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Sickle cell anemia is an inherited blood disorder that causes lifelong anemia and periodic pain and other complications. Hemoglobin is a protein carried by the red blood cells. In healthy individuals, the red blood cells containing hemoglobin A are round and soft. In people with sickle cell anemia, hemoglobin A has been replaced with sickle-shaped hemoglobin or hemoglobin S, which in addition to being abnormally shaped, are hard and rigid. This rigidity and distorted shape make it difficult for hemoglobin S to pass through small blood vessels, causing blockages that decrease the amount of oxygenated blood reaching tissues and vital body organs, causing not only damage but intense pain. The frequency and amount of pain varies from one patient to another. Pain is the principal symptom of sickle cell anemia in children and adults, alike. Sickle-shaped red blood cells have a short lifespan, which leads to a low level of hemoglobin (anemia).
Sickle cell anemia is found in many parts of Africa, North America, South America, Central America, the West Indies, the Middle East, India, and Mediterranean countries. It is genetically passed on from parents to their children. An individual who inherits a single sickle cell gene from one parent is known as a carrier. A carrier does not have any symptoms of the disease and can lead a normal, healthy life. Individuals with sickle cell anemia have inherited two sickle cell genes, one from each parent. These individuals will have symptoms of the disease.
There are three types: sickle cell anemia, sickle cell hemoglobin C disease, and sickle cell beta thalassemia disease.
People with sickle cell anemia experience periods of pain that affect different parts of the body, particularly the abdomen and joints. Feeling lethargic and suffering from chronic fatigue are also symptoms. Affected people are at risk for malnutrition, stunted growth, frequent infections, bone deformities, and visual impairment.
To diagnose sickle cell anemia, a simple blood test is carried out. If the test is positive, a second blood test called hemoglobin electrophoresis is performed to determine if the patient has the disease or is a carrier. This test can be done at any age. In Saudi Arabia, it is a routine screening test performed on all newborns.
The most effective way to prevent sickle cell anemia is to avoid marriage between carriers or between people who have the disease. If a carrier marries another carrier, the couple is at risk of having children with the disease or children who are carriers. To prevent this, carriers are advised to select marriage partners who are not carriers. This is best done through premarital screening and counseling. The goal of premarital screening and counseling is to promote health in families. Having a child with sickle cell anemia is difficult for not only the child but the entire family. Premarital screening for sickle cell anemia is required by the Saudi Ministry of Health. The screening procedure requires a simple blood test from the prospective wife and the prospective husband. If the prospective wife and the prospective husband are both carriers, they will be advised to consult a genetic counselor. The counselor will provide information to enable them to make appropriate decisions about marriage, reproduction, and health management.
The treatment for sickle cell anemia focuses on maintaining a normal hemoglobin level and managing the frequency, duration, and severity of pain. Blood transfusions are commonly used to treat anemia. Hydroxyurea treatments (a mild chemotherapy agent) significantly decrease episodes of pain. Drinking plenty of fluids also helps to prevent or minimize pain.