The Child with Hematologic or Immunologic Dysfunction

The Child with Hematologic or Immunologic Dysfunction Chapter 26 Mosby items and derived items © 2005, 2001 by Mosby, Inc. Assessment of Hematologic Function • Complete blood count • History and assessment findings • Child’s energy and activity level • Growth patterns Mosby items and derived items © 2005, 2001 by Mosby, Inc. Anemia • The most common hematologic disorder of childhood • Decrease in number of RBCs and/or hemoglobin concentration below normal • Decreased oxygen-carrying capacity of blood Mosby items and derived items © 2005, 2001 by Mosby, Inc. Classification of Anemias • Etiology and physiology – RBC and/or Hgb depletion • Morphology – Characteristic changes in RBC size, shape, and/or color Mosby items and derived items © 2005, 2001 by Mosby, Inc. Consequences of Anemia • Decrease in oxygen-carrying capacity of blood and decreased amount of oxygen available to tissues • When anemia develops slowly, child adapts Mosby items and derived items © 2005, 2001 by Mosby, Inc. Effects of Anemia on Circulatory System • Hemodilution • Decreased peripheral resistance • Increased cardiac circulation and turbulence – May have murmur – May lead to cardiac failure • Cyanosis • Growth retardation Mosby items and derived items © 2005, 2001 by Mosby, Inc. Diagnostic Evaluation • CBC – Decreased RBCs – Decreased Hbg and Hct • Other tests for particular type of anemia Mosby items and derived items © 2005, 2001 by Mosby, Inc. Therapeutic Management • Treat underlying cause – Transfusion after hemorrhage if needed – Nutritional intervention for deficiency anemias • Supportive care – IV fluids to replace intravascular volume – Oxygen – Bed rest Mosby items and derived items © 2005, 2001 by Mosby, Inc. Nursing Considerations • Prepare child and family for laboratory tests • Decrease oxygen demands • Prevent complications • Support family Mosby items and derived items © 2005, 2001 by Mosby, Inc. Iron Deficiency Anemia • Caused by inadequate supply of dietary iron • Generally preventable – Iron-fortified cereals and formulas for infants – Special needs of premature infants – Adolescents at risk due to rapid growth and poor eating habits Mosby items and derived items © 2005, 2001 by Mosby, Inc. Iron Deficiency Anemia (cont’d) • Pathophysiology • Therapeutic management • Prognosis • Nursing considerations Mosby items and derived items © 2005, 2001 by Mosby, Inc. Sickle Cell Anemia • A hereditary hemoglobinopathy • Ethnicity – Occurs primarily in blacks • • • Occurrence 1 in 375 infants born in US 1 in 12 have sickle cell trait Occasionally also in persons of Mediterranean descent • Also seen in South American, Arabian, and East Indian descent Mosby items and derived items © 2005, 2001 by Mosby, Inc. Etiology of Sickle Cell • In areas of world where malaria is common, individuals with sickle cell trait tend to have survival advantage over those without trait • Autosomal recessive disorder – 1 in 12 blacks are carriers (have sickle cell trait) – (If both parents have trait, each offspring will have 1 in 4 likelihood of having disease) Mosby items and derived items © 2005, 2001 by Mosby, Inc. Pathophysiology • Partial or complete replacement of normal Hgb with abnormal hemoglobin S (Hgb S) • Hemoglobin in the RBCs takes on an elongated ―sickle‖ shape • Sickled cells are rigid and obstruct capillary blood flow • Microscopic obstructions lead to engorgement and tissue ischemia • Hypoxia occurs and causes sickling Mosby items and derived items © 2005, 2001 by Mosby, Inc. Sickled Hemoglobin • (Note to instructor: Image available in Electronic Image Collection) Mosby items and derived items © 2005, 2001 by Mosby, Inc. Pathophysiology • Large tissue infarctions occur • Damaged tissues in organs; impaired function – Splenic sequestration • • May require splenectomy at early age Results in immunity Mosby items and derived items © 2005, 2001 by Mosby, Inc. Prognosis • No cure (except possibly bone marrow transplants) • Supportive care/prevent sickling episodes • Frequent bacterial infections may occur due to immunocompromise • Bacterial infection is leading cause of death in young children with sickle cell disease • Strokes in 5%-10% of children with disease – Result in neurodevelopmental delay, mental retardation Mosby items and derived items © 2005, 2001 by Mosby, Inc. Systems Affected • (Note to