Background: Patients with sickle cell disease cope with their disease in various ways, such as psychological counseling, hypnosis, medication, and prayer. Spirituality is a coping mechanism in a variety of diseases. This study evaluates the role of spirituality in patients coping with the pain of sickle cell disease.
Methods: Seventy-one patients from the Georgia Sickle Cell Clinic completed a questionnaire addressing their ability to cope with the pain of sickle cell disease and their degree of spirituality. A descriptive cross-sectional design was used. Correlation and multiple regression analyses were calculated for the relation between coping with the pain of sickle cell disease and spirituality.
Results: The questionnaire provided several scales with high internal consistency for measuring spiritual well-being and its two components, existential well-being and religious well-being, that show a correlation between high levels of spirituality and life control. The study population exhibited high levels of spirituality and religiosity, but the influence of these feelings on coping with sickle cell disease was variable. Spiritual well-being was correlated with life-control but not with perceived pain severity.
Conclusions: Existential well-being was associated with general coping ability. Spiritual well-being is important for some patients who must cope with the pain of sickle cell disease.
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Rheumatoid arthritis (RA) is rarely reported among patients with sickle cell disease (SCD). RA treatment in these patients is believed to be more challenging due to fear of increasing the risk of infection and complications of SCD. We are reporting 7 patients with concurrent SCD and RA.
View Article and Find Full Text PDFExpert Rev Respir Med
January 2025
Department of Surgery, Dentistry, Pediatrics and Gynaecology, Pediatric Division, University of Verona, Verona, Italy.
Introduction: Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by the production of sickle hemoglobin, leading to red blood cells sickling and hemolysis in hypoxic conditions. The resulting acute and chronic endothelial inflammation leads to chronic organ damage. Respiratory manifestations in SCD usually start from childhood and represent the leading causes of morbidity and mortality.
View Article and Find Full Text PDFHaematologica
January 2025
Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati OH; University of Cincinnati College of Medicine, Cincinnati OH; Global Health Center, Cincinnati Children's Hospital Medical Center, Cincinnati OH.
Over the past 40 years, the introduction and refinement of hydroxyurea therapy has led to remarkable progress for the care of individuals with sickle cell anemia (SCA). From initial small proof-of-principle studies to multi-center Phase 3 controlled clinical trials and then numerous open-label studies, the consistent benefits of once-daily oral hydroxyurea have been demonstrated across the lifespan. Elevated fetal hemoglobin (HbF) serves as the most important treatment response, as HbF delays sickle hemoglobin polymerization and reduces erythrocyte sickling.
View Article and Find Full Text PDFPediatr Blood Cancer
January 2025
Division of Pediatric Hematology/Oncology, Hasbro Children's Hospital, Providence, Rhode Island, USA.
Rev Med Interne
January 2025
Service de médecine interne, centre national de référence des syndromes drépanocytaires majeurs de l'adulte, hôpital européen Georges-Pompidou, AP-HP, 20, rue Leblanc, 75015 Paris, France; Inserm U1163, laboratoire « Mécanismes cellulaires et moléculaires des désordres hématologiques et implications thérapeutiques », institut Imagine, université Paris-Cité, 75015 Paris, France; Laboratoire d'Excellence GR-Ex, 75015 Paris, France; Faculté de santé, université Paris-Cité, 75006 Paris, France. Electronic address:
Introduction: Extramedullary hematopoiesis (EMH) is very rarely described during sickle cell disease (SCD). Our aim was to describe six cases of EMH occurring in adult SCD patients and to conduct a literature review.
Methods: Retrospective, descriptive, and monocentric study, identifying all cases of EMH recorded in our cohort of adult SCD patients, up to April 2024.
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