Sickle cell disease
Michael S. Marsch, Janet M. Rennie, Phillipa A. Groves in Clinical Protocols in Labour, 2020
The stress of labour may precipitate a sickle cell crisis. As a general rule patients with sickle cell disease should only be transfused because of a particular clinical situation, not just because they have sickle cell disease. Such situations might be their obstetric or haematological history, or clinical problems in a current pregnancy. The final decision on transfusion will normally be made together by the consultant obstetrician and haematologist. Possible indications include an ongoing high transfusion regime, multiple pregnancy, poor fetal growth or a haemoglobin 15% less than the steady state level. For all these reasons it is essential that: the request form for transfusion be clearly marked indicating that the patient has sickle cell disease and that the transfusion laboratory be given as much notice as possible that a transfusion is required (often at least 3 working days) unless the transfusion is required as a medical emergency.
Physical activity and sickle cell disease
Roy J. Shephard in Physical Activity and the Abdominal Viscera, 2017
This chapter looks at issues in the clinical management of athletes where the risk of splenic rupture is increased because of sickle cell disease. It also looks first at the physiopathology of sickling and its impact upon physical performance and other aspects of health. Sickle cell disease is often associated with poor physical and mental health, a low level of habitual physical activity, cardiac enlargement, non-specific ECG changes and an impaired physical performance. Given that inheritance influences many aspects of athletic performance, it is not surprising that sickle cell trait is associated with success in some types of competition. Some authors have described autonomic disturbances, abnormal capillary blood flow and sudden death in individuals with sickle cell trait even during sleep, but others argue that the evidence supporting sickling episodes while sleeping is not convincing. Homozygotes with overt sickle cell disease must expect painful sickling episodes, a reduced quality of life, pulmonary hypertension and orthopaedic or neurologic complications.
Sickle cell disease
Chris Carter in Critical Care Nursing in Resource Limited Environments, 2019
Sickle cell disease (SCD) is an inherited haemoglobin disorder that affects how the oxygen is transported within the blood. Individuals with sickle cell trait are generally asymptomatic and may be diagnosed accidently, for example from blood investigations prior to surgery or antenatal care. SCD is characterised by a change in the shape of red blood cells. In SCD when red blood cells are oxygenated, they retain the normal cell shape. However, when they become deoxygenated they polymerise causing them to change shape and become long fibres and ‘stick’ together, causing them to become sickle in shape. During a severe sickle cell crisis and acute deterioration patients may require admission to critical care units. Vaso-occlusive crises (VOC) occur when the sickle-shaped cells occlude blood vessels, preventing oxygen and nutrients reaching tissues; this leads to tissue hypoxia, tissue necrosis and severe pain. The underlying cause of VOC is often infection, therefore the cause should be identified and appropriately treated.
Decompensated Cirrhosis and Sickle Cell Disease: Case Reports and Review of the Literature
Published in Hemoglobin, 2017
Roberta D’Ambrosio, Marco Maggioni, Maria F. Donato, Pietro Lampertico, Maria D. Cappellini, Giovanna Graziadei
Although its prevalence is unknown, liver involvement by sickle cell disease is not uncommon and encompasses different clinical spectra including non cholestatic and cholestatic disorders. Few data have been provided on chronic sickle cell intrahepatic cholestasis (SCIC) clinical course, although cirrhosis has been reported in sickle cell disease. However, no effective therapeutic approaches have been recognized either to prevent or treat this condition. Here we present two cases of adult sickle cell disease patients with decompensated cirrhosis. Their liver biopsies showed sickle cell thrombi within the hepatic sinusoids. Despite erythroexchange (EEX) transfusions, both patients suffered from major sickle cell disease-related events, suggesting that EEX transfusions may not be enough to impact on advanced liver involvement by sickle cell disease.
Erythropoietin Levels in Patients with Sickle Cell Disease Do Not Correlate with Known Inducers of Erythropoietin
Published in Hemoglobin, 2014
E. Dianne Pulte, Steven E. McKenzie, Jaime Caro, Samir K. Ballas
Previous studies have suggested that erythropoietin (Epo) levels may be inappropriately low in patients with sickle cell disease compared to the extent of the related anemia they demonstrate. Here, we evaluate Epo level vs. renal function, oxygenation, and markers of inflammation for patients treated for sickle cell disease at our institution. Blood was drawn from 54 patients with sickle cell disease during routine visits to the outpatient hematology office and analyzed for hemoglobin (Hb) level, Epo, markers of inflammation, oxygenation, and renal function. Erythropoietin levels were lower than expected for patients with sickle cell disease, compared to the degree of anemia demonstrated in these patients. In addition, a correlation between Hb level and Epo was not consistently observed. Higher Epo levels were seen in patients receiving hydroxyurea (HU), but no correlation with oxygenation, hemolysis, renal function, or inflammation was observed.
Universal Screening Program in Pregnant Women and Newborns at-Risk for Sickle Cell Disease: First Report from Northern Italy
Published in Hemoglobin, 2017
Mariachiara Lodi, Elena Bigi, Giovanni Palazzi, Lara Vecchi, Riccardo Morandi, Monica Setti, Silvana Borsari, Giuliano Bergonzini, Lorenzo Iughetti, Donatella Venturelli
The implementation of screening programs for early detection of patients with sickle cell disease has become necessary in Italy as a result of the high rate of migration from areas with a high prevalence of the disease (Sub-Saharan Africa, Middle East and the Balkans). Following a pilot study performed in the province of Modena, Italy in 2011–2013, an official screening program was established on May 31 2014 for all pregnant women, free-of-charge for the family according to the National Guidelines for Physiological Pregnancy. Hemoglobin (Hb) profiles of pregnant women within 10 weeks of pregnancy, of new mothers at delivery and of the newborns of mothers with variant Hb profiles (newborns at-risk), were evaluated by high performance liquid chromatography (HPLC). Samples from 17,077 new mothers were analyzed and 993 showed alteration of Hb patterns (5.8%) (1.0% Hb AS carriers); of the 1011 at-risk newborns, four (0.4%) carried sickle cell disease and 90 (8.9%) were Hb AS carriers. These data show that early diagnosis of sickle cell disease or carrier status can be obtained in high-risk newborns, providing valuable information on the frequency of these conditions in geographic areas in which the disease is historically rare.
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