· Sickle hemoglobin C disease (also known as hemoglobin SC disease)
· Sickle beta thalassemia disease (also known as sickle beta plus thalassemia disease and sickle beta zero thalassemia disease)
· Sickle cell anemia (also known as hemoglobin SS disease, or homozygous sickle cell disease)
What is sickle cell disease?
Sickle cell disease is a group of blood disorders that prevent the normal flow of blood in the body because of the effect on the hemoglobin within red blood cells.
· Hemoglobin is the main ingredient in red blood cells, helping them carry oxygen from the lungs to other parts of the body.
· Normal red blood cells have hemoglobin A, which helps keep red blood cells soft and round so they flow easily through small blood vessels.
· People with sickle cell disease have mostly hemoglobin S (also called sickle hemoglobin) in their red blood cells.
· Hemoglobin S can form hard fibers inside the red cells, causing them to reshape into sickle (banana) shape. Abnormally shaped red blood cells cannot move through blood vessels easily and at times are blocked from delivering oxygen to some body tissues.
· Sickle cell disease is always inherited (passed down in families).
· When one parent has sickle cell disease and the other carries only one of the abnormal genes, such as hemoglobin S, their child has a 1 in 2 (50 percent) chance of having sickle cell disease.
· When both parents carry a sickle cell gene, but are not sick themselves, their child has a 25 percent (1 in 4) chance of having sickle cell disease.
How common is sickle cell disease?
· Sickle cell disease is the most common inherited blood disorder in the United States, affecting about 100,000 Americans.
· It can occur in all races but is most common in African-Americans and Hispanics. About one out of 500 African-American babies born in the US has sickle cell disease.
What are the symptoms of sickle cell disease?
Symptoms of sickle cell disease include:
· Painful swelling of hands and feet
· High fevers because of weakened defenses against bacterial infection
· Severe pain in various parts of the body—may require hospital stays
· Paleness, fatigue and rapid pulse because of anemia from fewer red blood cells
· Chest pain
· Trouble breathing
· Enlarged spleen
· Yellowing of eyes and skin (jaundice)
· Delayed growth or onset of puberty
· Abdominal (belly) pain
· Infections, including urinary tract infections and pneumonia
How is sickle cell disease treated?
· Transfusions of red blood cells — are given every three to four weeks. This is the main treatment for fighting the strokes that can occur in children with sickle cell disease.
· Complications can include iron overload, infection, antibody formation and unwanted transfusion reactions that can mimic symptoms, such as anemia.
· Hydroxyurea — is a drug that is used to help reduce cell sickling as well as many of the major complications of sickle cell disease
· Hydroxyurea is a once-daily medication taken by mouth.
· The treatment goal is to boost normal hemoglobin levels by increasing the patient’s level of fetal hemoglobin (HbF), which normally decreases very quickly after birth.
· Stem cell transplant — is the only cure for sickle cell disease.
· The cure was first performed successfully in 1983, when a St. Jude patient with leukemia and sickle cell disease received a bone marrow transplant. The procedure cured both diseases.
· During a transplant, the patient’s bone marrow is replaced with marrow from a person who does not have sickle cell disease. The transplanted cells grow into healthy blood cells.
· Even though bone marrow transplant is a cure for sickle cell disease, its use is limited because of the problems in finding a matched donor, and the complications associated with transplant.
· Some types of stem cell transplants may be called “bone marrow transplants” because the cells come from the donor’s bone marrow.
· Other treatments include antibiotics, pain medicines and psychological counseling. These are used mainly to treat side effects of sickle cell anemia.
What are the survival rates for sickle cell disease?
· Overall, the lives of people with any type of sickle cell disease are typically 20 to 30 years shorter than those of people who do not have it.
· Formerly, 50 percent of sickle cell disease patients did not live beyond age 20, and most did not survive to age 50. Today, with early diagnosis and use of recently developed treatments, the life expectancy of children with sickle cell disease has increased 98 percent. For example, people with hemoglobin SS may live to age 50 or older.
Source: St. Jude Children’s Research Hospital
May 15, 2015
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