Module 5: Emergencies of Hematological Disorders PDF

Module 5: Emergencies of Hematological Disorders  Objectives Upon completion of this module, the paramedic student will be able to: 1. Describe the physiology of blood and its components. 2. Discuss pathophysiology and signs and symptoms of specific hematological disorders. 3. Outline gene...

Module 5: Emergencies of Hematological Disorders  Objectives Upon completion of this module, the paramedic student will be able to: 1. Describe the physiology of blood and its components. 2. Discuss pathophysiology and signs and symptoms of specific hematological disorders. 3. Outline general assessment and management of patients with hematological disorders.  Blood and Blood Components Blood is composed of cells and formed elements surrounded by plasma. About 95% of the volume of formed elements consists of red blood cells (RBCs; erythrocytes). The remaining 5% consists of white blood cells (WBCs; leukocytes) and cell fragments (platelets). )figure and table) The continuous movement of blood keeps the formed elements dispersed throughout the plasma, where they are available to carry out their chief functions:  delivery of substances needed for cellular metabolism in the tissues,  defense against invading microorganisms and injury; and  acid–base balance. Next table Cellular Components of the Blood All types of blood cells are formed within the red bone marrow, which is present in all tissues at birth.  In adults, the red bone marrow is mainly found in membranous bones such as the vertebrae, pelvis, sternum, and ribs. Yellow marrow produces some WBCs but is composed mainly of connective tissue and fat. Other blood-forming organs include the following:  Lymph nodes, which produce lymphocytes and antibodies  The spleen, which stores large quantities of blood and produces lymphocytes, plasma cells, and antibodies  The liver, a blood-forming organ only during intrauterine life, which plays an important role in the coagulation process  Plasma  The clear portion of blood, is approximately 91.5% water. In addition to plasma proteins (7%), electrolytes (1%), , and inorganic compounds (0.5%).  Plasma contains three important proteins: I. Albumin—is the most plentiful protein that gives blood its gummy texture. The presence of these large proteins keeps the water concentration of blood low so that water diffuses from tissues into the blood. II. Globulins (alpha, beta, and gamma) transport other proteins and provide the body with immunity to disease. III. Fibrinogen is essential for blood clotting  Functions: maintaining blood pH; transporting fat-soluble vitamins, hormones, and carbohydrates; and allowing the body to digest them temporarily for food.  Red Blood Cells RBCs, the most abundant cells in the body, are mainly responsible for tissue oxygenation. They appear as small rounded disks with nearly hollowed-out centers They are composed mainly of water and the red-colored protein hemoglobin.  RBC production occurs in the bone marrow and continues throughout life to replace blood cells that grow old and die, are killed by disease, or are lost through bleeding. The new shape of the RBC increases the surface area of the cell and, therefore, its oxygen-carrying potential.  RBCs have a life span of about 120 days. As the cells age, their internal chemical machinery weakens, they lose elasticity, and they become trapped in small blood vessels in the bone marrow, liver, and spleen. They then are destroyed by specialized WBCs (macrophages).  Most components of destroyed hemoglobin molecules are used again, some are broken down to the waste product bilirubin Each RBC contains approximately 270 million hemoglobin molecules. Each hemoglobin molecule carries four oxygen molecules. Hb level is about 15 g/dl; No. of RBCs 4.2-6.2 Laboratory Tests Hematocrit is the fraction of the total volume of blood that consists of RBCs. For example, a value of 45% (the normal level) implies that there is 45 mL of RBCs in 100 mL of blood. The normal hematocrit for males is 40% to 54%. In females, a normal hematocrit is 38% to 47%. A low hematocrit value often indicates anemia, which may result from trauma, surgery, internal bleeding, nutritional deficiency (eg, iron or vitamin B12), bone marrow disease, or sickle cell disease. A high hematocrit value may be caused by dehydration, lung disease, certain tumors, and disorders of the bone marrow. The normal hemoglobin level for males is 13.5 to 18 g/dl. In women, a normal hemoglobin measurement is 12 to 16 g/dl. Reticulocyte count offers details about the rate of RBC production. A reticulocyte count of less than 0.5% of the RBC count usually indicates a deceleration in the process of RBC formation. A reticulocyte count greater than 1.5% usually indicates an acceleration of RBC formation.  