Management of Sickle Cell Disease in Children

Abstract

Sickle cell disease (SCD) is a heterogeneous inherited disorder of hemoglobin that causes chronic hemolytic anemia, vaso-occlusion, and endothelial dysfunction. These physiologic derangements often lead to multiorgan damage in infancy and throughout childhood. The most common types of SCD are homozygous hemoglobin S (HbSS disease), hemoglobin SC disease, and sickle β thalassemia. HbSS disease and sickle β(0) thalassemia often are referred to as sickle cell anemia because they have similar severity. Screening and preventive measures, including infection prophylaxis and vaccination, have significantly improved outcomes for children with SCD. Evidence-based therapies, such as hydroxyurea and transfusion, play an important role in preventing progression of select complications. Many chronic complications develop insidiously and require multidisciplinary care for effective treatment. Primary care physicians, as well as physicians in many other disciplines, may care for these patients and should be familiar with the potential acute and chronic complications of this disease. This review addresses healthcare maintenance guidelines, common complications, and recommendations for management of pediatric patients with SCD.

Interleukin 6 as a Marker of Severe Bacterial Infection in Children with Sickle Cell Disease and Fever: A Case-Control Study

“…Despite the low proportion of con rmed SBI in this cohort, almost all patients received at least one dose of antibiotic (96.2%), in agreement to current guidelines [18][19][20].This high use of antibiotics emphasizes the importance of de ning laboratory parameters that could early discriminate the etiology of fever in these patients, in order to optimize the antimicrobial prescription. Children with SBI presented more frequently with hemodynamic instability and had higher in ammatory parameters (neutrophils, CRP and procalcitonin) than the rest of the patients, similarly to what was described in other studies [1,[5][6][7][8][9].…”

<p>Depression and Anxiety as Moderators of the Pain-Social Functioning Relationship in Youth with Sickle Cell Disease</p>

“…It builds on previous work supporting the need for multidisciplinary approaches to care for youth with SCD who experience pain. 33,34 It also provides support for the applicability of Valrie's biopsychosocial model of pediatric pain 14 for understanding the experiences of youth with SCD, and rationale for future studies to investigate the possible moderating role of depression and anxiety symptoms on other functional outcomes in youth with SCD and other pediatric pain populations.…”

Child with unusual combination of sickle cell disease and autosomal recessive agammaglobulinemia associated with a novelCD79agene mutation

“…SCD is most common among people from Africa, Mediterranean countries, India and South and Central America. This disorder causes chronic haemolytic anaemia, vasoocclusion and major infection risk, specially by encapsulated bacteria due to splenic dysfunction and decreased opsonisation capacity 18 19. The association of these two conditions has obvious implications on patient immune function, significantly increasing the risk of recurrent and serious infections.…”