Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal crescent shape. (Red blood cells are normally shaped like a disc.)
Alternative NamesAnemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease
Causes, incidence, and risk factorsHemoglobin is a protein inside red blood cells that carries oxygen. Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin S distorts the shape of red blood cells, especially when exposed to low oxygen levels.
The distorted red blood cells are shaped like crescents or sickles. These fragile, sickle-shaped cells deliver less oxygen to the body's tissues. They can also clog more easily in small blood vessels, and break into pieces that disrupt healthy blood flow.
Sickle cell anemia is inherited from both parents. Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East.
Someone who inherits the hemoglobin S gene from one parent and normal hemoglobin (A) from the other parent will have sickle cell trait. People with sickle cell trait do not have the symptoms of true sickle cell anemia.
SymptomsSymptoms usually don't occur until after age 4 months.
Almost all patients with sickle cell anemia have painful episodes (crises), which can last from hours to days. These crises can affect the bones of the back, the long bones, and the chest.
Some patients have one episode every few years. Others have many episodes per year. The crises can be severe enough to require a hospital stay.
Common symptoms include:
Other symptoms include:
Tests commonly performed to diagnose and monitor patients with sickle cell anemia include:
Other tests may include:
Patients with sickle cell disease need ongoing treatment, even when they are not having a painful crisis. They should take supplements of folic acid (essential for producing red blood cells) because red blood cells are turned over so quickly.
The purpose of treatment is to manage and control symptoms, and to limit the frequency of crises.
During a sickle cell crisis, you may need certain treatments. Painful episodes are treated with pain medicines and by drinking plenty of fluids. It is important to treat the pain. Non-narcotic medications may be effective, but some patients will need large doses of narcotics.
Hydroxyurea (Hydrea) is a drug some patients use to reduce the number of pain episodes (including chest pain and difficulty breathing). It does not work for everyone.
Antibiotics and vaccines are given to prevent bacterial infections, which are common in children with sickle cell disease.
Blood transfusions are used to treat a sickle cell crisis. They may also be used on a regular basis to help prevent strokes.
Other treatments for complications may include:
Bone marrow or stem cell transplants can cure sickle cell anemia. However, transplants have many risks, including infection, rejection, and graft-vs-host disease. Therefore, they are currently not an option for most patients. Also, sickle cell anemia patients are often unable to find well-matched donors.
Support GroupsSickle cell anemia can cause great stress to the patient and family members. Joining a support group where members share common experiences and problems can relieve this stress.
See: Sickle cell anemia - support group
Expectations (prognosis)In the past, sickle cell patients often died from organ failure between ages 20 and 40. Thanks to a better understanding and management of the disease, today, patients can live into their 50s or beyond.
Causes of death include organ failure and infection. Some people with the disease experience minor, brief, infrequent episodes. Others experience severe, long-term, frequent episodes with many complications.
ComplicationsCall your health care provider if you have:
Sickle cell anemia can only occur when two people who carry sickle cell trait have a child together. Genetic counseling is recommended for all carriers of sickle cell trait. About 1 in 12 African Americans has sickle cell trait. It is possible to diagnose sickle cell anemia during pregnancy.
You can prevent sickling of red blood cells by:
Have physical exams every 3 - 6 months to ensure that you are getting enough nutrition and activity, and that you are receiving the proper vaccinations. Regular eye exams are also recommended.
PREVENTING INFECTIONS
PREVENTING CRISES
Parents should encourage children with sickle cell anemia to lead normal lives.
To reduce sickle cell crises, take the following precautions:
Be aware of the effects that chronic, life-threatening illnesses can have on siblings, marriages, parents, and the child.
ReferencesLee MT, Piomelli S, Granger S, et al. Stroke prevention trial in sickle cell anemia (STOP): extended follow-up and final results. Blood. 2006;108:847-852.
Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi C, et al. National Institutes of Health consensus development conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med. 2008;148:932-938.
Geller AK, O'Connor MK. The sickle cell crisis: a dilemma in pain relief. Mayo Clin Proc. 2008;83:320-323.
Hebbel RP. Pathobiology of sickle cell disease. In: Hoffman R, Benz EJ, Shattil SS, et al., eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone;2008:chap 42.
Saunthararajah Y, Vichinsky EP, Embury SH. Sickle cell disease. Clinical features and management. In: Hoffman R, Benz Jr. EJ, Shattil SS, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingston; 2008:chap 43.
U.S. Preventive Services Task Force. Screening for Sickle Cell Disease in Newborns: U.S. Preventive Services Task Force Recommendation Statement. Agency for Healthcare Research and Quality, Rockville, MD. Sep 2007:AHRQ Publication No. 07-05104-EF-2.
Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal crescent shape. (Red blood cells are normally shaped like a disc.)
Alternative NamesAnemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease
Causes, incidence, and risk factorsSickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen. Hemoglobin S changes the shape of red blood cells, especially when the cells are exposed to low oxygen levels. The red blood cells become shaped like crescents or sickles.
