Sickle cell disease is an inherited gene disease that manifests itself through affecting the red blood cells by modifying their shape and rigidity. Red blood cells have a round shape; they are flexible and smooth. Sickle cell patients have stiff red blood cells that portray a letter C in shape. Sickle cell disease is a gene disorder of the red blood cells that occurs when the hemoglobin protein is defective and deficient (Embury, 1994). This paper discusses the symptoms, care and management of sickle cell disease in children, as well as possible advancement of therapies available to patients. It also highlights the possible management of various disorders and disease conditions that may affect sickle cell patients.
Various researches indicate that sickle cell disease usually develops in people of African, African American and Hispanics origins. The medical fraternity has progressively tried to develop measures that could decrease the mortality of infants with sickle cell disease. Screening of infants carried in hospital helps detect those with sickle cell disease. This helps to manage proper comprehensive medical care of the affected children and provides psychological education on the disease management for their parents. Early researches documented high mortality rates of infants with sickle cell disease in their first five years. Children’s death was caused by different infections and sequestration of the spleen. Optimal health care maintenance for children with sickle cell disease involves integration of pediatric hematologists, nurses, social workers, psychologists and genetic counselors (Alston, Valrie, & East Carolina University, 2011). Pediatric hematologist educates the family on the needs of the infant or child and provides support for the family. Nurses take clinical examination of a child and prescribe drugs for any developing ailments. Psychologist offers emotional support to the family and educates parents on how to provide their affected child with support and care.
Sickle cell disease affects the shape of the red blood cells transforming them from round shape into C-shape. This derives from the deoxygenating of hemoglobin S, which causes replacement of glutamic acid that is in charge of interaction with the other hemoglobin molecule. This combination leads to an appearance of large polymers, which, through polymerization of hemoglobin S, results in shape distortion of red blood cell. This biological phenomenon makes the red blood cells support insufficient oxygen in the blood, which makes the patient’s health fragile because of low oxygen levels (Embury, 1994). Through research on this phenomenon, attempts to create medical solutions to the disease are progressing, though at a slow pace. There is a technological advancement suggesting that gene transplant would help manage the disease, though such method has some practical difficulties.
Patients with sickle cell disease require significant clinical care, especially when they are diagnosed with infections. There are fatal infections that often affect people with sickle cell disease causing aplastic crisis, acute chest syndrome, acute splenic sequestration, gallbladder disease and kidney failure. These infections require comprehensive pediatric tertiary care of the affected children. National health statistics indicates that slightly more than 70.000 children with sickle cell disease are born annually. There are different types of sickle cell disease, but the common one is sickle cell anemia. Although there is minimal progress in the treatment methodology of sickle cell diseases, there is a method of the stem cell transplant, which may cure some patients (Embury, 1994).
The main discomfort associated with sickle cell disease is acute pain in the body muscles and joints. Although the scientists are still in search of the best possible cure for sickle cell disease, it seems to be elusive, considering the cause and mode of infection. The best remedy to the disease discomforts is the use of various therapies. There are different management severity levels of sickle cell disease in the affected patients. The therapies may be clinical or contemporary depending on the severity of pain (Meyer & Robb, 2005). The study by Sibinga, Shindell, Casella, Duggan and Wilson (2006) has showed how useful contemporary and alternative (CAM) therapies are in managing pain and psychological stress in children with sickle cell disease. This study highlighted also the various alternative therapies, such as biochemical therapy that involves mega vitamins and herbal medications, bioenergetics therapies that involve spiritual exercising and prayers and biomechanical therapies that involve body massage.
Researches on the cure of sickle cell disease are in progress. Currently, different therapies are regarded as the only way of the disease management. Unfortunately, scientists do not clearly understand the biological process through which the disease manifests itself, though it helps to discover more gene modifications. It is critical to detect and manage any infections as early as possible in order to avoid complications that could arise from the reaction with the disease. Sickle cell disease is a gene disorder. Therefore, its treatment may be possible through gene transplant and modification. Children affected by sickle cell disease require comprehensive medical and psychological care and support that will help them fight the predicament. The use of both clinical and alternative contemporary therapeutic measures indicates that sickle cell disease is manageable.
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