White Blood Cells WBCs arise from the bone marrow and are released into the bloodstream. WBCs destroy foreign substances (eg, bacteria, viruses) and clear the bloodstream of debris. Leukocyte production increases in response to infection, which in turn causes an elevated WBC count in the blood. The bone marrow and lymph glands continually produce and maintain a reserve of WBCs. However, there are not many WBCs in the bloodstream of a healthy person—only 5,000 to 10,000 cells/mm2 Monocytes make up about 5% of the total WBC count, though their concentration increases with chronic infections. Lymphocytes account for about 27.5%, neutrophils about 65%, and eosinophils and basophils together about 2.5% of the total WBC count A rise in the number of WBCs aids in the diagnosis of some diseases. For example, an increased WBC count is suggestive of illnesses such as bacterial infection, inflammation, leukemia, trauma, and stress The differential count (also called the diff) identifies the different types of leukocytes (WBCs) present in blood. This test is performed by spreading a drop of blood on a microscope slide, staining the slide, and examining it under a microscope.  Cells are identified by the shape and appearance of the nucleus, the color of cytoplasm (the background of the cell), and the presence and color of granules. The percentage of each cell type is reported. At the same time, RBCs and platelets are examined for abnormalities in appearance.  Platelets Platelets (thrombocytes) are small, sticky cell fragments. They play an important role in blood clotting. When a blood vessel is cut, platelets travel to the site, where they swell into odd, irregular shapes and adhere to the damaged vessel wall. In this way, platelets plug the leak and allow other cells to stick to them and to form a clot. However, if the damage to the vessel is too great, the platelets chemically signal for the complex clotting process or clotting cascade to begin (described later). Platelets repair millions of ruptured capillaries each day and often render the rest of the clotting cascade unnecessary (BOX 32-2). Clotting Measurements Clotting time is normally 7 to 10 minutes. The patient bleeds if the clotting time is prolonged; he or she develops intravascular clots if the clotting time is less than normal. Prothrombin time (PT) measures the clotting time of plasma. The PT test is used to monitor patients taking certain medications (eg, warfarin), to monitor patients with liver failure, and to diagnose clotting disorders. It specifically evaluates the presence of factors V, VIIa, and X and of prothrombin and fibrinogen. A drop in the concentration of any of these factors will cause the blood to take longer to clot, so a prolonged PT time is considered abnormal. The PT test is used in combination with the partial thromboplastin time (PTT) to screen for hemophilia and other hereditary clotting disorders  Hemostasis Hemostasis is the initial physiologic response to wounding that causes bleeding to cease. The vascular reaction or physiology of hemostasis involves vasoconstriction, formation of a platelet plug, coagulation, and the growth of fibrous tissue into the blood clot that permanently closes and seals the injured vessel.  Vasoconstriction resulting from injury is rapid but temporary. In response to injury, severed blood vessels constrict and retract with the aid of the surrounding subcutaneous tissues. This vessel spasm slows blood loss immediately. Vasoconstriction may close the ends of the injured vessels completely. This response usually is sustained for as long as 10 minutes. During this time, blood coagulation mechanisms are activated to produce a blood clot  Hemostasis Platelets adhere to injured blood vessels and to collagen in the connective tissue that surrounds the injured vessel. As platelets contact collagen, they swell, become sticky, and secrete chemicals that activate other surrounding platelets. This process, which causes the platelets to adhere to one another, creates a platelet plug in the injured vessel.  If the opening in the vessel wall is small, the plug may be sufficient to stop blood loss completely. If the opening in the vessel is large, however, a blood clot is necessary to arrest the flow of blood.  