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Maternal-Child Nursing Care Optimizing Outcomes for Mothers, Children, & Families Chapter 33 Caring for the Child with a Hematological Condition Susan Ward Shelton Hisley A & P Review Blood Plasma (albumin, electrolytes, proteins, clotting factors, fibrinogen, globulins, and circulating antibodies) Cells RBCs (transport hemoglobin) WBCs (body’s protective system) Platelets (hemostasis and vascular repair after injury to a vessel wall) Common Hematological Conditions Anemia Signs and symptoms (initial signs) Fatigue, SOB, headache, difficulty concentrating, dizziness, and pale skin Nursing care Identify underlying cause Promote a healthy diet or a vitamin supplement Administer red blood cells (moderate anemia) Administer hematopoietic growth factors (specific types of anemia) Teach family (signs and symptoms, alter daily activities, evaluate status of anemia) Iron-Deficiency Anemia Signs and symptoms Asymptomatic (mild anemia) Decreased Hgb and Hct Irritability, fatigue, delayed motor development, SOB, decreased activity level, and pale skin Remember an overweight child can be diagnosed with irondeficiency anemia. Nursing care Identify early Understand that primary goal is prevention Facilitate nutritional counseling Assist with obtaining recommended iron-fortified formula and cereal See “What to say”—Nutritional counseling (p. 1074) Discuss the Women Infants and Children (WIC) program Administer oral iron supplements Epistaxis (Nosebleeds) Signs and symptoms Bleeding from the nose Nursing care Focus on prevention, family education Avoid the use of aspirin or NSAIDS Use a cool mist vaporizer Use first aid measures to stop bleeding Sickle Cell Disease (SCD) Signs and symptoms Result of vaso-occlusion Weakness Pallor Fatigue Tissue hypoxia Jaundice Pain Nursing care Identify early and treat hypoxic episodes Encourage rest Hydrate (1.5 times above normal calculated requirements) Maintain oxygenation (respiratory assessment) Control pain (oral, IV, or PCA) Interpret laboratory or radiographic studies Educate family Beta Thalassemia Signs and symptoms Enlarged liver and spleen Mild jaundice Growth retardation Moderate to severe anemia Bony deformities Increased susceptibility to infection Nursing care: Understand that the goal is to prevent hypoxia by providing blood transfusion therapy Promote growth and development Discuss bone marrow transplant Educate family (blood transfusion and chelation treatment, hand washing, when to seek medical attention) Refer for genetic counseling Hereditary Spherocytosis (HS) Signs and symptoms Hyperbilirubinemia Splenomegaly Negative Comb’s test Nursing care Give folic acid supplements Discuss splenectomy Educate family (folic acid supplementation and adhere to prescribed antibiotic regimen) Promote good hand washing Instruct parents on temperature taking and when to seek medical attention, evaluate blood counts and immunizations Provide support to help them cope with lifelong illness Hemophilia Signs and symptoms Present with bleeding or known family history of bleeding disorders Hemarthrosis Soft tissue bleeding Nursing care Initiate prompt treatment of bleeding episodes Ensure patient safety and prevent complications For severe hemophilia, administer recombinant factor products Educate family about proper administration of factor products von Willebrand’s Disease Signs and symptoms Epistaxis Bleeding from the oral cavity Menorrhagia Easy bruising Nursing care Administer Desmopressin (DDAVP) Administer Humate-P and/or cryoprecipitate or fresh frozen plasma (FFP) Recommend medical alert Instruct parents about common sites of bleeding (nose, gums, internal, and menses) Control bleeding (pressure, ice, and seek medical attention) Avoid the use of aspirin or NSAIDS Idiopathic Thrombocytopenia Purpura (ITP) Signs and symptoms After a recent viral infection Petechiae Bruising Mucocutaneous bleeding Epitaxis Menorrhagia (adolescent) Nursing care Administer steroids, Intravenous Immune Gamma Globulin (IVIG), or Anti-D antibody (WhinRho®) Restrict activities Instruct parents on how to manage a bleeding episode Avoid aspirin, injections, tampons, nothing via the rectum Teach family to report signs and symptoms of bleeding Encourage child to wear a medical alert bracelet Disseminated Intravascular Coagulation (DIC) Signs and symptoms Excessive bleeding from orifices Petechia, purpura, and hypotension Multi-organ failure Nursing care Identify the underlying cause Monitor for signs of hemorrhage, bleeding petechiae, cutaneous oozing, dyspnea, lethargy, pallor, increased heart rate, decreased blood pressure, headache, dizziness, muscle weakness, restlessness, and internal bleeding Do not disturb clots, use pressure and ice to control bleeding Obtain laboratory tests and administer blood and factor products Inform family about plan of care and course of treatment Aplastic Anemia Signs and symptoms Pancytopenia Anemia, pallor, dizziness, and fatigue Petechia, epistaxis Increased susceptibility to infections and oral ulcerations Nursing care Remove causative agent Administer antithymocyte globulin (ATG) and high-dose cyclosporine (Neoral®, Gengraf®) Administer hematopoietic growth factors Discuss hematopoietic stem cell transplant (HSCT) Support and educate family Note: Traditional s/s of infection are not evident (swelling, pus, and redness) Neutropenia Signs and symptoms Lymphadenopathy, organomegaly, pallor, bruising, petechia Absolute neutrophil count (ANC) Total number of white blood cells multiplied by the percentage of neutrophils (segs and bands) Nursing care Evaluate etiology Understand that treatment ranges from supportive measures to administration of colony-stimulating factors (GCSF [Neupogen ®]) to bone marrow transplant Monitor for infections (broad-spectrum antibiotic) Inform about bone marrow transplant procedure Educate family (hand washing, taking temperature, oral hygiene, skin care, keep away from other sick children) See Critical Nursing Action—The