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Stack #84152
| Peds Hematologic Immunologic | Answer |
|---|---|
| Assessment of hematologic function. | cbc, Hx and assessment, energy & activity level,growth patterns |
| What is the most common hematologic disorder in children? | Anemia |
| What is anemia? | Decreased RBC's or hemaglobin concentration below normal level. |
| How does this effect the body? | Decreases onygen-carrying capacity of blood. |
| Etiology and Physiology of anemia? | RBC and/or Hgb depletion. |
| Morphology of RBC's | Characteristic changes in RBC size, shape, and/or color. |
| Physical manifestations of decresed RBC production? | pallor,tachycardia,fatigue, shortness of breath muscle weakness, synoatrial heart murmur, |
| Consequences of anemia | decrease in 02 carrying capacity of blood and decreased amount of 02 available to tissues. |
| When anemia develops slowly what does the child do? | Adapts. |
| What are the effects of anemia on the circulatory system? | Hemodilution, decreased peripheral resistance, increased cardiac circulation and turbulence,(may have murmur, may lead to cardiac failure), cyanosis, growth retardation. |
| What is the theraputic management of anemia? | transfusion after hemorrhage if needed, nutritional intervention for deficiency anemias, IV fluids to replace intravaxcular volume, O2, bedrest. |
| What are the nursing considerations for anemia? | Prepare child & family for lab tests, decrease 02 demands, prevent complications, support family |
| What is the cause of iron deficient anemia? | Inacequate supply of dietary iron. |
| How to prevent iron deficient anemia? | iron-fortified cereals and formulas for infants, special needs of premature infants, adolescents at risk due to rapid growth and poor eating habits. |
| What is sickle cell Anemia? | A hereditary hemoglobinopathy. |
| Who does sickle cell effect? | primarily african americans,occasionally occurs in mediterranean decent, also seen in south americans, arabian, and east indian descent. |
| How often does sickle cell occur ? | 1 in 375 infants born in the US, 1 in 12 have sickle cell trait, |
| What is the etiology of sickle cell? | Autosomal recessive disorder, 1 in 12 african americans are carriers(have trait), if each parent has trait each offspring has 1 in 4 chance of having disease. |
| What is the patho of sickle cell? | partial or complete replacement of normal Hgb with abnormal hemagloin S(Hgb S), |
| What do the blood cells look like with sickle cell? | Hemaglobin in the RBCs takes on an elongated "sickle" shape |
| When the RBCs are sickled shape how does this effect blood flow? | The sicked cells are rigid and obstruct capillary blood flow, microscopic obstructions lead to engorgement and tissue ischemia/death, hypoxia(lack of 02 ) occurs and causes sickling,large tissue infarctions occur, damaged tissue in organs impairs fuction |
| What types of surgery might occur with sickle cell? | splenectomy at early age |
| What is the prognosis for pt. with sickle cell? | No cure, supportive care/prevent sickling episodes, frequent bacterial infectionds due to immunocompromise, strokes in 5% to 10% of children with disease. |
| What is the leading cause of death for chidren with sickle cell? | bacterial infections |
| What are the precipitating factors of a sickle cell crisis? | anything that increases body's need for 02 or alters transport of 02, trauma, infection, fever, stress, dehydration(increased blood viscosity), hypoxia(from high altitude poorly pressurized) |
| What are the manifestations of a sickle cell crisis? | Acute exacerbations that vary in severity and frequency |
| Types of sickle cell crisis? | vaso-occlusive[VOC] thrombotic, splenic sequestion, aplastic crisis |
| What is Vaso-occlusive thromotic sickle cell crisis? | most common type of crisis-very painful, stasis of blood with clumping of cells in microcirculation-ischemia-infarction |
| What are the signs of Vaso-occlusive thrombotic sickle cell crisis? | fever, pain, tissue engorgement |
| What is splenic sequestration sickle cell crisis? | life threatening-death can occur within hours, blood pools in the spleen |
| What are the signs of splenic sequestration sickle cell crisis? | profound anemia, hypovolemia, and shock |
| What is aplastic crisis in sickle cell? | diminished production and increased destruction of RBCs, triggered by viral infection or depletionof folic acid |
| What are the sighs of aplastic crisis in sickle cell? | profound anemia, pallor |
