Child Health Nursing

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

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

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

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 Anemialab 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 anemialab 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

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

Iron Deficiency AnemiaComplications • 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 AnemiaTreatment • 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

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 AnemiaEvaluation • 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

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 cell disease

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

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? Difference in Hgb

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. BindlerChild Health Nursing: Partnering with Children & Families © 2006 by Pearson Education, Inc.Upper Saddle River, New Jersey 07458All rights reserved.

Sickle Cell AnemiaSickling • 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.

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

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)

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

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 AClinical 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: • 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 ANursing 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 APsychosocial 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

Child Health Nursing