In the U.S., the common SCD genotypes include: sickle cell anaemia (HbSS), HbS/β° thalassaemia, and HbS/β+ thalassaemia [7]. Hydroxyurea can cause serious side effects, like a plunge in the number of white blood cells that help your body fight infections. Jude Children's Research Hospital: "Bone Marrow (Stem Cell) Transplant for Sickle Cell Disease," “Red Blood Cell Transfusions for Sickle Cell Disease.”The Internet Stroke Center: "Stroke as a Complication of Sickle Cell Disease," “Blood Transfusions for Children With Sickle Cell Disease.”American Family Physician: “Practical Tips for Preventing a Sickle Cell Crisis.”Mayo Clinic: "Sickle Cell Anemia: Symptoms and Causes,” “Sickle Cell Anemia: Diagnosis and Treatment. It may therefore enhance HbF production indirectly, by killing rapidly proliferating late erythroid cells [41,42].

Mean age when respondents started using Hydroxyurea was 4.8 years (SD=2.6), with median duration of use at 2.1 years (IQR=1.3-4). However, in SCD, low oxygen conditions result in the polymerisation of the pathological haemoglobin which downstream leads to the SCD signs and symptoms, including vaso-occlusive events, chronic haemolytic anaemia, organ dysfunction, and increased infections.A comprehensive review of literature was conducted using the computerised search databases, PubMed and Ovid MEDLINE. Hydroxycarbamide, also known as hydroxyurea, is a medication used in sickle-cell disease, chronic myelogenous leukemia, cervical cancer, and essential thrombocythemia.
Hydroxyurea is also used to reduce pain episodes and the need for blood transfusions in people with sickle cell anemia. However, the process is multifaceted and dynamic incorporating stimulatory interactions of the vascular endothelium to increase numerous adhesion molecules, such as integrin. In numerous studies in both children and adults with sickle cell anemia, hydroxyurea treatment has been associated with fewer painful crises, fewer acute chest syndrome (lung complication) events, reduction in the need for transfusion, and reduction in the need for hospitalization. A small Indian based study also reported that children treated with hydroxyurea experienced decreased numbers of vaso-occlusive events, hospitalisations, and blood transfusions required, despite the low doses of drug administered [50]. Hydroxyurea is used to treat chronic myeloid leukemia, ovarian cancer, and certain types of skin cancer (squamous cell cancer of the head and neck). Despite much evidence supporting the clinical efficacy for SCD hydroxyurea treatment, its HbF induction mechanism of action continues to be largely unknown [39].Hydroxyurea is a short-acting cytotoxic ribonucleotide reductase inhibitor which acts to arrest S-phase cells by impairing their DNA replication [40].

Thus, to summarise, SCD is a complex hereditary disorder in which for a medical practitioner to effectively manage requires a comprehensive understanding of both normal haemoglobin physiology and its pathophysiology.1. Background Hydroxyurea is an effective treatment for sickle cell anemia, but few studies have been conducted in sub-Saharan Africa, where the burden is greatest. THe medicines hydroxyurea (Droxia, Hydrea, Siklos) and voxelotor (Oxbryta) prevent abnormal red blood cells from forming. Ⓒ 2020 About, Inc. (Dotdash) — All rights reserved What Patients Should Consider Treatment With Hydroxyurea? Hydroxyurea also increases your However, this hydroxyurea recommendation is weak due to the limited sample population when likened to the size of HbSS data.To conclude, this literature review has discussed the normal physiology of haemoglobin, pathogenesis of sickle cell disease as well as its course of treatment. NO regulates vasodilation via the stimulation of cGMP dependent protein kinases, it also decreases platelet aggregation, and inhibits the release of endothelin-1, the powerful vasoconstrictor [27,28]. Chromosome 16 contains the genes for the α-chain and chromosome 11 has those for the β-chain [3].The other type of haemoglobin is foetal haemoglobin (HbF) and is clinically and therapeutically significant in SCD, as discussed below.

It is important to note, however, that most individuals in this study had abnormal sperm prior to commencing hydroxyurea treatment, thus it was difficult to establish the precise contribution of hydroxyurea. It is a safe drug and there is no evidence to suggest increases the risk of cancer in patients with sickle cell disease.

There is opportunity for future research into both the exact hydroxyurea mechanism of action in SCD patients and hydroxyurea efficacy and dangers when used to treat SCD patients expressing one of the rare genotypes. It is taken by mouth.

However, two cohort studies from Italy and Greece have shown that hydroxyurea efficacy observed in HbSS individuals also extends to those with HbS/β+ thalassaemia [54,55]. Subsequent systemic disturbances and pain can be felt downstream from the blockage, caused by ischemia, necrosis and organ dysfunction. Hydroxyurea has multiple posited mechanisms of action but more importantly is the only SCD treatment that targets the underlying pathology.