| How do they diagnose sickle cell? | cord blood, newborn screen (43 states) genetic testing to identify carriers and children who have disease, sickle turbidity test (quick screening purposes in children less than 6 months old) |
| What is the medical management for sickle cell? | aggressive treatment of infection, possible prophylactic ATBs from 2 months to 5 years, monitor reticulocyte count regularyly to evaluate bone marrow function, blood transfusion(if given early in crisis may reduce ischemia) |
| What does frequent transfusion do in sickle cell crisis? | decreases hemosiderosis ( iron in tissues) |
| How do you treat hemosiderosis(iron in tissues)in sickle cell crisis? | treat with iron chelation such as feroxamine, + vitamin C to promote iron excretion |
| Rx medications used in sickle cell? | hydoxyurea (cytotoxic) decreases production of abnormal blood cells and decreases pain |
| What is the nursing considerations for child with sickle cell crisis? | Monitor childs growth-watch for failure to thrive, careful mutisystem assessment, assess pain, observe for presence of inflammation or possible infection, carefully monitor for signs of shock |
| What are the phychosocial needs of children/family with sickle cell? | coping mechanisms, support with genetic counseling, financial needs, caregiver role strain, living with chronic illness int he family |
| What is thalassemia? | inherited blood disorders of hemoglobin synthesis, classified by Hgb chain affected and by amount of effect, autosomal reciessive with varying expressivity(both parents must be carriers to have child with disease) |
| What is the pathophysiology of Thalassemia? | Anemia results from defective sythesis of Hgb, structurally impaired RBCs, and shortened life of RBCs,chronic hypoxia, detected in infancy or toddlerhood. |
| What are the S/S of chroic hypoxia with thalassemia? | headache, irritability, precordial and bone pain, exercise intolerance, anorexia, epistaxis. |
| Physical manifestations of thalassemia? | pallor, FTT, Hepatosplenomegaly, severe anemia (Hgb less than 6) |
| Who does it affect/what happens in a-Thalassemia? | Alpha chains affected, occurs in chinese, thai, african and mediterranean people |
| Who does b-Thalassemia occur in ? | Occurs in greeks, italians and syrians, b is most common and has four forms. |
| Thalassemia minor | asymptomatic silent carrier |
| Thalassemia trait | mild microcytic anemia |
| Thalassemia intermediate | moderate to severe anemia plus splenomegaly |
| Thalassemia major "Cooley's anemia" | severe anemia requiring transfusions to survive |
| What are the four types of b-Thalassemia? | Thalassemia minor, Thalassemia trait, Thalassemia intermediate, Thalassemia major "Cooley's anemia" |
| Diagnosis of Thalassemia? | by hemoglobin electrophoresis, RBC changes often seen by 6 weeks of age, child presents with severe anemia, FTT |
| Medical management of Thalassemia? | blood transfusion to maintain normal Hgb levels, side effect-hemosiderosis (treat with ironochelating drugs such as deferoxamine (Desferal-binds excess iron for excretion by kidneys)) |
| Nursing care for Thalassemia? | Observe for complications of transfusion, emotional support to family, encourage genetic counseling, parent and patient teaching for self-care |
| Prognosis for child with Thalassemia ? | retarded growth, delayed or absent secondary sex characteristics, expect to live well into adulthood with proper clinical management, bone marrow transplant is potential cure |
| What is aplastic anemia? | All formed elements of the blood are simultaneously depressed ("pancytopenia") |
| What is hypoplastic anemia? | profound depression of RBCs but normal WBCs and platelets |
| Etiology of Aplastic anemia? | primary (congenital) secondary (acquired), diagnostic evaluation, therapeutic management, nursing consideration |
| What is hemophilia? | A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors. |
| Types of hemophilia? | Hemophilia A, Hemophilia B |
| What is hemophilia A? | "Classic hemophilia", deficiency of factor Vlll, accounts for 80% of cases of hemophilia, occurence: 1 in 5000 males |
| What is Hemophilia B? | Also known as christmas disease, caused by defiency of factor lX, accounts for 15% of cases of hemophilia |
| What is the etiology of hemophilia A? | X-linked recessive trait, males are affected, females are carriers, degree of bleeding depends on the amount of clotting factor and severity of injury, up to 1/3 of cases have no known family hx (in these cases caused by a new mutation) |
