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The Child with Hematologic or
Immunologic Dysfunction
Chapter 35
Assessment of
Hematologic Function




Complete blood count
History and assessment findings
Child’s energy and activity level
Growth patterns
Mosby items and derived items © 2007, 2003 by Mosby, Inc., an affiliate of Elsevier Inc.
Slide 2
Anemia



The most common hematologic disorder of
childhood
Decrease in number of RBCs and/or
hemoglobin concentration below normal
Decreased oxygen-carrying capacity of blood
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Slide 3
Classification of Anemias

Etiology and physiology


RBC and/or Hgb depletion
Morphology

Characteristic changes in RBC size, shape, and/or
color
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Slide 4
Consequences of Anemia


Decrease in oxygen-carrying capacity of
blood and decreased amount of oxygen
available to tissues
When anemia develops slowly, child adapts
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Slide 5
Effects of Anemia on
Circulatory System


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Hemodilution
Decreased peripheral resistance
Increased cardiac circulation and turbulence

May have murmur
 May lead to cardiac failure


Cyanosis
Growth retardation
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Slide 6
Diagnostic Evaluation

CBC



Decreased RBCs
Decreased Hbg and Hct
Other tests for particular type of anemia
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Slide 7
Therapeutic Management

Treat underlying cause



Transfusion after hemorrhage if needed
Nutritional intervention for deficiency anemias
Supportive care



IV fluids to replace intravascular volume
Oxygen
Bed rest
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Slide 8
Nursing Considerations
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


Prepare child and family for laboratory tests
Decrease oxygen demands
Prevent complications
Support family
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Slide 9
Blood Transfusion Therapy
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

Verify identity of recipient and donor’s blood
group
Monitor VS
Use appropriate filter
Use blood within 30 minutes of arrival
Infuse over 4 hours maximum
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Slide 10
Transfusion Reactions
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
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Hemolytic: the most severe, but rare
Febrile reactions: fever, chills
Allergic reactions: urticaria, pruritus, laryngeal
edema
Air emboli: may occur when blood is
transfused under pressure
Hypothermia
Electrolyte disturbances: hyperkalemia from
massive transfusions or with renal problems
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Slide 11
Nursing Responsibilities
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


Identify donor and recipient blood types and
groups before transfusing
Transfuse slowly for first 15 to 20 minutes
Observe carefully for patient response
Stop transfusion immediately if signs or
symptoms of transfusion reaction; notify
practitioner
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Slide 12
Delayed Reactions to
Blood Transfusion

Transmission of infection



Hepatitis, HIV, malaria, syphilis, other
Blood banks test vigorously and discard units of
infected blood
Delayed hemolytic reaction


Destruction of RBCs and fever 5 to 10 days after
transfusion
Observe for posttransfusion anemia
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Slide 13
Family Concerns

Difficult decision for HTSC transplant


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Child faces death without the transplant
Preparing the child for transplant places the child
at great risk
No “rescue” procedure if complications follow
HTSC transplants
Nursing considerations
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Slide 14
Apheresis


Definition: the removal of blood from an
individual; separation of the blood into its
components
Nursing considerations
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Slide 15
Iron Deficiency Anemia


Caused by inadequate supply of dietary iron
Generally is preventable

Iron-fortified cereals and formulas for infants
 Special needs of premature infants
 Adolescents at risk due to rapid growth and poor
eating habits
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Slide 16
Iron Deficiency Anemia (cont.)




Pathophysiology
Therapeutic management
Prognosis
Nursing considerations
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Slide 17
Sickle Cell Anemia


A hereditary hemoglobinopathy
Ethnicity

Occurs primarily in African-Americans
• Occurrence 1 in 375 infants born in U.S.
• 1 in 12 have sickle cell trait
• Occasionally also in people of Mediterranean descent
• Also seen in South American, Arabian, and East Indian
descent
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Slide 18
Etiology of Sickle Cell


In areas of the world where malaria is
common, individuals with sickle cell trait tend
to have a survival advantage over those
without the trait
Autosomal recessive disorder


1 in 12 African-Americans are carriers (have sickle
cell trait)
If both parents have trait, each offspring will have
1 in 4 likelihood of having disease
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Slide 19
Pathophysiology