instructor: Image available in Electronic Image Collection) Mosby items and derived items © 2005, 2001 by Mosby, Inc. Sickle Cell Crisis • Precipitating factors – Anything that increases body’s need for oxygen or alters transport of oxygen – Trauma – Infection, fever – Physical and emotional stress – Increased blood viscosity due to dehydration – Hypoxia • From high altitude, poorly pressurized airplanes, hypoventilation,vasoconstriction due to hypothermia Mosby items and derived items © 2005, 2001 by Mosby, Inc. Sickle Cell Crisis (cont’d) • Acute exacerbations that vary in severity and frequency • Types – Vaso-occlusive [VOC] thrombotic • • • Most common type of crisis—very painful Stasis of blood with clumping of cells in microcirculation→ischemia→infarction Signs: fever, pain, tissue engorgement Mosby items and derived items © 2005, 2001 by Mosby, Inc. Sickle Cell Crisis (cont’d) • Types (cont’d) – Splenic sequestration • • • Life threatening—death can occur within hours Blood pools in the spleen Signs – Profound anemia, hypovolemia, and shock Mosby items and derived items © 2005, 2001 by Mosby, Inc. Sickle Cell Crisis (cont’d) • Types (cont’d) – Aplastic crises • • • Diminished production and increased destruction of RBCs Triggered by viral infection or depletion of folic acid Signs include profound anemia, pallor Mosby items and derived items © 2005, 2001 by Mosby, Inc. Diagnosis of Sickle Cell • Cord blood in newborns • Newborn screening done in 43 states • Genetic testing to identify carriers and children who have disease • Sickle-turbidity test – Quick screening purposes in children >6 mos of age Mosby items and derived items © 2005, 2001 by Mosby, Inc. Medical Management • Aggressive treatment of infection • Possible prophylactic antibiotics from 2 mos-5 yrs Mosby items and derived items © 2005, 2001 by Mosby, Inc. Medical Management (cont’d) • Monitor reticulocyte count regularly to evaluate bone marrow function • Blood transfusion, if given early in crisis, may reduce ischemia Mosby items and derived items © 2005, 2001 by Mosby, Inc. Medical Management (cont’d) • Frequent transfusion decreases hemosiderosis (iron in tissues) – Treat with iron-chelation such as feroxamine + vitamin C to promote iron excretion • Rx—Hydronurea (Cytotoxic) decreases production of abnormal blood cells and decreases pain Mosby items and derived items © 2005, 2001 by Mosby, Inc. Nursing Management • Monitor child’s growth—watch for failure to thrive • Careful multi-system assessment • Assess pain • Observe for presence of inflammation or possible infection • Carefully monitor for signs of shock Mosby items and derived items © 2005, 2001 by Mosby, Inc. Psychosocial Needs • Coping mechanisms • Support with genetic counseling • Financial needs • Caregiver role strain • Living with chronic illness in the family Mosby items and derived items © 2005, 2001 by Mosby, Inc. Thalassemia • Inherited blood disorders of hemoglobin synthesis • Classified by Hgb chain affected and by amount of effect • Autosomal recessive with varying expessivity – Both parents must be carriers to have offspring with disease Mosby items and derived items © 2005, 2001 by Mosby, Inc. Pathophysiology • Anemia results from defective synthesis of Hgb, structurally impaired RBCs, and shortened life of RBCs • Chronic hypoxia – Headache, irritability, precordial and bone pain, exercise intolerance, anorexia, epistaxis • Detected in infancy or toddlerhood – Pallor, FTT, hepatosplenomegaly, severe anemia (Hgb <6) Mosby items and derived items © 2005, 2001 by Mosby, Inc. Thalassemia • -Thalassemia – Alpha chains affected – Occurs in Chinese, Thai, African, and Mediterranean people • -Thalassemia – Occurs in Greeks, Italians, and Syrians –  is most common and has four forms Mosby items and derived items © 2005, 2001 by Mosby, Inc. -Thalassemia • Four types – Thalassemia minor—asymptomatic silent carrier – Thalassemia trait—mild microcytic anemia – Thalassemia intermediate—moderate to severe anemia + splenomegaly – Thalassemia major (―Cooley’s anemia‖) — severe anemia requiring transfusions to survive Mosby items and derived items © 2005, 2001 by Mosby, Inc. Diagnosis • By hemoglobin electrophoresis • RBC changes often seen by 6 weeks of age • Child presents with severe anemia, FTT Mosby items and derived items © 2005, 2001 by Mosby, Inc. Medical Management • Blood transfusion to maintain