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.
Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain.
The sickling of red blood cells causes the signs and symptoms of sickle cell disease. When red blood cells sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain. A particularly severe complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of adults with sickle cell disease and can lead to heart failure.
Abnormal versions of beta-globin can distort red blood cells into a sickle shape. The sickle-shaped red blood cells die prematurely, which can lead to anemia. Sometimes the inflexible, sickle-shaped cells get stuck in small blood vessels and can cause serious medical complications. Usually, your red blood cells are flexible and round, moving smoothly through your blood vessels. In sickle cell anemia, the red blood cells become rigid and sticky and have sickle-like or crescent moon shape. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.
Sickle cells break apart easily and die, leaving you without enough red blood cells. Red blood cells usually live for about 120 days before they replace it. But sickle cells often die in 10 to 20 days, leaving a shortage of red blood cells (anemia).
Without enough red blood cells, your body can’t get the oxygen; it needs to feel energized, causing fatigue.
Periodic appearance of illness, called crises, is a significant symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen, and joints. Pain can also occur in your bones.
Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers, and other causes. Painful swelling of hands and feet is by sickle-shaped red blood cells blocking blood flow to the hands and feet.
Sickle cells can damage an organ that fights infection (spleen), leaving you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life-threatening diseases, such as pneumonia.
You can live a full, active life when you have sickle cell disease. Making smart choices is essential in keeping the condition from flaring into a crisis.
Until Next Time,
Team Doctor ASKY!