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What is Sickle Cell Disease?

It is a genetic disorder which is inherited from both the parents. This disorder affects the Hemoglobin, which is a protein found inside Red Blood Cells, which carries oxygen from the lungs, to different parts of the body. A person with the Sickle Cell Disease has two abnormal genes, (One from each parent) One HBS gene from one parent, and the other, with the individual Sickle Cell Trait.

In some cases, these individuals generally do not experience symptoms of the disease but can pass on this gene to their children and so forth.

Who Has Sickle Cell Disease?

In the United States, approximately 100,000 people living with Sickle Cell Disease. Many more Americans as many as 2.5 million including 1 in 12 African-Americans are carriers for Sickle Cell (also called sickle cell trait or SCD). SCD affects African Americans, Hispanics and is common among those whose ancestors come from sub-Saharan Africa, regions in the Western Hemisphere, Saudi Arabia, India, and Mediterranean countries. Sickle Cell Disease is an inherited disorder which constitutes a global public health problem. SCD causes the destruction of red blood cells due to the presence of the abnormal hemoglobins, resulting in anemia and vaso-occlusion blocking of blood vessels by sticky and inflexible red blood cells. Children with Sickle Disease include early childhood deaths from infection stroke in young children and adults, infection of the lungs similar to pneumonia, pulmonary hypertension, chronic damage to organs such as the kidney resulting in chronic kidney failure, and frequent severe painful episodes. These unpredictable, intermittent, devastating pain events can begin as early as six months of age and can span a lifetime, impacting school attendance and the workforce. Early detection and treatment have proven to be effective in preventing infections such as Pneumonia, Meningitis, and Sickle Cell related infants’ deaths. In New York State, all newborn are routinely screened for Sickle Cell Disease & Trait. However, there remains a significant lack of progress in the broader public health and social issues of educational, vocational, support for personal, family and community relations, practitioner and public education and mental health aspects of the lives of these individuals. There is an emerging body of promising experimental and practice-based research that suggest improvements in the overall quality of life for persons with Sickle Cell Disease and their families can be achieved. However, the lack of resources for such efforts remains problematic. Prompt treatment for acute complications, along with counseling and support will enable individuals with sickle cell to live longer and healthier lives. Appropriate Diagnosis, Counseling, and Education are essential for the affected families and their children.

The way in which Sickle Cell Disease affects individuals varies. Most individuals suffer recurrent episodes of Pain & Hospitalizations, while others may have relatively few Sickle Cell related complications. Many individuals affected by the disease can experience acute painful episodes known as "Sickle Cell Crisis". Some people have pain "Crisis" over and over again.

The abnormal hemoglobin causes Red Blood Cells to become "sickle-shaped"' and therefore blocking the blood vessels, and causing pain and damage to the body, one of the many damages caused is a "stroke".

Stroke is one of the worst problems that Sickle Cell can cause. Sickle Cell Disease can also cause progressive damage to organs such as the Liver, Spleen, Kidney, and the retina of the Eye.