SICKLE CELL DISEASE

Sickle Cell Disease

Sickle Cell Disease

Introduction

Sickle cell anemia is a genetic disease responsible for abnormal hemoglobin in red blood cells. Hemoglobin is responsible for transporting oxygen in the tissues. The abnormal hemoglobin (hemoglobin S), under certain conditions (cold, fever, dehydration, etc.), will make red blood cells rigid, they lose their natural ability to deform to pass through the tiny blood vessels.

Symptoms and Consequences

The abnormal hemoglobin will lead to several consequences which are mentioning below;

- Red blood cells are fragile and are destroyed by excess: the hemoglobin is low, defining chronic anemia.

- Children are always anemic, but generally adapt very well. The only visible signs may be fatigue and a yellow eye (because by breaking the red blood cells release a yellow pigment, bilirubin).

- Red blood cells are rigid; they will form plugs and block blood vessels. These accidents are called "vaso-occlusive crises." Some triggers are sometimes but not always identified: cold, dehydration, stress, infection. Some preventive measures reduce the risk of occurrence of a crisis, but crises are unpredictable (Mayfield, 1999).

- In terms of vessel members and extremities (feet, hands). Their bite is responsible for some severe pain, the member concerned may be hot, swollen, and its mobilization can become painful.

- The occlusion of vessels feeding the spleen is very early. It loses its function as anti-bacterial defense. The affected children are thus highly vulnerable to certain infections. This risk is greatest in children younger than 3 years, then decreases but continues throughout life. The most common infections are pulmonary, bone infections but are also possible (Satyen and Mehta, 2006).

Sickle cell disease has a severity varies considerably among children. Some children are very few complications (Keith, 2001). A minority of people frequent and severe complications, the average child is still hospitalized about a week a year (for a pain crisis, infection or worsening of anemia).

Treatment

Genetic testing of healthy carriers can be offered with a history or suspicion of the presence of disorder in the family, and requires genetic counseling. Prenatal diagnosis is feasible in turn by chorionic villus sampling or amniotic fluid. The management of patients from birth includes infection prevention, pain and complications, but also the social and psychological support the patient. Blood transfusion is a treatment modality base. The bone marrow transplant is planned in the event of cerebral vasculopathy. Gene therapies have shown some efficacy in animal models of disease (Satyen and Mehta, 2006). Sickle cell disease is not yet cure able. It is well known, however, treat or prevent symptoms;

- Young children receive daily antibiotic syrup and are vaccinated against certain germs which they are particularly vulnerable as the pneumococcus.

- The daily intake of folic acid prevents certain worsening of anemia.

- Children should drink regularly to avoid dehydration.

- Children should avoid exposure to cold.

- If pain is administered analgesics of increasing power, including in school thanks to Project Home Individual (PAI).

Some of them depending on the severity of the disease may benefit from treatment much heavier (blood ...