| What are the manifestations of hemophilia? | bleeding tendencies rage from mild to severe, symptoms may not occur until 6 months of age (mobility leads to injuries from falls and accidents), Hemarthrosis (bleeding into joint spaces of knee, ankle, elbow leading to impaired mobility, Ecchymosis |
| What clinical therapy can be done? | Can be diagnosed through amniocnetesis, genetic testing of family members to identify carriers, diagnosis on basis of hx, labs and exam. |
| What labs help diagnose hemophilia? | Low levels of factor Vll or lX, prolonged PTT, normal platelet count, PT and fibrinogen |
| Medical management of hemophilia? | DDAVP- IV, causes 2 to 4 times increase in factor Vlll activity, used for mild hemophilia, replace missing clotting factors, transfusions-at home with prompt interventionto reduce complications, following major surgery or minor hemorrhage |
| Prognosis for hemophiliacs? | Historically, most died by 5 years old, now mild to moderate hemophilia pt. live near normal lives, gene tharapy for future- infused carrier oranisms act on target cells to promote manufacture of deficient clotting factor. |
| Interventions for hemophiliacs? | close supervision & safe invironment, dental procedures in controlled situation, shave only with electric razor, superficial bleeding -apply pressure for at least 15 minutes + ice to vasoconstrict, If significant bleeding -transfuse for factor replacement |
| Management of Hemarthrosis? | During bleeding episoses, elevate and immobilize joint, ice, analgesics, ROM after bleeding stops to prevent contractures, PT, Avoid Obesity to minimize joint stress. |
| What is von Willebrand Disease? | A hereditary bleeding disorder, involving deficiency of von Willebrand factor( a plasma protein, & the carrier for factor Vlll), von Willebrand factor is necessary for platelet adhesion, transmitted as autosomal dominant trait, males &females,chromosome12 |
| What are the manifestations of von Willebrand disease? | easy bruising, epistaxis, gingival bleeding, excessive bleeding with lacerations or surgeries, menorrhagia |
| Lab test for von Willebrands? | decreased von Willebrand factor & antigen levels, decreased platelet agglutination, prolonged bleeding time, PTT may be normal or prolonged. |
| Treatment of von Willebrand disease? | Infusion of von Willebrand's protein concentrate, DDAVP infusion before surgery or to treat bleeding episode, Aminocaproic acid to treat bleeding in mucous membranes(in some cases) |
| Interventions for von Willebrands disease? | Avoid aspirin or NSAIDs (increase bleeding time and inhibit platelet fuction), manage bleeding episodes with prompt infusion therapy, children with von Willebrand have normal life expectancy if well managed. |
| Idiopathic Thrombocytopenic Purpera (ITP) | An acquired hemorrhagic disorder characterized by: thrombocytopenia(excessive destruction of platelets), Purpura(discoloration caused by petechiae beneath the skin |
| Forms of Idiopathic Thrombocytopenic Purpura? | Acute self-limiting-often follows URI or other infections, chronic ( greater than 6 months duration) |
| What is Disseminated intravascular Coagulation (DIC)? | Disorder of coagulation that occurs as complication of numerous pathologic processes such as hypoxia, acidosie, shock, endothelial damage |
| Pathophysiology of DIC? | Coagulation process is abnormally stimulated, Excessive amounts of thrombin are generated, fibrinolytic mechanisms are activated and cause extensive destruction of clotting factors. |
| Process of DIC? | Trigger mechanism, intrinsic coagulation process, consumption of platelets and clotting factors-(bleeding tendency) or fibrin deposits in microcirculation(secondary fibrinolysis)-( ischemic tissue damage and RBC damage and hemolysis) |
| ICP | diagnostic evaluation, therapeutic management prognosis nursing considerations |
| DIC | diagnostic evaluation, therapeutic management, nursing considerations |
| Epistaxis (Nosebleed) | Isolated and transient epistaxis is common in childhood, Recurrent or severe episodes may indicate underlying disease- vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases(von Willebrand and hemophilia) |
| Nursing considerations for Epistaxis? | remain calm, keep child calm, have child sit up and lean forward pressure to nose further evaluation if bleeding continues |
| Neoplastic Disorders in children? | leading cause of death from disease in children bast infancy, almost half of all childhood cancers involve blood or blood-forming organs |
