 25% reduction in their emergency hospital attendance for pain management. The clinical response was gradual and increased the longer patients stayed on the program. The earliest sign of clinical response was a reduction in the length of stay when these patients were hospitalised, indicating that a reduction in the severity of crises precedes the reduction in their frequency. Automated red cell exchange also appeared to be beneficial for patients with recurrent leg ulcers and severe, drug resistant stuttering priapism, while patients with pulmonary hypertension showed a dramatic improvement in their symptoms as well as echocardiographic parameters.
| S-EPMC7918980 | biostudies-literature
Project description:Sickle cell disease results in numerous complications that can lead to significant morbidity and mortality. Amongst them, acute chest syndrome is the leading cause of mortality. As a result, most providers are in tune with this complication and well versed with management. As sickle cell patients now live longer, they face a multitude of other complications that if left unattended, can lead to significant morbidity and mortality as well. It is critical to look beyond acute chest syndrome and adopt a more comprehensive approach to the management of the sickle cell patient.
| S-EPMC7920619 | biostudies-literature
Project description:Sickle cell disease is the most common inherited blood disorder in the United States. This disorder of hemoglobin structure leads to a chronic hemolytic anemia and complex chronic disease manifested by sudden, severe, and life-threatening complications. These acute complications can occur in any organ system beginning in early childhood and lasting throughout life. The intermittent nature and acuity of these complications lend the emergency department to be an important site of care. The hallmark of sickle cell disease is the vasoocclusive painful event. Other more "typical" complications include fever, acute chest syndrome, priapism, and ischemic stroke. Children with sickle cell disease can also present with other "atypical" complications that can have devastating consequences if they are unrecognized. Detailed discussion of these "atypical" sickle cell disease complications, organized by organ system involved, will be the focus of this article.
| S-EPMC3172721 | biostudies-other
Project description:Sickle cell disease (SCD) is a life-threatening genetic disorder characterized by chronic hemolytic anemia, vascular injury and multiorgan dysfunctions. Over the last few decades, there have been significant improvements in SCD management in Western countries, especially in pediatric population. An early onset of prophylaxis with Penicillin and a proper treatment of the infections have increased the overall survival in childhood. Nevertheless, management of painful episodes and prevention of organ damage are still challenging and more efforts are needed to better understand the mechanisms behind the development of chronic organ damages. Hydroxyurea (Hydroxycarbamide, HU), the only medication approved as a disease-modifying agent by the United States Food and Drug Administration and the European Medicines Agency, is usually under-used, especially in developing countries. Currently, hematopoietic stem-cell transplantation is considered the only curative option, although its use is limited by lack of donors and transplant-related toxicity. SCD symptoms are similar in children and adults, but complications and systemic organ damages increase with age, leading to early mortality worldwide. Experts in comprehensive care of young patients with SCD, especially those approaching the transition age to adulthood, are missing, leading people to rely on urgent care, increasing health care utilization costs and inappropriate treatments. It would be important to establish programs of comprehensive healthcare for patients with SCD from birth to adulthood, to improve their quality and expectancy of life.
| S-EPMC4737690 | biostudies-literature
Project description:Pain management in an acute vaso-occlusive episode for pediatric patients with sickle cell disease (SCD) is challenging and often is focused on opioids, IV fluids, regional anesthesia, ketamine infusions, and non-steroidal anti-inflammatory drugs (NSAIDs). Acupuncture has long been studied as an effective method of pain relief, although the use of acupuncture in pediatric patients with SCD during an acute vaso-occlusive pain episode is vastly understudied. This article provides a review of current research regarding the use of acupuncture as a pain treatment strategy for pediatric patients with SCD experiencing acute pain. A literature review of scientific papers published within the last ten years was conducted on the topic. Five primary literature articles on acupuncture for pain management in pediatric patients with SCD were reviewed. Acupuncture is feasible and acceptable, with statistically significant findings for effectiveness as an adjunct treatment for pain in this setting. It is concluded that acupuncture is a promising and understudied therapy for the treatment of pain during an acute pain episode in pediatric patients with SCD. Hopefully, this paper stimulates interest in this specific area of medicine and prompts future research studies to be conducted to reveal conclusive outcomes.
| S-EPMC9324601 | biostudies-literature
Project description:Objective:To describe self-management recommendations for sickle cell disease (SCD) care among health professionals who manage SCD in Ghana. Method:Nine health care professionals (nurses, doctors, and physician assistants) who work in SCD were interviewed. The semistructured interviews were recorded, transcribed, and analysed using the qualitative content analysis method. Self-management recommendations were conceptualised as preventive health, self-monitoring, self-diagnosis, self-treatment, and self-evaluation. Results:Preventive health recommendations were the commonest, where the professionals described similar topics including avoidance of cold temperature, frequent oral hydration, and healthy nutrition. Self-monitoring recommendations included regular checks for pallor, urine colour, and splenic enlargement. Self-diagnosis recommendations were captured as warning signs and included pain, fever, unusual feelings, and enlarged spleen. Pain and fever management were the focus of most self-treatment advice, and there were some self-treatment recommendations for dactylitis, anaemia, and priapism. There was considerable variation in the strategies recommended for the management of individual SCD-related problems. Conclusion:Ghanaian health professionals involved in SCD care provide limited and inconsistent self-management recommendations. There is a need for the development of SCD standards and guidelines that support effective self-management. Health professionals working in SCD require continuing education in self-management.
| S-EPMC6242362 | biostudies-other
Project description:Because self-management is central to sickle cell disease (SCD) management, this descriptive study of 18 young adults with SCD, ages 19-39, was conducted to understand their pain experience and to identify the specific home activities they use for pain prevention and management prior to care-seeking. Participants completed two baseline surveys and one semi-structured, individual interview. Content analysis of the interview transcripts yielded two themes: difficulty in describing pain and living with pain. Participants used pharmacological and non-pharmacological strategies to alleviate pain and avoid disease complications but report barriers to using these strategies. Healthcare providers should use study findings to provide appropriate care to and improve pain outcomes for young adults with SCD. In addition, interventions aimed at addressing barriers and optimizing self-management are needed.
| S-EPMC6269114 | biostudies-other
Project description:Introduction:Sickle cell disease (SCD) is an orphan disease in the United States, but is highly prevalent worldwide. Only two drugs, hydroxyurea and L-glutamine, are approved for this disease. With an improved understanding of the pathophysiology of SCD as well as the success of several recently approved drugs for other orphan diseases, there is an increased interest in the development of drugs for SCD. Areas covered:This review summarizes published studies of drug therapies and ongoing trials of novel agents. Expert opinion:The development of drugs with different mechanisms of action offers opportunities for combination and individualized therapy in SCD. In addition to acute pain crisis, the evaluation of other SCD-related complications, exercise capacity, patient reported outcomes and validated surrogate endpoints are necessary to advance drug development. It is important to involve sites in sub-Saharan Africa and India, which have the highest burden of SCD, in trials of novel therapies.
| S-EPMC6411095 | biostudies-literature
Project description:Red blood cell transfusions have reduced morbidity and mortality for patients with sickle cell disease. Transfusions can lead to erythrocyte alloimmunization, however, with serious complications for the patient including life-threatening delayed hemolytic transfusion reactions and difficulty in finding compatible units, which can cause transfusion delays. In this review, we discuss the risk factors associated with alloimmunization with emphasis on possible mechanisms that can trigger delayed hemolytic transfusion reactions in sickle cell disease, and we describe the challenges in transfusion management of these patients, including opportunities and emerging approaches for minimizing this life-threatening complication.
| S-EPMC3401213 | biostudies-literature