Sickle-Cell Pain: Advances in Epidemiology and Etiology

Abstract

New epidemiological findings recast pain in sickle-cell disease (SCD) as being more often a chronic manifestation than was previously thought, although acute pain is still the hallmark of the disease. SCD pain intensity, the number of painful locations, and the frequency of hospitalizations due to SCD pain may worsen with age. In adults and even in children, the quantity and severity of SCD pain may be vastly underestimated, because most of the "iceberg" of SCD pain is "submerged" at home, and only the tip of the iceberg is seen by health care providers when acute SCD care is rendered in emergency rooms and hospitals. Implications of this "iceberg phenomenon" are significant for clinicians, researchers, employers, policy makers, and the public. Nevertheless, both emergency room and hospital utilization for SCD pain remain prevalent. Often, utilization recurs early, perhaps emblematic of poor acute pain management. New data show the protean impacts of SCD pain on health-related quality of life, sleep, and psychological and social health. The relationship of the severity of SCD pain to the severity of underlying sickle vasculopathy is unclear, but epidemiologic evidence and patient descriptors suggest a temporal evolution of pain mechanisms. At first, increasingly worse nociceptive pain from vaso-occlusion and local lesions may evolve over the first two decades of life. Then, in the third and following decades, central neuropathic pain may also evolve due to past and continuing nociceptive stimuli. New findings confirm environmental contributors to SCD pain, including seasonal (colder) temperatures, barometric pressure, and wind speed.

Impact of hydroxyurea on clinical events in the BABY HUG trial

“…However, particularly for painful events, self-report is required to identify the extent of disease. 35,36 Study coordinators were diligent in documenting clinical events at each monthly study visit, records were obtained from pediatricians and outside hospitals/emergency departments to document events, and the MCC reviewed source data for 100% of events.…”

The impact of the oral condition of children with sickle cell disease on family quality of life

“…1 The severity of clinical manifestations depends on the genotype and the environment. 1 The homozygous form (HBSS) is the most severe genotype.…”

“…1 The severity of clinical manifestations depends on the genotype and the environment. 1 The homozygous form (HBSS) is the most severe genotype. 2 Oral complications have been reported but these are not as common as other complications.…”

Avaliação da qualidade de vida em pacientes com anemia falciforme: diferenças entre adultos e crianças

“…In relation to satisfaction with personal relationships (friends, relatives, acquaintances, colleagues), 44.4% considered themselves satisfied, and being that same percentage was found as they researched to satisfaction with sex life, that is, the majority stated be satisfied. More than half 5,10 however, our findings may reflect an appropriate medical monitoring, as well as patient compliance due to greater availability of effective treatment and also currently offered by specialized services.…”

“…1,2 Because it is the most prevalent hereditary disease in the country, and frequently present delayed diagnosis with high morbidity and mortality, sickle cell anemia should be considered a public health problem in Brazil and in the world. 3,4,5 It is believed that patients with sickle cell disease has a quality of life (QoL) very low and that their overall socio-economic and cultural deficiency also point to several aspects, 6,7 so that the they has a better health status and QoL is needed proper guidance and assistance, both in medical and psychosocial terms, as well as studies that address the public health aspects.…”