Enhancing the care of patients with sickle cell disease.

Enhancing the care of patients with sickle cell disease. Nurs Stand. 2019 Aug 19;: Authors: De D, Blackmore A, Taylor H Abstract When typically round red blood cells intermittently elongate and become lodged in the body's microcirculation, this can result in painful vaso-occlusive crises, often referred to as 'sickle cell crises'. The sickling and unsickling process can result in acute pain, chronic anaemia, ischaemic injury and multiple organ damage. One of the main concerns raised by patients with sickle cell disease is the lack of knowledge and understanding of their condition among healthcare professionals in acute care settings. Therefore, this article aims to enhance nurses' understanding of sickle cell disease and the effective management of painful vaso-occlusive crises. While sickle cell disease was traditionally perceived to only occur in people of black African or African-Caribbean ethnic origin, this article seeks to challenge this belief and reconsider sickle cell as a public health concern for all. PMID: 31468941 [PubMed - as supplied by publisher]
Source: Nursing Standard - Category: Nursing Authors: Tags: Nurs Stand Source Type: research

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Inherited hemoglobin disorders, including beta-thalassemia (BT) and sickle-cell disease (SCD) are the most common monogenic diseases worldwide, with a global carrier frequency of over 5%. With migration they are becoming more common worldwide, making their management and care an increasing concern for health care systems.BT is characterized by an imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and compensatory haemopoietic expansion. Globally, there are over 25,000 births each year with transfusion-dependent thalassemia (TDT). The current available tr...
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Source Type: research
Abstract Sickle cell disease afflicts millions of people worldwide and approximately 100,000 Americans. Complications are myriad and arise as a result of complex pathological pathways 'downstream' to a point mutation in DNA, and include red blood cell membrane damage, inflammation, chronic hemolytic anemia with episodic vaso-occlusion, ischemia and pain, and ultimately risk of cumulative organ damage with reduced patient lifespan. The National Heart, Lung, and Blood Institute's 2014 evidence-based guideline for sickle cell disease -management states additional research is needed before investigational curative the...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
BackgroundSickle cell disease (SCD) is characterized by hemoglobin polymerization leading to vaso-occlusion, chronic hemolysis and vasculopathy. Parvovirus B19 (B19) infection causes an acute arrest of erythropoiesis leading to reticulocytopenia and potentially life-threatening anemia in patients with chronic hemolysis such as SCD. Diagnosis of B19 infection relies on clinical history in combination with expensive and often belated serological and molecular testing. The immature reticulocyte fraction (IRF) is a relatively new reticulocyte parameter available on automated hematology analyzers that assesses the maturity of c...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster III Source Type: research
Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disorder characterized by chronic hemolytic anemia in association with acute and chronic life-threatening complications mainly related to acute vaso-occlusive events (VOCs) due to amplified inflammatory response and defective pro-resolving events.Imatinib is an oral Tyrosine (Tyr)-kinase inhibitor, developed for the treatment of chronic myeloid leukemia (CML). Few case reports on SCD patients with CML undergoing imatinib treatment highlight the beneficial impact of imatinib on severity and recurrence of acute VOCs in SCD. In red blood cells (RBCs), Im...
Source: Blood - Category: Hematology Authors: Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Sickle Cell Disease-Genomic, Gene Regulation, and Pain Mechanisms Source Type: research
Pain is one of the most dreadful symptoms in sickle cell disease (SCD) and is often refractory to currently available analgesics. Besides acute painful vaso-occlusive crises, SCD is also accompanied by intractable chronic pain. This persistent, and often unrelieved, pain starts early in childhood and continues throughout life. The neurobiological mechanisms of chronic pain in SCD remain unclear, which markedly limits effective pain management and the quality of life in patients with SCD. Taking advantage of two humanized mouse models of SCD, this study aimed to investigate protein phosphorylation mechanisms for chronic pai...
Source: Blood - Category: Hematology Authors: Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster III Source Type: research
Conclusions: Adults with sickle cell disease on chronic full agonist opioid therapy can be safely converted to buprenorphine. Acute care utilization has dropped significantly for patients post induction. Assessment of patient reported outcomes such as quality of life and pain interference are being collected. Buprenorphine appears to be a safe and effective medication in the management of pain in adults with SCD.Figure.DisclosuresLanzkron: PCORI: Research Funding; GBT: Research Funding; Prolong: Research Funding; Pfizer: Research Funding; selexys: Research Funding; NHLBI: Research Funding; HRSA: Research Funding; Ironwood: Research Funding.
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Pain and Pain Management in Sickle Cell Disease Source Type: research
Authors: Matthie N, Ross D, Sinha C, Khemani K, Bakshi N, Krishnamurti L Abstract Acute, intermittent vaso-occlusive pain is the hallmark of sickle cell disease (SCD) and is associated with substantial morbidity and impaired quality of life (QOL). The subgroup of adults with SCD who transition from recurrent, acute pain to chronic, persistent pain have even greater QOL impairment and higher rates of healthcare utilization. Self-management is central to SCD management; however, its role in chronic pain management is not established. This qualitative study was conducted to answer the following research questions: (1)...
Source: Journal of the National Medical Association - Category: General Medicine Tags: J Natl Med Assoc Source Type: research
Discussion Potassium (K+) is an alkali metal (Group 1 of periodic table with Hydrogen, Lithium and Sodium) with an anatomic number of 19. Its chemical symbol K, comes from the medieval Latin, kalium which means potash (mainly potassium carbonate or potassium hydroxide), the substance it was first isolated from. Potassium is an important cation and it mainly resides in the intracellular fluid with only a small amount in the extracellular fluid. Potassium regulates cell volume, pH and enzyme functions. Hyperkalemia is defined as a potassium level> 5.5 mEq/L in children and> 6.0 mEq/L in newborns. Hyperkalemia incre...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
This report would examine developments at the state and federal-level, court cases, and current views from stakeholders. Policy Questions Which states have PAS laws and what do those laws provide? What protections against abuse of PAS?What have the Supreme Court and lower courts held regarding individuals’ rights under PAS laws? The laws themselves?Is there evidence that persons with disabilities are being denied treatment by insurance companies but offered PAS instead, as NCD predicted?How is PAS viewed by disability organizations? Has this evolved in the past 13 years? If so why? If not, why?Are persons with disabi...
Source: blog.bioethics.net - Category: Medical Ethics Authors: Tags: Health Care syndicated Source Type: blogs
Sickle cell disease (SCD) is an inherited red blood cell (RBC) disorder caused by the presence of an abnormal hemoglobin, called hemoglobin S (HbS) or sickle hemoglobin. It is the most prevalent genetic blood disorder worldwide.1,2 The two major characteristics are chronic hemolytic anemia and intermittent vaso-occlusion that results in tissue ischemia and causes acute, severe pain episodes. SCD is a chronic disease that has detrimental effects on the entire body and requires a multidisciplinary team for management.
Source: Disease a Month - Category: Infectious Diseases Authors: Source Type: research
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