Sicklecell disease is a genetic blood disorder that damages and deforms red blood cells, leading to anemia and episodes of sudden, sometimes severe pain.
This is a life-threatening complication of sicklecell anemia, similar to pneumonia, that is caused by infection or trapped sickledcells in the lung.
Sicklecell anemia patients with severe chest or back pain that prevents them from breathing deeply may be able to avoid lung complications by using an incentive spirometer.
Sickle Cell Disease: Practical Tips for Preventing a Sickle Cell Crisis -- familydoctor.org(Site not responding. Last check: 2007-10-25)
Sicklecell disease, also called sicklecell anemia, is a hereditary (you inherit it from your parents) problem that causes a type of faulty hemoglobin in red blood cells.
The sicklecells also die earlier than normal blood cells, which can cause a shortage of red blood cells in the body.
A sicklecellcrisis happens when sickled red blood cells block small blood vessels that carry blood to your bones.
Sicklecell is an inherited alteration of haemoglobin.
Sicklecell occurs as a result of a person inheriting a sickle haemoglobin S gene from one or both parents.
Sicklecell disease (SCD) is the name for a group of diseases where a person has inherited only sickle haemoglobin S genes (one from each parent) or a sickle haemoglobin S gene from one parent plus another unusual haemoglobin gene from the other parent.
Nitric Oxide Gas May Treat, Prevent Sickle Cell Crisis(Site not responding. Last check: 2007-10-25)
In sicklecell disease, a genetic disorder, affected individuals have an abnormal form of hemoglobin, the protein in red blood cells that carries oxygen from the lungs to tissues and organs throughout the body.
The result is sicklecellcrisis, excruciating pain in the affected area that can require hospitalization in the most serious cases.
In eight of the nine sicklecell disease patients, breathing nitric oxide caused their red cells to give up oxygen less readily than before, while the cells from the normal patients showed no change.
Evidence suggests that hemoglobin, which is released in large amounts by the abnormal sickle red blood cells, removes nitric oxide, which may be responsible for the blood vessels constriction and pain in sicklecell diseases.
Fortunately, with screening tests for sicklecell now required for newborns in most states and with the use of preventive antibiotics in babies who are born with the disease, this terrible mortality rate has dropped significantly.
Sickling can also cause bone loss, particularly the top of the thighbone, and pain in the hands and feet of children, which is known as the hand-foot syndrome.
Sicklecell is a hereditary condition that may occur as the “trait” (you carry one mutated gene from one of your parents) or as the disease (you carry two mutated genes, one from each of your parents).
The sicklecell abnormality is found predominantly in people of African ancestry but may also be found in those who can trace their roots to the Mediterranean area, South and Central America, the Middle East, India, and the Caribbean.
Signs of sicklecellcrisis include fever, mild jaundice (yellowing of the skin and whites of the eyes), chest pain and shortness of breath.
Sickle-Cell Disease(Site not responding. Last check: 2007-10-25)
Sicklecells disease is a result of changes in hemoglobin S: The destructive nature of the sickle hemoglobin develops when it loses oxygen.
Sicklecells also have a shorter life span (10 to 20 days) than that of normal red blood cells (90 to 120 days).
Hemoglobin removes nitric oxide and because abnormal sicklecells release such hemoglobin, patients with the disease are deficient in nitric oxide, which may play an important role in blood vessels constriction and pain in sicklecell diseases.
Sickle Cell Anemia(Site not responding. Last check: 2007-10-25)
SickleCell Anemia (SCA) is caused by a change in the chemical composition of the protein (hemoglobin or Hgb) that carries the oxygen inside of the red blood cells (RBC's).
At the same time, sicklecell patients are in a continuous state of hyperdynamic circulation and therefore, frequently exhibit systolic murmurs.
The primary goal of therapy is to reduce the frequency, duration, and severity of the "sicklecellcrisis episodes" and to maintain an adequate supply of RBC's to nourish the tissues.
If follow-up studies prove successful, patients might someday treat or prevent sicklecellcrisis symptoms by self-administering nitric oxide with inhalers similar to those used by asthma patients.
The current report describes how nitric oxide causes sickle hemoglobin molecules to bind oxygen with greater affinity, which should reduce formation of the sicklecells.
To pursue this question, Head entered into a collaboration with several local sicklecell specialists, including Carlo Brugnara, MD, of Children's Hospital and Bridges, as well as MGH investigators specializing in nitric oxide research.
Open Directory - Health: Conditions and Diseases: Blood Disorders: Sickle Cell(Site not responding. Last check: 2007-10-25)
The American SickleCell Anemia Association - A non-profit organisation based in Cleveland Ohio whose objectives are to raise awareness of the condition.
SickleCell Anaemia - Written by a UK sufferer which details the causes, symptoms and normal treatments of sicklecell anaemia.
SickleCell Disease: Information for Health Professionals - Basic facts, symptoms, how to recognise a crisis, diagnosis and management are some of the topics covered.
BOSTON — September 2, 1997—A study by researchers at the Massachusetts General Hospital (MGH) and other Boston hospitals suggests that inhaled nitric oxide gas might successfully treat sicklecell disease and its characteristic episodes of debilitating pain, called sicklecellcrisis.
After this abnormal hemoglobin releases its oxygen, it clumps together into an abnormal shape, deforming the red blood cells — normally flexible discs — into rigid, elongated "sicklecells." These sicklecells can become stuck in tiny blood vessels, blocking blood flow to various parts of the body.
They first added low concentrations of nitric oxide — similar to those used therapeutically — to normal red blood cells and those from sicklecell disease patients.
Schechter, A. N., Rodgers, G. P., Adams-Graves, P., Heltsley, C., Deitcher, S. Hydroxyurea in SickleCell Disease.
Koshy, M., Dorn, L., Bressler, L., Molokie, R., Lavelle, D., Talischy, N., Hoffman, R., van Overveld, W., DeSimone, J. 2-deoxy 5-azacytidine and fetal hemoglobin induction in sicklecell anemia.
