14 Steps to Help Those With Sickle Cell Disease!

Sickle cell disease primarily affects people of African, Mediterranean, Middle Eastern, and Asian Indian ancestry. There is also a growing segment in the Latino-American population particularly those of Caribbean, Central American, and South American ancestry. In the United States one out of every 400 births has this disease. The most common type of sickle cell disease is sickle cell anemia. In our companion article "Understanding Sickle Cell Disease" we cover a lot of material about the cause and symptoms of this disease. The following paragraph is a very brief overview.

All types of sickle cell disease are caused by a genetic change in the hemoglobin portion of the red blood cell. Hemoglobin is the oxygen-carrying protein inside the red blood cell. Normal red blood cells are oval and flexible. Red blood cells in sickle cell disease have a tendency to reshape themselves into rod-like structures that resemble the curved blade of a sickle; thus, the term sickle cell. Sickle cells have a shorter life span than normal red blood cells. This results in chronic anemia and reduced oxygen to the tissues of the body. In addition, sickle cells are less flexible than normal red blood cells. This presents a problem since they can become trapped in the small blood vessels preventing blood flow to the body's tissues. This compromise in the delivery of oxygen to the tissues results in pain and potential damage to the associated tissues and organs.

Recent Research Highlights the Importance of Nitric Oxide!
Researchers at Duke University and Howard Hughes Medical Institute recently discovered one of the keys to the cause of pain in sickle cell disease. Their findings were reported in the January 31, 2005 Proceedings of the National Academy of Sciences . Their research showed that when normal red blood cells move through the arteries they release a signaling molecule that tells the arterial walls to expand. The signaling molecule is nitric oxide. Nitric oxide causes the smooth muscle in the blood vessel wall to relax which opens up the vessel to allow the cells to pass through.

For those with sickle cell anemia, when the red blood cells are distorted into the sickle cell shape, the researchers at Duke University discovered that the walls of the arteries don't expand. This distorted shape of the sickle cells, combined with the fact that they tend to clump together, ends up blocking blood flow through these small arteries and capillaries. The Duke researchers noted that as the blood pulses, the walls of the arteries didn't expand like they do with normal red blood cells.

Their research also noted that the degree of nitric oxide deficiency directly correlated with symptom severity. This means that the less nitric oxide produced the greater the pain. This Duke/HHMI study found that when nitric oxide was administered to people with sickle cell anemia their symptoms were relieved. One of the conclusions from this study was that abnormal nitric oxide processing may be the real cause of sickle cell circulatory restrictions. In addition to the Duke/HHMI study, several other studies found that the administration of nitric oxide to people with sickle cell anemia relieved symptoms.