Hemostasis Blood coagulation occurs as a result of a chemical process that begins within seconds of a severe vessel injury. Coagulation progresses rapidly; Within 3 to 6 minutes after the rupture of a vessel, the entire end of the vessel is filled with a clot. Within 30 minutes, the clot retracts, and the vessel is sealed further.  The blood-clotting mechanism is a complex process that includes three mechanisms (FIGURE 32-3): 1. Prothrombin activator is formed in response to rupture or damage of the blood vessel. 2. Prothrombin activator stimulates the conversion of prothrombin to thrombin. 3. Thrombin, in the presence of calcium ions, acts as an enzyme to convert fibrinogen into fibrin threads. These threads entrap platelets, blood cells, and plasma to form the clot. Specific Hematologic Disorders Hematological disorders presented in this chapter are anemia, leukemia, leukopenia, lymphomas, polycythemia, disseminated intravascular coagulopathy, hemophilia, sickle cell disease, and multiple myeloma (BOX 31-3)  Anemia Anemia is a condition in which the concentration of hemoglobin or erythrocytes in the blood is below normal (TABLE 31-2). Anemia is not a disease itself, but rather a symptom of a disease. Precipitating causes of anemia include chronic or acute blood loss, decreased production of erythrocytes, and increased destruction of erythrocytes. In the case of cancer and chemotherapy, anemia sometimes can be caused by the treatment itself.  Two common forms of anemia that are iron- deficiency anemia and hemolytic anemia.  Iron-Deficiency Anemia Iron is the critical part of a hemoglobin molecule, giving it the ability to bind oxygen (FIGURE 32-4). The lack of iron associated with iron-deficiency anemia prevents the bone marrow from making enough hemoglobin for the RBCs. The RBCs produced are small, have a pale center, and have a reduced oxygen- carrying capacity. The most common cause of iron deficiency anemia in adults is blood loss from menstrual bleeding or intestinal bleeding A diet that is low in iron usually is the cause of iron deficiency anemia in children. Vitamin deficiencies also can produce anemia. Lack of folic acid (one of the B vitamins) is the most common form of vitamin-deficiency anemia (Box 32-4). Hemolytic Anemia Premature destruction of RBCs in the blood (hemolysis) causes hemolytic anemia. This destruction can result from an inherited disorder inside the RBC, or it can result from a disorder outside the cell, which is usually acquired later in life. Inherited Disorders. Hemolysis can occur because of abnormal rigidity of the cell membrane. This rigidity causes the cell to become trapped at an early stage of its life span in smaller blood vessels (usually within the spleen). In these smaller blood vessels, the RBC is destroyed by macrophages. This type of anemia can occur from a genetic defect in the hemoglobin within the cell (eg, sickle cell anemia, thalassemia). It also can occur from a defect in one of the enzymes in the cell that helps to protect the cell from chemical damage during infectious illness. A deficiency of one of the enzymes, glucose-6-phosphate dehydrogenase (G6PD), is common in people from parts of Asia, Africa, the Middle East, and the Mediterranean. Acquired Disorders Acquired hemolytic anemia results from one of three conditions: Disorders in which normal RBCs are disrupted by mechanical forces (eg, abnormal blood vessel linings or blood clots) Autoimmune disorders, which can destroy RBCs with antibodies that are produced by the immune system (eg, drug-induced hemolytic anemia or an incompatible blood transfusion) Conditions that can cause hemolytic anemia when RBCs are destroyed by microorganisms in the blood (eg, malaria)  All forms of anemia share common signs and symptoms, including fatigue and headaches, sometimes a sore mouth or tongue, brittle nails, and, in severe cases, breathlessness and chest pain (TABLE 31-3). Other patient complaints are related to an Signs and abnormal decrease in the number of WBCs Symptoms of (leukopenia) or a reduction in platelets (thrombocytopenia) and may include the Anemia following: Bleeding from mucous membranes Cutaneous bleeding Fatigue Fever Lethargy  The patient’s signs and symptoms, history, and blood, as examined through blood tests and bone marrow biopsy, indicate a diagnosis of most Diagnosis forms of anemia. For example, in iron-deficiency anemia, laboratory examination usually reveals and RBCs that are smaller than normal. Treatment  In hemolytic anemia, the examination shows immature and abnormally shaped RBCs. After diagnosis, treatment is begun to correct, modify, or diminish the mechanism or process that is leading to defective RBC production or reduced RBC survival.  