CHAPTER ONE
Introduction
Sickle cell disease is a group disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have typical hemoglobin molecule called hemoglobin S, which can distort red blood cells into a sickle or crescent shape.
Signs and symptoms of sickle cell disease usually begins in early childhood. Characteristic feature of this disorder includes a low number of red blood cell (anemia), repeated infection and periodic episode of pain the severity of the symptom varies from person to person some people have mild symptoms while others are frequently hospitalized for more seri9ous complications.
The signs and symptoms of sickle cell disease are caused by the sicking of red blood cells. When red bloos cell sickle, they break down prematurely which can lead to anemia. Anemia can cause shortness sof breath, fatique, and the delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin which are signs of jaundice. Painful episode can occur when sickle red blood cells which are stiff and inflexible gets stuck in small blood vessels. These episode deprive tissues and organs of oxygen rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen and brain. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supplies the lungs (pulmonary hypertension), pulmonary hypertension occurs in about 1/3 of adult in sickle cell disease and can lead to heart failure.
A problem in the hemoglobin-beta gene found in chromosome II the defects forms abnormal hemoglobin molecules.
Both once parents needs to pass the abnormal hemoglobin gene onto one in order for one to develop the disease. If both parents carry the defective gene, one has a one in four chance of inheriting the disease and becoming sick with it. If a child is born with one defective hemoglobin –betagene he may become a carrier of the disease. Carriers usually don’t develop SCD symptoms but they can pass the unto future children if their partners also carry the sickle cell threads.
Types of Sickle cell Disease
There are several different forms of sickle cell disease. The type a parent or child inherent depends on many things, including the specific type of hemoglobin one has hemoglobin SS also called sickle cell anemia is usually the most severe type of this disorder, other common forms includes:
- Hemoglobin SC (usually Mild)
- Hemoglobin SB (Thalassemia)
Raretypes are:
Hemoglobin SD
Hemoglobin SE
Hemoglobin SO
In the U.S new born screening programme requires that all babies are tested for sickle cell disease shortly after birth.
Treatment of Sickle Disease
Finding a widely available cure of sickle cell Anemia (HbSS) still remains a challenge one hundred years after its discovery as a genetically inherited disease.
However, growing interest in the nutritional of the disease has created a body of literature from researchers seeking nutritional alternatives as a means of decreasing mobility and improving quality of life among HbSS patient. This review demonstrated the over past 30 yrs, the role of protein/Energy deffiency in HbSS has been more clearly defined via direct measurement, leading to the concept of a relative shortage of nutrients for growth and development, despite apparently adequate dietary intakes. Although there is still lack of data supporting the efficacy of macro nutrients supplementation, it is becoming clearer that recommended dietary allowances (RDAs) for the general population are insufficient for the sickle cell patients, a similar shortage is likely to be true for micro nutrients deficiencies including findings of vitamin D deficiency that might be associated with incomplete ossification and bone disease, which are well known complication of HbSS disease.
We conclude that there is need for more efforts and resources to be dedicated to research (including supplementation study of larger sample size) aimed at establishing specific RDAs for HbSS patients, much like specific RDAs develo0ped for pregnancy and growth within the general population.
Statement of the Problem
Many raw conclusions have been made on the topic. While many see sickle disease as a death sentence others see it as incurable and many assumptions and fallacies have been told.
Many people do not understand that it can be totally cured or managed effectively and also can be avoided by taking major tests (genotype testing) before taking partners. Whereby those with sickle cell disease are seen as not worth leaving, many parents see such children as burden upon them. Many have ignored that before modern medicine came to light these disease are been managed by local food and health. Therefore attention must be paid on the food they consume.
Objectives of Study
At the end of this research the following objectives should be seen through:
- To educate the public on the disease
- To make them understand that more than the RDA included is needed for effective management of the disease.
- To known the prevalence rate of SCD in the area.
- To identify micro nutrients that have been found essential in the disease condition and examine the extent to which they have been useful in the management of sickle cell disease.
- To explore the metabolic function they carry out which alleviates the sickle cell disease state.
- To gather informations and document sources of these micro nutrients for the benefits of the generality of our society as a means of pointing out the need for more research into the important area that have received little attention, at least, in the Saharan African region.
Significance of study
The study is important as it will bring about ideas that will correct abnormal nutritional status of patients with sickle cell disease and its dietary management and equally educate them on a benefit of maintaining normal nutritional status.
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