blood disorders
Sickle Cell Disease
Symptoms of Sickle Cell Disease
People who have sickle cell disease always have some degree of anemia (often causing fatigue, weakness, and paleness) and may have mild Jaundice in Adults (yellowing of the skin and whites of the eyes). Some people have few other symptoms. Others have severe, recurring symptoms that cause enormous disability and early death. In people with sickle cell trait, red blood cells are not fragile and do not break easily. Sickle cell trait does not cause painful crises, but rarely, people die suddenly while undergoing very strenuous exercise that causes severe dehydration, such as during military or athletic training. People with sickle cell trait are at increased risk of Chronic Kidney Disease and Anything that reduces the amount of oxygen in the blood, such as vigorous exercise, mountain climbing, flying at high altitudes without sufficient oxygen, or an illness, may bring on a sickle cell crisis (also called an exacerbation). A sickle cell pain (vaso-occlusive) crisis is an episode of increased symptoms and can consist of a sudden worsening of anemia, pain (often in the abdomen or long bones of the arms and legs), fever, and sometimes shortness of breath. Abdominal pain may be severe, and vomiting may occur. Sometimes, additional complications occur with a pain crisis, including Aplastic crisis: Production of red blood cells in the bone marrow stops during infection with some viruses Acute chest syndrome: Caused by blockage of capillaries in the lungs Acute splenic or hepatic (liver) sequestration (a large accumulation of cells in an organ): Rapid enlargement of the spleen or liver Acute chest syndrome can occur in people of all ages, but it is most common among children. It is usually characterized by severe pain and difficulty breathing. Acute chest syndrome can be fatal. In children, acute sequestration of sickled cells in the spleen (sequestration crisis) may occur causing an enlarged spleen and worsening anemia. Acute hepatic (liver) sequestration is less common and can occur at any age. Most people who have sickle cell disease develop an Enlarged Spleen during childhood because sickled cells become trapped in the spleen. By the time the person reaches adolescence, the spleen is often so badly injured that it shrinks and no longer functions. Because the spleen helps fight infection, people with sickle cell disease are more likely to develop The liver can become progressively larger throughout life (causing upper abdominal fullness), and Gallstones often form from the pigment of broken-apart red blood cells. The heart usually enlarges, and an enlarged heart is less effective in pumping blood to the body, possibly leading to Heart Failure (HF) . Heart murmurs are common. Children who have sickle cell disease often have a relatively short torso but long arms, legs, fingers, and toes. Changes in the bones and bone marrow may cause bone pain, especially in the hands and feet. Episodes of joint pain with fever may occur, and the hip joint may become so damaged that it eventually needs to be replaced. Poor circulation to the skin may cause sores on the legs, especially at the ankles. Young men may develop persistent, often painful erections ( Persistent Erection ). Episodes of priapism may permanently damage the penis so that the man can no longer have erections. Blocked blood vessels may cause strokes that damage the nervous system. In older people, lung and kidney function may deteriorate. 