Genetic Disorder

Sickle Cell Anemia

Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape. Symptoms include, fever, swelling of the hands and feet, pain in the chest, abdomen, limbs, and joints, nosebleeds and frequent upper respiratory infections, fatigue and shortness of breath (signs of anemia), irritability, jaundice (yellowish discoloration of the skin and eyes), delayed puberty (in young teenagers), severe joint pain, progressive anemia, Leg sores, gum disease, vision problems. Blood tests can determine whether an individual has sickle cell trait or sickle cell disease. Treatment goals for sickle cell disease aim to relieve pain, prevent infections, and manage complications. Antibiotics, usually penicillin, are commonly given to infants and young children, as well as adults, to help prevent infections. Pain relief medication ranging from nonprescription nonsteroidal anti-inflammatory drugs (NSAIDs) to opiods are given to control pain. Hydroxyurea is prescribed for patients with moderate-to-severe sickle cell disease to help reduce the frequency of pain episodes and acute chest syndrome. There has not been a method proven to prevent sickle cell disease. But by taking the correct precautions to health problems is showing patients to live longer. The expected life expectancy with sickle cell disease is 42 years old for males and 48 for females.