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SICKLE CELL DISEASE Definition: • Sickle cell anemia is a disease in which your body produces abnormally shaped red blood cells. The cells are shaped like a crescent or sickle. They don't last as long as normal, round red blood cells, which leads to anemia. The sickle cells also get stuck in blood vessels, blocking blood flow. This can cause pain and organ damage. A genetic problem causes sickle cell anemia. People with the disease are born with two sickle cell genes, one from each parent. If you only have one sickle cell gene, it's called sickle cell trait. About 1 in 12 African Americans has sickle cell trait. A blood test can show if you have the trait or anemia. Most states test newborn babies as part of their newborn screening programs. Rbc life span reduced to 6-20 days only • • Anatomy & Physiology: Red blood cells (erythrocytes)  The blood cells that carry oxygen. Red cells contain hemoglobin and it is the hemoglobin which permits them to transport oxygen (and carbon dioxide). Hemoglobin, aside from being a transport molecule, is a pigment. It gives the cells their red color (and their name).  An erythrocyte is a disc-shaped cell with a thick rim and a thin sunken centre. The plasma membrane of a mature RBC has glycoproteins and glycolipids that determine a person's blood type. On its inner surface are two proteins called spectrin and actin that give the membrane resilience and durability. This allows the RBCs to stretch, bend and fold as they squeeze through small blood vessels, and to spring back to their original shape as they pass through larger vessels.  Circulating erythrocytes live for about 120 days. As a RBC ages, its membrane grows increasingly fragile. Without key organelles such as a nucleus or ribosomes, RBCs cannot repair themselves. Many RBCs die in the spleen, where they become trapped in narrow channels, broken up and destroyed. Haemolysis refers to the rupture of RBCs, where haemoglobin is released leaving empty plasma membranes which are easily digested by cells known as macrophages in the liver and spleen. The Hb is then further broken down into its different components and either recycled in the body for further use. Hemoglobin o Heme is the pigmented iron-containing nonprotein part of the hemoglobin molecule. There are four heme groups in a hemoglobin molecule, each consisting of a cyclic structure of four pyrrole residues, called protoporphyrin, and an atom of iron in the center. Heme binds and carries oxygen in the red blood cells, releasing it to tissues that give off excess amounts of CO2. o Globin is a long polypeptide chain. One of the component protein molecules found in hemoglobin. Normal adult hemoglobin has a pair each of alpha-globin and betaglobin molecules. Etiology: Sickle cell anemia is an inherited disease. People who have the disease inherit two genes for sickle hemoglobin—one from each parent. Symptomatology: The signs and symptoms of sickle cell anemia vary. Some people have mild symptoms. Others have very severe symptoms and often are hospitalized for treatment. Sickle cell anemia is present at birth, but many infants don't show any signs until after 4 months of age. The most common signs and symptoms are linked to anemia and pain. Other signs and symptoms are linked to the disease's complications. Signs and Symptoms Related to Anemia The most common symptom of anemia is fatigue (feeling tired or weak). Other signs and symptoms of anemia may include:       Shortness of breath Dizziness Headaches Coldness in the hands and feet Paler than normal skin or mucous membranes (the tissue that lines your nose, mouth, and other organs and body cavities) Jaundice (a yellowish color of the skin or whites of the eyes) Signs and Symptoms Related to Pain Sudden pain throughout the body is a common symptom of sickle cell anemia. This pain is called a sickle cell crisis. Sickle cell crises often affect the bones, lungs, abdomen, and joints. These crises occur when sickled red blood cells block blood flow to the limbs and organs. This can cause pain and organ damage. The pain from sickle cell anemia can be acute or chronic, but acute pain is more common. Acute pain is sudden and can range from mild to very severe. The pain usually lasts from hours to as long as a week or more. Many people who have sickle cell anemia also have chronic pain, especially in their bones. Chronic pain often lasts for weeks or months and can be hard to bear and mentally draining. Chronic pain may limit your daily activities. Almost all people who have sickle cell anemia have painful crises at some point in their lives. Some have these crises less than once a year. Others may have crises once a month or more. Repeated crises can damage the bones, kidneys, lungs, eyes, heart, and liver. This type of damage happens more often in adults than in children. Many factors can play a role in sickle cell crises. Often, more than one factor is involved and the exact cause isn't known. You can control some factors. For example, the risk of a sickle cell crisis increases if you're dehydrated (your body doesn't have enough fluids). Drinking plenty of fluids can lower the risk of a painful crisis. You can't control other factors, such as infections. Painful crises are the leading cause of emergency room visits and hospital stays for people who have sickle cell anemia. Medical Management:  Laboratory