Management of chronic respiratory complications in children and adolescents with sickle cell disease

Abstract

Sickle cell disease (SCD) is a life-threatening hereditary blood disorder that affects millions of people worldwide, especially in sub-Saharan Africa. This condition has a multi-organ involvement and highly vascularised organs, such as the lungs, are particularly affected. Chronic respiratory complications of SCD involve pulmonary vascular, parenchymal and airways alterations. A progressive decline of lung function often begins in childhood. Asthma, sleep-disordered breathing and chronic hypoxaemia are common and associated with increased morbidity. Pulmonary hypertension is a serious complication, more common in adults than in children. Although there is a growing attention towards respiratory care of patients with SCD, evidence regarding the prognostic meaning and optimal management of pulmonary issues in children with this condition is limited.This narrative review presents state-of-the-art evidence regarding the epidemiology, pathophysiology and therapeutic options for chronic respiratory complications commonly seen in paediatric patients with SCD. Furthermore, it highlights the gaps in the current knowledge and indicates future directions for studies that aim to improve our understanding of chronic respiratory complications in children with SCD.

Sickle Cell Disease and Its Respiratory Complications

“…The symptoms of PH are nonspecific and can present as shortness of breath or dyspnea on exertion, fatigue, chest pain, and syncope. Signs of right heart failure, such as edema and jugular venous distension, can also be present on examination but usually appear in the late stage of the disease [32]. There are two factors causing hemolysis in SCD.…”

Anemia de células falciformes: Evaluación de la mecánica pulmonar y pruebas para medir los cambios en la difusión pulmonar

“…En la enfermedad de anemia de células falciformes (ACF) se presentan alteraciones pulmonares, y es una causa de morbimortalidad. El daño pulmonar puede obedecer a lesiones en el parénquima pulmonar , las vías respiratorias y a nivel vascular con lo que puede haber complicaciones agudas y crónicas [1,2]. Durante la realización de las pruebas de función pulmonar son normales, pero puede experimentar alteraciones conforme van creciendo (probable por las alteraciones crónicas en los órganos) [3][4][5][6][7].…”

Pediatric pulmonary year in review 2021: Sleep medicine

“…Causes of nocturnal hypoxemia in patients with SCD may be broad. Chronic lung disease and associated ventilation‐perfusion mismatch, 17,18 pulmonary hypertension and intracardiac/intrapulmonary shunting, 19–21 and severe anemia and right shift of the oxygen‐hemoglobin dissociation curve 22 can all contribute to hypoxemia. Nourani et al 23 explored the association between nocturnal hypoxemia and acute chest syndrome (ACS), a severe complication of SCD that can lead to respiratory failure and is the leading cause of death in this population 24 .…”