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pediatric hema diso

components, anemia, sickle cell, betathalasemia, aplastic anemia, thromboctpn pr

QuestionAnswer
formed elements are what: Erythrocytes. Leukocytes. Thrombocytes.
formation of bone: marrow of long bones hematopoeis in adolescence, erythropoietin regulates production
Lymphatic system: drains regions of the body to lymph nodes (organism destroyed & antibodies produced).  Lymphocytes. Lymphatic vessels. Lymph nodes. Spleen. Tonsils, adenoids, and thymus gland.
What are the three formed elements of blood? Erythrocytes, leukocytes and thrombocytes.
reduction i hte number of rbc or amount of hemoglobn below normal, when is the hematologic disorder most common and what effects are profound? most common in infancy and childhood (not a disease but an indication of an underlying problem) decreased O2 carrying capacity & availability
what about anemia causes a murmr? Severe anemia decreases peripheral resistance. Increased amount of blood returned to heart. Murmur.
Diagnostic Evaluation of anemia: CBC - Decreased RBCs, HgB and Hct. 1st indication Fatigue, lack of energy and pallor.
Therapeutic Management: Supportive. Supplemental oxygen. Bedrest. IV fluids. Treat underlying cause. Seek anemia reversal.
Prepare for tests. Multiple sticks EMLA
Note signs of exertion: Tachycardia and palpitations. Tachypnea. Dyspnea and shortness of breath. Hyperpnea or breathlessness. Dizziness or lightheadedness. Diaphoresis.
nursing considerations: diversion, allow parents to stay with child, provide o2, prevent infection, observe signs of infection
What is one nursing responsibility when caring for a child with anemia? Prepare the child and family for lab tests, decrease tissue oxygen needs and prevent complications.
what is the most prevalant nutritional disorder? at what age are pts at highest risk,and why? inadequate supply of dietary Iron. Children 12 to 36 months are at highest risk. Cow’s milk major staple.Premature infants are at risk. Reduced fetal Iron supply give iron supplement with juice
what factors may cause iron deficiency? Decrease supply of Iron (hemorrhage) Impair its absorption Increase body’s need for Iron (excess growth) Hgb synthesis affect Inadequate diet
therapy for iron deficiency Diet counseling and iron supplements. Ferrous Sulfate Supplement with iron-fortified infant formula and cereal. Transfuse if severely anemic. Packed RBC’s No whole blood – circulatory overload
what instruction may help parents on iron administrattion Give as prescribed (two divided doses between meals). Juice with the medication helps absorption. Iron turns: Stools tarry green. Vomiting and diarrhea can occur. Liquid iron may stain teeth: Give medication with a syringe, straw or dropper.
how are injections made to ease? Use Z-track method to administer IM and do not massage site.
prevent nutritional anemia by educating teens, and what? Administer iron supplementation. Give an iron-fortified formula. Overweight does not mean healthy.
Name three causes of iron-deficiency anemia. Severe hemorrhage, inability to absorb iron, excessive growth requirements, inadequate diet, and GI bleeding r/t lactose intolerance.
Inherited defect: normal adult hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin a normal red blood cell is shaped like an intertube, a sickled cell looks shredded like a slug out of its shell.
why are newborns with sickle cell generally asymptomatic? Due to fetal Hbg Decrease rapidly in 1st year
even though it happens mostly in African Americans, when does a child have a sickle cell trait? When the child inherits both HbA and HbS, they are said to have the sickle cell trait. When the child inherits predominately HbS, they have sickle cell anemia.
how does sickle cell lead to cell death? Features are a result of obstruction caused by the sickled RBCs and increased RBC destruction.Obstruction causes vaso-occlusion. Leads to hypoxia, tissue ischemia and cell death.
acute pain results from infarct at the major organs and systems such as? Spleen Brain Heart Lungs GI tract Kidneys bones
clinical manifestations may vary, but what is sickle cell crisis? Sickle cell crisis is a period of exacerbation of symptoms and is painful and can be fatal.
two types of sickle cell aplastic, hyperhemolytic.
acute chest syndrome Chest pain. Fever. Cough. Tachypnea. Wheezing. Hypoxia.
dx eval for sickle cell Newborn screening. Sickledex. Hemoglobin electrophoresis.
therapeutic goals Prevent conditions that enhance sickling. Treat medical emergencies.
Prevention = maintaining hemodilution. Medical management is supportive and symptomatic. Rest. Hydration. Electrolyte replacement. Analgesics. Blood transfusion. Antibiotics.
Administer all vaccines as recommended. Short term oxygen may be helpful. Exchange transfusions. Splenectomy. Treat chronic pain: Ibuprofen or acetaminophen. Codeine. PCA. Steroids. NO Demerol
Educate family and child: Disease. When to seek intervention. Give meds as ordered. Recognize signs and symptoms of impending crises. Treat the child normally.
