Imagine an illness at birth that causes lifelong complications for the child, the family, and the healthcare providers. Sickle-cell is a disease that affects thousands of people around the world and unfortunately that number continues to grow. Sickle-cell is a disease that many healthcare workers will encounter, especially in the acute care setting. It is important that nurses can recognize the symptoms and know how to treat these patients. With proper treatment and care, many of these patients will survive and live longer lives. Pathophysiology/Description
Sickle-cell disease is caused by a gene transferred from parents to child. This gene affects the shape of red blood cells changing them from a circular shape to a crescent moon shape. As a result, loss of elasticity occurs reducing the ability to carry oxygen to organs. Misshaped RBC's can become lodged in blood vessels blocking the flow of blood causing pain, vessel occlusion, and ischemia. Because of the nature of the disease and its effects on the cells, the spleen becomes engorged multiple times, causing infarction of the spleen. Bone marrow attempts to compensate by creating new red cells, but it cannot keep up with the destruction rate as sickle cells last 10 to 20 days ("Sickle Cell Disease", 2012). Signs and Symptoms/Labs
There are several signs and symptoms of Sickle-cell disease and include some form of anemia. Acute chest syndrome is an inflammation, infection, and occlusion of small vessels, symptoms include chest pain, coughing, difficulty breathing, and fever. Hand-foot syndrome is the painful swelling of hands and feet of infants and is the first sign on this syndrome. Because of the reduced amount of oxygen in the system, those affected by the disease are more prone to infections resulting in high fevers. Prolonged pain throughout the chest, abdomen, and joints is another leading indicator. As the spleen becomes engorged from trapping the deformed RBC's, anemia results causing paleness, weakness, and fatigue. Trapped sickle cells may also result in stroke.
To diagnose one with Sickle Cell Disease different tests must be completed. A Sickle Turbidity test tube will identify the hemoglobin S, the Sickle Cell disease trait. Hgb Electrophoresis determines if it is Sickle Cell anemia versus the Sickle Cell trait. Erythrocyte Fragility tests how long the RBC survives and indicates any abnormality of the RBC ("Blood Test: Hemoglobin Electrophoresis", 2012). Once diagnosed other tests may need to be done to show if any organ damage and/or bone damaged has occurred. Medical Treatment
There is no cure for Sickle Cell Disease except possibly a Bone Marrow Transplant. When providing care of the patient presenting this disease there are two objectives, to manage the pain, and to manage the complications of the disease.
Vaso-occlusive crises (VSO) cause severe deep gnawing and throbbing pain followed frequently by a post-crises residual aching pain. According to "Managing The Problem Of Pain In Adolescents With Sickle Cell" (2004), "The pain is challenging to treat as its duration, location, severity, and frequency changes" (Para. 13). NSAIDs are often used but must be done with care as they could damage the kidneys that may already be damaged by SCD. Those adolescents with severe SCD may have to be treated with opiates. The half-life of morphine in children with SCD is 80 minutes, 40 minutes faster than usual, leading to a greater risk of frequent re-medication and opiate tolerance ("Managing The Problem Of Pain In Adolescents With Sickle Cell", 2004).
Titrated oral morphine with adjunctive use of NSAIDs can be for the acute pain, particularly when the vaso-occlusion affects the bones. Subcutaneous morphine is also effective and can be used as a PCA pump only with proper patient, family, and staff education along with protocols in place. Pain Management may also include nerve blocks, massage, hydrotherapy, and...
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