What is Sickle Cell Anaemia?

The disorder affects the red blood cells which contain a special protein called haemoglobin (Hb for short). The function of haemoglobin is to carry oxygen from the lungs to all parts of the body.
People with Sickle Cell Anaemia have Sickle haemoglobin (HbS) which is different from the normal haemoglobin (HbA). When sickle haemoglobin gives up its oxygen to the tissues, it sticks together to form long rods inside the red blood cells making these cells rigid and sickle-shaped. Normal red blood cells can bend and flex easily.

Because of their shape, sickled red blood cells can't squeeze through small blood vessels as easily as the almost donut-shaped normal cells. This can lead to these small blood vessels getting blocked which then stops the oxygen from getting through to where it is needed. This in turn can lead to severe pain and damage to organs.

Everyone has two copies of the gene for haemoglobin; one from their mother and one from their father. If one of these genes carries the instructions to make sickle haemoglobin (HbS) and the other carries the instructions to make normal haemoglobin (HbA) then the person has Sickle Cell Trait and is a carrier of the sickle haemoglobin gene. This means that this person has enough normal haemoglobin in their red blood cells to keep the cells flexible and they don't have the symptoms of the sickle cell disorders. They do however have to be careful when doing things where there is less oxygen than normal such as scuba diving, activities at high altitude and under general anaesthetics.

If both copies of the haemoglobin gene carry instructions to make sickle haemoglobin then this will be the only type of haemoglobin they can make and sickled cells can occur. These people have Sickle Cell Anaemia and can suffer from anaemia and severe pain. These severe attacks are known as Crises. Over time Sickle Cell sufferers can experience damage to organs such as liver, kidney, lungs, heart and spleen. Death can be a result.

Another problem is that red blood cells containing sickle haemoglobin do not live as long as the normal 120 days and this results in a chronic state of anaemia. In spite of this, a person with sickle cell disorder can attend school, college and work. People with sickle cell disorder need regular medical attention particularly before and after operations, dental extraction and during pregnancy. Many hospitals arrange follow-up appointments and it is advisable to discuss with the doctors questions concerning schooling, strenuous exercise, family planning, suitable types of employment and air travel. When a person is found to have a sickle cell disorder it is important that all members of the family be tested. They will not necessarily have sickle cell disorder but may be healthy carriers of a sickle cell trait.

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Press Release

Contact Information:
Charles Hartman
P.O. Box 87010
Baton Rouge, Louisiana 70879
chartman@stopsicklecell.com
www.stopsicklecell.com

FOR IMMEDIATE RELEASE: (January 30, 2005)

A Baton Rouge biotech firm believes it has the technology that will allow sufferers of Sickle Cell Disease and Myelodysplastic Syndrome to lead normal lives.

Baton Rouge, LA - A Baton Rouge biotech firm believes it has the technology that will allow sufferers of sickle cell disease and myelodysplastic syndrome to lead normal lives.

Paul Robichaux, founder of Sickle Cell Therapeutics International, LLC, said his firm is engaged in research to treat and possibly eliminate the symptoms of the two diseases through the use of adult stem cells.

The treatment process uses adult stem cells from a person’s own body, which are expanded a thousand times faster than the cells are reproduced within the body. This expansion of the adult stems employs technology developed by NASA. Through its licensor, SCTI has a licensing agreement with NASA to use this proprietary process, said Robichaux, who also owns Robichaux Equipment Inc.

For the past 17 years, NASA has developed the process of expanding cells on numerous Space Shuttle Flights and the Mir International Space Laboratory. The process is unique because the cells form in three-dimensional colonies as they do in a human body and maintain their cell-to-cell geometry.
Sickle cell disease is a painful genetic disorder that obstructs normal blood flow, limiting critical oxygen delivery to tissues. Myelodysplastic syndrome is a disorder in which the bone marrow does not produce enough blood cells, and, like sickle cell disease, there is no known cure. Once diagnosed, life expectancy for those suffering from myelodysplastic is two to 10 years.

Jack Rome Jr., chairman of SCTI board of directors, said, “When the science involved is proven and taken to the market, it will make a significant difference in the lives of many, many people around the world.”

“This has without a doubt been one of the most exciting projects I have worked on in my 30-year career,” said Rome, chairman and president of Jack Rome Jr. and Associates, a Baton Rouge professional management consulting firm that has developed a master plan to move SCTI’s scientific research forward.

Other members of the board are Robichaux, Howard F. Jeter, former U.S. Ambassador to Nigeria, Deputy Assistant Secretary for West African Affairs, and the President’s special envoy to Liberia; Dr. Ernest Simo, the CEO and founder of Space2000 and two-time NASA Astronaut Program finalist.; recently retired Gen. Buford Blount , who was commanding general of the 3rd Infantry Division during Iraqi Freedom and the U. S. Army’s assistant deputy chief of staff for operations.
Heading the medical advisory board is Dr. Mehboob Hussain, a research associate and assistant professor of endocrinology at the University of Chicago, division of biological sciences’ department of medicine. Dr. Hussain received his medical degree from the University of Zurich and completed a fellowship at Harvard Medical School and Massachusetts General Hospital.

Currently, SCTI plans to provide a grant to a university capable of conducting specific research necessary for SCTI’s technology. The company will be seeking to raise funds from public and private entities over the next four years to fund this research and develop therapies.

Once funded, SCTI plans to hire a chief scientist to manage all research and development of technology. The company’s goal is to begin research within a major university by the end of the first quarter of 2005.





 
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