The fragile, sickle-shaped cells deliver less oxygen to the body's tissues. They can also get stuck more easily in small blood vessels, and break into pieces that interupt healthy blood flow.
Sickle cell anemia is inherited from both parents. If you inherit the hemoglobin S gene from one parent and normal hemoglobin (A) from your other parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell anemia.
Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East.
SymptomsSymptoms usually don't occur until after age 4 months.
Almost all patients with sickle cell anemia have painful episodes (called crises), which can last from hours to days. These crises can affect the bones of the back, the long bones, and the chest.
Some patients have one episode every few years. Others have many episodes per year. The crises can be severe enough to require a hospital stay.
Common symptoms include:
Other symptoms include:
Tests commonly performed to diagnose and monitor patients with sickle cell anemia include:
Other tests may include:
The goal of treatment is to manage and control symptoms, and to limit the number of crises.
Patients with sickle cell disease need ongoing treatment, even when they are not having a painful crisis.
Folic acid supplements should be taken. Folic acid is needed to make red blood cells.
Treatment for a sickle cell crisis includes:
Other treatments for sickle cell anemia may include:
Treatments for complications of sickle cell anemia may include:
Bone marrow or stem cell transplants can cure sickle cell anemia. However, they are current not an option for most patients. Sickle cell anemia patients are often unable to find well-matched donors.
Support GroupsJoining a support group where members share common experiences can relieve the stress related to caring for someone with a chronic disease. See: Sickle cell anemia - support group
Expectations (prognosis)In the past, sickle cell patients often died from organ failure between ages 20 and 40. Thanks to a better understanding and management of the disease, today, patients can live into their 50s or beyond.
Causes of death include organ failure and infection. Some people with the disease experience minor, brief, infrequent episodes. Others experience severe, long-term, frequent episodes with many complications.
ComplicationsCall your health care provider if you have:
Sickle cell anemia can only occur when two people who carry sickle cell trait have a child together. Genetic counseling is recommended for all carriers of sickle cell trait. About 1 in 12 African Americans has sickle cell trait.
It is possible to diagnose sickle cell anemia during pregnancy.
If you have sickle cell anermia, you can prevent the change in red blood cell shape by:
Have a physical exam every 3 - 6 months to make sure that you are getting enough nutrition and activity, and that you are receiving the proper vaccinations. Regular eye exams are recommended.
PREVENTING CRISES
It is important to maintain good oxygen levels and to prevent dehydration. The following steps can help prevent a sickle cell crisis:
Consider having the child with sickle cell anemia wear a Medic Alert bracelet. Share the above information with teachers and other caretakers, when necessary.
PREVENTING INFECTIONS
Lee MT, Piomelli S, Granger S, et al. Stroke prevention trial in sickle cell anemia (STOP): extended follow-up and final results. Blood. 2006;108:847-852.
Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi C, et al. National Institutes of Health consensus development conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med. 2008;148:932-938.
Geller AK, O'Connor MK. The sickle cell crisis: a dilemma in pain relief. Mayo Clin Proc. 2008;83:320-323.
Hebbel RP. Pathobiology of sickle cell disease. In: Hoffman R, Benz EJ, Shattil SS, et al., eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone;2008:chap 42.
Saunthararajah Y, Vichinsky EP, Embury SH. Sickle cell disease. Clinical features and management. In: Hoffman R, Benz Jr. EJ, Shattil SS, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingston; 2008:chap 43.
U.S. Preventive Services Task Force. Screening for Sickle Cell Disease in Newborns: U.S. Preventive Services Task Force Recommendation Statement. Agency for Healthcare Research and Quality, Rockville, MD. Sep 2007:AHRQ Publication No. 07-05104-EF-2.
Reviewed ByReview Date: 02/28/2011
David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital.
C. Sickle-cell anemia
Sickle cell anemia.
its optional hemophilia or sickle cell anaemia
yes - no its not because a sickle cell is basically a diseased cell that is shapped like a sickle.. a disease in bloodcell - it cannot get as much oxygen as healthy blood cells
codominant
You get Sickle-Cell Anemia by Birth,it is a genetic disorder.
An example of point-mutation is sickle-cell anemia. Sickle-cell disease is hereditary.
Sickle cell anemia is an autosomal recessive disease. Carriers have sickle cell trait, which confers resistance to malaria.
Yes, Sickle Cell Anemia is in fact a genetic disorder.
An example of point-mutation is sickle-cell anemia. Sickle-cell disease is hereditary.
sickle cell anemia
Sickle cell anemia -yes it is hereditary
Sickle cell anemia was discovered in the 1870's and carried many names, but 1922 it was officially named sickle cell anemia.
It sounds like you are looking for Sickle Cell Anemia.
Sickle cell anemia is genetic. It is an autosomal recessive disease.
Yes, drepanocytosis(sickle cell anemia) is a type of poikilocytosis.
While technically there are more than one, the main one by far is sickle cell anemia.