sources:

http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html
http://www.innvista.com/health/ailments/anemias/sickhist.htm
http://www.webmd.com/pain-management/pain-management-sickle-cell-disease

Sickle Cell

Foundations;

Sickle Cell Disease Association of America, Inc (SCDAA):http://www.sicklecelldisease.org/

This national fundraising and awareness initiative is designed to focus on education and funding for sickle cell disease programs, treatment and research. The campaign will motivate and empower communities nationally to go above and beyond the call of duty to enhance and save the many lives affected by this disease.

Current research

St. Jude Children's Research Hospital has one of the largest and most active Sickle Cell Disease Programs in the nation. St. Jude treats approximately 800 children per year with sickle cell disease. St. Jude has several labs, which perform various research on sickle cell disease. These labs perform basic science and translational research. Basic science involves theoretical research which is conducted in the lab. Translational research brings the research from the lab to the patient.
In 2008, the National Heart, Lung and Blood Institute named St. Jude as one of 12 institutions nationwide to participate in the Basic and Translational Research Program on sickle cell disease. The BTRP funded 2 projects at St. Jude under this program.

• One project funded by in the BTRP is led by Dr. Derek Person. It is designed to include a gene therapy solution on sickle cell disease. It has both a basic science and translational research component.
• The second project is led by Dr. Tuomanen, Infectious Disease chair. This project will study pneumococcal infection in patients with sickle cell disease from a lab prospective. This project also has both basic science and translational components.

Prevention

• Getting enough fluids in your body.
• Getting enough oxygen.
• Quickly treating infections.

Treatment

• Thers is NO known cure besides a bone marrow transplant.

How is sickle-cell diagnosed?

• A hemoglobin electrophoresis is a blood test that can determine if a person is a carrier of a specific sickle cell trait, or has any of the diseases associated with the sickle cell gene.
• Early diagnosis in providing proper preventative treatment for some of the devastating complications of the disease.
• Many states provide routine newborn screening blood tests in order to begin proper treatment as soon as possible.

Causes

• A genetic abnormality in the gene for hemoglobin, which results in the production of sickle hemoglobin.
• When oxygen is released from sickle hemoglobin, it sticks together and forms long rods, which damage and change the shape of the red blood cell. The sickle red blood cells causes the symptoms of sickle cell disease.
• When a child inherits two sickle hemoglobin genes, one from each parent.
• People who inherit only one sickle hemoglobin gene are carriers (sickle cell trait) and do not have anemia or painful sickle cell crises.
• The 11th pair of chromosomes contains a gene responsible for normal hemoglobin production.
• Recessive needs two defective genes for sickle cell.

Symptoms

• Severe pains
• Anemia
• Chest pain and difficulty breathing
• Strokes
• Joint pain and arthritis
• Blockage of blood flow in sperm in the spleen or liver
• Severe infection

History

Sickle cell has been known to the people of Africa for many years. Many tribes gave the disease different names.

Ga tribe: chwechweechwe
Faute tribe: nwiiwii
Ewe tribe: nuidudui
Twi tribe: ahotutuo

Sickle cell disease went unreported in African medical literature until the 1870s. The symptoms were very similar to those of other tropical diseases in Africa and blood was not usually examined. Children born with sickle cell disease usually died in infancy and were typically not seen by a doctor. Most of the earliest published reports of the disease involved black patients living in the US. This first sickle cell patient came to Chicago in 1904 to study dentistry in one of the best schools of the country and was likely the only black student there. He was a wealthy man from the West Indies; and, repeated hospitalizations for his illness. Studies have shown that African Americans, who have lived in malaria-free areas for as long as ten generations, have lower sickle cell gene frequencies than Africans and the frequencies have dropped more than those of other, less harmful African genes.

What is sickle-cell disease?

Sickle Cell is a disorder in which the body makes sickle-shaped red blood cells. “Sickle-shaped” means that the red blood cells are shaped like a crescent. People who do not have sickle-cell have normal red blood cells and look like disc-shaped.They move easily through your blood vessels. Red blood cells contain an iron-rich protein called hemoglobin . Hemoglobin carries oxygen from the lungs to the rest of the body. Sickle cells contain abnormal hemoglobin called sickle hemoglobin. Sickle hemoglobin causes the cells to develop a sickle, or crescent, shape. Sickle cells are stiff and sticky. They tend to block your blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain, serious infections, and organ damages.