Paediatric Association of Nigeria Guidelines on the Management of Acute Chest Syndrome in Children with Sickle Cell Disease (2023)

Abstract

Background
Acute Chest Syndrome (ACS) is a major cause of hospitalisation, a potentially life-threatening complication and a leading cause of mortality in children with Sickle Cell Disease (SCD). The affected child commonly presents with respiratory symptoms such as cough, breathlessness, chest pain, jitteriness, and confusion, with or without fever. The cause of this condition is multifactorial and sometimes unidentified, but the majority are due to infection, infarction, and fat embolism. ACS and its related complications may be minimised by prompt intervention and appropriate therapy, including the use of incentive spirometry and  blood transfusion.

Objective
The development of a national guideline on the management of ACS in children with SCD under 18 years in Nigeria is meant to enhance early diagnosis and prompt treatment of ACS to improve the quality of care and clinical outcome and prevent death from ACS. It is intended to enhance the clinician's diagnostic capability and ensure that children with ACS receive the best available care.

Methods
This evidence-based guideline was adapted from the British Society of Haematology (BSH) and the American Society of Hematology (ASH) guidelines using the ADAPTE (Resource tool kit version 2.1) and AGREE II  methods.

Results
The PAN Guideline Panel reached a consensus on 25 recommendations, three of which were modified and adapted for local use. The recommendations reflect a broad definition of ACS and a management approach, including blood transfusion and incentive spirometry, such as blowing latex balloons in the absence of a spirometer.

Conclusions
Most recommendations are conditional because of low-certainty evidence and closely balanced benefits and harms (benefits of therapy to patients and availability of such therapy). Patient preferences should drive clinical decisions. Randomised controlled trials and comparative-effectiveness studies are needed for optimal management of blood transfusion, fluid therapy, and use of oxygen.