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Child Health Nursing Chapter 28 Alterations in Hematologic Function Kristine Ruggiero CPNP, MSN, RN © 2006 Pearson Education, Inc. Pearson Prentice Hall.

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Presentation on theme: "Child Health Nursing Chapter 28 Alterations in Hematologic Function Kristine Ruggiero CPNP, MSN, RN © 2006 Pearson Education, Inc. Pearson Prentice Hall."— Presentation transcript:

1 Child Health Nursing Chapter 28 Alterations in Hematologic Function Kristine Ruggiero CPNP, MSN, RN © 2006 Pearson Education, Inc. Pearson Prentice Hall Upper Saddle River, NJ 07458

2 Structure and Function of Blood Componenets Review of Hematologic System –Blood formation Red Blood Cells White Blood Cells Platelets

3 FIGURE 28–1 Types of blood cells. Jane W. Ball and Ruth C. Bindler Child Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc. Upper Saddle River, New Jersey 07458 All rights reserved.

4 Hematologic System Bone marrow contains the essential element in the hematologic system…. –The STEM CELL aka the pluripotential stem cell, meaning it has the ability to transform into more than one type of blood cell. –Remember, every blood cell in the body arises from a stem cell. –Although it’s fluid, blood is one of the body’s major tissues.

5 Blood Formation In utero, the process of blood formation, called hematopoiesis, occurs in the liver and spleen. These organs retain some hematopoietic ability throughout life. After birth, the red bone marrow becomes the main site of hematopoiesis. The stem cells contained in the red marrow create blast cells. These are precursors to –RBCs, WBCs and PLTs

6 Blood Components Blood is composed on plasma and cells –90% water –10% solutes, such as proteins, electrolytes, albumin, clotting factors, anticoagulants, antibodies and dissolved nutrients. 3 main cell types –RBCs or erythrocytes –WBC or leukocytes –Platelets, or thrombocytes –See Table 28-1 for normal pediatric values

7 Red Blood Cells Carry O2 to the tissues, and CO2 away from tissues During times of hypoxia, a hormone from the kidneys (erythropoietin) stimulates the bone marrow to produce more RBCs. –Synthetic forms now available Life of RBC= 120 days An important waste product of RBC death is bilirubin

8 White Blood Cells Fight different types of infection in our body; each type has it’s own role 2 main categories of WBC’s –Granular leukocytes (granulocytes) Neutrphils, decour invading microorganisms by phagocytosis Eosinophils, act in allergic rxns, defend against parasites and lung and skin infections Basophils, release heparin and histamine, involved in inflammatory and infectious rxns, aka mast cells in body tissues –Nongranular leukocytes (agranulocytes) Lymphocytes, which are the main cells that fight infections and include B and T cells Monocytes, work with neutrophils to help devour invading organisms


10 Platelets Adhere to one another and plug holes in vessels or tissues where there’s bleeding. This action is part of a larger coagulation process PLTs also release serotonin at injury sites –Serotonin is a vasoconstrictor, decreases blood flow to injured areas

11 Iron Deficiency Anemia A disorder of O2 transport in which the production of hgb is inadequate. Without sufficient iron, the body can’t produce the Hgb molecure, b/c the heme component is primarily iron

12 Iron Deficiency Anemia The cause? –Inadequate intake of iron in the diet, malabsorption of iron through the GI tract, or chronic blood loss –Last trimester of pregnancy, the fetus draws what iron it needs for the next 6-12 months If mother is deficient in iron or Baby is more than 4 weeks premature (32 weeks) may not have sufficient iron intake Anemia will usually present in 2 nd year of life

13 Iron Deficiency Anemia Who knew? About 80% of iron used in building Hgb is actually reabsorbed in the GI tract from dead RBCs that have broken up. Therefore, problems w/ GI absorption causes iron deficiency –Cow’s milk allergy (common in Blacks and Asians) causes inflammation of GI tract –In adolescents- fad diets

14 Iron Deficiency Anemia Clinical Manifestations: –Range from mild to severe –Pale appearance and decreased activity –Toddlers may have h/o prematurity and poor weight gain –Other sxs include Fatigue, inability to concentrate, palpitations, dyspnea on exertion, craving for nonnutritive substances such as ice, tachycardia, dry brittle nails, concave or “spoon-shaped fingernails

15 Iron Deficiency Anemia lab values Tests: Hgb levels are routinely screened, and a CBC is typically done at 9-12 months and 24 month well-baby check-ups and at-risk populations Iron deficiency is a microcytic, hypochromic anemia, meaning the RBC’s are small and pale. RBCs w/ decreased iron appear bleached out B/c the cells are small, the Mean corpuscular volume (MCV), the Mean corpuscular hemoglobin (MCH) and the Mean corpuscular hemoglobin concentrations are low. Serum iron levels are decreased

16 Iron deficiency anemia lab values Hemoglobin Hematocrit Reticulocyte count= Hemoglobin 9.5-11 g/dL= Mild iron deficiency Hemoglobin 8-9.4 g/dL= Moderate iron deficiency anemia Hemoglobin less than 8 g/dL= severe iron deficiency anemia

17 Iron Deficiency Anemia So what is the greatest nutritional risk factor for developing iron deficiency anemia?

18 Iron Deficiency Anemia Complications Untreated, anemia can cause stress on all body tissues, w/ decreased oxygenation, especially respiratory and cardiovascular systems Decreased ability to concentrate Poor muscle development Decreased performance on developmental tests

19 Iron Deficiency Anemia Treatment The AAP recommends if Hct less than 34% or Hgb less than 11.3 g/dL begin iron supplementation Main treatment: –Treat underlying problem GI bleeding, chronic blood loss Lack of iron from diet –Iron Supplementation ORAL ferrous sulfate at 3-6 mg/kg/day for 4 weeks, then repeat Hgb/Hct –Administer through a straw, nipple –Administer on empty stomach –Administer with source of vitamin C

20 Iron Deficiency Anemia Iron Rich Foods –Iron fortified cereal and formula –Enriched bread –Dark green vegetables –Legumes (kidney and pinto beans) –Figs, raisins –Meats, fish, poultry –Dried appricots

21 Iron Deficiency Anemia Evaluation With tx, reticulocyte count increases w/in 3-5 days. Indicates + therapeutic response Hgb should normalize w/in 4-8 weeks When lab values are nml, wean from iron supplements Repeat labs in 6 months, monitor wt/ development

22 Sickle Cell Anemia

23 Sickle cell disease Sickle cell anemia (SS) is an inherited, autosomal recessive genetic disease that affects the RBC’s, which become acutely sickle-shaped. Involves RBCs and their ability to carry oxygen Pathophysiology of the disease –Results from a single amino acid substitution (valine for glutamine) in position 6 of the beta globin chain of hemoglobin –What does this mean? unstable RBC w/ a shortened survival that under stress becomes sickle shaped

24 Sickle cell disease How is the individual affected? –Short Hgb life span Chronically anemic –Sickle cells risk being destroyed by the spleen…? Implications Damage to the spleen w/o normal functioning spleen at risk for infections

25 Sickle cell disease Age: –Hematologic changes evident as early as 10 weeks, though usually delayed until age 6-12 months--? Why do you think this is –Beta-chain (adult) hemoglobin is not prominent until the age of 3 months

26 Difference in Hgb Normal Hgb cells Live for 120 days Round Smooth Flexible, like a letter “o” so they can move through vessels easily Sickle Hgb cells Live for about 15 days Stiff Sticky Form into the shape of a sicle, or the letter “C”, when they loose Oxygen Cluster together…what would this lead to in the body?

27 FIGURE 28–5 The clinical manifestations of sickle cell anemia result from pathologic changes to structures and systems throughout the body. Jane W. Ball and Ruth C. Bindler Child Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc. Upper Saddle River, New Jersey 07458 All rights reserved.

28 Sickle Cell Anemia Sickling Triggered by fever, emotional stress, physical stress States of hypoxia –High altitudes –Hypoventilation –Poorly pressurized aircrafts Dehydration Cold

29 Sickle Cell Crisis SS crisis are acute exacerbations of the disease Vary in severity and frequency Three most common types –Vaso-occlusive crisis –Sequestration crisis –Aplastic crisis

30 Vaso-occlusive Crisis “Pain Crisis” Aka thrombotic crisis Most common type of crisis Precipitated by dehydration, exposure to cold, acidosis or localized hypoxemia Extremely painful Caused by stasis of blood w/ clumping of cells in the microciruclation, ischemia and infarction Thrombosis and infarction of tissue may occur if crisis not reversed Clinical manifestations include fever, pain, tissue engorgement, swelling of joints, hands and feet, priapism and severe abdominal pain

31 Splenic Sequestration Life-threatening crisis: death can occur w/in hours; high mortality (up to 50%) Caused by pooling of blood in the spleen Spleen can hold up to 1/5 th of body’s blood supply at one time—leads to CV collapse Clinical manifestations include profound anemia, hypovolemia and shock Occurs b/t 4 months-3 years Tx- blood transfusions, emergent splenectomy

32 Aplastic Crisis Diminished erythropoiesis and increased destruction of RBCs –(bone marrow depression resulting from a viral infection) Triggered by viral infection or depletion of folic acid Clinical manifestations include profound anemia, pallor, fatigue

33 Acute Chest Syndrome This is similar to pneumonia, with symptoms such as difficulty breathing, chest pain and fever. It can be caused by an infection or by blocked blood vessels in the lung. This potentially life-threatening disorder should be treated in the hospital. Treatments may include antibiotics, blood transfusions, pain medications, oxygen and medicines that help open up blood vessels and improve breathing.

