Sickle cell anemia, a serious congenital inherited disease usually diagnosed in childhood, causes red blood cells to develop a sickle rather than an oval shape. Red blood cells carry oxygen to the body on hemoglobin, a protein that gives blood its color. Sickle cells clump together, blocking blood vessels, and don't live as long as normal red blood cells. The child with sickle cell anemia can't make new red blood cells fast enough to replace the sickle cells, and anemia--a low red blood count--results. Sickle cell anemia has a number of serious effects on a child.
Cause
To inherit sickle cell disease, a child must inherit the sickle cell gene from both parents. Most people who carry the sickle cell gene in the United States are black, the Merck Manual states, although people of Indian, Mediterranean, Hispanic, Middle Eastern and Spanish descent may also carry the gene. Around 10 percent of black children in the United States carry the gene, while 0.3 percent of black children in the United States have sickle cell anemia, the Merck Manual reports. If both parents carry the gene, a child's risk of having the disease is 25 percent.
Symptoms
The sickle cell gene causes red blood cells to contain an abnormal type of hemoglobin, called hemoglobin S. Some cells have more hemoglobin S than others, which causes some cells, but not all, to sickle. A child with sickle cell always has anemia, which causes shortness of breath, pallor and fatigue. Swollen hands and feet caused by blood flow blockage often occurs as the first sign of disease in infants.
Sickle cells can block blood flow to any organ, including the brain, kidneys, spleen, bones or heart, causing severe pain and interfering with the organ's functioning. A child with sickle cell anemia may have an increased risk of infection due to problems with the spleen, which helps fight infection, MayoClinic.com states. Children with sickle cell anemia may have a short torso but long arms and legs.
Diagnosis
Over 40 states require newborn blood testing for sickle cell anemia, KidsHealth reports. If the screening test is positive, a second blood test, called a hemoglobin electrophoresis, confirms the diagnosis.
Treatment
There's no cure for sickle cell anemia. Regular blood transfusions may reduce the risk of stroke in susceptible children, MayoClinic.com advises. Staying current with immunizations and antibiotics given daily help reduce the chance of infection in children. The only cure for the disease, a bone marrow transplant, carries significant risks and the need to take immunosuppressive medications for life.
Complications
Sickle cell crisis, which occurs whenever a child's oxygen levels drop due to exertion, high altitudes or illness, can cause severe chest pain and shortness of breath. Severe abdominal pain, fever and vomiting may also occur, says the Merck Manual. Infections occur frequently due to spleen damage. Stroke can occur if blood flow to the brain becomes blocked, leading to facial droop, seizures, weakness, speech problems or loss of consciousness, Nemours KidsHealth warns.