normal Hgb levels • Side effect—hemosiderosis • Treat with iron chelating drugs such as deferoxamine (Desferal) – Binds excess iron for excretion by kidney Mosby items and derived items © 2005, 2001 by Mosby, Inc. Nursing Management • Observe for complications of transfusion • Emotional support to family • Encourage genetic counseling • Parent and patient teaching for self-care Mosby items and derived items © 2005, 2001 by Mosby, Inc. Prognosis • Retarded growth • Delayed or absent secondary sex characteristics • Expect to live well into adulthood with proper clinical management • Bone marrow transplant is potential cure Mosby items and derived items © 2005, 2001 by Mosby, Inc. Aplastic Anemia • All formed elements of the blood are simultaneously depressed: ―pancytopenia‖ • Hypoplastic anemia: profound depression of RBCs but normal WBCs and platelets Mosby items and derived items © 2005, 2001 by Mosby, Inc. Aplastic Anemia (cont’d) • Etiology – Primary (congenital) – Secondary (acquired) • Diagnostic evaluation • Therapeutic management • Nursing consideration Mosby items and derived items © 2005, 2001 by Mosby, Inc. Hemophilia • A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors Mosby items and derived items © 2005, 2001 by Mosby, Inc. Types of Hemophilia • Hemophilia A – ―Classic hemophilia‖ – Deficiency of factor VIII – Accounts for 80% of cases of hemophilia – Occurrence: 1 in 5000 males Mosby items and derived items © 2005, 2001 by Mosby, Inc. Types of Hemophilia (cont’d) • Hemophilia B – Also known as Christmas disease – Caused by deficiency of factor IX – Accounts for 15% of cases of hemophilia Mosby items and derived items © 2005, 2001 by Mosby, Inc. Etiology of Hemophilia A • X-linked recessive trait • Males are affected • Females may be carriers • Degree of bleeding depends on amount of clotting factor and severity of a given injury • Up to ⅓ of cases have no known family history – In these cases disease is caused by a NEW mutation Mosby items and derived items © 2005, 2001 by Mosby, Inc. Manifestations of Hemophilia • Bleeding tendencies range from mild to severe • Symptoms may not occur until 6 mos of age – Mobility leads to injuries from falls and accidents • Hemarthrosis – Bleeding into joint spaces of knee, ankle, elbow leading to impaired mobility • Ecchymosis Mosby items and derived items © 2005, 2001 by Mosby, Inc. Manifestations (cont’d) • Epistaxis • Bleeding after procedures – Minor trauma, tooth extraction, minor surgeries – Large subcutaneous and intramuscular hemorrhages may occur – Bleeding into neck, chest, mouth may compromise airway Mosby items and derived items © 2005, 2001 by Mosby, Inc. Clinical Therapy • Can be diagnosed through amniocentesis • Genetic testing of family members to identify carriers • Diagnosis on basis of hx, labs, and exam – *Labs: Low levels of factor VIII or IX, prolonged PTT – *Normal: platelet count, PT, and fibrinogen Mosby items and derived items © 2005, 2001 by Mosby, Inc. Medical Management • DDAVP – IV – Causes 2-4 X increase in factor VIII activity – Used for mild hemophilia • Replace missing clotting factors • Transfusions – At home with prompt intervention to reduce complications – Following major or minor hemorrhages Mosby items and derived items © 2005, 2001 by Mosby, Inc. Prognosis • Historically, most died by 5 yrs age • Now mild to moderate hemophilia patients live near normal lives • Gene therapy for future – Infused carrier organisms act on target cells to promote manufacture of deficient clotting factor Mosby items and derived items © 2005, 2001 by Mosby, Inc. Interventions • Close supervision and safe environment • 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 occurs, transfuse for factor replacement Mosby items and derived items © 2005, 2001 by Mosby, Inc. Managing Hemarthrosis • During bleeding episodes, elevate and immobilize joint • Ice • Analgesics • ROM after bleeding stops to prevent contractures • PT • Avoid obesity to minimize joint stress Mosby items and derived items © 2005, 2001 by Mosby, Inc. Von Willebrand Disease • A hereditary bleeding disorder, involving deficiency of Von Willebrand factor, (a plasma protein, and the carrier for factor VIII) • Von Willebrand factor is necessary for platelet adhesion • Transmitted as autosomal dominant trait • Occurs in males and females • Gene for disease is located on chromosome 12 Mosby