| Leukemias in children | Most common form of childhood cancer, 3 or 4 cases per 100,000 caucasian children less than 15 year old,more frequently in males greater than 1, peak onset between 2 & 6 years old, survivability. |
| Classification of Leukemias | Leukemia: broad group of malignant diseases of bone marrow and lymphatic system, complex disease with varying heterogeneity, classifications are increasingly complex |
| Mophology of leukemia | Acute lymphoid leukemia (ALL), Acute nonlymphoid (myelogenous) leukemia (ANLL or AML), Stem cell or blast cel leukemia |
| Symptoms of Leukemia? | ALL: lymphatic, lymphocytic, lyphoblastic, and lymphoblastoid leukemia, AML: granulocytic, myelocytic, monocytic myelogenous, monoblasic, and nonmyeloblastic leukemia |
| Chromosome studies for leukemia | children with trisomy 21: 20 times greater risk for developing ALL, Children with more than 50 chromosomes on the leukemic cells have the best prognosis, Specicic translocations of chromosmes on leukemic cells can affect prognosis. |
| Pathophysiology of leukemia | Leukemia is a n unrestricted proliferation of immature WBCs in the blood-forming tissues of the body, liver & spleen ar the most severly affected organs |
| More pathophisiology of leukemia | Although leukemia is an overproduction of WBCs, often acute form causes low leukocyte count, cellular destruciton takes place by infiltation and subsequent competition for metabolic elements. |
| Consequences of Leukemia | Anemia from decreased RBCs, infection from neutropenia, bleeding tendencies from decreased platelet produciton, spleen, liver, and lymph glands sho marked infiltration, enlargement, and fibrosis |
| Diagnostic evaluation? | Based on hx, physical manifestations, peripheral blood smear(immature leukocytes, frequently low blood counts), LP to evaluate CNS involvement, bone marrow aspiration or biopsy |
| Therapeutic Management | Chemotherapeutic agents, cranial irradiation (in some cases) |
| Four phases of Therapy for leukemia | Induction to therapy:4 to 6 weeks, CNS prophylactic therapy: intrathecal chemotherapy, Intensification(consolidation) therapy: to eradicate residual leukemic cells and prevent leukemic clones, Maintenance therapy: to preserve remission |
| Hematopoietic stem cell trsplatation(HSCT) for leukemia patients | donors may be relatives or nonrelatives, antigen natched or mismatched, peripheral stem cells may be used, stem cells form umbilical cord blood. |
| Risks of HSCT for leukemia patients | significant risk of morbitiy and mortality, graft vs. host disease(GVHD), overwhelming infection, severe organ damage, cure after HSCT: up to 60% or 70% |
| Prognosis of HSCT leuekemia patients | If released after HSCT: dismal prognosis, identified factors for determining prognosis: initial WBC count, age at the time of diagnosis, type of cell involvement, gender, karyotype analysis |
| Nursing considerations for leukemia patients recieving HSCT | Assessment, nusing diagnosis, planning, implementation: prepare child and family for procedures, pain management, prevent complications of myelosuppression |
| Increased susceptibility to infection in leukemia patients | At time of diagnosis and relaspe, during immunosuppressive therapy, after prolonged antibiotic therapy that predisposes to the growth of resistant organism. |
| Infection control for leukemia patients | hand washing, environment, visitor restriction |
| Managing chemotherapeutic agents with leukemia | "Vesicants"-sclerosing agents even in minute amounts, interventions for extravasation, risk for anaphylaxis |
| Managing problems of drug toxiciy in leukemia | Nausea/vomiting, Anorexia, mucosal ulceration, neuropathy, hemorrhagic cystitis, alopecia, mood changes, moon face |
| Nursing diagnoses for leukemia patients | Risk for injury related to malignant process & treatment, risk for deficient fluid volume related to nausea/vomiting, risk for imbalanced nutrition, impaired skin integrity, altered family processes, fear related to diagnosis, procedures, treatments |
| Types of Lymphomas | Hodgkin disease-15 to 19 years of age, non-hodgkin lyphoma(NHL)- more prevalent in children less than 14 years old. |
| Hodgkin disease | neoplastic disease originating in lymph system, often metastasizes to spleen, liver, bone marrow, lungs, and other tissues. |
| What are the four types of hodgkin disease? | lymphocytic predominance, nodular sclerosis, mixed cellularity, lyphocytic depletion, accurate staging is basis for treatment and prognosis |
