What is Sickle Cell?
Sickle cell changes normal, round red blood cells into crescent-like shapes. “Sickle” describes the shape of the cells. When red blood cells have the sickle shape they are not able to move throughout the body as easily as round cells. The cells can block blood vessels, preventing oxygen from reaching certain parts of the body. This causes pain and damage to organs, muscles and bones. There is no cure for sickle cell anemia but there are treatments that can relieve pain and prevent other problems associated with the disorder.
What are the causes?
- A mutation in the gene causes your body to make hemoglobin (part of the red blood cell that makes it red in color).
- When the gene mutates, hemoglobin s (for “sickle”) is made rather than hemoglobin a (healthy hemoglobin). Blood disorders occur if the body does not produce healthy hemoglobin. Hemoglobin is important because it carries oxygen from the lungs to the body and transports waste from the body back to the lungs to be exhaled.
- Sickle cell is inherited.
- In order to have the disease you have to receive the gene from BOTH your mother and your father. If you only get the gene from one parent, you are a carrier. A carrier will not have any symptoms of the disease but has the potential to pass the disease on to his/her children.
Treatments for Sickle Cell:
- Medications – antibiotics and pain relievers
- Blood transfusions – increasing the number of round red blood cells decreases the risk of stroke
- Supplemental oxygen – to help your child breathe more easily
- Bone marrow transplant – this procedure places healthy bone marrow cells into your child. The cells will replace the bone marrow that is not working properly and start to produce healthy red blood cells. This procedure will cure sickle cell if successful. However, it is very high risk and extremely difficult to find matching donors.
What can I do?
- Ensure your child receives all of his/her immunizations
- Learn what environmental or activity triggers cause painful events (i.e., low oxygen due to high altitude) and avoid them.
- Encourage a healthy diet with plenty of water
- Ask your doctor about experimental treatments
Learn more about the Sickle Cell program at Miller Children's.
It is important to remember the health information found on this Web site is for reference only not intended to replace the advice and guidance of your health care provider. Always seek the advice of your physician with any questions you may have regarding a medical condition. If you think you may have a true medical emergency, call 911 immediately.
Symptoms
- Anemia – a shortage of red blood cells because sickle cells break apart easily and die. This also causes fatigue because oxygen brings energy to the body.
- Pain – when the sickle cells block vessels you feel pain that can last a few hours up to a few weeks. This pain may require hospitalization
- Hand-foot syndrome – swollen hands and feet caused by sickle cells blocking blood flow out of hands and feet
- Jaundice – a yellow coloring of skin and eyes. This indicates liver damage. The liver has to work very hard to filter out all of the broken or dead sickle cells.
- Frequent infections – sickle cells damage the spleen (the organ that fights infection).
- Delayed growth and puberty – a shortage of oxygen in cells means less (or delayed) growth.
- Vision problems – the blood vessels leading to the eyes can become blocked with sickle cells.
Centers & Programs
Providing quality, compassionate care to children with cancer, sickle cell disease and other serious blood disorders through comprehensive psychological programs, research and clinical trails, a specially trained care team and survivorship programs.
There are many types of blood disorders, which can involve problems with bone marrow, blood vessels, lymph nodes, platelet, proteins red blood cells or white blood cells, involved in bleeding and/or clotting. Patients and families receive care from experienced hematologists that treat a wide range of serious blood disorders.