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Partial or complete replacement of normal
Hgb with abnormal hemoglobin S (HgbS)
Hemoglobin in the RBCs takes on an
elongated (“sickle”) shape
Sickled cells are rigid and obstruct capillary
blood flow
Microscopic obstructions lead to
engorgement and tissue ischemia
Hypoxia occurs and causes sickling
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Slide 20
Sickled Hemoglobin
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Slide 21
Pathophysiology


Large tissue infarctions occur
Damaged tissues in organs lead to impaired
function

Splenic sequestration
• May require splenectomy at early age
• Results in decreased immunity
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Slide 22
Prognosis



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No cure (except possibly bone marrow
transplants)
Supportive care/prevent sickling episodes
Frequent bacterial infections may occur due
to immunocompromise
Bacterial infection is leading cause of death in
young children with sickle cell disease
Strokes in 5% to 10% of children with disease

Result in neurodevelopmental delay, mental
retardation
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Slide 23
Sickle Cell Crisis

Precipitating factors

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Anything that increases the body’s need for
oxygen or alters transport of oxygen
Trauma
Infection, fever
Physical and emotional stress
Increased blood viscosity due to dehydration
Hypoxia
• From high altitude, poorly pressurized airplanes,
hypoventilation, vasoconstriction due to hypothermia
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Slide 24
Sickle Cell Crisis (cont.)


Acute exacerbations that vary in severity and
frequency
Types
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Vaso-occlusive (VOC) thrombotic
• Most common type of crisis—very painful
• Stasis of blood with clumping of cells in microcirculation
→ ischemia → infarction
• Signs: fever, pain, tissue engorgement
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Slide 25
Sickle Cell Crisis (cont.)

Types (cont.)
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Splenic sequestration
• Life threatening—death can occur within hours
• Blood pools in the spleen
• Signs:

Profound anemia, hypovolemia, shock
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Slide 26
Sickle Cell Crisis (cont.)

Types (cont.)
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Aplastic crises
• Diminished production and increased destruction of
RBCs
• Triggered by viral infection or depletion of folic acid
• Signs include profound anemia, pallor
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Slide 27
Diagnosis of Sickle Cell



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Cord blood in newborns
Newborn screening done in 43 states
Genetic testing to identify carriers and
children who have disease
Sickle turbidity test

Quick screening purposes in children older than 6
months
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Slide 28
Medical Management


Aggressive treatment of infection
Possibly prophylactic antibiotics from
2 months to 5 years
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Slide 29
Medical Management (cont.)


Monitor reticulocyte count regularly to
evaluate bone marrow function
Blood transfusion, if given early in crisis, may
reduce ischemia
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Slide 30
Medical Management (cont.)

Frequent transfusion leads to hemosiderosis
(iron in tissues)


Treat with iron-chelation such as feroxamine +
vitamin C to promote iron excretion
Rx—hydronurea (cytotoxic) leads to
decreased production of abnormal blood cells
and less pain
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Slide 31
Nursing Management





Monitor child’s growth—watch for failure to
thrive
Careful multisystem assessment
Assess pain
Observe for presence of inflammation or
possible infection
Carefully monitor for signs of shock
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Slide 32
Sickle Cell Pain
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Slide 33
Psychosocial Needs





Coping mechanisms
Support with genetic counseling
Financial needs?
Caregiver role strain
Living with chronic illness in the family
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Slide 34
Thalassemia



Inherited blood disorders of hemoglobin
synthesis
Classified by Hgb chain affected and by
amount of effect
Autosomal recessive with varying expressivity

Both parents must be carriers to have offspring
with disease
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Slide 35
Pathophysiology


Anemia results from defective synthesis of
Hgb, structurally impaired RBCs, and
shortened life of RBCs
Chronic hypoxia


Headache, irritability, precordial and bone pain,
exercise intolerance, anorexia, epistaxis
Detected in infancy or toddlerhood

Pallor, FTT, hepatosplenomegaly, severe anemia
(Hgb <6)
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Slide 36
Thalassemia

-Thalassemia
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

α chains affected
Occurs in Chinese, Thai, African, and
Mediterranean people
-Thalassemia


Occurs in Greeks, Italians, and Syrians
 is most common and has four forms
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Slide 37
-Thalassemia

