Sickle Cell Anemia
Sickle cell disease (SCD) is a blood disorder. The shape of some red blood cells (RBCs) change to a sickle (crescent moon) shape. The sickle RBCs can block blood flow in blood vessels. This will slow or stop the flow of blood and oxygen to tissues and organs.
The body will also destroy these RBCs because they are abnormal. This means you have fewer overall RBCs. Low levels of RBCs is a condition called anemia. This makes it harder for the body to get the oxygen it needs.
|Red Blood Cells: Normal and Sickle|
|Copyright © Nucleus Medical Media, Inc.|
A defect in the genes causes SDC. This gene causes a problem with a part of the RBC called hemoglobin.
Parents pass the defect to their children. SCD will only develop if you get the defective gene from both of your parents.
If you only get one defective gene, you will not have SCD. But, you can pass this gene on to your children.
Symptoms may be noticed as early as 6 months old. They are different in everyone depending on where problems are in the body. The most common include:
- Fatigue—caused by anemia
- Painful swelling of hands and feet
- Yellowing of the skin and eyes— jaundice
- Belly pain
- Pale skin
Signs of acute chest syndrome (ACS):
- Breathing problems
- Chest pain
- Fast heart rate
SDC crisis may cause:
- Sudden pain—mainly in the back, chest, arms, or legs
- Pain may be throbbing or sharp
- Pain may be in one place or move around
SCD may cause other serious problems such as a stroke , or painful erections lasting 4 hours or more. These problems need immediate care.
The goal of care is to lower the chances of health problems. It will include both medical care and lifestyle habits:
Young children may be given penicillin daily. It can help to lower the risk of certain infections. It’s given until the age 5 years. Penicillin is also used for people who had their spleen removed.
People with SCD have a higher risk of infections. Infections can also trigger other health problems. The doctor may advise:
- Pneumococcal vaccine (PCV-13) series
- Influenza (flu) vaccine
- Meningococcal Vaccine
Hydroxyurea is a pill that can help lower the risk of or prevent health problems. It increases the production of healthy hemoglobin.
Hydroxyurea lowers the chances of sickle cell crisis. It’s not right for everyone. It’s also unsafe for pregnant women.
Blood transfusions raise the number of healthy RBCs. Transfusions treat anemia. They also help with liver problems or stroke.
Hematopoietic Stem Cell Transplantation (HSCT)
HSCT is currently the only cure. It's not right for everyone. A donor with matching blood proteins will be needed. This is most often found in a brother or sister.
There are also a number of medical risks involved. For one, the immune system will need to be suppressed for the rest of your life. This can increase the risk for other illnesses. HSCT may be considered in children who have had severe complications.
Regular medical care is important. Keep in touch with your healthcare team. Early care of health problems may keep them from getting worse.
Lifestyle steps that may help prevent problems:
- Daily folic acid supplements. It will help to build new RBCs.
- Drink plenty of water. Try to avoid dehydration which can trigger problems.
- Avoid places that are very hot or very cold.
- Avoid physical exhaustion and stress.
- Get plenty of rest.
This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.
Edits to original content made by Denver Health.
Copyright © EBSCO Information Services
All rights reserved.
a (SCD; Sickle Cell Anemia)
American Sickle Cell Anemia Association http://www.ascaa.org
Sickle Cell Information Center http://scinfo.org
About Kids Health—The Hospital for Sick Children https://www.aboutkidshealth.ca
Sickle Cell Foundation of Alberta https://ourscfa.org
Sickle cell disease. Family Doctor—American Academy of Family Physicians website. Available at: https://familydoctor.org/condition/sickle-cell-disease. Updated September 8, 2017. Accessed July 18, 2018.
Sickle cell disease. Kids Health—Nemours Foundation website. Available at: http://kidshealth.org/en/parents/sickle-cell-anemia.html. Updated May 2018. Accessed July 18, 2018.
Sickle cell disease. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease. Accessed July 18, 2018.
Sickle cell disease (SCD). Centers for Disease Control and Prevention website. Available at: https://www.cdc.gov/ncbddd/sicklecell/index.html. Updated June 18, 2018. Accessed July 18, 2018.
Sickle cell disease in adults and adolescents. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T902929/Sickle-cell-disease-in-adults-and-adolescents . Updated January 19, 2018. Accessed July 18, 2018.
Sickle cell disease in infants and children. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T902928/Sickle-cell-disease-in-infants-and-children . Updated May 7, 2018. Accessed July 18, 2018.
1/4/2011 DynaMed's Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T902929/Sickle-cell-disease-in-adults-and-adolescents : Vichinsky EP, Neumayr LD, Gold JI, et al. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2010;303(18):1823-1831.