Leukemia Leukemia refers to any of several types of cancer in which an abnormal proliferation of WBCs occurs, usually in the bone marrow (FIGURE 31-5). The excess production of leukemic cells crowds and impairs the normal production of RBCs, WBCs, and platelets. Leukemia is more common in males than in females. Exact cause of leukemia is not known; however, genetics may play a role. Abnormal chromosomes associated with congenital disorders (eg, Down syndrome) and HIV-type viruses are associated with certain forms of this disease. Other factors that may play a role in the development of leukemia include exposure to radiation, viral infections, immune defects, and exposure to various chemicals in home and work environments.   Classifications Leukemia is classified as either acute or chronic. Cancer cells in acute leukemia begin proliferating at an early stage of their development; that is, their development is arrested when they are immature cells. Chronic leukemia implies an abnormal proliferation of more mature but not fully differentiated cells. Leukemias are classified further according to the type of WBC involved (BOX 31-5).  Two common forms of leukemia are acute lymphocytic leukemia (ALL) and acute myelogenous leukemia (AML).  ALL affects mostly children younger than 15 years and, therefore, is sometimes called childhood leukemia. AML affects mostly middle-aged adults. In both types, abnormal WBCs are produced in such large amounts that they eventually accumulate in the vital organs— for example, the liver, spleen, lymph, and brain. This accumulation impedes the function of these organs and leads to death.  Chronic forms of leukemia can develop slowly, often over many years. Cases of disease often are discovered by chance during routine blood analysis  The proliferation of leukemic cells or the resulting inadequate production of other normal blood cells makes the patient with leukemia highly susceptible to serious infections, anemia, and bleeding episodes. Signs and symptoms of leukemia include the following: Abdominal fullness Bleeding Bone pain Signs and Elevated body temperature and diaphoresis (ie, sweating) Symptoms Enlargement of lymph nodes Enlargement of the liver, spleen, and testes Fatigue Frequent bruising Headache Heat intolerance Night sweats Weight loss  The diagnosis of leukemia is confirmed by bone marrow biopsy. The severity of the disease is assessed by the degree of liver and spleen enlargement, extent of anemia, and lack of platelets in the blood. Diagnosis  Treatment includes the transfusion of blood and platelets, antibiotic therapy to manage anemia and and infection, and the use of anticancer drugs and Treatment sometimes radiation to destroy the leukemic cells. In some cases, the leukemia is treated with a bone marrow transplant (BOX 31-6).  Patients with chronic leukemia can be managed effectively with medication. Many patients require no treatment in its preliminary stages  Lymphomas Lymphoma is a general term applied to any neoplastic disorder of the lymphoid tissue.  Hodgkin lymphoma non-Hodgkin lymphomas. All lymphomas are malignant—that is, cancerous tumors that tend to metastasize  Hodgkin Lymphoma Hodgkin lymphoma (formerly known as Hodgkin disease) is characterized by painless, progressive enlargement of lymphoid tissue found mainly in the lymph nodes and spleen (FIGURE 31-6). Left unchecked, these cancer cells multiply and eventually displace healthy lymphocytes, suppressing the immune system.  Signs and symptoms swollen lymph nodes in the neck, axillae, or groin; fatigue; chills; and night sweats. Some patients also experience severe itching, persistent cough, weight loss, shortness of breath, and chest discomfort. Hodgkin lymphoma is a rare cancer of unknown cause that may have a heritable component; the risk of developing this disease is higher when a young sibling has the disease.  The disease is more common in males than in females, with two peak incidences—first in the 20s and later in people older than 55 years.  The disease is confirmed by the identification of Reed- Sternberg cells in lymph nodes or organs affected by the cancer. Treatment depends on the level of lymph node and organ system involvement (the stage of the disease) and can consist of radiation and chemotherapy with anticancer drugs. Hodgkin lymphoma is one of the most curable cancers.  