SICKLE CELL DISEASE

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SICKLE CELL DISEASE

PREFACE

Sickle cell disease is a disorder that affects the red blood cells, which use a protein called haemoglobin to transport oxygen from the lungs to the rest of the body. Sickle cell disease is inherited from parents who are AS. This means that a child cannot inherit the disease unless the parents pass down a detective copy of the gene. Unlike normal red blood cells, which can live for 120 days, sickle – shaped cells live only 10 to 20 days. Sickle cell anaemia is mostly dominated in African countries, e.g. Nigeria where most people get married blindly without going to the hospital to know their genotype. The high prevalence of the defective gene in these region may be due to the fact that carriers of a mutation in the beta – subunit of haemoglobin. The term paper consider the symptoms, treatment and prevention.

TABLE OF CONTENTS

Cover Page i

Title Page ii

Acknowledgementiii

Table of Contents iv

Prefacev

CHAPTER ONE

Introduction 1

CHAPTER TWO

History of Sickle Cell, Causes Signs and Symptoms 3

CHAPTER THREE

Diagnosis, Pathophysiology 6

Effect of Sickle Cell Anamia 8

CHAPTER FOUR

Management and Treatment, Prevention 10

CHAPTER FIVE

Conclusion 12

References

INTRODUCTION

Sickle cell disease is a disorder that affects the red blood cells, which use a protein celled haemoglobin to transport oxygen from the lungs to the rest of the body (Miller, 2011). Normally, red blood cells are round and flexible so they can travel freely through the narrow blood cells vessels (Arteries and Veins). The haemoglobin molecules have two parts: an alpha and beta. Patients with sickle cell disease have a mutation in a gene on chromosome ll that codes for the beta subunit of the haemoglobin protein (Adams, 2001).

As a result, haemoglobin molecules do not form properly, causing red blood cells to be rigid and have a concave shape (Like a sickle used to cut wheat and other grains). These irregular shaped cells get stuck in the blood vessels and are unable to transport oxygen effectively, causing the pain and damage to the organs (Adams, 2007). Unlike normal red blood cells, which can live for 120 days, sickle shaped cells live only 10 – 20 days.

Sickle cell disease is most common among people from African countries such as those from India, Caribbean, the middle East, and the Mediterranean etc .The high prevalence of the defective gene in these regions may be due to the fact that carriers of a mutation in the beta – subunit of haemoglobin are more resistant to malaria. Malaria is a disease caused by a parasite that is transmitted to a person when they are been bitten by infected mosquito (Martin-Cartajal and Dunlop, 2004).

Sickle cell disease prevents Oxygen from reaching the spleen, liver, kidney, lungs, heart or other vital organs, causing a lot of damage. Without oxygen, the cell that makes up these organs will begin to die. For example, the spleen is often destroyed in these patients resulting in some loss of immune function. As a result, these patients experience frequent infections (Chrouser, 2011).

The red blood cells of patients with sickle cell disease do not live as long as healthy red blood cells. As a result, people with this disorder often have low red blood cell count (Anaemia) that is why this disease is commonly referred to as sickle cell anaemia (Robert, 2005). The term disease, is also applied to sickle cell because the inherited abnormality causes a pathological condition that can lead to death and severe complications. Not all inherited variants of haemoglobin are detrimental, a concepts known as genetic polymorphism. There are also sickle cell crisis or sickling crisis, which is used to describe several independent acute conditions occurring in patients with sickle cell disease (Elagouz, 2010). It can be of different types which include the vaso – occlusive crisis – caused by sickle – shape red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in ischaemia, pain necrosis and often organ damage (Elagouz,2010).

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