Tests Tests commonly performed to diagnose and monitor patients with sickle cell anemia include: Bilirubin Blood oxygen Complete blood count (CBC) Hemoglobin electrophoresis Serum creatinine Serum potassium Sickle cell test  Medications o Antibiotics. Children with sickle cell anemia may begin taking the antibiotic penicillin when they're about 2 months of age and continue taking it until they're 5 years old. Doing so helps prevent infections, such as pneumonia, which can be life-threatening to an infant or child with sickle cell anemia. Antibiotics may also help adults with sickle cell anemia fight certain infections. o Pain-relieving medications. To relieve pain during a sickle crisis, your doctor may advise over-the-counter pain relievers and application of heat to the affected area. You may also need stronger prescription pain medication. o Hydroxyurea (Droxia, Hydrea). When taken daily, hydroxyurea reduces the frequency of painful crises and may reduce the need for blood transfusions. It may be an option for adults with severe disease. Hydroxyurea seems to work by stimulating production of fetal hemoglobin — a type of hemoglobin found in newborns that helps prevent the formation of sickle cells. Hydroxyurea increases your risk of infections, and there is some concern that long-term use of this drug may cause tumors or leukemia in certain people.  Treatment o Analgesics/narcotic for pain o Antibiotics for infection o Hydration- oral and IV o Bed rest o oxygenation Nursing Diagnoses:  Acute pain r/t tissue hypoxia  Ineffective tissue perfusion r/t accumulation of sickle red blood cells within vessels  Deficient knowledge r/t sickle crisis prevention  Risk for infection r/t altered immune system s/t immunosuppressants  Risk for powerlessness r/t illness Nursing Management: * Manage acute crises. During an acute crisis, comfort measures, use of analgesics, and complementary approaches such as massage and distraction are key. Healthcare providers focus on hydration, prevention of infections, and early recognition of complications. Pharmacologic treatment is started immediately to manage pain. Some individuals need blood transfusions to manage severe anemia. Indications for blood transfusions include splenic sequestration, acute chest syndrome, severe anemia, and stroke. * Teach effective pain management measures. Pain is a recurring factor in SCD. Pharmacologic and nonpharmacologic measures to control and/or decrease the severity of pain episodes are essential. These include physical and occupational therapy, physiotherapy, cognitive-behavioral therapy, and support groups. If your patient is a child, her parents need to understand effective measures and provide them to control pain. Reading and watching TV/videos can be effective distraction techniques. For the teen exploring hobbies, choosing physical activities such as swimming, especially in a heated pool, can improve pain management and physical stamina while limiting stress on joints. * Teach effective lifestyle modifications. It's crucial that children and adults who have SCD learn effective lifestyle modifications. Parents need to encourage their child to engage in normal childhood activities when possible. Play is crucial, but some activities may need to be modified. For the teen, the task is to recognize what triggers a crisis, manage or eliminate these factors, and compensate for less appropriate activities with alternatives that bring a sense of accomplishment and enjoyment. * Instruct when to contact the healthcare provider. Symptoms to be reported include fever; swelling of the hands or feet; swelling of the abdomen and lower left side with tenderness; pain in the joints, chest, or muscles; pale skin or nail beds or a yellowish skin color (jaundice); sudden fatigue with less interest in surroundings; and an erection of the penis that won't go away. * Teach stress management techniques. Stress triggers sickle cell crises and decreases coping abilities. Learning how to effectively reduce stress through breathing, muscle relaxation, meditation, and guided imagery can assist your patient in gaining a sense of control. Maintaining a balance of nutrition, rest, and exercise is also important. A child with SCD may not yet be able to identify how to effectively balance rest and exercise, so parents need to manage this. A teen with SCD should be encouraged to participate in the process. Joining a support group for people who have SCD or chronic diseases may be helpful. The use of social networking sites such as Facebook and Twitter may decrease isolation and stress. * Encourage the acknowledgment of feelings. Many people living with SCD find it helpful to line up supportive individuals, such as parents, teachers, and friends. Others talk with a therapist or join a support group specifically for people with their condition. It's important for your patient to acknowledge her emotions, talk about feelings of anger and dependence, and recognize these as part of the illness. Encourage strategies that increase a sense of control over your patient's life to decrease feelings of powerlessness and hopelessness. Families may become overprotective of their children, stifling their growth and ability to manage their disease. Adolescents, especially, need to be allowed to determine treatment strategies. Teens may be concerned with, and need to discuss, body image issues related to delayed sexual maturity.