Four types

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

Thalassemia minor: asymptomatic silent carrier
Thalassemia trait: mild microcytic anemia
Thalassemia intermediate: moderate to severe
anemia + splenomegaly
Thalassemia major “Cooley anemia”: severe
anemia requiring transfusions to survive
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Slide 38
Patient with Thalassemia
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Slide 39
Diagnosis



By hemoglobin electrophoresis
RBC changes often seen by 6 weeks of age
Child presents with severe anemia, FTT
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Slide 40
Medical Management


Blood transfusion to maintain normal Hgb
levels
Side effect—hemosiderosis

Treat with iron-chelating drugs such as
deferoxamine (Desferal)
• Binds excess iron for excretion by kidney
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Slide 41
Nursing Management




Observe for complications of transfusion
Emotional support to family
Encourage genetic counseling
Parent and patient teaching for self-care
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Slide 42
Prognosis
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

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Retarded growth
Delayed or absent secondary sex
characteristics
Expect to live well into adulthood with proper
clinical management
Bone marrow transplant is potential cure
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Slide 43
Aplastic Anemia
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
All formed elements of the blood are
simultaneously depressed: “pancytopenia”
Hypoplastic anemia: profound depression of
RBCs but normal WBCs and platelets
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Slide 44
Aplastic Anemia (cont.)

Etiology





Primary (congenital)
Secondary (acquired)
Diagnostic evaluation
Therapeutic management
Nursing considerations
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Slide 45
Hemophilia

A group of hereditary bleeding disorders that
result from deficiencies of specific clotting
factors
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Slide 46
Types of Hemophilia

Hemophilia A

Classic hemophilia
 Deficiency of factor VIII
 Accounts for 80% of cases of hemophilia
 Occurrence: 1 in 5000 males
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Slide 47
Types of Hemophilia (cont.)

Hemophilia B
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

Also known as Christmas disease
Caused by deficiency of factor IX
Accounts for 15% of cases of hemophilia
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Slide 48
Etiology of Hemophilia A
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

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X-linked recessive trait
Males are affected
Females may be carriers
Degree of bleeding depends on amount of
clotting factor and severity of a given injury
Up to one third of cases have no known
family history

In these cases disease is caused by a new
mutation
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Slide 49
Manifestations


Bleeding tendencies from mild to severe
Symptoms may not occur until 6 months of
age


Hemarthrosis


Mobility leads to injuries from falls and accidents
Bleeding into joint spaces of knee, ankle, elbow,
leading to impaired mobility
Ecchymosis
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Slide 50
Manifestations (cont.)
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
Epistaxis
Bleeding after procedures

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Minor trauma, tooth extraction, minor surgeries
Large subcutaneous and intramuscular
hemorrhages may occur
Bleeding into neck, chest, mouth may compromise
airway
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Slide 51
Clinical Therapy



Can be diagnosed through amniocentesis
Genetic testing of family members to identify
carriers
Diagnosis on basis of history, labs, and exam


Labs: low levels of factor VIII or IX, prolonged PTT
Normal: platelet count, PT, and fibrinogen
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Slide 52
Medical Management

DDAVP

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IV
Causes 2 to 4 times increase in factor VIII activity
Used for mild hemophilia
Replace missing clotting factors
Transfusions


At home with prompt intervention to decrease
complications
Following major or minor hemorrhages
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Slide 53
Prognosis



Historically, most died by age 5 years
Now those with mild to moderate hemophilia
live near-normal lives
Gene therapy for the future

Infuse carrier organisms into patient; these act on
target cells to promote manufacture of deficient
clotting factor
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Slide 54
Interventions


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Close supervision and safe environment
Dental procedures in controlled situation
Shave only with electric razor
Superficial bleeding—apply pressure for at
least 15 minutes and ice to vasoconstrict
If significant bleeding occurs, transfuse for
factor replacement
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Slide 55
Managing Hemarthrosis

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During bleeding episodes, elevate and
immobilize the joint
Ice
Analgesics
ROM after bleeding stops to prevent
contractures
PT
Avoid obesity to minimize joint stress
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Slide 56
von Willebrand Disease (vWD)





