Plerixafor use in patients with sicklecelldisease (SCD) who are eligible for treatment with exagamglogene autotemcel (ages 12 years and above) NHS England » Plerixafor use in patients with sicklecelldisease (SCD) who are eligible for treatment with exagamglogene autotemcel (ages 12 years and above) Skip to main content Cookies on the NHS England websiteWe’ve put some small files called * Publications * Statistics * Blogs * Events * Contact us Search Search Menu * About us * Our work * Commissioning * Get involved Plerixafor use in patients with sicklecelldisease (SCD) who are eligible for treatment with exagamglogene autotemcel (ages 12 years and above)Document first published: 25 February 2025 Page updated: 27 February 2025 Topic: Specialised commissioning Publication type: Policy
Casgevy (exagamglogene autotemcel) and Lyfgenia (lovotibeglogene autotemcel) for individuals 12 years and older with sicklecelldisease (SCD) and recurrent vaso-occlusive crises (VOC): A therapeutics bulletin of the American College of Medical Genetics ACMG THERAPEUTICS BULLETINCasgevy (exagamglogene autotemcel) and Lyfgenia(lovotibeglogene autotemcel) for individuals 12 yearsand older with sicklecelldisease (SCD) and recurrentvaso-occlusive crises (VOC): A therapeutics bulletin of theAmerican College of Medical Genetics and Genomics (ACMG)Harry Lesmana1,2,3, Sun Young Kim4,5, Andr ́es Morales Corado6, Sheri A. Poskanzer7;onbehalf of the ACMG Therapeutics Committee8,*ARTICLE INFOArticle history:Received 16 July 2024Accepted 18 July 2024Available online 10 September
ASH Clinical Practice Guidelines on SickleCellDisease (SCD) 1 | 2019-2020 ASH Clinical Practice Guidelines on SickleCellDisease: What You Should KnowFor more information on the 2019-2020 ASH Clinical Practice Guidelines on SickleCellDisease, visit www.hematology.org/SCDguidelines.The American Society of Hematology (ASH) (www.hematology.org) is the world’s largest professional society of hematologists dedicated to furthering the understanding, diagnosis, treatment, and prevention of disorders affecting the blood. For more than 60 years, the Society has led the development of hematology as a discipline by promoting research, patient care, education, training, and advocacy in hematology.2019-2021 ASH Clinical Practice Guidelines on SickleCellDisease (SCD): What You Should
Black Americans with sicklecelldisease (SCD) demonstrate accelerated epigenetic pace of aging compared to Black Americans without SCD. Sicklecelldisease (SCD) is a chronic medical condition characterized by red blood cell sickling, vaso-occlusion, hemolytic anemia, and subsequently, end-organ damage and reduced survival. Because of this significant pathophysiology and early mortality, we hypothesized that patients with SCD are experiencing accelerated biological aging compared to individuals without SCD. We utilized the DunedinPACE measure to compare the epigenetic pace of aging in 131 Black Americans with SCD to 1391 Black American veterans without SCD. SCD patients displayed a significantly accelerated pace of aging (DunedinPACE mean difference of 0.057 points) compared to the veterans
Riociguat in patients with sicklecelldisease and hypertension or proteinuria (STERIO-SCD): a randomised, double-blind, placebo controlled, phase 1-2 trial. Although nitric oxide based therapeutics have been shown in preclinical models to reduce vaso-occlusive events and improve cardiovascular function, a clinical trial of a phosphodiesterase 5 inhibitor increased rates of admission to hospital for pain. We aimed to examine if riociguat, a direct stimulator of the nitric oxide receptor soluble guanylate cyclase, causes similar increases in vaso-occlusive events. This was a phase 1-2, randomised, double blind, placebo-controlled trial. Eligible patients were 18 years or older, had confirmed sicklecelldisease documented by haemoglobin electrophoresis or HPLC fractionation (haemoglobin SS, SC
Exagamglogene autotemcel (Casgevy) in beta-thalassaemia or sickle-celldisease Prescrire IN ENGLISH - Spotlight ''Exagamglogene autotemcel (Casgevy°) in beta-thalassaemia or sickle-celldisease'', 1 March 2025 {1}##LOC[OK]## {1} ##LOC[OK]## ##LOC[Cancel]## {1}##LOC[OK]####LOC[Cancel]## Register online| Log in| My Prescrire Issue contents * Current issue * Last 12 issues * All issues Topics Subscription Rate * Subscribers: register online * Prescrire's other products * Free Special Edition * Sign up to receive the newsletter english.prescrire.org > Spotlight > 100 most recent > Exagamglogene autotemcel (Casgevy°) in beta-thalassaemia or sickle-celldisease SpotlightEvery month, the subjects in Prescrire’s Spotlight. 100 most recent: 1|10|20|30|40|50|60|70|80|90 Spotlight * 100 most recent