Emphasize the importance of hydration.Provide supportive therapy: Manage pain. Apply heat & keep child on bed rest. Observe signs and symptoms of transfusion reaction. Be aware of spleen size. Recognize other complications. Support family to include siblings.
What is the most common type of sickle cell crisis? Vaso-occlusive.
What nursing interventions are appropriate to manage the pain a child experiences during a vaso-occlusive crisis? Provide analgesics as ordered; notify the provider if medication is ineffective.
Beta-Thalassemia Refers to inherited blood disorders characterized by deficiencies in the rate of production of specific globin chains in Hgb.
Occurs most often in persons leaving near the Mediterranean sea. four forms are Thalassemia minor, trait, intermedia, and major or Cooley minor which is an asymptomatic form.trait, which produces a mild microcytic anemia.intermedia which is manifested as Splenomegaly and moderate to severe anemia.Cooley is severe leads to cardiac fail/death
Occurs most often in persons leaving near the Mediterranean sea. four forms are Thalassemia minor, trait, intermedia, and major or Cooley minor which is an asymptomatic form.trait, which produces a mild microcytic anemia.intermedia which is manifested as Splenomegaly and moderate to severe anemia.Cooley is severe leads to cardiac fail/death
how does Beta-thalassemmia lead to severe anemia? Partial or complete deficiency in the synthesis of hemoglobin. Compensatory increase in different chains. Increases the production of defective hemoglobin. Damages the RBC causing severe anemia.
Beta-Thalassemia Diagnostic Evaluation Hematologic studies: changes in RBCs. Low Hgb and Hct. Hgb electrophoresis. X-rays (show minor or major)
Beta-Thalassemia Therapeutic Management Maintain sufficient Hgb levels. Provide sufficient RBC. Transfusions are necessary. An iron chelating agent is given with oral supplements of vitamin C. A splenectomy may be necessary.
Beta-Thalassemia Nursing Considerations Objectives: Promote compliance. Assist with coping. Foster adjusting. Observe for complications. Refer for genetic counseling. Support the family.
Which form of Thalassemia is the most severe form with the potential to lead to death if untreated? Thalassemia major known as Cooley anemia.
Beta-Thalassemia Nursing Considerations Objectives: Promote compliance. Assist with coping. Foster adjusting. Observe for complications. Refer for genetic counseling. Support the family.
Which form of Thalassemia is the most severe form with the potential to lead to death if untreated? Thalassemia major known as Cooley anemia.
Aplastic Anemia Definition: a bone marrow failure condition in which the formed elements of blood are simultaneously depressed (pancytopenia).
Aplastic Anemia Etiology Can be primary or secondary. Fanconi syndrome occurs (pancytopenia, hypoplasia of bone marrow and patchy brown discolor).
Fanconi anemia: Fanconi anemia. Patient shows characteristic café-au-lait skin pigmentation. Generalized hyperpigmentation, increasing with age, and areas of hypopigmentation may occur.
Aplastic Anemia Factors Infection. Irradiation. Drugs. Exposure to household or industrial chemicals. Idiopathic.
Aplastic Anemia Diagnostic Evaluation Anemia. Leukopenia. bone marrow transplant, clinical manifestations: Decreased platelet count.
Aplastic Anemia Therapeutic Management Immunosuppression therapy. Replacement of bone marrow. Antilymphocyte globulin (ALG) or antithymocyte globulin (ATG). Hemopatopoietic stem cell transplantation.
Aplastic Anemia Nursing Considerations Prepare the family and child for procedures. Prevent complications from the severe pancytopenia. Emotional support. If ATG is administered, it must go through a central vein. If chemotherapy is given, monitor the child for side effects.
Hemophilia Definition: a disorder where the blood does not clot normally and even the slightest injury can cause severe bleeding.
what is the inheritance pattern The inheritance pattern is demonstrated as X-linked recessive so it affects males almost exclusively, but is transmitted by symptom free females via a defective gene on the X (female) chromosome.
what are the most common forms of hemophilia Factor VIII deficiency (hemophilia A). Factor IX deficiency (hemophilia B). von Willebrand disease (hemophilia A affecting males & females).
how does coagulation degree effect pt bleeding? Factor VIII is needed for forming thromboplastin (1st phase of coagulation). Less factor VIII = more severe disease. Leads to patients bleeding longer but not faster.
Hemophilia Diagnostic Evaluation,evidence of x-linked inheritance and lab findings Prolonged bleeding. Hemorrhage. Subcutaneous and intramuscular hemorrhages. Hemarthrosis. Hematomas. Spontaneous hematuria.
Hemophilia Therapeutic Management Primary therapy: replacing missing clotting factor. Corticosteroids. NSAIDS with caution. Regular exercise is encouraged. Prophylactic infusions of factor VIII concentrate.
