Hematological Diseases and Syndromes in the Neonate: Haemoglobin, Haemoglobinopathies, and Oxygen Therapy

Abstract

Fetal haemoglobin is the main haemoglobin in the fetus, newborn, and the neonate. Besides HbF, there are several embryonic Hbs in the fetal life. Increase in their concentration and presence of abnormal HB is a cause of fetal demise and early neonatal death. Various hematological diseases and clinical syndromes can have severe adverse consequences on the growth of the fetus. The diagnosis can be extremely difficult in the neonate, and it requires early detection by perinatal and neonatal screening, especially in babies of mothers with a positive family history. Undiagnosed syndromes (sickle cell disease, thalassemia, variants of Hb) can impact the surgery and intra operative course, with high risk of complications, need for blood transfusion and its risks, and poor postoperative outcomes. Oxygen is the most common drug used in newborns, right since birth, in ICU and during anaesthesia and in the perioperative period. It is important for the anaesthesiologist and clinicians caring for these babes to have detailed knowledge about the dosing, indications, modes of therapy, the risks involved, and measures recommended, because both high and low oxygen are deleterious to them unlike in adults. Close observation of the baby for development of new signs and exquisite communication between the neonatologist, anaesthesiologist and the surgeon can go a long way in improving their survival after surgery under general anaesthesia. This chapter will be dealt in four sections: (1) Haemoglobin, (2) sickle cell disease, (3) thalassemia, and (4) oxygen therapy.