Sickle Cell Anemia

Sickle Cell Anemia

Pathophysiology:
Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal crescent shape. The “sickling” occurs because of a mutation in the hemoglobin gene. Sickle cells are stiff and sticky. They tend to block blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain, serious infections, and organ damage. Life expectancy in people who have this disease is shortened. Anemia is a condition in which your blood has a lower than normal number of red blood cells. This condition also can occur if your red blood cells don't contain enough hemoglobin. Red blood cells are made in the spongy marrow inside the large bones of the body. Bone marrow is always making new red blood cells to replace old ones. Normal red blood cells live about 120 days in the bloodstream and then die. They carry oxygen and remove carbon dioxide from your body. In sickle cell anemia, the number of red blood cells is low because sickle cells don't last very long. Sickle cells usually die after only about 10 to 20 days. The bone marrow can't make new red blood cells fast enough to replace the dying ones.
Sickle cell anemia is an inherited, lifelong disease. People who have the disease are born with it. They inherit two genes for sickle hemoglobin—one from each parent. People who inherit a sickle hemoglobin gene from one parent and a normal gene from the other parent have a condition called sickle cell trait. Sickle cell trait is different than sickle cell anemia. People who have sickle cell trait don't have the disease, but they have one of the genes that cause it. Like people who have sickle cell anemia, people who have sickle cell trait can pass the sickle hemoglobin gene on to their children.
Symptoms:
Symptoms usually don't occur until after age 4 months. Almost all patients that are diagnosed with sickle cell anemia have crises. Crises are painful episodes that can affect the bones of the back, the chest and long bones. These crises vary among all sickle cell patients. Some may have a crisis once every few years. Others can have multiple crises per year. These crises can be severe enough to keep the patient at a hospital.
Common symptoms include: * Attacks of abdominal pain * Bone pain * Breathlessness * Delayed growth and puberty * Fatigue * Fever * Paleness * Rapid heart rate * Ulcers on the lower legs * Yellowing of the eyes and skin
Other symptoms include: * Chest pain * Excessive thirst * Frequent urination * Painful and prolonged erection * Poor eyesight/blindness * Strokes * Skin ulcers

Treatment: Sickle cell anemia has no widely available cure, so people who have sickle cell anemia need regular medical care. Some doctors and clinics specialize in treating people who have the disease. Hematologists specialize in treating adults and children who have blood diseases or disorders. Treatments can help relieve symptoms and treat complications. The goals of treatment are to manage and control symptoms, and to limit the number of crises. Patients with sickle cell disease need ongoing treatment, even when they are not having a painful crisis. Folic acid supplements should be taken. Folic acid is needed to make red blood cells.

Treatment for a sickle cell crisis includes:

* Blood transfusions (This procedure can also be done regularly to prevent strokes) * Folic acid supplements should be taken. (Folic acid is needed to make red blood cells) * Pain medicines * Plenty of fluids

RX: There is no real cure for Sickle cell anemia. There are ways to help suppress the number of crises, the pain and to help prevent any infections that may occur. Patients are given antibiotics, and pain-relieving medications, and Hydroxyurea, a medicine that may help reduce the number of crises (including chest pain and difficulty breathing) in some people. This medicine prompts your body to make fetal hemoglobin. Fetal hemoglobin, or hemoglobin F, is the type of hemoglobin that newborns have. Fetal hemoglobin helps prevent red blood cells from “sickling” and improves anemia in people who have sickle cell anemia.

Nursing Application:
1. Nursing Diagnosis: Altered health maintenance R/T lack of knowledge regarding long term care. Nursing Intervention: Teach, educate, and instruct the patient and/or caregivers about the signs and symptoms to watch for. Evaluation/Outcome: Check the patient to see if he maintaining and improving his condition.
2. Nursing Diagnosis: Potential for alteration in self concept, related to: chronic health condition, loss or impairment of body functions, perception of being different from peer, disturbance of body image, disease potentially fatal. Nursing Intervention: Encourage counseling, encourage participation in group activities, Nurse to be available weekly if needed for assistance. Evaluation/Outcome: Patient will feel comfortable about talking and sharing with peers and staff about disease, patient will understand how to deal with the disease and take care of themselves.