Sickle cell disease interferes with hemoglobin production, the part of the red blood cell that carries oxygen, and causes red blood cells to change shape. The altered shape resembles a sickle or letter C. The abnormal red blood cells die early, interfere with circulation and do not carry oxygen well. Sickle cell anemia affects every system of the body and threatens the life of the patient.
Anemia
Healthy red blood cells and hemoglobin carry oxygen to the body's tissues. Anemia is a condition of inadequate red blood cells or hemoglobin. In sickle cell disease, both of these problems occur. Sickle cells die earlier than healthy red blood cells, so the number of mature red blood cells remains low. In addition, the hemoglobin cannot bind with oxygen normally. These effects create the condition of anemia. Patients experience fatigue and shortness of breath. Oxygen therapy decreases symptoms of anemia. In some cases blood transfusions are used to relieve anemia.
Infection
The effects of sickle cell anemia include frequent and serious infections, because sickle cells damage the spleen. The spleen stores and produces blood cells necessary to fight infection. Sickle cell disease also damages the bone marrow, an important source of white blood cells that support the immune system. The National Heart Lung and Blood Institute says that these effects of sickle cell anemia make patients immunocompromised and susceptible to pneumonia, meningitis, influenza and hepatitis infections, all of which can be fatal.
Infarction
An infarction is a lack of blood flow to an area of the body. Sickle cells clump together and block small blood vessels, cutting off blood and oxygen supply to tissues. Any part of the body can be affected, according to Donna D. Ignatavicius, MS, RN, and M. Linda Workman, Ph.D., authors of "Medical-Surgical Nursing: Critical Thinking for Collaborative Care." Symptoms can include stroke, blindness, kidney failure, skin ulcers and swelling, pain and loss of function in extremities. An infarction in the heart may trigger irregular heart rhythms that cause sudden death. An infarction in the bone damages bone marrow, leading to osteoporosis.
Embolism
Another effect of sickle cell anemia is embolism. An embolism, or blood clot, can break free from the blood vessel where it formed and travel anywhere in the body. A pulmonary embolism causes sudden shortness of breath, pain and low oxygen saturation. Low oxygen saturation triggers sickle cell crisis and exacerbates the pulmonary embolism symptoms. An embolism may travel to the brain, causing symptoms of a stroke such as sudden weakness, fainting, loss of speech or vision disturbances. Embolisms are medical emergencies and require immediate care.
Pain
Pain, often acute and for some a chronic symptom, diminishes a sickle cell patient's quality of life. Pain is a result of many of the effects of sickle cell anemia. Anemia and infarction damage tissues and cause pain. Patients in crisis can have severe chest, back and abdominal pain. Chronic pain symptoms affect the muscles and joints.
References
- National Heart Lung and Blood Institute: Sickle Cell Anemia-Signs and Symptoms
- "Medical-Surgical Nursing: Critical Thinking for Collaborative Care"; Donna D. Ignatavicius & M. Linda Workman; 2006
