Sickle cell Disease was first described by James B. Herrick in November 1910. Since its first record, it has been detected in many part of the world including Orissa.
Sickle Cell Disease is a chronic inherited disease transmitted as Mendelian recessive character. It is a disease of hemoglobin structure where the glutamic acid in the 6th position of Beta Chain is replaced by valine. This disease may be present either in homozygous state or in conjunction with other type of normal or abnormal haemoglobin. The clinically significant sickle cell haemoglobinopathy include Sickle Cell Trait, Sickle Cell Disease, Sickle Beta thalassemia, Sickle Cell ‘C' Disease, Sickle Cell ‘D’ disease and Sickle ‘0’ Arab disease etc. These may present with different clinical severity ranging from mild symptoms to fatal complications. They usually present as chronic haemolysis, Sickle Cell crisis, growth retardation, increased susceptibility for infection and variety of other acute and chronic, complications that produce multi system organ damage, disability and death. Their clinical severity depends upon presence of precipitating factors or association with other abnormal or normal haemoglobin variants.
The most important clinical presentation is Sickle Cell crisis. The Sickle Cell Crisis was defined by Diggs L.W. in 1965 as “a sharp turn on the course of the disease with rapid development of new symptoms not explained by any other cause". There are four types of crises, such as vaso-occlusive crisis, Aplastic crisis, Haemolytic crisis and Sequestration crisis. The Vaso-occulusive crisis is the commonest and is very painful. The Vaso-occlusive crisis is defined as sudden onset of excruciating pain in different parts of the body particularly extremities, chest, abdomen etc. due to occlusion of microcirculation by the sickled erythrocytes. The crises are precipitated by many factors like anoxia, infection, acidosis, dehydration, exposure to cold, pregnancy, emotional stress etc.
Despite substantial increase in knowledge in cellular, in pathological and physical basis of sickling phenomenon, there is some therapy available for prevention of crisis. There is some hope in gene replacement therapy and bone marrow transplantation in future and cure of the disease.
Currently accepted management of painful crisis includes.
1. Prophylactic measures to reduce the incidence or crisis and
2. Therapy to relieve- or reverse the established crisis by
a) Early detection and prompt management of precipitating factor- infection should be treated with suitable antibiotic, hypoxaemia with oxygen, acidosis with alkali and dehydration with adequate IV fluid.
b) Analgesics for control of pain
c) Stabilization of Red Blood Cell membrane
d) Vasodilatation to improve microcirculation
Many drugs like phenothiazine, Nitrates, Urea, Nicotinic acid, Dextran, alkali, Androgen, Aspirin, Desmopressin acetate, 5 azacytidine etc. have been tried with partial or no benefit and none of them are also safe. New compounds continue to be sought, that might interfere with sickling and be useful clinically. Aspartame is the only agent that can prevent sickling and reverse sickling tested in-vitro and in-vivo so far (Manion et al, 2001). Therefore this study is undertaken to know the efficacy of drug (Aspartame) in sickle cell disease and sickle cell crisis.
A glucose analogue aspartame (L. aspartyl L. Phenylalanine) a sweetener prepared from 2 amino acids -  - aspartic acid and L-phenylalanine is appears to be beneficial effects in sickle cell disease and sickle cell crisis. This drug can prevent fibril formation and / or interaction with or by getting inserted into the EF acceptor site and prevent the  - helix that contain valine  from its delectation binding to the EF acceptor site and thus obstruct sickling. A possibility of functional interaction of intracellular biopolymerization may occur appears to be possible mechanism (Manion et al, 2001).