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

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

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.

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.

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 In infants and young children, all bones contain red bone marrow. And are therefore, capable of hematopoiesis. However, as the child approaches adolescence and bone growth stops, the bone marrow in many bones can’t form blood cells because the marrow has transformed to yellow bone marrow (fat deposits), although it can usually revert to red bone marrow during times of increased blood cell demand. Only the ribs, sternum, vertebra and pelvis continue to contain red bone marrow and produce blood cells.

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

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 Bilirubin binds with albumin for transport to the liver cells to conjugate with glucuronide, forming direct bilirubin. Because unconjugated bili is fat-soluble and can’t be excreted in urine or bile, it may escape to extravascular tissue, especially fatty tissue and the brain, restulting in hyperbilirubinemia O2 Is carried in the cell in a protein (globin) and iron (heme) structure known as hemoglobin. If adequate amounts of Iron aren’t available, the protein structure can’t be formed and RBCs cant carry their normal amount of oxygen.

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

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

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

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 2nd year of life

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

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

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

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 Reticulocyte count= helps distinguish a hypoproductive anemia from a destructive process decrease= indicates bone marrow disorders or aplastic crisis increase= indicates hemolytic process or active blood loss

Iron Deficiency Anemia So what is the greatest nutritional risk factor for developing iron deficiency anemia? Intro of cow’s milk in first year of life

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

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 SES of iron administration include- black tarry stools Constipation GI discomfort Foul aftertaste

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

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

Sickle Cell Anemia

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?...an unstable RBC w/ a shortened survival that under stress becomes sickle shaped Background: Sickle cell disease is an inherited disorder of hemoglobin synthesis. The resulting abnormality produces a normocytic, hemolytic anemia with multiple diversely shaped RBCs that are susceptible to morphologically changing into a sickle shape. The sickle cells produce thrombosis and obstruction in small vessels, leading to ischemia and necrosis of distal tissue. Pathophysiology: Sickle cell disease results from a single amino acid substitution (valine for glutamate) in position 6 of the beta-globin chain of hemoglobin. This genetic alteration yields an unstable RBC with a shortened survival that under stress becomes sickle shaped.

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 Sickle cells only live for about 15 days, while normal hemoglobin can live up to 120 days. Also, sickle cells risk being destroyed by the spleen because of their shape and stiffness. The spleen is an organ that helps filter the blood of infections and sickled cells get stuck in this filter and die. Due to the decreased number of hemoglobin cells circulating in the body, a person with sickle cell disease is chronically anemic. The spleen also suffers damage from the sickled cells blocking healthy oxygen carrying cells. Without a normal functioning spleen, these individuals are more at risk for infections. Infants and young children are at risk for life-threatening infections.

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 Age: Hematologic changes indicative of the disorder are evident as early as the age of 10 weeks, though symptoms are usually delayed until the age of 6-12 months….why do you think this is?? because of high levels of circulating fetal hemoglobin. Beta-chain (adult) hemoglobulin is usually not prominent until the age of 3 months. After infancy, erythrocytes of patients with sickle cell anemia contain approximately 90% hemoglobin S (HbS), 2-10% hemoglobin F (HbF), a normal amount of minor fraction of adult hemoglobin (HbA2), and no hemoglobin A (HbA).

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? Sickle cell disease involves the red blood cells, or hemoglobin, and their ability to carry oxygen. Normal hemoglobin cells are smooth, round, and flexible, like the letter "O," so they can move through the vessels in our bodies easily. Sickle cell hemoglobin cells are stiff and sticky, and form into the shape of a sickle, or the letter "C," when they lose their oxygen. These sickle cells tend to cluster together, and cannot easily move through the blood vessels. The cluster causes a blockage and stops the movement of healthy, normal oxygen-carrying blood. This blockage is what causes the painful and damaging complications of sickle cell disease.

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.

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

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

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

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 1/5th 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

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

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.

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. ACUTE CHEST SYNDROME - when sickling is in the chest. This can be a life-threatening complication of sickle cell disease. It often occurs suddenly, when the body is under stress from infection, fever, or dehydration. The sickled cells stick together and block the flow of oxygen in the tiny vessels in the lungs. It resembles pneumonia and can include fever, pain, and a violent cough. Multiple episodes of acute chest syndrome can cause permanent lung damage. 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. Recent data from the Clinical Course of Sickle Cell Disease Cooperative Study indicate that this complication occurs with an incidence of 10,500/100,000 patients/year(2). ACS occurs most often as a single episode, but certain patients have multiple episodes. 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. It is believed that Hb F exerts a protective effect on those less than 2 years of age. Furthermore, the decline observed in older age groups is partially related to at least two other factors: (a) excess mortality in the group which had an ACS, and (b) fewer viral episodes in adults due to acquired immunity.

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 Pain episodes. This is the most common symptom of sickle cell disease. Some affected individuals have one or fewer pain episodes a year, while others may have 15 or more.1,2 Pain episodes usually last a few hours to a few days, but they may sometimes last for weeks. Pain can occur in any organ or joint in the body, wherever sickle-shaped cells pile up and block blood vessels. Mild pain episodes can be treated at home with over-the-counter pain medications (such as acetaminophen and ibuprofen) and heating pads. But some pain episodes may be severe and need to be treated in the hospital with strong pain-killing drugs given intravenously (in a vein).   Until recently, there was no effective treatment to prevent the sickling that causes a pain crisis other than blood transfusions. A 1995 study reported that treatment with a drug called hydroxyurea reduced the number of pain episodes in some of the severely affected adults by about 50 percent.3 In 1998, the Food and Drug Administration (FDA) approved the use of this drug in patients over 18 years of age who have had at least three painful episodes in the previous year. A 2003 study that followed the same patients for nine years also found that treatment with hydroxyurea reduced deaths by 40 percent.4 Several smaller studies have found that hydroxyurea is effective and well tolerated in children in the short term. However, the drug is not yet routinely recommended in children because it is not known whether it has any adverse effects on growth and development. Researchers continue to study the long-term safety of the drug in children.

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

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) It is important for babies and children with sickle cell disease to receive regular childhood vaccinations. The Hib (Hemophilus influenzae b) vaccine and the pneumococcal vaccine (Prevnar) help protect against potentially life-threatening bacterial infections. These vaccines are recommended for all babies starting at 2 months of age. Children with sickle cell disease should receive additional vaccinations. These include a yearly flu (influenza) shot, beginning at 6 months of age; another type of pneumococcal vaccine (23-valent pneumococcal vaccine), which protects against additional types of bacteria at 2 and 5 years of age; and the meningococcal vaccine, which protects against meningitis, at age 5.1 

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 hydroxyurea (a medication recently developed that may help reduce the frequency of pain crises and acute chest syndrome; it may also help decrease the need for frequent blood transfusions. The long-term effects of the medication are unknown.)

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

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

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

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

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

Hemophilia A Treatment: Factor Replacement Therapy 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

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)

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

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

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

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

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

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

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 Children w/ wWD usually do not have hemarthrosis

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

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

DIC The sequence of events for DIC Clinical Manifestations: Treatment: – 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

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

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

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

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

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

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

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