Non-Hodgkin Lymphomas Non-Hodgkin lymphoma includes numerous lymphomas that vary in their malignancy according to the nature and activity of the abnormal cells.  At least 10 types of non-Hodgkin lymphomas have been identified, each of which is graded as low, intermediate, or high based on how aggressively the disease behaves. Low-grade diseases usually progress slowly and tend not to spread beyond the lymphatic system. By comparison, high-grade diseases can spread to distant organs within a few months.  Signs and symptoms include painless swelling of one or more groups of lymph nodes, enlargement of the liver and spleen, fever, and, in rare cases, abdominal pain and gastrointestinal bleeding. The cause of these cancers is largely unknown. One form, Burkitt’s ymphoma, is a childhood cancer. In Africa, it is strongly associated with infection by Epstein-Barr virus. Other types worldwide have been linked to infection by HIV-type viruses and other conditions that affect the immune system (e.g., organ transplantation, radiation and chemotherapy, lupus, and rheumatoid arthritis). Treatment consists of radiation therapy, administration of anticancer drugs, and sometimes bone marrow transplantation  Polycythemia is an increase in the total RBC mass of the blood.  The condition may occur for unknown reasons (primary polycythemia) or be a natural response to chronic hypoxia Polycythemia (secondary polycythemia). Polycythemia also can result from dehydration (apparent polycythemia), in which case the RBC production does not exceed the upper limits of normal.  Secondary polycythemia can be naturally present in people who live in or visit areas of high altitude. Polycythemia in such instances is due to reduced air pressure and low oxygen concentration. When the oxygen supply to the blood is reduced, the kidneys produce the hormone erythropoietin, Secondary which stimulates RBC production in the bone marrow to compensate for the reduced oxygen Polycythemia supply. The result is an increase in the oxygen- carrying efficiency of the blood. The RBC numbers return to normal when the person returns to sea level.  Secondary polycythemia also can be present in heavy smokers. The disease can be caused by chronic bronchitis and conditions that increase erythropoietin production (eg, liver cancer, some kidney disorders).  Primary polycythemia, also known as polycythemia vera, is a rare disorder of the bone marrow in which increased production of RBCs causes the blood to thicken. This condition mainly develops in people older than 50 years and can lead to several physiologic problems, such as the following: Blurred vision Dizziness Generalized itching Primary Headache Hypertension Polycythemia Red hands and feet; red-purple complexion Splenomegaly Other complications associated with primary polycythemia include platelet disorders, which cause bleeding or clot formation; stroke; and the development of other bone marrow diseases (eg, leukemias). Treatment consists of phlebotomy—the slow removal of blood through a vein—as well as anticancer drug therapy to control the overproduction of RBCs in the marrow Disseminated intravascular Coagulopathy (DIC) Disseminated intravascular coagulation (DIC) is a complication of severe injury, trauma, or disease. DIC is a common abnormal clotting disorder. The disease most often is seen in the critical care setting. It disrupts the balance among procoagulants, inhibitors, thrombus formation, and lysis. Signs and symptoms of DIC include dyspnea and bleeding and symptoms associated with hypotension and hypoperfusion. DIC occurs in two phases: 1. characterized by free thrombin in the blood, fibrin deposits, and aggregation of platelets 2. characterized by hemorrhage caused by the depletion of clotting factors. The clinical consequences of these processes predispose the patient to multiple-system organ failure from bleeding and coagulation disorders caused by the following: Loss of platelets and clotting factors Fibrinolysis Fibrin degradation interference Small vessel obstruction, tissue ischemia, RBC injury, and anemia from fibrin deposits Diagnosis of DIC involves a combination of laboratory tests and clinical evaluation. Laboratory findings suggestive of DIC include a I. Low platelet count II. Decreased fibrinogen concentration, III. Prolongation of clotting times such as prothrombin time (PT). In an effort to control the depletion of clotting factors, in-hospital