Dmg For The Treatment Of Sickle Cell Anemia

In this Article

In this Article

In this Article

Background: Sickle cell disease causes significant morbidity and mortality and affects the economic and healthcare status of many countries. Yet historically, the disease has not had commensurate outlays of funds that have been aimed at research and development of drugs and treatment procedures for other diseases. Individuals with sickle cell disease. This publi-cation, which was developed by physicians, nurses, psychologists, and social workers who specialize in the care of children and adults with sickle cell disease, describes the current approach to counseling and also to manage-ment of many of the medical complications of sickle cell disease. Sickle cell disease, also known as sickle cell anemia, is inherited. People who have the disease inherit two copies of the sickle cell gene—one from each parent. The gene codes for production of an abnormal hemoglobin. If a person inherits only one copy of the sickle cell gene, he or she will have sickle cell trait.

Sickle cell disease (SCD) is the most common inherited blood disorder. That means it’s passed down through families. You’re born with SCD. It is not something you catch or develop later in life.

The disease gets its name because when you have SCD, your red blood cells look like a sickle, which is a C-shaped farm tool.

Red blood cells contain a molecule called hemoglobin, which carries oxygen throughout the body. In a healthy person, hemoglobin is smooth, round, and flexible. That allows red blood cells to glide easily through your bloodstream. But if you have SCD, the hemoglobin’s shape is abnormal. It forms rods that clump together. That causes red blood cells to become rigid and curved. The odd-shaped cells block blood flow. It’s dangerous, and can cause extreme pain, anemia, and other symptoms.

Dmg

Jan 29, 2020 The Stroke Prevention Trial in Sickle Cell Anemia (STOP) showed that regular blood transfusions produced a marked (90%) reduction in first stroke in asymptomatic high-risk children who had 2 abnormal transcranial Doppler (TCD) studies with velocities of 200 cm/s or greater.

  1. Apr 12, 2012  The NHLBI and Sickle Cell Disease. The NHLBI has researched sickle cell disease since its founding as the National Heart Institute in 1948. Since 1972, when the National Sickle Cell Anemia Control act was passed, the NHLBI has spent more than $1 billion researching the condition.
  2. Children with sickle cell anemia should receive prophylactic penicillin from birth through at least five years of age, and all persons with sickle cell disease require vaccination to prevent.
Anemia

About 100,000 people in the United States have sickle cell disease. Most of them are African-Americans.

What Causes Sickle Cell Disease?

A problem in the hemoglobin-beta gene found on chromosome 11. The defect forms abnormal hemoglobin molecules.

Both your parents need to pass the abnormal hemoglobin gene on to you in order for you to develop the disease. If both your parents carry the defective gene, you have a 1 in 4 chance of inheriting the disease and becoming sick with it.

If a child is born with one defective hemoglobin-beta gene, he may become a carrier of the disease. Carriers usually don’t develop SCD symptoms. But, they can pass the disease on to future children if their partner also carries the sickle cell trait.

Types of Sickle Cell Disease

There are several different forms of sickle cell disease. The type you or your child inherit depends on many things, including the specific type of abnormal hemoglobin you have.

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Hemoglobin SS, also called sickle cell anemia, is usually the most severe type of this disorder.

Other common forms include:

  • Hemoglobin SC (usually mild)
  • Hemoglobin Sβ thalassemia

Rare types are:

Dmg For The Treatment Of Sickle Cell Anemia Symptoms

  • Hemoglobin SD
  • Hemoglobin SE
  • Hemoglobin SO

Dmg For The Treatment Of Sickle Cell Anemia Genetics

In the U.S., newborn screening programs require that all babies are tested for sickle cell disease shortly after birth.