Sickle Cell Disease: Child and Adolescent Development
Sickle Cell Disease (Anemia)
Child and Adolescent Development (PSC 1246A)
Sickle Cell Disease, commonly referred to as Sickle Cell Anemia, is a blood disease which red blood cells form an abnormal sickle or crescent shape. Red blood cells carry oxygen to the body and are normally shaped like a disc. This disease is genetically inherited from both parents. If you inherit the sickle cell gene from only one 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. Sickle cell anemia is caused by an abnormal type of hemoglobin[->0] called hemoglobin S. A single cell causes this disease. Hemoglobin is a protein inside red blood cells that carries oxygen. The fragile, sickle-shaped cells deliver less oxygen to the body’s tissues, and also get stuck more easily in small blood vessels, as well as break into pieces that can interrupt healthy blood flow. These problems decrease the amount of oxygen flowing to body tissues even more, sometimes causing a crisis.
A crisis is a painful episode, sometimes lasting from hours to days, of pain in the back, long bones and the chest. These episodes often start after four months of age, usually lasting until death. In young children these episodes include, attacks of abdominal pain, fatigue, jaundice, rapid heart rate, shortness of breath and paleness.
As a person grows older with the disease, small blood vessels may become blocked by the abnormal cells causing painful and prolonged erection commonly known as priapism, poor eyesight or even blindness, problems thinking or confusion caused by small strokes, and ulcer on the lower legs of adults and adolescents.
Usually a person with the disease will sooner or later lose their spleen and have other symptoms and
Child and Adolescent Development (PSC 1246A)
Sickle Cell Disease, commonly referred to as Sickle Cell Anemia, is a blood disease which red blood cells form an abnormal sickle or crescent shape. Red blood cells carry oxygen to the body and are normally shaped like a disc. This disease is genetically inherited from both parents. If you inherit the sickle cell gene from only one 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. Sickle cell anemia is caused by an abnormal type of hemoglobin[->0] called hemoglobin S. A single cell causes this disease. Hemoglobin is a protein inside red blood cells that carries oxygen. The fragile, sickle-shaped cells deliver less oxygen to the body’s tissues, and also get stuck more easily in small blood vessels, as well as break into pieces that can interrupt healthy blood flow. These problems decrease the amount of oxygen flowing to body tissues even more, sometimes causing a crisis.
A crisis is a painful episode, sometimes lasting from hours to days, of pain in the back, long bones and the chest. These episodes often start after four months of age, usually lasting until death. In young children these episodes include, attacks of abdominal pain, fatigue, jaundice, rapid heart rate, shortness of breath and paleness.
As a person grows older with the disease, small blood vessels may become blocked by the abnormal cells causing painful and prolonged erection commonly known as priapism, poor eyesight or even blindness, problems thinking or confusion caused by small strokes, and ulcer on the lower legs of adults and adolescents.
Usually a person with the disease will sooner or later lose their spleen and have other symptoms and
References: 1. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-2031. 2. 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. 3. 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. 4. Geller AK, O 'Connor MK. The sickle cell crisis: a dilemma in pain relief. Mayo Clin Proc. 2008;83:320-323. Review Date: 2/7/2012. Reviewed by: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc