child with hematologic or immunologic dysfunction

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The Child with Hematologicor Immunologic Dysfunction

ERNIE B. GUEVARRA, M.D.


Assessment of Hematologic Function

• Complete blood count

• History and assessment findings

• Child’s energy and activity level

• Growth patterns


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


Classification of Anemias

• Etiology and physiology

– RBC and/or Hgb depletion

• Morphology

– Characteristic changes in RBC size, shape, and/or color


Consequences of Anemia

• Decrease in oxygen-carrying capacity of blood and decreased amount of oxygen available to tissues

• When anemia develops slowly, child adapts


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


Diagnostic Evaluation

• CBC

– Decreased RBCs

– Decreased Hbg and Hct

• Other tests for particular type of anemia


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


Nursing Considerations

• Prepare child and family for laboratory tests

• Decrease oxygen demands

• Prevent complications

• Support family


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


Iron Deficiency Anemia (cont’d)

• Pathophysiology

• Therapeutic management

• Prognosis

• Nursing considerations


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


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)


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


Pathophysiology

• Large tissue infarctions occur

• Damaged tissues in organs; impaired function

– Splenic sequestration

• May require splenectomy at early age

• Results in immunity


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


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


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)


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


-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


Diagnosis

• By hemoglobin electrophoresis

• RBC changes often seen by 6 weeks of age

• Child presents with severe anemia, FTT


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


Nursing Management

• Observe for complications of transfusion

• Emotional support to family

• Encourage genetic counseling

• Parent and patient teaching for self-care


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


Aplastic Anemia

• All formed elements of the blood are simultaneously depressed: “pancytopenia”

• Hypoplastic anemia: profound depression of RBCs but normal WBCs and platelets


Aplastic Anemia (cont’d)

• Etiology

– Primary (congenital)

– Secondary (acquired)

• Diagnostic evaluation


• Nursing consideration


Hemophilia

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


Types of Hemophilia

• Hemophilia A

– “Classic hemophilia”

– Deficiency of factor VIII

– Accounts for 80% of cases of hemophilia

– Occurrence: 1 in 5000 males


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


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


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


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


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


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

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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


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


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


Idiopathic Thrombocytopenic Purpura (ITP)

• An acquired hemorrhagic disorder characterized by

– Thrombocytopenia: excessive destruction of platelets

– Purpura: discoloration caused by petechiae beneath the skin


ITP Forms

• Acute self-limiting

– Often follows URI or other infection

• Chronic (>6 months duration)


ITP



• Prognosis



Disseminated Intravascular Coagulation (DIC)

• Disorder of coagulation that occurs as complication of numerous pathologic processes

– Hypoxia

– Acidosis

– 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


DIC





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)


Nursing Considerations—Epistaxis

• Remain calm, keep child calm

• Have child sit up and lean forward

• Pressure to nose

• Further evaluation if bleeding continues


Neoplastic Disorders

• Leading cause of death from disease in children past infancy

• Almost half of all childhood cancers involve blood or blood-forming organs


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


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


Morphology

• Acute lymphoid leukemia (ALL)

• Acute non-lymphoid (myelogenous) leukemia (ANLL or AML)

• Stem cell or blast cell leukemia


Symptoms

• ALL: lymphatic, lymphocytic, lymphoblastic, and lymphoblastoid leukemia

• AML: granulocytic, myelocytic, monocytic, myelogenous, monoblastic, and non-myeloblastic leukemia


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


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


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


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


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



• Chemotherapeutic agents

• Cranial irradiation (in some cases)


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


Nursing Considerations

• Assessment

• Nursing diagnosis

• Planning

• Implementation

– Prepare child and family for procedures

– Pain management

– Prevent complication of myelosuppression


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


Infection Control

• Environment

• Hand hygiene

• Visitor restriction


Managing Chemotherapeutic Agents

• “Vesicants”—sclerosing agents even in minute amounts

• Interventions for extravasation

• Risk for anaphylaxis


Managing Problems of Drug Toxicity

– Nausea/vomiting

– Anorexia

– Mucosal ulceration

– Neuropathy

– Hemorrhagic cystitis

– Alopecia

– Mood changes

– Moon face


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


Lymphomas

• Hodgkin disease

– More prevalent in 15-19 yrs of age

• Non-Hodgkin lymphoma (NHL)

– More prevalent in children <14 yrs of age


Hodgkin Disease

• Neoplastic disease originating in lymphoid system

• Often metastasizes to spleen, liver, bone marrow, lungs, and other tissues


Four Types of Hodgkin Disease

• Lymphocytic predominance

• Nodular sclerosis

• Mixed cellularity

• Lymphocytic depletion

• Accurate staging is basis for treatment and prognosis


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


Diagnostics

• Clinical manifestations of Hodgkin disease

• Lymph node biopsy for diagnosis and staging

• Presence of Reed-Sternberg cells is characteristic of Hodgkin disease



• Radiation

• Chemotherapy (alone or with radiation)

• Prognosis



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


NHL (cont’d)



• Prognosis

• Nursing consideration


Immunologic Deficiency Disorders

• HIV/AIDS

– Epidemiology

– Etiology and pathophysiology

– Clinical manifestations

– Diagnostics

– Therapeutic management


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


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

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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


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


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



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


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


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 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


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


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

child with hematologic or immunologic dysfunction

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