care includes the replacement of platelets, coagulation factors, and blood. At the same time, attempts are made to manage the primary process  Hemophilia is a medical condition that causes uncontrolled bleeding and that involves the loss of Hemophilia bleeding control mechanisms; it comprises a group of inherited bleeding disorders (BOX 31-7)  Hemophilia A is caused by a deficiency in factor VIII, which is essential to the process of blood clotting (TABLE 31-4). Another, less common form of hemophilia, caused by a deficiency of factor IX, is known as hemophilia B or Christmas disease (named for a man first diagnosed with the disease in 1952). Hemophilia All types of hemophilia present with similar problems, but the specific factor involved determines the severity of bleeding. About 18,000 people in the United States have hemophilia; about 400 are born with the disorder each year. Bleeding from hemophilia can occur even after minor injury and during some medical procedures (eg, tooth extraction). Although hemorrhage can occur anywhere in the body, bleeding into joints, deep muscles, the urinary tract, and intracranial sites is the most common. Head trauma is potentially life threatening in these patients. Central nervous system bleeding is the major cause of death for patients with hemophilia in all age groups. Hemophilia is controlled by infusions of concentrates of factor VIII (or factor IX for people with type B hemophilia). These infusions can be administered by the patient. However, serious or unusual bleeding often requires hospitalization. People with hemophilia are advised to avoid activities that may increase their risk of injury (eg, contact sports). Most patients with hemophilia are knowledgeable about their disease and seek emergency care only when complicated problems and trauma-related issues arise.  Thrombocytopenia Thrombocytopenia is a low platelet count. In healthy people, blood normally contains 150,000 to 450,000 platelets/micL of blood. At levels of 20,000 to 30,000 platelets/mcL of blood, bleeding can occur with relatively minor trauma. At levels below 20,000 platelets/micL of blood, spontaneous bleeding can occur, increasing the risk for shock and death. This risk is especially true if bleeding occurs in the brain. Bleeding on the skin is usually the first sign of a low platelet count and may have the following appearance: Small red or purple spots on the skin (petechiae), often on the lower legs Purple, brown, and red bruises (purpura) that happen easily and often Prolonged bleeding, even from minor cuts Bleeding or oozing from the mouth or nose, especially nosebleeds or bleeding from brushing teeth Unusually heavy menstrual flow  Thrombocytopenia Thrombocytopenia can occur if the body does not produce enough platelets or destroys too many platelets, or if the spleen retains too many platelets. This disease is often associated with leukemia or lymphoma, aplastic anemia, vitamin B12 or folic acid deficiency anemias, an enlarged spleen, infectious diseases such as HIV/AIDS, and massive blood transfusions. Increased destruction of platelets may result in the following two conditions  Two diseases that occur because of increased destruction of platelets are: Idiopathic thrombocytopenic purpura (ITP). ITP occurs when antibodies attack and destroy the body’s platelets for unknown reasons. In children, ITP can be an acute condition that occurs after infection. It is most frequently affects women ages 20 to 40 years. --Thrombotic thrombocytopenic purpura (TTP). TTP is a life-threatening disease that occurs when small blood clots form suddenly throughout the body. It can result in cardiac hemorrhage and death. TTP occurs more often in women and is associated with pregnancy, metastatic cancer, chemotherapy, HIV/AIDS, and some prescription drugs. Patients with TTP experience kidney failure or decreased kidney function, fever, and neurologic complications Treatment for thrombocytopenia depends on the disease’s cause and severity. Some patients will require only careful monitoring of their platelet counts. Other, more serious cases may be treated with administration of corticosteroids (prednisone), transfusion of platelets, and, rarely, surgical removal of the spleen.  