34 Acute chest syndrome The acute chest syndrome (ACS) in sickle cell disease (SCD) can be defined as: –a new infiltrate on chest x-ray –associated with one or more NEW symptoms: fever, cough, sputum production, dyspnea, or hypoxia.. A past history of an ACS is associated with early mortality compared to those who have never had an episode. The disorder is most common in the 2 to 4 year age group and gradually declines in incidence with age.

35 Nursing Dx: Pain r/t sickling of RBCs Pain can occur in any organ or joint in the body Pain can be reversed –Oxygenation –Hydration –Pain Management –Rest Mild pain episodes can be treated w/ OTC pain meds (tylenol, ibuprofen) and heating pads More severe episodes require hospitalization and IV pain meds Hydroxyurea –1998 FDA approved this drug for use in tx of SCD

36 Nursing Dx: Risk for Infection Infants and young children w/ SCD are especially vulnerable to serious bacterial infections Major cause of death in children w/ SCD Daily prophylactic Pen VK 125 mg BID from 2 months- 5 years of age Erythromycin for children w/ PCN allergies

37 Nursing Dx: Risk for Infection Important to receive regular childhood vaccinations (Hib and PCV 7) –In addition children w/ SCD should also receive a yearly flu shot (influenza) beginning at 6 mos of age –Another type of pneumoccocal vaccine (PCV 23)—protects against additional bacteria b/t 2- 5 years of age –Meningococcal vaccine (protects against meningitis at age 5 and beyond)

38 Treatment for Sickle Cell Anemia Treatment consists of sx management The primary focus being on prevention of sickle cell crisis –Education Blood transfusions Hydration: Drinking plenty of water daily –(8 to 10 glasses) or receiving fluid intravenously (to prevent and treat pain crises) Pain Management

39 Medications for SS anemia Hydroxyurea 15-20 mg/kg/day to start and increase until therapeutic response (not more than 35 mg/kg) –A chemotherapeutic drug used in CA tx –Shown to decrease the number and severity of crises –Increases production of Hemoglobin F –Side effects include bone marrow supression, HA’s dizziness, N/V

40 Clotting Disorders Hemophilia A (Factor VIII deficiency) Von Willerbrand Disease Disseminated Intravascular Coagulation (DIC) Idiopathic Thrombocytopenic Purpura (ITP)

41 Hemophilia A Hereditary bleeding disorder, that result from deficiency of specific clotting factors –Hemophilia A aka Factor VIII is most common type –80% of people w/ hemophilia X-linked recessive traits, which manifests as affected males, and carrier females 30% of cases are new mutations Range of manifestations of disease from mild to severe

42 Hemophilia A Clinical Manifestations Children usually do not manifest sxs until after 6 months of age (begin moving around, loosing teeth) Spontaneous bleeding Hemarthrosis (bleeding into joint space) Deep tissue hemorrhage Nosebleeds Easy bruising (ecchymosis) Hematuria Life-threatening bleeding includes: –Head/ intracranial –Neck and throat –Abdominal/GI –Iliopsoas muscle with decrease hip ROM

43 Hemophilia A Complications from bleeding include: –Bone changes –Contractures –Disabling deformities result from immobility and from bleeding into joint spaces –Muscle contractures –Joint arthritis –Chronic pain –Muscle atrophy –Compartment syndrome –Neurologic impairment

44 Hemophilia A Treatment: –Goal to control bleeding by replacing the missing clotting factor and prevent complications Factor Replacement Therapy –On demand –Prophyllaxis IV infusions consist of –Fresh frozen plasma –Cryoprecipitate –Factor VIII

45 Treatment of Hemophilia A Prophylaxis: –Scheduled infusions of factor 2-3 X/ week DDAVP (Desmopressin acetate) –An analog of vasopressin, causes a 2-4 fold increase in factor VIII –Not to be confused w/ DDAVP for nocturnal enuresis –Synthetic vasopressin –MOA: release of stores from endothelial cells raising factor VIII and vWD serum levels –Administered IV, sub-Q or nasally (stimate)