| Classifications of Hodgkin disease? | class A: asymptomatic, class B: temperature of 38C or higher for 3 consecutive days, night sweats, unexplained wt. loss of 10% or more over previous 6 months. |
| Diagnostic studies for Hodgkin disease? | clinical manifestations of hodgkin disease, lymph node biopsy for diagnosis and staging, presence of Reed-Sternberg cells is characteristic of Hodgkins disease. |
| Therapeutic management of Hodgkin? | Radiation, chemotherapy (alone or with radiation), prognosis, nursing considerations |
| Non-Hodgkin lymphoma | Approximately 60% of pediatric lymphomas are NHL |
| Clinical appearance of Non-Hodgkin lymphoma | Disease usually diffuse rather than nodular, cell type undefferentiated or poorly differentiated, dissemination occurs early, often, and rapidly, mediastinal involvementand invasion of meninges. |
| Non-hodgkin lymphoma | Diagnostic evaluation, therapeutic management, prognosis, nursing considerations |
| Immunologic Deficiency Disorders | HIV/AIDS-Epidemiology, etiology and pathophysiology, clinical manifestions, diagnostics, therapeutic management |
| Sever combined Immunodificiency Disease (SCID) | therapeutic management, nursing considerations |
| Wiskott-Aldrich Syndrome | Thrombocytopenia, Eczema, Immunodeficiency of selective functions of B-lymphocytes and T-lyphocytes, x-linked recessive |
| Blood transfusion Therapy for Wiskott-Aldrich syndrome | verify identity of recipient and verification of donor's blood group, monitor VS, use appropriate filter, Use blood within 30 minutes of arrival, infuse over 4 hours maximum. |
| Transfusion Reactions with Wiskott-Aldrich syndrome | Hemolytic:the most severe, but rare, Febrile reactions: fever chills, Allergic reaction: urticaria, pruritis, laryngeal edema, Air emboli: may occur when blood is transfused under pressure, Hypothermia, Electrolyte disturbances: hyperkalemia |
| Nursing responsibilities with Wiskott-Aldrich syndrome blood transfusion | Identify donor and recipient blood types and groups before transfusing, transfuse slowly for first 15 to 20 minutes, observe carefully for patient response, stop transfusion immediately fi signs/symptoms of transfusion reaction; notify practitioner. |
| Delayed reactions to blood transfusion | transmission of infection--Hepatitis, HIV, malaria, syphillis, other, blood banks test vigorously and discard units of infected blood. |
| Family concerns for wiskott-aldrich sydrome | difficult decision for HTSC transplant- child faces death without transplant, preparing child for transplant places child at great risk, No "rescue" procedure if complications follow HTSC transplant, nursing considerations |
| Apheresis in Wiskott-Aldrich syndrome | Definition: removal of blood from an individual; separation of the blood into its components |
| Hematopoietic stem cell transplantation(HSCT) | Used to establish healthy cells in both malignant and nonmalignant disease, Ablative Therapy: high-dose combinatin chemo (with or without radiation) to eradicate unhealthy cells and suppress immune system to prevent rejection of transplanted marrow. |
| Hematopoietic stem Cell Transplantation(HSCT) | Stem cells harvested from bone marrow, peripheral blood, or umbilical vein of placenta, stem cells given to patient by IV transfusion, Newly transfused stem cells repopulate ablative bone marrow. |
| Allogeneic Bone Marrow Transplant (BMT) | Involves matching histocompatible donor with recipient, Limited by presence of suitable marrow donor |
| Umbilical Cord Blood Stem Cell Transplantation | Rich source of hematopoietic cells for for kids with cancers, Stem cells found with high frequency in circulation of newborns |
| Benifit of umbilical cord blood | blood's relative immunodeficiency at birth, allowing for partially matched unrelated cord blood transplants to be successful. |
| Autologous Bone marrow transfusion | Uses pt. own marrow collected from disease-free tissue, frozen and somtimes treated to remover malignant cells, Has been used to treat neuroblastoma, Hodgkins, NHL, Wilms' tumor, rhabdomyosarcoma, and Ewing sarcoma |
| Peripheral Stem Cell Transplants (PSCT) | A type of autologous transplant, different type of collection from pt., stimulate producitonof high number of stem cells, then collect stem cells by an apheresis machine, stem cells separated from whole blood |
| PSCT continued | Remaining blood cells and plasma returned to pt. after apheresis, stem cells frozen for later transfusion to pt. |
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esther502000
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