Sickle Cell Disease (SCD) is an inherited multisystem disease characterized by irregular, rigid and sickle-shaped red blood cells. The normal red blood cells are round and flexible, which enable them to move freely through the thin blood vessels. Sickle cell disease causes the red blood cells to become hard and assume an irregular (crescent) shape as opposed to the normal disc shape, causing them to get stuck within the narrow blood vessels, and consequently, block. The decreased flexibility of the red blood cells result into a series of complications from periodic painful episodes, chronic anemia, to damage of body organs. It is vital to note that the complications differ in various persons depending on the sickle cell disease type one has. While some people manage to live relatively healthy lives, others have to be hospitalized frequently to manage the disease. There are various forms of sickle cell disease depending on the genes that a person inherits from the parents. Sickle cell anemia, which is the most common sickle cell disease, (hemoglobin SS disease) occurs when an individual inherits sickle cell genes from both parents.However, when a person inherits a sickle-cell gene from a single parent, in addition to a normal gene from the other, he ends up with a sickle cell trait. People having sickle cell traits have no sickle cell disease and exhibits no symptoms of the disorder. Nevertheless, there is a high likelihood of them passing the gene of the disease to their offspring. Other kinds of sickle cell disease are sickle beta-zero-thalassaemia, sickle-haemoglobin C and sickle beta-plus-thalassaemia diseases.

Sickle-cell disease is more common in persons coming from the tropical and subtropical areas where malaria is prevalent. It is estimated that a third of all native dwellers of Sub-Saharan Africa are carriers of the gene. It is estimated that 1 in every 500 African-American children born America have sickle-cell anemia. Other people who are more likely to be affected by the disease include African- Caribbeans, Asians, and people from the Mediterranean region (Greece, Italy and Turkey). It is uncommon to find people from North Europe with the disease. In the United Kingdom, sickle cell disease has become one of the most popular genetic conditions in newly-born babies. It is estimated that, on average, 1 in every 2,400 babies born in England have the disease. In the 1990s, people with sickle cell disease lived an average of 42 and 48 years for males and females respectively. However, advancements in treatment and early diagnosis have made it possible for children with the disorder to live relatively productive and healthy lives. Currently, the life expectancy of persons with SCD in the United Kingdom is approximated to be between 53 and 60 years old. The effects of SCD range from physical, academic, to psychological such as severe pain, stroke, ulcerations, hearing and vision loss, low self-esteem, social withdrawal, depression etc. This proposal is aimed at examining the effects of sickle cell disease on the intellectual capacity of children in the UK.

Problem Statement

It is estimated that currently about 15% of school going children in regular classrooms, are suffering from some chronic health conditions. It is crucial that teachers and parents have knowledge of such health conditions and the impact of the conditions on the children’s intellectual capability. That is why collaboration and communication amongst parents, teachers and healthcare providers is necessary to meet the educational and medicinal needs of the children. Research done in South Carolina revealed that nearly 30% of children suffering from sickle cell disease has difficulties performing well in school. The disorder can cause a child to suffer from silent stroke, which usually goes unrecognized, but leads to a child having learning problems such as difficulty in sustaining and focusing attention, as well as delays in development of their vocabulary. In addition, the disorder can cause anemia in children, which in turn causes fatigue that limits the ability of the child to pay attention in the classroom. Consequently, the performance standards of children with sickle cell disease tend to be lower than that of other normal children. However, without the knowledge of SCD and its impacts, and how children with sickle cell disease can be assisted to remain at per with the rest of the students, such children will continue to suffer silently, and perform poorly in school without their problem being noticed. This study will be very significant in providing information that will be instrumental to parents and educators in the development of an educational and medical plan, which will enable children with sickle cell disease to live normal productive lives.

Objectives

The main objective of this research study will be to examine how sickle cell affects the intellectual capacity of children. Other objectives include examining:

1) Sickle cell

2) Effects of sickle cell

3) Sickle cell and how it affects children’s intellectual capacity

Research Questions

The main research question of this study will be how sickle cell affects the development of children. In view of this, the following questions will be asked:

1) What is sickle cell?

2) What are the affects of sickle cell on children’s intellectual capacity?

3) Does sickle cell disease have negative or positive impact on a child’s intellectual capacity?