Antioxidant supplementation for sicklecelldisease. Sicklecelldisease (SCD) refers to a group of genetic disorders characterized by the presence of an abnormal haemoglobin molecule called haemoglobin S (HbS). When subjected to oxidative stress from low oxygen concentrations, HbS molecules form rigid polymers, giving the red cell the typical sickle shape. Antioxidants have been shown to reduce oxidative stress and improve outcomes in other diseases associated with oxidative stress. Therefore, it is important to review and synthesize the available evidence on the effect of antioxidants on the clinical outcomes of people with SCD. To assess the effectiveness and safety of antioxidant supplementation for improving health outcomes in people with SCD. We used standard, extensive Cochrane search
Voxelotor (Oxbryta) in sickle-celldisease Register online | Log in | My PrescrireISSUE CONTENTSTOPICSABOUT PRESCRIREOFFERSenglish.prescrire.org > Spotlight > 100 most recent > Voxelotor (Oxbryta°) in sickle-cell diseaseSpotlightEvery month, the subjects in Prescrire’s Spotlight.100 most recent : 1 | 10 | 20 | 30 | 40 | 50 | 60 | 70 | 80 | 90Spotlight100 most recentArchivesVoxelotor (Oxbryta °) in sickle-celldisease Marketing Authorisations Voxelotor binds to haemoglobin and increases its affinity for oxygen. It is claimed that this prevents sickling and premature destruction of red blood cells. A reduction in oxygen release by haemoglobin would be expected in its presence. Voxelotor has been authorised in the European Union for the treatment of haemolytic anaemia due to sickle-celldisease
.................................................................................................................................... 17 Appendix 1 – Example of an SCD Clinical Pathway ....................................................................... 18 Appendix 2 – The ACT NOW* paradigm ........................................................................................ 19 RCEM Best Practice Guideline - Management of Acute Presentations of SickleCellDisease Page 5 This guideline has been developed to provide recommendations on the initial care of patients presenting to the emergency department (ED) with acute sicklecelldisease (SCD) presentations and its potential complications. The aim is to increase Emergency Department (ED) awareness of SCD, optimise and standardise the management of such patients, and ensure equity of access to acute care and specialist services. The process of creating this guideline ha..
statement Patients admitted on hydroxyurea shall not be given hydroxyurea prescription on discharge. Haematology is to prescribe in order to monitor blood work. 2.0 Definitions Hydroxyurea (HU) is a chemotherapy medicine that has been used to treat many disorders, including sicklecelldisease (SCD). Research has shown that patients with sicklecelldisease who take hydroxyurea are admitted at the Hospital for Sick Children. Use of this guideline in any setting must be subject to the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside SickKids. Page 2 of 4 3.0 Guideline Pain assessment and consult completed of child with SickleCellDisease (SCD)Prior to starting hydroxyurea ensure that the following
Exagamglogene autotemcel (Exa-cel, Casgevy) for the treatment of beta-thalassaemia and severe sicklecelldisease. Exagamglogene autotemcel (Exa-cel, Casgevy®) for the treatment of beta-thalassaemia and severe sicklecelldisease - Repository of AIHTA GmbH English | Deutsch Atom RSS 1.0 RSS 2.0 * Simple search * Advanced search * Help * Services * Login * Browse * Type * Subject * Author / Editor * Institution * Year AIHTA - Publications - Search - Exagamglogene autotemcel (Exa-cel, Casgevy®) for the treatment of beta-thalassaemia and severe sicklecelldisease Grössmann-Waniek, N. and Wild, C. and Malíková, E. and Wolf, S. and Fabian, D. and Sehic, O. and Zechmeister-Koss, I.(2025):Exagamglogene autotemcel (Exa-cel, Casgevy®) for the treatment of beta-thalassaemia and severe sicklecelldisease
