Sickle Cell - New

WELCOME TO FEMALE MEDICAL WARD IN-SERVICE Sickle Cell Disease By: Ghada Al-Omaireen

TABLE OF CONTENTS 1. DEFINITION. 2. HOW IS SICKLE CELL DIAGNOSED? 3. TYPES OF SICKLE CELL. 4. HERTIANCE. 5. SIGNS AND SYMPTOMS. 6. COMPLICATIONS. 7. MANAGEMENT. 8. NURSING INTERVENTIONS.

What is Sickle Cell Disease? Sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell disease have red blood cells that contain mostly hemoglobin* S, an abnormal type of hemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels. When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There is currently no universal cure for

Sickle Cell Distribution

How is sickle cell diagnosed?

Sickle cell disease usually is diagnosed at birth with a blood test during routine newborn screening tests [amniocentesis ]. If a child tests positive on the screening test, a second blood test (called a hemoglobin electrophoresis) should be performed to confirm the diagnosis. Because kids with sickle cell disease are at an increased risk of infection and other health complications, early diagnosis and treatment to prevent problems is

:Types of Sickle Cell Disease 

There are several types of sickle cell disease. The most common are:

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HbSS - People who have two sickle cell genes (“S”), one from each parent. This is commonly called “sickle cell anemia” and is usually the most severe form of the disease. HbSC - People who have one sickle cell gene and one gene from an abnormal type of hemoglobin called “C.” This is usually a milder form of the disease. HbS beta thalassemia - People who have one sickle cell gene and one gene for beta thalassemia, another type of anemia. There are two types of beta thalassemia. “O” and “+.” Those with HbS beta O-thalassemia usually have a severe form of the disease. People with HbS beta +-thalassemia tend to have a milder form of the disease.

HbAS - People who have one sickle cell gene and one normal gene. This is called sickle cell trait.  Sickle Cell trait (AS) is an inherited condition in which both hemoglobin A and S are produced in the red blood cells, always more A than S. Sickle cell trait is not a type of sickle cell disease. People with sickle cell trait are generally healthy and do not have any of the symptoms of the disease and live a normal life, but they can pass the disease on to their children.

:Inheritance

:Signs and Symptoms Vaso-occlusive crisis - is caused by sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in ischemia, pain, and often organ damage. Aplastic crises - are acute worsenings of the patient's baseline anaemia, producing pallor, tachycardia, and fatigue. This crisis is triggered by parvovirus B19, which directly affects erythropoiesis (production of red blood cells). Splenic sequestration crises - are acute, painful enlargements of the spleen. The abdomen becomes bloated and very hard. Hemolytic crises - are acute accelerated drop in hemoglobin level. The red blood cells break down at a faster rate. This is particularly common in patients with co-existent G6PD deficiency.

:Complications      

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Anemia – patient will be pale, tire easily and have shortness of breath. Infections - are a major cause of death in children with sickle cell disease, such as pneumonia and meningitis. Pain episodes – it’s can be treated by strong pain-killer. Hand-foot syndrome - may swell when small blood vessels become blocked. This may be the first symptom of sickle cell disease in babies. Stroke - can occur when sickle-shaped cells block a blood vessel in the brain. Splenic crisis - Symptoms include pain on the left side of the abdomen, weakness and rapid heart rate. If the condition recurs frequently, the provider may recommend regular blood transfusions or removal of the spleen. Acute chest syndrome: This is similar to pneumonia, with symptoms such as difficulty breathing, chest pain and fever. It can be caused by an infection or by blocked blood vessels in the lung. Vision problems: When tiny blood vessels in the eye become blocked with sickle-shaped cells, vision problems and even blindness can result.

Slow growth – due to anemia.

:Management 

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Penicillin and appropriate immunizations - including the pneumococcal vaccine and influenza and meningococcal vaccines. Although penicillin isn't a cure, it can help prevent life-threatening infections. transfusions of red blood cells – for patients who develop serious complications (such as acute chest syndrome, severe anemia, or stroke. Vitamins supplement such as Folic acid - can help them with the disorder produce new RBCs. Hydroxyurea + exjade – to increases the amount of hemoglobin in blood cells. Hydration. Analgesics. bone marrow transplant.

Can a woman with sickle cell ?disease have a safe pregnancy 

Yes. However, women with sickle cell disease are at increased risk for Complications that can affect their health and that of their babies. During pregnancy, sickle cell disease may become more severe, and pain episodes may occur more frequently. A pregnant woman with sickle cell disease is at increased risk for miscarriage, for preterm labor, and for having a low-birthweight baby. However, with early prenatal care and careful monitoring throughout pregnancy, women with sickle cell disease can have a healthy pregnancy. Treatment with hydroxyurea is not recommended during pregnancy because it can increase the risk of birth defects.

Nursing Care: 1. Assessment of level of pain. 2. Avoid to hot or cold whether. 3. Avoid extreme exercise. 4. Limit emotional stress. 5. Avoid high attitude levels. 6. Encourage to increase fluid intake.

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