Neutropenic Child (p. 1089) Blood Transfusion Therapy Reasons for transfusion Hematological illness Oncological illness Chronic conditions Blood Transfusion Therapy Nurse responsibilities Be knowledgeable about blood transfusion Review the plan of care with the family Explain in detail the indications and process Obtain blood consent Jehovah Witnesses Obtain the required blood samples Administer based on policy and procedures The Administration of Blood Strict observance to the institutional policy regarding the administration of blood products cannot be stressed enough. The accuracy of patient verification is a critical nursing action that can help prevent a transfusion reaction. Transfusion Reactions Most transfusion reactions occur during the initiation of a transfusion, but a reaction can occur at any time during this process. These reactions can vary from a mild reaction, such as mild fever, to the most severe complication of death. Children who have received multiple transfusions are at higher risk for developing a transfusion reaction. Febrile Reaction Child develops a fever greater than 1.8˚F from the baseline temperature Occurs on initiation of the transfusion, but has been known to occur up to 12 hours posttransfusion Signs and symptoms—fever and chills, which then may progress to more serious complications such as tachycardia, tachypnea, and hypotension Nursing care—premedicating with acetaminophen (children’s Tylenol) can sometimes prevent this type of reaction. Monitor the child’s temperature to recognize febrile reactions early and prevent progression. Stop the transfusion, monitor vital signs closely, and notify the physician. Allergic Reaction Occurs during a transfusion in which the child has had a previous exposure to a particular allergen in the blood product Signs and symptoms—rash, hives, pruritus, swelling of the lips, wheezing, and anxiety Nursing care—stop the transfusion immediately, monitor vital signs closely, and notify the physician. The administration of an antihistamine such as diphenhydramine (Benadryl) resolves an allergic response. Circulatory Overload Occurs when the infusion is given too rapidly or an excessive quantity of blood is given Signs and symptoms—dry cough, dyspnea, rales, distended neck veins, hypertension/hypotension, bradycardia/tachycardia, clammy skin, and cyanosis of the extremities Nursing care—accurate verification of physician orders, doublechecking the volume to be infused, and the use of an intravenous pump. If any of the signs and symptoms are identified, the nurse must immediately stop the transfusion, monitor vital signs closely, place the child upright with feet in a dependent position to increase venous resistance, notify physician, and prepare emergency care. Acute Hemolytic Transfusion Reaction Occurs when the donor RBCs and the recipient plasma are incompatible and there is an ABO mismatch. Acute hemolytic transfusion reactions occur upon initiation after exposure to a small amount of blood. Signs and symptoms—fever, shaking chills, pain at the intravenous site, tightness of the chest, difficulty breathing, impending sense of doom, pallor, jaundice, nausea/vomiting, red or black urine, flank pain, and progressive signs of shock such as tachycardia and hypotension Nursing care—stop the transfusion, monitor vital signs closely, start a normal saline infusion, verify patient identification, notify the physician, and prepare emergency care. Other nursing responsibilities include obtaining blood and urine samples and sending them to the laboratory to analyze for the presence of hemoglobin, which indicates intravascular hemolysis. Insert a urinary catheter to monitor the child’s output more accurately. Bacterial Contamination Occurs during the initiation of the infusion Guidelines from the AABB (2004) require strict adherence to the completion of all transfusions in 4 hours or less to prevent this from happening. Signs and symptoms—shaking chills, fever, vomiting, diffuse erythema, and the onset of hypotension that may progress to shock. In severe cases, hemoglobinuria, actual renal failure, and DIC may develop. Nursing care—stop the transfusion immediately, monitor vital signs closely, start a normal saline infusion, notify the physician, and prepare emergency care. Nursing responsibilities also include obtaining blood samples for culture and sensitivity and sending the blood product with tubing to the blood bank also to be cultured. Bone Marrow Transplantation Hematopoietic stem cell transplant (HSCT) Bone marrow transplantation (BMT) Bone Marrow Transplantation Preparative regimen Administration of “near lethal” doses of chemotherapy and/or radiation Three types of hematopoietic stem cell transplants (HSCT) Autologous transplant (child’s own donor of stem cells) Allogeneic transplant (matched compatible donor, usually a sibling) Syngeneic (identical sibling) Bone Marrow Transplantation Complications Acute and chronic graft versus host disease (GVHD) Infection Immunosuppression Organ dysfunction Psyosocial impact Apheresis The process of apheresis is the selective removal of a specific blood component from a donor or child while re-transfusing the remaining components. Blood is removed from the child, pumped through a special cell separator in the apheresis machine that removes the specific desire component by centrifugal force, and then is returned to the patient. Thrombosis Risk factors Prolonged immobility Disease states, obesity, medications, hereditary factors Major surgery or trauma Complications Stroke Deep vein thrombosis Pulmonary emboli Signs and symptoms Lungs—SOB, lightheaded, and increased heart rate Kidney—blood in the urine Skin—hemorrhagic spots Artery or extremity—cold, pale, blue, and absent pulse Thrombosis Nursing care Thoroughly assess risk factors Apply compression stockings Use an intermittent pneumatic compression device Perform passive range of motion Administer low–molecular-weight heparin Promote early ambulation Administer heparin in some cases