46 Treatment of Hemophilia A Amicar (epsilon amino caproic acid) Antifibrinolytic Uses: –Mucocutaneous bleeding –50-100 mg/kg q 6 hours Contraindications: –hematuria

47 Hemophilia A Complications of Treatment: –Inhibitors/antibody development IgG antibody to infused factor VIII concentrates which occurs after exposure to the extraneous VIII protein 20-30% of pts w/ severe hemophilia A –Blood-borne illnesses Hep A,B and C HIV

48 Hemophilia A Nursing Considerations Factor replacement given on time Lab monitoring as ordered Increase metabolic states will increase factor requirements Factor coverage for invasive procedures Document- infusion and response to tx NO NSAIDS NO HEAT NO IM injections Utilize Hemophilia Center staff for ???s

49 Hemophilia A Nursing Considerations Avoid taking temperatures rectally or giving suppositories Check Bp by cuff as little as possible Avoid IM or subcutaneous injections Use only paper or silk tape for dressings Perform mouth care w/ glycerin swab Limit venipunctures Do not give aspirin

50 Hemophilia A Psychosocial Issues Guilt Challenge of hospitalizations Control issues Financial/ insurance challenges Feeling different/ unable to do certain activities Counseling needs Refer for genetic counseling after dx

51 Von Willebrand Disease A hereditary bleeding disorder vWF Involved w/ platelet adhesion Most common form of disorder is autosomal dominant trait Disease can occur in both males and females equally Manifestations: –Easy bruising –Epistaxis

52 Von Willebrand Disease Other clinical manifestations include: –Gingival bleeding –Ecchymosis –Increased bleeding w/ lacerations or during surgery and dental extractions –Menorrhagia (increased menstrual bleeding) –GI bleeding

53 Von Willebrand Disease Treatment: –Similar to Hemophila A –Restore clotting factor and prevent complications associated w/ bleeding Infusion of vWB protein concentrate DDAVP Amicar for mucous membrane bleeding Nursing Management: –Similar to Hemophilia A

54 Disseminated Intravascular Coagulation (DIC) Life-threatening process which occurs as complication of other serious illnesses in infants and children Most common cause of DIC is infection An acquired pathologic process in which the clotting system is abnormally activated, resulting in widespread clot formation in the small vessels throughout the body. These changes slow blood circulation, cause tissue hypoxia and results in tissue necrosis. The circulating fibrin also interfere w/ clotting process and bleeding and hemmorrhage result

55 DIC The sequence of events for DIC –– please see p 1027 Clinical Manifestations: –see chart on page 1028 Treatment: –Controlling bleeding, identifying and correcting the primary cause of the disorder, and preventing further activations of clotting mechanisms

56 DIC Nursing Assessment and Diagnosis: –Involves all body systems, so frequent thorough assessment of entire body is critical –Observe for petechiae, ecchymoses, and oozing every 1-2 hours –Observe for pooling of blood in dependent areas –Assess IV site Q 15 minutes for oozing –Examine stool for presence of blood –Assess extremities for cap refill, warmth and pulses –Frequently assess VS and LOC –I’s and O’s –Monitor O2 sat and ABG’s –ID family’s coping strategies and support systems

57 Idiopathic Thrombocytopenic Purpura (ITP) Aka autoimmune thrombocytopenic purpura Most common bleeding disorder in children –Occurs in children 2-10 years-old, peaks b/t 2-5 y.o A disorder characterized by increased destruction of platelets in the spleen, even though plt production in the bone marrow is normal Autoimmune –Plts are destroyed as a result of the binding of autoantibodies to PLT antigens After a viral illness

58 ITP Clinical Manifestations: –Multiple ecchymoses –Petechiae –Purpura (purplish areas where blood has collected d/t bleeding from blood vessels) –Bleeding from gums –nosebleeds –Hematuria –heme in stools

59 ITP Dx is made by Hx and PE and lab findings Tx: depends on PLT counts and clinical presentation –Corticosteroids –IVIG –PLT administration only if hemorrhage occurs –If no response to therapy in 6mos-1 year, splenectomy may be tx of choice –Spontaneous remission in 90% of cases

60 Nursing Care of the Child with a Hematologic Disorder Based on the Disorder RBC’s –Oxygenation –Circulation –Fluid –Nutrition –Pain Management

61 Nursing Care of a Child with a Hematologic Disorder Based on the Disorder WBC’s –Infection –Oxygenation –Nutrition Platelets and bleeding disorders –Bleeding –Oxygenation –Circulation –Injury Prevention

62 Collaborative Care for a Child with a Hematologic Disorder Team Approach Family Involved –Decisions w/ family and child

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