Interventions for chronic kidney disease in people with sicklecelldisease. Sicklecelldisease (SCD), one of the commonest severe monogenic disorders, is caused by the inheritance of two abnormal haemoglobin (beta-globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Kidney disease is a frequent and potentially severe complication , outcomes are poor as a result of the progression to proteinuria and chronic kidney insufficiency. Up to 12% of people who develop sickle cell nephropathy will develop end-stage renal disease. This is an update of a review first published in 2017. To assess the effectiveness of any intervention for preventing or reducing kidney complications or chronic kidney disease in people with sicklecelldisease
Exagamglogene Autotemcel (Casgevy) - sicklecelldisease View of Exagamglogene Autotemcel (Casgevy) | Canadian Journal of Health Technologies Return to Article DetailsExagamglogene Autotemcel (Casgevy)
Terms of Reference, visit the MSAC website 1. Purpose of application An application requesting the addition of sicklecelldisease (SCD) to newborn bloodspot screening (NBS) was received from Australian Sickle Cell Advocacy Inc by the Department of Health and Aged Care. During the initial review stage, the SickleCellDisease Expert Working Group recommended that beta(β)-thalassaemia, Haemoglobin E-β deferred its advice regarding adding sicklecelldisease (SCD) and beta (β)-thalassaemia to newborn bloodspot screening (NBS) programs. MSAC noted that SCD and β-thalassaemia are rare but severe conditions with high morbidity and mortality, especially SCD. MSAC considered that while adding these haemoglobinopathies to NBS would result in an earlier diagnosis than symptomatic presentation, the strength
of autopsy practice, the evidence level for the majority of the material in this text is Grade D. Nonetheless, many of the clinicopathological scenarios that occur in sicklecelldisease (SCD) and sickle trait persons have been investigated in Coroners’ courts, along with clinical expert witness contributions, and several have gone to High Court litigation for resolution on causality. Thus, the following ; pneumococcal antigen test if suspected. PGD 160823 20 V3 Final Appendix B Cardiac complications in sicklecelldisease Researched and drafted by Dr Naoimh Herlihy. Cardiovascular causes are reported to account for up to 32% of deaths in SCD.28 Several autopsy series have reported cardiac pathologies including heart failure, myocardial ischaemia and infarction, sudden cardiac death and cardiac arrhythmias
Effectiveness of a home-based therapeutic exercise program on lower back pain and functionality in SickleCellDisease (SCD) patients. To investigate the effectiveness of a home-based therapeutic exercise program on lower back pain and functionality of SCD patients. A Hematology and Transfusion Medicine Center, University of Campinas (HEMOCENTRO-UNICAMP). This was a prospective study , with a three-month follow-up of SCD patients with lower back pain. The lumbar spine functionality was evaluated by questionnaires, trunk flexion and extension analyses by fiber-optic-electrogoniometry and measurements of muscle strength of trunk flexor and extensors. The Intervention Group (IG) comprised 18 volunteers, median age 44y (28-58) and the control group (CG) comprised 15 volunteers, median age 42y
Effects of home-based inspiratory muscle training on sicklecelldisease (SCD) patients. Inspiratory muscle training (IMT) has been shown to be an efficient method of improving exercise tolerance and inspiratory and expiratory muscle strength in several diseases. The effects of IMT on patients with sickle cell anemia (SCD) are relatively unknown. Our study aimed to evaluate the effects of IMT on adult SCD patients, regarding respiratory muscle strength (RMS) variables, lung function, exercise tolerance, blood lactation concentration, limitation imposed by dyspnea during daily activities and impact of fatigue on the quality of life. This was a randomized single-blind study, with an IMT design comprising true load (TG) and sham load (SG) groups. Initial assessment included spirometry
Hydroxyurea (hydroxycarbamide) for sicklecelldisease. Sicklecelldisease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea (hydroxycarbamide), an oral chemotherapeutic drug, ameliorates some of the clinical problems of SCD, in particular that of pain, by raising foetal haemoglobin (HbF ). This is an update of a previously published Cochrane Review. The aims of this review are to determine through a review of randomised or quasi-randomised studies whether the use of hydroxyurea in people with SCD alters the pattern of acute events, including pain; prevents, delays or reverses organ dysfunction; alters mortality and quality of life; or is associated with adverse effects. In addition, we hoped