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VOLUME 16 , ISSUE 3 ( July-September, 2022 ) > List of Articles

REVIEW ARTICLE

Hemoglobinopathies in Perinatal Medicine: Challenges in Management

Aliyu Labaran Dayyabu

Keywords : Challenges, Hemoglobinopathies, Management, Medicine, Perinatal

Citation Information : Dayyabu AL. Hemoglobinopathies in Perinatal Medicine: Challenges in Management. Donald School J Ultrasound Obstet Gynecol 2022; 16 (3):222-237.

DOI: 10.5005/jp-journals-10009-1936

License: CC BY-NC 4.0

Published Online: 18-10-2022

Copyright Statement:  Copyright © 2022; The Author(s).


Abstract

Introduction: Hemoglobinopathies are a group of genetic disorders of hemoglobin (Hb) structure or synthesis. They are among the most common inherited diseases. Approximately 7% of the global population is a carrier, and 300,000–500,000 children are born with a severe Hb disorder annually. They are classified according to the impaired globin chains and whether the disorder leads to reduced production of a normal chain or an abnormal tertiary structure of globin chains. There are two types of hemoglobinopathies, sickle cell disease (SCD), and thalassemia. For years, these diseases impair the quality of life of those afflicted. Impairment in the quality of life can be due to the disease, its complications or even as a result of its treatment. Mortality and morbidity from these diseases are staggering, and many of those affected do not survive to adulthood. However, as medical science develops, the etiology and treatment of the disease are now almost completely unraveled. These have enabled the provision of quality care to those affected with concomitant improvement in quality of life and survival for those affected. Those that get pregnant now constitute a high-risk cohort who require meticulous quality prenatal, intrapartum, and postpartum care to survive. Such care is widely available in developed countries; however, in developing countries where the burden of the disease is highest, such care is scarce, and maternal and perinatal morbidity and mortality remain high. Methods: This review is done through extensive literature search from various sources. It first discusses sickle disease and then the thalassemias. Aims: The review is aimed at summarizing the disease and its management in pregnancy with the hope that physicians will use the acquired knowledge of the disease and the various strategies to improve the quality of life and survival of patients and their fetuses in pregnancy and postpartum. Conclusion: Hemoglobinopathies are a group of genetic disorders of hemoglobin (Hb) structure or synthesis. There are two types of hemoglobinopathies, sickle cell disease (SCD), and thalassemia. Mortality and morbidity from these diseases are staggering, and many of those affected do not survive until adulthood. Those that get pregnant now constitute a high-risk cohort who require meticulous quality prenatal, intrapartum, and postpartum care to survive. The aim of the review is to provide current overview of the disease and its management with the hope that physicians will use the knowledge to improve the quality of life and survival of pregnant women afflicted with the disease to go through pregnancy safely.

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