Hemophilia Nursing Considerations Maintain a high level of suspicion. Prevent bleeding by preventing trauma. Educate the family. Use an extra soft toothbrush. Medical alert bracelet. Educate the family on measures to control bleeding:RICE
Hemophilia Nursing Considerations Con’t Prevent crippling effects of bleeding. Eat appropriate diet. Support the family. Recommend genetic counseling. Support groups.
Define hemophilia. A genetic disorder where blood does not clot normally.
What does RICE stand for and when should it be used? RICE stands for Rest, Ice, Compression and Elevation and is used to help control bleeding episodes.
Idiopathic Thrombocytopenic Purpura Definition: an acquired platelet disorder that occurs in childhood.
what is ITP characterized by? Thrombocytopenia: excessive destruction of platelets. Purpura: a discoloration caused by petechiae. Normal bone marrow with normal or increased number of immature platelets and eosinophils.
Idiopathic thrombocytopenic Purpura dx eval Easy bruising, petechiae, ecchymosis. Bleeding from mucous membranes, epistaxis, bleeding gums and internal hemorrhage. Hematomas over lower extremities.Test to rule out other disorders. SLE, leukemia, lymphoma
Therapeutic Management: Primarily supportive. Restrict activity. Treat symptoms. Splenectomy if ITP has persisted for >1 yr.
Nursing Care: Supportive. Teach family to limit activity. Teach family not to use aspirin or NSAIDS.
What is the cause of ITP? Unknown, thought to be an auto immune system reaction to a virus.
Leukemia Definition: a malignant disease of the blood forming organs of the body that results in an uncontrolled growth of immature white blood cells (blasts or stem cells).
5 facts about leukemia Most common form of childhood cancer. 3-4 cases per 100,000 children under 15 years. More frequent in males. Peak onset is between 2-6 years of age. Chromosomal abnormalities identified in many cases.
how does over production of immature WBC's lead to bone weakness and diminished organ function? Immature cells cannot fight infection. Infiltration of WBCs of platelet forming centers in the marrow cause bleeding tendencies and weakening of bones. Infiltration in spleen, liver and lymph glands results in diminished function.
Cancerous cells invade the CNS and other organs. leukemia is Classified according to type: Acute Lymphocytic (ALL) : most common. Acute Non-Lymphocytic (myelogenous),ANLL/AML.
Initial phase of leukemia manifestations Low-grade fever. Pallor. Tendency to bruise. Leg & joint pain, pathologic fractures. Listlessness. Abdominal pain. Enlargement of lymph nodes.
Leukemia Manifestations Hepatomegaly & splenomegaly. Anemia despite transfusions. Lemon-yellow skin. Petechiae & purpura. Ulcerations. Anorexia, N/V, weight loss. Dyspnea. Enlargement of kidneys & testicles. Hematuria.
Leukemia Diagnosis History & symptoms. Blood tests. Bone marrow aspiration. Spinal tap. Kidney & liver function tests.
Leukemia Treatment Multidisciplinary. Specialized facility. Chemotherapeutic agents with steroids. Antibiotics, sedatives & analgesics. Bone marrow transplant. Blood transfusions. TPN.
Leukemia Nursing Care Promote rest & reduce fatigue. Prevent skin breakdown. Stimulate appetite. Help child & family cope.Assist with administration of blood products. Pain relief. Minimize risk of infection. Prevent hemorrhage.
: Name three diagnostic tests associated with leukemia. Bone marrow aspirate; Kidney and liver function tests; Spinal tap; Blood tests; X-rays.
Name two ways to provide appropriate nursing care for a child with leukemia. 1. Pain relief. 2. Promote rest and reduce fatigue.
Hodgkin’s Disease Pathophysiology Twice as common in males. a malignancy of the lymph system may metastasize, shows reed sternberg cells, non hodgkins lymphoma prevalant in < 14 yrs while hodgkins is prevalant in 15-19
manifestations of hodgkins Painless lump along neck. Unexplained low-grade fever, night sweats. Anorexia, unexplained weight loss, malaise. Rash & pruritis.
Hodgkin’s Disease Diagnosis X-ray, body scan, lymphangiogram & node biopsy. staging: I: Restricted. II: Two or more lymph nodes. III: Involves nodes on both sides of diaphragm. IV: Diffuse disease.
Hodgkin’s Disease Treatment Radiation therapy. Chemotherapy: MOPP. Mustargen, Oncovin, Prednisone, Procarbazine ABVD. Adriamycin, Bleomycin, Vinblastine Sulfate Splenectomy.
Hodgkin’s Disease Nursing Care Symptomatic relief. Education. Prevent over fatigue. Inform adolescents of side effects of treatment. Age appropriate emotional support.
What is a definitive test for Hodgkin's lymphoma? A blood test that identifies Reed-Sternberg cells.
main points: Components of the blood. Anemia. Sickle cell disease. Beta-Thalassemia. Aplastic Anemia. Hemophilia. Idiopathic Thrombocytopenic Purpura (ITP). Leukemia. Hodgkin's disease.
Created by: redhawk101