Child With Hematologic Or Immunologic Dysfunction

The Child with Hematologic or Immunologic Dysfunction ERNIE B. GUEVARRA, M.D.

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Assessment of Hematologic Function • Complete blood count • History and assessment findings • Child’s energy and activity level • Growth patterns

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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

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Classification of Anemias • Etiology and physiology – RBC and/or Hgb depletion • Morphology – Characteristic changes in RBC size, shape, and/or color

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Consequences of Anemia • Decrease in oxygen-carrying capacity of blood and decreased amount of oxygen available to tissues • When anemia develops slowly, child adapts

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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

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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

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Nursing Considerations • Prepare child and family for laboratory tests • Decrease oxygen demands • Prevent complications • Support family

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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

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Sickle Cell Anemia • A hereditary hemoglobinopathy • Ethnicity – Occurs primarily in blacks •

Occurrence 1 in 375 infants born in US

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1 in 12 have sickle cell trait

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Occasionally also in persons of Mediterranean descent

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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)

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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.

Pathophysiology • Large tissue infarctions occur • Damaged tissues in organs; impaired function – Splenic sequestration •

May require splenectomy at early age

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Results in immunity

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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.

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 ∀α-Thalassemia – Alpha chains affected – Occurs in Chinese, Thai, African, and Mediterranean people ∀β-Thalassemia – Occurs in Greeks, Italians, and Syrians – β is most common and has four forms Mosby items and derived items © 2005, 2001 by Mosby, Inc.

β-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

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Diagnosis • By hemoglobin electrophoresis • RBC changes often seen by 6 weeks of age • Child presents with severe anemia, FTT

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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

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Nursing Management • Observe for complications of transfusion • Emotional support to family • Encourage genetic counseling • Parent and patient teaching for self-care

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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

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Aplastic Anemia • All formed elements of the blood are simultaneously depressed: “pancytopenia” • Hypoplastic anemia: profound depression of RBCs but normal WBCs and platelets

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Aplastic Anemia (cont’d) • Etiology – Primary (congenital) – Secondary (acquired) • Diagnostic evaluation • Therapeutic management • Nursing consideration

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Hemophilia • A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors

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Types of Hemophilia • Hemophilia A – “Classic hemophilia” – Deficiency of factor VIII – Accounts for 80% of cases of hemophilia – Occurrence: 1 in 5000 males

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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

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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

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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

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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

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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

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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.

Idiopathic Thrombocytopenic Purpura (ITP) • An acquired hemorrhagic disorder characterized by – Thrombocytopenia: excessive destruction of platelets – Purpura: discoloration caused by petechiae beneath the skin

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ITP Forms • Acute self-limiting – Often follows URI or other infection • Chronic (>6 months duration)

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ITP • Diagnostic evaluation • Therapeutic management • Prognosis • Nursing considerations

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Disseminated Intravascular Coagulation (DIC) • Disorder of coagulation that occurs as complication of numerous pathologic processes – Hypoxia – Acidosis – Shock – Endothelial damage

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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

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DIC • Diagnostic evaluation • Therapeutic management • Nursing considerations

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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)

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Nursing Considerations— Epistaxis • Remain calm, keep child calm • Have child sit up and lean forward • Pressure to nose • Further evaluation if bleeding continues

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Neoplastic Disorders • Leading cause of death from disease in children past infancy • Almost half of all childhood cancers involve blood or blood-forming organs

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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

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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

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Morphology • Acute lymphoid leukemia (ALL) • Acute non-lymphoid (myelogenous) leukemia (ANLL or AML) • Stem cell or blast cell leukemia

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Symptoms • ALL: lymphatic, lymphocytic, lymphoblastic, and lymphoblastoid leukemia • AML: granulocytic, myelocytic, monocytic, myelogenous, monoblastic, and nonmyeloblastic leukemia

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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

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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

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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

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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

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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

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Therapeutic Management • Chemotherapeutic agents • Cranial irradiation (in some cases)

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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.

Nursing Considerations • Assessment • Nursing diagnosis • Planning • Implementation – Prepare child and family for procedures – Pain management – Prevent complication of myelosuppression

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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

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Infection Control • Environment • Hand hygiene • Visitor restriction

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Managing Chemotherapeutic Agents • “Vesicants”—sclerosing agents even in minute amounts • Interventions for extravasation • Risk for anaphylaxis

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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

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Hodgkin Disease • Neoplastic disease originating in lymphoid system • Often metastasizes to spleen, liver, bone marrow, lungs, and other tissues

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Four Types of Hodgkin Disease • Lymphocytic predominance • Nodular sclerosis • Mixed cellularity • Lymphocytic depletion • Accurate staging is basis for treatment and prognosis

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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

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Diagnostics • Clinical manifestations of Hodgkin disease • Lymph node biopsy for diagnosis and staging • Presence of Reed-Sternberg cells is characteristic of Hodgkin disease

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Therapeutic Management • Radiation • Chemotherapy (alone or with radiation) • Prognosis • Nursing considerations

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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

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Immunologic Deficiency Disorders • HIV/AIDS – Epidemiology – Etiology and pathophysiology – Clinical manifestations – Diagnostics – Therapeutic management

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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

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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

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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

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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

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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

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Allogeneic Bone Marrow Transplant (BMT) • Involves matching histocompatible donor with recipient • Limited by presence of suitable marrow donor

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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

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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

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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.