Alterations in Hematologic Function

2006 Pearson Education, Inc. Pearson Prentice Hall

Upper Saddle River, NJ 07458

Structure and Function of Blood Components

Review of Hematologic System Blood formation Red Blood Cells White Blood Cells Platelets

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, is a component that 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 its fluid, blood is one of the bodys 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. Formation of blood cells begins as early as week 2 of intrauterine life. By month 2 of intrauterine life liver and spleen begin forming blood components. After birth, the red bone marrow becomes the main site of hematopoiesis. Total blood volume upon BIRTH is roughly 85 ml/kg. 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

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. Approximately 5 million RBCs per cubic millimeter of blood at birth. Life of RBC= 120 days An important waste product of RBC death is bilirubin

RBC has its components that allows to carry out

and transport oxygen, it is called HEMOGLOBIN. Two Components of Hemoglobin: A.HEME- an iron- containing pigment, it is the portion that combines with oxygen and carbon dioxide for transport. B.GLOBIN- protein dependent on nitrogen metabolism for its formation. At birth 40 70 % of the child hemoglobin is composed of FETAL HEMOGLOBIN. (hemoglobin F) Fetal hemoglobin is replaced by adult hemoglobin (hemoglobin A) during the first 6 months of life. Hemoglobin levels are highest at birth (13.7- 20.1 g/100 ml)

White Blood Cells

Fight different types of infection in our

body; each type has its own role 2 main categories of WBCs

Granular leukocytes (granulocytes) Neutrophils- 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

Total WBC count in newborns is

approximately 20, 000 per cubic millimeter. By 14- 30 days of life, the total WBC count falls to approximately 12, 000 per cubic millimeter.

Platelets
It is a round, non-nucleated bodies formed by

bone marrow which major functions is to maintain capillary hemostasis and primary coagulation Adhere to one another and plug holes in vessels or tissues where theres 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

Care for a Child with Hematologic disorder

Assessing the child with a Hematologic disorder: History Chief concern: fatigue, easy bruising, epistaxis. Pregnancy Hx.: Low birth weight, blood loss at birth, lack of vitamin K administration at birth. Nutrition: Picky eater, or presence of pica. Increased milk intake. Past illnesses: History of recent illness, history of recent medicine ingestion. Family Hx.: Inherited blood disorders, parents known to have sickle- cell trait, thalassemia minor, or hemophilia in family.

Physical Assessment
General Appearance: Obese infant, fatigue Eyes: Retinal hemorrhage Face: Bossing of maxillary bone Mouth: Pale, mucous membrane, ecchymotic or

bleeding gum line. Heart: Increased rate, possible murmur Skin: Petechiae, ecchymosis, blood oozing from wound or injection point, jaundice, pallor, bronze color. Abdomen: pain on palpation, increased liver or spleen size Genitourinary: delayed secondary sex characteristics Extremities: Spoon nails, joint swelling, pain

Disorder of Red Blood Cell

ANEMIA- occurs when there is reduction in the

number or function of erythrocytes. Also it happens when the rate of RBC production falls below that of cell destruction. POLYCYTHEMIA- over production of RBC in the system.

TYPES OF ANEMIA
NORMOCHROMIC, NORMOCYTIC ANEMIAS ACUTE BLOOD- LOSS ANEMIA ANEMIA OF ACUTE INFECTION ANEMIA OF RENAL DISEASE ANEMIA OF NEOPLASTIC DISEASE APLASTIC ANEMIA HYPOPLASTIC ANEMIA HYPOCHROMIC ANEMIA IRON- DEFICIENCY ANEMIA MACROCYTIC (MEGALOBLASTIC) ANEMIA ANEMIA OF FOLIC ACID DEFICIENCY PERNICIOUS ANEMIA( VITAMIN S2 DEFICIENCY) HEMOLYTIC ANEMIA AUTOIMMUNE ACQUIRED ANEMIA

NORMOCHROMATIC

NORMOCYTIC ANEMIA- marked by impaired production of erythrocytes by the bone marrow, or by abnormal or uncompensated loss of circulating RBCs such as in acute hemorrhage. ACUTE BLOOD LOSS ANEMIA- cause from a trauma thus causing blood loss decreasing the number of RBC in the circulation, trauma such as: automobile accident with internal bleeding, acute nephritis with severe hematuria, or a newborn born from a pregnancy disorder such as placenta previa, abruptio placenta , trauma to cord or placenta. Tx.: control bleeding by addressing the underlying cause., place in supine position, keep warm, BT, plasma expanders (plasma or

ANEMIA OF ACUTE INFECTION Acute infection or inflammation, especially in infants may lead to increased destruction of erythrocytes and therefore decreases erythrocyte level in circulation. Common causes: Osteomyelitis Ulcerative colitis Advanced renal disease Mgt. Treating the underlying condition will increase the number of erythrocytes that leads correct the anemia. Antibiotic therapy

ANEMIA OF RENAL DISEASE Due to the dysfunction of kidney cells that decreases the erythropoietin production thus decreases also the total number of RBCs in the system causing anemia. COMMON CAUSES: Irritation of nephrons Autoimmune disorder Mgt. Administration of recombinant human erythropoietin.