A hereditary bleeding disorder involving
deficiency of von Willebrand factor (a plasma
protein and the carrier for factor VIII)
von Willebrand factor needed for platelet
adhesion
Transmitted as autosomal dominant trait
Occurs in both males and females
The gene for the disease is located on
chromosome 12
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Slide 57
Manifestations

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Easy bruising
Epistaxis
Gingival bleeding
Excessive bleeding with lacerations or
surgeries
Menorrhagia
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Slide 58
Diagnosis:
Laboratory Findings

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
Decreased von Willebrand factor levels
von Willebrand antigen levels
Decreased platelet agglutination
Prolonged bleeding time
PTT may be normal or prolonged
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Slide 59
Treatment of vWD



Infusion of von Willebrand protein
concentrate
DDAVP infusion before surgery or to treat
bleeding episode
Aminocaproic acid to treat bleeding in
mucous membranes (in some cases)
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Slide 60
Interventions



Avoid aspirin or NSAIDs (increase bleeding
time and inhibit platelet function)
Manage bleeding episodes with prompt
infusion therapy
Children with vWD have normal life
expectancy if well managed
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Slide 61
Idiopathic Thrombocytopenic
Purpura (ITP)

An acquired hemorrhagic disorder
characterized by:


Thrombocytopenia: excessive destruction of
platelets
Purpura: discoloration caused by petechiae
beneath the skin
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Slide 62
ITP Forms

Acute, self-limiting


Often follows URI or other infection
Chronic (more than 6 months’ duration)
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Slide 63
ITP
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
Diagnostic evaluation
Therapeutic management
Prognosis
Nursing considerations
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Slide 64
Disseminated Intravascular
Coagulation (DIC)

A disorder of coagulation that occurs as a
complication of numerous pathologic
processes

Hypoxia
 Acidosis
 Shock
 Endothelial damage
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Slide 65
Pathophysiology of DIC



Coagulation process is abnormally stimulated
Excessive amounts of thrombin are
generated
Fibrinolytic mechanisms are activated and
cause extensive destruction of clotting factors
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Slide 66
Mechanisms of DIC
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Slide 67
DIC



Diagnostic evaluation
Therapeutic management
Nursing considerations
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Slide 68
Immunologic Deficiency
Disorders

HIV/AIDS

Epidemiology
 Etiology and pathophysiology
 Clinical manifestations
 Diagnostics
 Therapeutic management
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Slide 69
Components of
Immune
System
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Slide 70
Common Clinical Manifestations
of HIV Infection in Children


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



Lymphadenopathy
Hepatosplenomegaly
Oral candidiasis
Chronic or recurrent diarrhea
Failure to thrive
Developmental delay
Parotitis
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Slide 71
Common AIDS-Defining
Conditions in Children








Pneumocystis carinii pneumonia
Lymphoid interstitial pneumonitis
Recurrent bacterial infections
Wasting syndrome
Candidal esophagitis
HIV encephalopathy
CMV
Cryptosporidiosis
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Slide 72
Severe Combined Immunodeficiency
Disease (SCID)


Absence of both humoral and cell-mediated
immunity
Pathophysiology



Graft-versus-host reaction
Therapeutic management
Nursing considerations
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Slide 73
Nursing Care of the
Child with a Neurologic
Disorder
Chapter 37
Assessment of Cerebral Function




Infants and young children: observe
spontaneous and elicited reflex responses
Family history
Health history
Physical examination
Increased Intracranial Pressure
(ICP)

Early signs and symptoms may be subtle

As pressure increases, signs and symptoms
become more pronounced and level of
consciousness (LOC) deteriorates
Clinical Manifestations of
Increased ICP in Infants






Irritability, poor feeding
High-pitched cry, difficult to soothe
Fontanels: tense, bulging
Cranial sutures: separated
Eyes: setting-sun sign
Scalp veins: distended
Clinical Manifestations of
Increased ICP in Children




Headache
Vomiting: with or without nausea
Seizures
Diplopia, blurred vision
Behavioral Signs of
Increasing ICP





Irritability, restlessness
Drowsiness, indifference, decrease in physical
activity and motor skills
Complaint of fatigue, somnolence
Inability to follow commands, memory loss
Weight loss
Late Signs of Increasing ICP







Decreased LOC
Decreased motor response to command
Decreased sensory response to painful
stimuli
Alterations in pupil size and reactivity
Papilledema
Decerebrate or decorticate posturing
Cheyne-Stokes respirations
Levels of Consciousness
(in Descending Order)




Full consciousness
Confusion: impaired decision making
Disorientation: to time and place
Lethargy: sluggish speech
Levels of Consciousness (in
Descending Order; cont.)