Sickle Cell Disease It is an inherited blood disorder that affects RBCs. Their are various types of sickle cell disease, the most common is sickle cell disease. Sickle cell disease is a debilitating and unpredictable genetic illness. It affects persons of African descent and, less commonly, persons of Mediterranean origin. Signs and symptoms of sickle cell disease include the following: Delayed growth, development, and sexual maturation in children Jaundice Splenomegaly Acute renal failure Stroke Pathophysiology of Sickle cell disease Sickle cell disease produces an abnormal type of hemoglobin, called hemoglobin S, that has an inferior oxygen-carrying capacity. When hemoglobin S is exposed to low oxygen states, it crystallizes, which distorts the RBCs into a sickle shape (FIGURE 31-8).  The sickle-shaped cells are fragile and easily destroyed. They are unable to pass easily through the tiny blood vessels and consequently block flow to various organs and tissues, most commonly in bones and bone marrow. This causes a vasoocclusive sickle cell crisis that can life threatening. As fewer RBCs pass through congested vessels, tissues and joints become starved for oxygen and other nutrients, causing severe pain, most frequently in the back or extremities. Three less common types of sickle cell crisis are aplastic, hemolytic, and splenic sequestration. In aplastic crisis the bone marrow temporarily stops producing RBCs. In hemolytic crisis the RBCs break down too rapidly to be replaced adequately. Splenic sequestration usually is a childhood difficulty that occurs when blood becomes trapped in the spleen. This causes the organ to enlarge and possibly may lead to death. Pathophysiology of Sickle cell disease Other signs and symptoms of sickle cell disease are increased weakness, aching, chest pain with shortness of breath, sudden and severe abdominal pain, bony deformities, icteric (jaundice) sclera (figure 31-9) , fever, and arthralgia (joint pain). Sickle cell crisis can occur in any part of the body and can vary in intensity from one person to the next, and from one crisis to the next. Over time, the crises can destroy the spleen, kidneys, gallbladder, and other organs. Sickle cell crisis may occur for no apparent reason, or it may be triggered by conditions such as the following: Dehydration Exposure to extremes in temperature Infection Lack of oxygen Strenuous physical activity Stress  In some cases, sickle cell disease can be cured with a bone marrow transplantation. Because of the eventual damage that occurs to the spleen, patients with sickle cell disease are at increased risk for septicemia if infected by certain types of bacteria. Children with the disease should be kept current with all immunizations. The main care objectives for patients in sickle cell crisis are to provide: (1) Supplemental oxygen by face mask or nasal cannula to Management help counter tissue hypoxia and reduce cell clumping if the arterial blood gas is less than 95%; of SCD (2) Maintenance hydration (intravenous [IV] therapy if unable to tolerate oral liquids); (3) Electrolyte replacement, because hypoxia results in metabolic acidosis, which also promotes sickling; (4) Analgesics for the severe pain from vaso-occlusion; (5) Blood replacement as needed to treat anemia and to reduce the viscosity of the sickled blood. Antibiotics are also given to manage infection (6) Antibiotics to manage infection,  Most patients with diagnosed hematologic disorders are knowledgeable about their disease. Often, they call General emergency medical services to help Assessment and manage a “change” in their condition. Management of They also may call to arrange for transport Patients With to an emergency department for physician Hematologic evaluation. The situations that invoke a Disorders call for emergency care vary by patient and disease. Common chief complaints can be classified by body system (TABLE 31-5).  In many cases, the prehospital care for a patient with a hematologic disorder will be mainly supportive.  the paramedic should perform a general assessment, a focused history, and a focused physical examination. These measures will guide patient care and help to determine the urgency of emergency transport..  As referenced in Table 31-5, a patient with a Pre-hospital hematologic disorder may have a variety of complaints and physical findings. Some patient complaints may be Care vague (eg, fever, fatigue, headache), which can further complicate the paramedic’s assessment. After ensuring adequate airway, ventilatory, and circulatory status, the paramedic should assess vital signs and perform a physical examination. The patient’s skin should be assessed for color and turgor, noting any cyanosis or jaundice, warmth or coolness, bruising, edema, or ulcerations. Thank you