Diagnosing sickle cell disease involves the use of the more expensive gene analysis or hemoglobin electrophoresis, isoelectric focusing, and high-performance liquid chromatography. There is no curative treatment for sickle cell disease, except for bone marrow stem cell transplantation, which is extremely limited in use. The most potent current therapy available is the anticancer drug hydroxyurea, with its ability to raise the levels of fetal hemoglobin and thereby reduce the frequency and intensity of the life-threatening crisis events of sickle cell disease. Managing pain is the key to the satisfactory management of sickle cell disease. However, the lack of adequate knowledge of pain management techniques quite often leads to unsatisfactory outcomes from the patient’s perspective. Research continues in the hope of finding an effective cure for sickle cell disease
Gulbis et al, 2005 p.309, describes sickle cell disease as “a genetic disorder involving hemoglobin designated as hemoglobin S, an autonomic recessive hereditary disease. This inherited disease causes the normally round and flexible red blood cells to become stiff and shaped like a sickle, from whence it derives its name. The stiff sickle-shaped blood cells no longer flow through blood vessels easily, hampering blood supply to the tissues. The resultant inadequate blood supply is responsible for the characteristic features of pain and respiratory difficulties experienced by patients suffering from sickle cell disease, and it is also responsible for organ damage in sickle cell disease. These sickle-shaped blood cells die much faster than normal red blood cells, which lead to anemia. In essence, the genetic alteration that occurs in sickle cell disease is the single base-pair change in the gene that is responsible for encoding a part of the hemoglobin protein. Such alteration results in distorted hemoglobin protein production, which does not function adequately.