Hypothesis

The hypothesis of the study will be:

Ho – sickle cell disease does not affect a child’s intellectual capacity

H1 – sickle cell disease affects a child’s intellectual capacity

Scope of the Study

This research will be conducted using a sample of thirty children (n = 30) between the ages of seven and seventeen years living with sickle cell disease in the United Kingdom.

Literature Review

Sickle Cell Disease

Sickle cell disease (SCD) is a heritable disease of the red blood cells (RBC). People with SCD have abnormal hemoglobin, which causes the RBC to be stiff and sickle-shaped. Red blood cells are normally flexible and round, which makes it easy to flow via the blood vessels. However, due to the stiffness of the RBC of people with SCD, they tend to stick within the narrow blood vessels, thus cutting off the supply of blood.This either causes pain to the person suffering from the disease or even organ damage. It is important to note that RBC Which are sickle-shaped break down and die faster compared to normal red blood cells, and this causes sickle cell anemia. The most popular types of sickle cell disease are Hemoglobin SS, in which an individual inherits a single sickle cell gene from every parent; Hemoglobin SC, in which a person inherits a single sickle cell gene, and an abnormal hemoglobin type called, C and; Hemoglobin S-beta thalassemia, in which a person inherits a single sickle cell gene, in addition to another gene for, betathalassemia.

Studies done by Adams on brain function of children suffering from sickle cell disease revealed that certain children with the disease (even those who have not suffering from stroke), suffer from silent brain injury. Though there are no clear changes on brain scans, such children have been found to have a certain degree of cognitive dysfunction that appears to worsen as they grow up. As discussed earlier, stroke is one of the complications of sickle cell disease, with varied effects in a child’s life such as learning difficulties, long-term brain-damage, paralysis, death etc.

The above study is seconded by the research done by Trial Investigator, to compare the cognitive functioning of African-American adults with sickle cell disease and those without. According to the study, sickle cell patients performed relatively lower than their healthy counterparts in areas of intellectual ability, processing speed, short-term memory and attention. It is important to point out that all the participants had no history of stroke, organ-failure, high blood pressure etc that would otherwise impact negatively on their brain function. This indicates that people suffering from sickle cell disease, both children and adults are likely to develop cognitive problems i.e. difficulty in organizing thoughts, learning, or decision making, regardless of whether they suffered sickle cell disease-related complications like stroke.

The long-term illness associated with sickle cell disease tends to slow growth and development of children with SCD, and; therefore, they grow more slowly than normal children, and enter into puberty two to three years later than normal. Children with sickle cell disease on entering adolescence, find themselves lighter, smaller and less developed sexually in comparison to their peers. The hair patterns in the body signifying maturity, appear late in both sexes, and boys tend to have lower testosterone levels and relatively smaller testicles compared to healthy boys. Girls, on the other hand, tend to lag behind in breast development, as well as, starting their menstruation, and their menstruation tend to be more painful and irregular. A report by the Cooperative Study of Sickle Cell Disease on serial cognitive testing revealed that children with Sickle Cell Disease exhibited declining scores in verbal IQ over a 5-year period. However, these results lacked a control group for comparison purposes, which weakened the validity of the data. This research will be very important in adding to the body of knowledge with regards to the sickle cell disease and its impact on the development and intellectual ability of children, by making a reference to children living with sickle cell in the UK. By focusing on this chronic illnesses and its impact on children, the findings of this study will equip policy makers with the relevant information to enable them design and enact policies that will assist in the normal development of children.

This research will be conducted on thirty children between the ages of seven and seventeen years living with sickle cell disease in the United Kingdom. Since sickle cell disease is very serious, getting first-hand information from the subjects with the disease is critical. Therefore, data collection will involve primary data collection methods such as intercept survey and interview to obtain the facts as they are. Some of the questions that i will ask the respondents include 1) what is sickle cell? 2) What are the affects of sickle cell on children’s intellectual capacity?

Results, Discussion and Analysis

The results of the study will be subjected to Chi-Square Test for analysis, and compared with the findings in the literature review to find out if the effects of sickle cell disease on a child’s intellectual ability in literature match with my findings. The results of this study will be very instrumental in providing information that will enable parents and educators, to develop an educational and medical plan that will help children with sickle cell disease, to live normal productive lives