items and derived items © 2005, 2001 by Mosby, Inc. Manifestations of Von Willebrand Disease • Easy bruising • Epistaxis • Gingival bleeding • Excessive bleeding with lacerations or surgeries • Menorrhagia Mosby items and derived items © 2005, 2001 by Mosby, Inc. Diagnosis Laboratory Findings • Decreased Von Willebrand factor levels • Von Willebrand antigen levels • Decreased platelet agglutination • Prolonged bleeding time • PTT may be normal or prolonged Mosby items and derived items © 2005, 2001 by Mosby, Inc. Treatment of Von Willebrand Disease • Infusion of Von Willebrand’s protein concentrate • DDAVP infusion prior to surgery or to treat bleeding episode • Aminocaproic acid to treat bleeding in mucous membranes (in some cases) Mosby items and derived items © 2005, 2001 by Mosby, Inc. Interventions • Avoid aspirin or NSAIDs (increased bleeding time and inhibit platelet function) • Manage bleeding episodes with prompt infusion therapy • Children with Von Willebrand’s have normal life expectancy if well managed Mosby items and derived items © 2005, 2001 by Mosby, Inc. Idiopathic Thrombocytopenic Purpura (ITP) • An acquired hemorrhagic disorder characterized by – Thrombocytopenia: excessive destruction of platelets – Purpura: discoloration caused by petechiae beneath the skin Mosby items and derived items © 2005, 2001 by Mosby, Inc. ITP Forms • Acute self-limiting – Often follows URI or other infection • Chronic (>6 months duration) Mosby items and derived items © 2005, 2001 by Mosby, Inc. ITP • Diagnostic evaluation • Therapeutic management • Prognosis • Nursing considerations Mosby items and derived items © 2005, 2001 by Mosby, Inc. Disseminated Intravascular Coagulation (DIC) • Disorder of coagulation that occurs as complication of numerous pathologic processes – Hypoxia – Acidosis – Shock – Endothelial damage Mosby items and derived items © 2005, 2001 by Mosby, Inc. 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 Mosby items and derived items © 2005, 2001 by Mosby, Inc. DIC • Diagnostic evaluation • Therapeutic management • Nursing considerations Mosby items and derived items © 2005, 2001 by Mosby, Inc. Epistaxis (Nosebleeding) • 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 disease and hemophilia) Mosby items and derived items © 2005, 2001 by Mosby, Inc. Nursing Considerations— Epistaxis • Remain calm, keep child calm • Have child sit up and lean forward • Pressure to nose • Further evaluation if bleeding continues Mosby items and derived items © 2005, 2001 by Mosby, Inc. Neoplastic Disorders • Leading cause of death from disease in children past infancy • Almost half of all childhood cancers involve blood or blood-forming organs Mosby items and derived items © 2005, 2001 by Mosby, Inc. Leukemias • Most common form of childhood cancer • 3-4 cases per 100,000 Caucasian children <15 yrs old • More frequent in males >1 yr old • Peak onset between 2 and 6 yrs of age • Survivability Mosby items and derived items © 2005, 2001 by Mosby, Inc. Classification of Leukemias • Leukemia: A broad group of malignant disease of bone marrow and lymphatic system • Complex disease with varying heterogeneity • Classifications are increasingly complex Mosby items and derived items © 2005, 2001 by Mosby, Inc. Morphology • Acute lymphoid leukemia (ALL) • Acute non-lymphoid (myelogenous) leukemia (ANLL or AML) • Stem cell or blast cell leukemia Mosby items and derived items © 2005, 2001 by Mosby, Inc. Symptoms • ALL: lymphatic, lymphocytic, lymphoblastic, and lymphoblastoid leukemia • AML: granulocytic, myelocytic, monocytic, myelogenous, monoblastic, and nonmyeloblastic leukemia Mosby items and derived items © 2005, 2001 by Mosby, Inc. Chromosome Studies • Children with Trisomy 21:20X greater risk for developing ALL • Children with more than 50 chromosomes on the leukemic cells have the best prognosis • Specific translocations of chromosomes on leukemic cells can affect prognosis Mosby items and derived items © 2005, 2001 by Mosby, Inc. Pathophysiology • Leukemia is an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body • Liver and spleen are the most severely affected organs Mosby items and derived items © 2005, 2001 by Mosby, Inc. Pathophysiology (cont’d) • Although leukemia is an overproduction of WBCs, often acute form causes low leukocyte count • Cellular destruction takes place by infiltration and subsequent competition