Transcript Child Health Nursing - Salem State University

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
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 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
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 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 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
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 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
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?
manifestations of sickle cell anemia result from pathologic changes to
structures and systems throughout the body.
FIGURE 28–5
The clinical
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.
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 25 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 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 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
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
– – 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
Oxygenation
Circulation
Injury Prevention
Collaborative Care for a Child with
a Hematologic Disorder
• Team Approach
• Family Involved
– Decisions w/ family and child