Haematologyhttp://hdl.handle.net/20.500.12904/1672024-03-28T09:23:34Z2024-03-28T09:23:34ZThe indignities of shielding during the COVID-19 pandemic for people with sickle cell disorders: an interpretative phenomenological analysisWebster, Amyhttp://hdl.handle.net/20.500.12904/183612024-03-13T15:21:03Z2024-02-13T00:00:00ZThe indignities of shielding during the COVID-19 pandemic for people with sickle cell disorders: an interpretative phenomenological analysis
Webster, Amy
This article seeks to understand the first-hand experiences of people with sickle cell, a recessively inherited blood disorder, who were identified as clinically extremely vulnerable during the COVID-19 pandemic. Part of a larger sequential mixed-methods study, this article uses a selective sample of eight qualitative semi-structured interviews, which were analysed using interpretative phenomenological analysis (IPA). The first stage of IPA focused on practical concerns participants had correlated to understanding shielding and their feelings about being identified as clinically extremely vulnerable. In a secondary stage of analysis, we examined the emotions that it brought forth and the foundations of those based on discriminations. This article adds to our theoretical understanding of embodiment and temporality with respect to chronicity and early ageing. It explains how people with sickle cell disorders have an embodied ethics of crisis and expertise. It also elucidates how people's experiences during the pandemic cannot be seen in void but illustrates ableism, racism, and ageism in society writ large.
2024-02-13T00:00:00ZLong-term treatment with rilzabrutinib in patients with immune thrombocytopeniaGarg, Mamtahttp://hdl.handle.net/20.500.12904/183442024-03-13T14:25:11Z2024-02-22T00:00:00ZLong-term treatment with rilzabrutinib in patients with immune thrombocytopenia
Garg, Mamta
mmune thrombocytopenia (ITP) is an autoimmune disease associated with autoantibody-mediated platelet destruction and impaired platelet production, resulting in thrombocytopenia and a predisposition to bleeding. The ongoing, global phase 1/2 study showed that rilzabrutinib, a Bruton tyrosine kinase inhibitor specifically developed to treat autoimmune disorders, could be an efficacious and well-tolerated treatment for ITP. Clinical activity, durability of response, and safety were evaluated in 16 responding patients who continued rilzabrutinib 400 mg twice daily in the long-term extension (LTE) study. At LTE entry, the median platelet count was 87×109/L in all patients, 68×109/L in those who had rilzabrutinib monotherapy (n=5), and 156×109/L in patients who received concomitant ITP medication (thrombopoietin-receptor agonists and/or corticosteroids, n=11). At a median duration of treatment of 478 days (range, 303-764), 11 of 16 (69%) patients continued to receive rilzabrutinib. A platelet count of ≥50×109/L was reported in 93% of patients for more than half of their monthly visits. The median percentage of LTE weeks with platelet counts ≥30×109/L and ≥50×109/L was 100% and 88%, respectively. Five patients discontinued concomitant ITP therapy and maintained median platelet counts of 106×109/L at 3-6 months after stopping concomitant ITP therapy. Adverse events related to treatment were grade 1 or 2 and transient, with no bleeding, thrombotic, or serious adverse events. With continued rilzabrutinib treatment in the LTE, platelet responses were durable and stable over time with no new safety signals. This trial is registered at www.clinicaltrials.gov NCT03395210 and https://www.clinicaltrialsregister.eu EudraCT 2017-004012-19.
2024-02-22T00:00:00ZMyocardial calcium handling in type 2 diabetes: a novel therapeutic targetDattani, AbhishekSingh, AnveshaMcCann, Gerry PGulsin, Guarav Shttp://hdl.handle.net/20.500.12904/182052024-02-02T14:43:08Z2023-12-23T00:00:00ZMyocardial calcium handling in type 2 diabetes: a novel therapeutic target
Dattani, Abhishek; Singh, Anvesha; McCann, Gerry P; Gulsin, Guarav S
Type 2 diabetes (T2D) is a multisystem disease with rapidly increasing global prevalence. Heart failure has emerged as a major complication of T2D. Dysregulated myocardial calcium handling is evident in the failing heart and this may be a key driver of cardiomyopathy in T2D, but until recently this has only been demonstrated in animal models. In this review, we describe the physiological concepts behind calcium handling within the cardiomyocyte and the application of novel imaging techniques for the quantification of myocardial calcium uptake. We take an in-depth look at the evidence for the impairment of calcium handling in T2D using pre-clinical models as well as in vivo studies, following which we discuss potential novel therapeutic approaches targeting dysregulated myocardial calcium handling in T2D.
2023-12-23T00:00:00ZGuidelines for the diagnosis and management of adult aplastic anaemia: A British Society for Haematology GuidelineGarg, Mamtahttp://hdl.handle.net/20.500.12904/182042024-02-02T13:40:04Z2024-01-21T00:00:00ZGuidelines for the diagnosis and management of adult aplastic anaemia: A British Society for Haematology Guideline
Garg, Mamta
Pancytopenia with hypocellular bone marrow is the hallmark of aplastic anaemia (AA) and the diagnosis is confirmed after careful evaluation, following exclusion of alternate diagnosis including hypoplastic myelodysplastic syndromes. Emerging use of molecular cyto-genomics is helpful in delineating immune mediated AA from inherited bone marrow failures (IBMF). Camitta criteria is used to assess disease severity, which along with age and availability of human leucocyte antigen compatible donor are determinants for therapeutic decisions. Supportive care with blood and platelet transfusion support, along with anti-microbial prophylaxis and prompt management of opportunistic infections remain key throughout the disease course. The standard first-line treatment for newly diagnosed acquired severe/very severe AA patients is horse anti-thymocyte globulin and ciclosporin-based immunosuppressive therapy (IST) with eltrombopag or allogeneic haemopoietic stem cell transplant (HSCT) from a matched sibling donor. Unrelated donor HSCT in adults should be considered after lack of response to IST, and up front for young adults with severe infections and a readily available matched unrelated donor. Management of IBMF, AA in pregnancy and in elderly require special attention. In view of the rarity of AA and complexity of management, appropriate discussion in multidisciplinary meetings and involvement of expert centres is strongly recommended to improve patient outcomes.
2024-01-21T00:00:00Z