for metabolic elements Mosby items and derived items © 2005, 2001 by Mosby, Inc. Consequences of Leukemia • Anemia from decreased RBCs • Infection from neutropenia • Bleeding tendencies from decreased platelet production • Spleen, liver, and lymph glands show marked infiltration, enlargement, and fibrosis Mosby items and derived items © 2005, 2001 by Mosby, Inc. Diagnostic Evaluation • Based on history, physical manifestations • Peripheral blood smear – Immature leukocytes – Frequently low blood counts • LP to evaluate CNS involvement • Bone marrow aspiration or biopsy Mosby items and derived items © 2005, 2001 by Mosby, Inc. Therapeutic Management • Chemotherapeutic agents • Cranial irradiation (in some cases) Mosby items and derived items © 2005, 2001 by Mosby, Inc. Four Phases of Therapy • Induction therapy: 4-6 weeks • CNS prophylactic therapy: intrathecal chemotherapy • Intensification (consolidation) therapy: To eradicate residual leukemic cells and prevent resistant leukemic clones • Maintenance therapy: to preserve remission Mosby items and derived items © 2005, 2001 by Mosby, Inc. Hematopoietic Stem Cell Transplantation (HSCT) • Donors may be relatives or non-relatives • Antigen-matched or mismatched • Peripheral stem cells may be used • Stem cells from umbilical cord blood Mosby items and derived items © 2005, 2001 by Mosby, Inc. Risks of HSCT • Significant risk of morbidity and mortality • Graft vs. host disease (GVHD) • Overwhelming infection • Severe organ damage • Cure after HSCT: up to 60%-70% Mosby items and derived items © 2005, 2001 by Mosby, Inc. Prognosis • If relapse after HSCT: dismal prognosis • Identified factors for determining prognosis – Initial WBC count – Age at time of diagnosis – Type of cell involved – Gender – Karyotype analysis Mosby items and derived items © 2005, 2001 by Mosby, Inc. Nursing Considerations • Assessment • Nursing diagnosis • Planning • Implementation – Prepare child and family for procedures – Pain management – Prevent complication of myelosuppression Mosby items and derived items © 2005, 2001 by Mosby, Inc. Increased Susceptibility to Infection • At time of diagnosis and relapse • During immunosuppressive therapy • After prolonged antibiotic therapy that predisposes to the growth of resistant organism Mosby items and derived items © 2005, 2001 by Mosby, Inc. Infection Control • Environment • Hand hygiene • Visitor restriction Mosby items and derived items © 2005, 2001 by Mosby, Inc. Managing Chemotherapeutic Agents • ―Vesicants‖—sclerosing agents even in minute amounts • Interventions for extravasation • Risk for anaphylaxis Mosby items and derived items © 2005, 2001 by Mosby, Inc. Managing Problems of Drug Toxicity – Nausea/vomiting – Anorexia – Mucosal ulceration – Neuropathy – Hemorrhagic cystitis – Alopecia – Mood changes – Moon face Mosby items and derived items © 2005, 2001 by Mosby, Inc. Nursing Diagnoses • Risk for injury related to malignant process, treatment • Risk for fluid volume deficit related to nausea, vomiting • Altered nutrition • Impaired skin integrity • Altered family processes • Fear related to diagnosis, procedures, treatments Mosby items and derived items © 2005, 2001 by Mosby, Inc. Lymphomas • Hodgkin disease – More prevalent in 15-19 yrs of age • Non-Hodgkin lymphoma (NHL) – More prevalent in children <14 yrs of age Mosby items and derived items © 2005, 2001 by Mosby, Inc. Hodgkin Disease • Neoplastic disease originating in lymphoid system • Often metastasizes to spleen, liver, bone marrow, lungs, and other tissues Mosby items and derived items © 2005, 2001 by Mosby, Inc. Four Types of Hodgkin Disease • Lymphocytic predominance • Nodular sclerosis • Mixed cellularity • Lymphocytic depletion • Accurate staging is basis for treatment and prognosis Mosby items and derived items © 2005, 2001 by Mosby, Inc. Classifications of Hodgkin Disease • Classification A: asymptomatic • Classification B: temperature of 38°C or higher for three consecutive days, night sweats, unexplained wt loss of 10% or more over previous 6 mos Mosby items and derived items © 2005, 2001 by Mosby, Inc. Diagnostics • Clinical manifestations of Hodgkin disease • Lymph node biopsy for diagnosis and staging • Presence of Reed-Sternberg cells is characteristic of Hodgkin disease Mosby items and derived items © 2005, 2001 by Mosby, Inc. Therapeutic Management • Radiation • Chemotherapy (alone or with radiation) • Prognosis • Nursing considerations Mosby items and derived items © 2005, 2001 by Mosby, Inc. Non-Hodgkin Lymphoma • Approximately 60% of pediatric lymphomas are NHL • Clinical appearance – Disease usually diffuse rather than nodular – Cell type undifferentiated or poorly differentiated – Dissemination occurs early, often, and rapidly – Mediastinal involvement and invasion of meninges Mosby items and derived items © 2005, 2001 by Mosby, Inc. NHL (cont’d) • Diagnostic evaluation • Therapeutic management • Prognosis • Nursing consideration Mosby items and derived items © 2005, 2001 by Mosby, Inc. Immunologic Deficiency Disorders • HIV/AIDS – Epidemiology – Etiology and pathophysiology – Clinical manifestations – Diagnostics – Therapeutic management Mosby items and derived items © 2005, 2001 by Mosby, Inc. Severe Combined Immunodeficiency Disease (SCID) • Therapeutic management • Nursing considerations Mosby items and derived items © 2005, 2001 by Mosby, Inc. Wiskott-Aldrich Syndrome • Thrombocytopenia • Eczema • Immunodeficiency of selective functions of B and T lymphocytes • X-linked recessive Mosby items and derived items © 2005, 2001 by Mosby, Inc. Blood Transfusion Therapy • 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 Mosby items and derived items © 2005, 2001 by Mosby, Inc. Transfusion Reactions • 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 from massive transfusions or pt with renal problems Mosby items and derived items © 2005, 2001 by Mosby, Inc. Nursing Responsibilities • Identify donor and recipient blood types and groups before transfusing • Transfuse slowly for first 15-20 minutes • Observe carefully for patient response • Stop transfusion immediately if signs/symptoms of transfusion reaction; notify practitioner Mosby items and derived items © 2005, 2001 by Mosby, Inc. Delayed Reactions to Blood Transfusion • Transmission of infection – Hepatitis, HIV, malaria, syphilis, other – Blood banks test vigorously and discard units of infected blood • Delayed hemolytic reaction – Destruction of RBCs and fever 5-10 days after transfusion – Observe for posttransfusion anemia Mosby items and derived items © 2005, 2001 by Mosby, Inc. Family Concerns • 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 transplants • Nursing considerations Mosby items and derived items © 2005, 2001 by Mosby, Inc. Apheresis • Definition: removal of blood from an individual; separation of the blood into its components • Nursing considerations Mosby items and derived items © 2005, 2001 by Mosby, Inc. Hematopoietic Stem Cell Transplantation (HSCT) • Used to establish healthy cells in both malignant and nonmalignant disease • Ablative therapy: high-dose combination chemo (with or without radiation) to eradicate unhealthy cells and suppress immune system to prevent rejection of transplanted marrow Mosby items and derived items © 2005, 2001 by Mosby, Inc. HSCT (cont’d) • 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 Mosby items and derived items © 2005, 2001 by Mosby, Inc. Allogeneic Bone Marrow Transplant (BMT) • Involves matching histocompatible donor with recipient • Limited by presence of suitable marrow donor Mosby items and derived items © 2005, 2001 by Mosby, Inc. Umbilical Cord Blood Stem Cell Transplantation • Rich source of hematopoietc stem cells for use in children with cancers • Stem cells found with high frequency in circulation of newborns • Benefit of umbilical cord blood is blood’s relative immunodeficiency at birth, allowing for partially matched unrelated cord blood transplants to be successful Mosby items and derived items © 2005, 2001 by Mosby, Inc. Autologous BMT • Uses patient’s own marrow collected from disease-free tissue, frozen and sometimes treated to remove malignant cells • Has been used to treat neuroblastoma, Hodgkin disease, NHL, Wilms' tumor, rhabdomyosarcoma, and Ewing sarcoma Mosby items and derived items © 2005, 2001 by Mosby, Inc. Peripheral Stem Cell Transplants (PSCT) • A type of autologous transplant • Different type of collection from patient • Stimulate production of high number of stem cells then collect stem cells by an ―apheresis‖ machine • Stem cells separated from whole blood • Remaining blood cells and plasma returned to patient after apheresis • Stem cells frozen for later transfusion to patient Mosby items and derived items © 2005, 2001 by Mosby, Inc.