Sickle Cell Disease in Early Infancy: A Case Report

Abstract

Sickle cell disease (SCD) refers to a group of hereditary disorders that result in faulty hemoglobin carriage by the red blood cells. This paper discusses an atypical presentation of SCD in early infancy. Despite current literature suggesting protection by fetal hemoglobin in the first few months of life, we report a diagnosis of SCD at 2 months of age with severe symptoms requiring hospitalization. It is therefore important for clinicians to raise their clinical index of suspicion of SCD in children presenting with severe anemia even though they are less than 6 months old and do not present with classic dactylitis or pain syndromes. Expansion and sustained newborn screening programs for SCD in developing countries could help clinicians and parents plan for early treatment, appropriate prophylaxis, and improved management of SCD complications.

Keywords:

• sickle cell disease
• severe anemia
• malaria
• invasive pneumococcal disease
• newborn screening
• infancy

Abbreviations

SCD, sickle cell disease; RBC, red blood cell; HbF, fetal haemoglobin; IPD, invasive pneumococcal disease; BCG, Bacille Calmette-Guerin; DPT, diphtheria-pertussis-tetanus vaccine; PCV, pneumococcal conjugate vaccine; OPV, oral polio vaccine; HB, hemoglobin; VOC, vaso-occlusive crisis.

Ethics Statement

Ethical clearance for the parent study was obtained from the University of Zambia Biomedical Research ethics Committee. Written informed consent was obtained from the participant’s mother for study participation and publication of case details. The case details cannot be made publicly available because it contains human research participant data; however, de-identified data can be made available to any interested researchers upon request through the Centre for Infectious Disease Research in Zambia (CIDRZ) Ethics and Compliance Committee. To request data access, one must write to the Secretary to the Committee/Head of Research Operations through this email address: [email protected], mentioning the intended use for the data, contact information, a research project title, and a description of the analysis being proposed as well as the format it is expected. The requested data should only be used for purposes related to the original research or study. The CIDRZ Ethics and Compliance Committee will normally review all data requests within 48–72 hours (Monday–Friday), and provide notification if access has been granted or additional project information is needed.

Acknowledgments

We would like to acknowledge the CIDRZ Chawama clinical research site team for their support during the data collection process.

Disclosure

All authors report no conflicts of interest in this work.

Additional information

Funding

The funding for the parent trial is from MRC grant MR/T030321/1.