ANEMIA OF NEOPLASTIC DISEASE It occurs when a neoplastic cells proliferate inside the bone marrow that causing impairment of the RBC production, it forms a normochromic normoctic anemia. Mgt. Remission of neoplastic process. Blood transfusion APLASTIC ANEMIA Results from depression of hematopoietic activity in the bone marrow thus affecting the development of WBC, platelets and RBCs.

A.Congenital Aplastic Anemia (Fanconis Syndrome) Is an inherited autosomal recessive trait. Manifestation: Children who are having skeletal and renal abnormalities. Hypogenitalism And short stature Usually happens between ages 4- 12 years of age. The child begins manifesting symptoms of PANCYTOPENIA- reduction of all blood cell components. Tx: Bone marrow transplantation

B. ACQUIRED APLASTIC ANEMIA Is a decrease in bone marrow production that occurs during a child has excessive exposure to radiation, drugs, chemicals that are known to cause bone marrow damage. CONTRIBUTING DRUGS : chloramphenicol, sulfonamides, arsenic(common in rat poison), hydatoin, benzene or quinine., chemotherapeutic drugs. S/Sx: pallor, easy fatigability, anorexia, thrombocytopenia ( bleeding), increase in WBC count (Leukopenia).petechiae, easy bruising.

Treatment: Administration of Antithymocyte globulin (ATG) and Cyclosporine testosterone that stimulates RBC growth. Reminders: ATG should be given cautiously because it is high risk to cause anaphylaxis. Transfusion of new blood elements. (packed RBC and platelets) Erythropoietin administration Colony stimulating factor- helps improved bone marrow functioning. Oral corticosteriods ( Prednisone) Stem cell transplantation.

HYPOPLASTIC ANEMIA Also results from depression of hematopoietic activity of bone marrow; they can be congenital or acquired. Unlike APLASTIC anemia, it only affects RBCs. Types of Hypoplastic anemia: A. Congenital aka BLACKFAN-DIAMOND syndrome Rare disorder revealed in 6-8 months of life which Tx,: corticosteriod usually caused by inherited defect in RBC formation. B. Acquired-usually caused by infection with

Long term transfusion of PRBCs needed to raise

erythrocyte levels thus necessary to increase also times of transfusion that causes deposition of iron tissue in the body called HEMOSIDEROSIS. Tx. For Hemosiderosis: IRON CHELATION PROGRAM Using a subcutaneous infusion of Hypodermoclysis Such as DEFEROXAMINE (Desferal)- binds with iron and aids on its excretion from the body in urine form. Given for 5 to 6 days a week over an 8 hour period. Common site for infusion is on area beside scapula or on the thigh.

Iron Deficiency Anemia

A disorder of O2 transport in which the

production of hgb is inadequate. Without sufficient iron, the body cant produce the Hgb molecure, b/c the heme component is primarily iron

Iron Deficiency Anemia

CAUSES: 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

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:
Cows milk allergy (common in Blacks and

Asians) causes inflammation of GI tract In adolescents- fad diets

Iron Deficiency Anemia

CLINICAL MANIFESTATION:
Range from mild to severe Pale appearance and decreased activity Toddlers may have h/o prematurity and poor

weight gain

Other Sx 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 RBCs are small and pale. RBCs w/ decreased iron appear bleached out 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

Iron Deficiency Anemia

So what is the greatest nutritional risk factor

for developing iron deficiency anemia?

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 Association of American Pediatrics

recommends if Hct less than 34% or Hgb less than 11.3 g/dL begin iron supplementation(4-6 weeks) 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 4-6 weeks, then repeat Hgb/Hct
Administer through a straw, nipple Administer on empty stomach

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 RBCs, 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

Sickle cell disease

Sickle cells only live for about 15 days, while normal hemoglobin can live up to 120 days. How is the individual affected? Short Hgb life span Chronically anemic Sickle cells risk being destroyed by the spleen Implications Damage to the spleen

Sickle cell disease

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

Difference in Hgb
Normal Hgb cells Live for 120 days Round Smooth Flexible, like a letter Sickle Hgb cells Live for about 15 days Stiff Sticky Form into the shape of

o so they can move through vessels easily

a sicle, or the letter C, when they loose Oxygen Cluster togetherwhat would this lead to in 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,

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 bodys blood supply at one timeleads 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

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, HAs 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

type 80% of people w/ hemophilia

Hemophilia A aka Factor VIII is most common

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:
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. Administered IV, sub-Q or nasally

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 .

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

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

DIC
The sequence of events for DIC Clinical Manifestations: 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 Is and Os Monitor O2 sat and ABGs ID familys coping strategies and support systems

Idiopathic Thrombocytopenic Purpura (ITP)

Aka autoimmune thrombocytopenic

purpura Most common bleeding disorder in children

y.o

Occurs in children 2-10 years-old, peaks b/t 2-5

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

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
IVIG

Corticosteroids 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 RBCs
Oxygenation Circulation Fluid Nutrition Pain Management

Nursing Care of a Child with a Hematologic Disorder

Based on the Disorder WBCs
Infection Oxygenation Nutrition

Platelets and bleeding disorders

Bleeding Oxygenation Circulation Injury Prevention

Collaborative Care for a Child with a Hematologic Disorder

Team Approach Family Involved
Decisions w/ family and child