Obtundation: arouses with stimulation
Stupor: responds only to vigorous and repeated
stimulation
Coma: no motor or verbal response to noxious
stimuli
Persistent vegetative state: permanently lost
function of cerebral cortex
Pediatric Glasgow Coma Scale

Three part assessment:





Eyes
Verbal response
Motor response
Score of 15: unaltered LOC
Score of 3: extremely decreased LOC
(worst possible score on the scale)
Pediatric
Glasgow
Coma Scale
Neurologic Exam
Vital signs
Skin
Eyes
Motor function
Posturing
Reflexes
Variations in Pupil Size with
Altered States of Consciousness
Decorticate/Decerebrate
Posturing
Special Diagnostic Procedures





Lab tests: glucose, CBC, electrolytes, blood
culture if fever; evaluate for toxic substances,
liver function
Imaging: CT, MRI, echoencephalography,
ultrasound, nuclear brain scan, PET
Lumbar puncture
EEG
X-ray (rule out skull fractures, dislocations;
evaluate degenerative changes, suture lines)
Nursing Care of the
Unconscious Child


The outcome and recovery of the unconscious
child may depend on the level of nursing care
and observational skills
Emergency management



Airway
Reduction of ICP
Treatment of shock
Assessment Parameters




LOC
Pupillary reaction
Vital signs
Frequency of assessment depends on
condition: range from every 15 minutes
to 2 hours
Pain Management for the
Comatose Child




Signs of pain
Increased agitation and rigidity
Pain increases ICP
Alterations in vital signs

Usually increase in HR, RR, BP and decrease in
oxygen saturation
Drug Therapies for Pain



Opioids
Fentanyl + midazolam + vecuronium
Acetaminophen and codeine
Respiratory Management





Airway management is primary concern
Cerebral hypoxia lasting more than 4 hours
may cause irreversible brain damage
CO2 causes vasodilation, increased cerebral
blood flow, and increased ICP
May have minimal gag and cough reflexes
Risk of aspiration of secretions
ICP Monitoring

Indications for ICP monitoring

Glasgow coma scale <7
 Glasgow coma scale <8 with respiratory distress
 Deteriorating neurologic condition
 Subjective judgment
Nursing Care for Child with
Increased ICP




Patient positioning
Avoid activities that may increase ICP
Eliminate or minimize environmental noise
Suctioning issues
Nutrition and Hydration




IV administration of fluids and parenteral
nutrition
Caution with overhydration
Later begin gastric feedings via NG or GT
Patient may continue to have risk of
aspiration
Altered Pituitary Secretion

Syndrome of inappropriate antidiuretic
hormone (SIADH) may accompany CNS
diseases


Decreased urine output with hyponatremia and
hyposmolality
Treatment of SIADH

Fluid restriction, observe for electrolyte balance,
vasopressin administered
Medications (as Indicated)





Osmotic diuretics for cerebral edema
Antiseizure medications with or without
sedatives
Controversy with barbiturates
Paralyzing agents
Antipyretics
Nursing Care Needs






Elimination
Hygienic care
Position to prevent skin breakdown
Exercise- range of motion
Stimulation
Family support
Nursing Diagnoses




Disturbed Sensory Perception related to CNS
impairment
Self-Care Deficits related to physical
immobility, perceptual and cognitive
impairments
Risk for Aspiration related to depressed
sensorium, impaired motor function
Risk for Injury related to depressed
sensorium
Head Injury: Etiology



Falls
Motor vehicle injuries
Bicycle injuries
Falls – Leading Cause of Head
Injury under 5 years
Pathophysiology of Head Injury


Force of intracranial contents cannot be
absorbed by the skull and
musculoligamentous support of the head
Especially vulnerable to accelerationdeceleration injuries
Primary Head Injuries


Those injuries that occur at a time of trauma
Include:

Skull fracture
 Contusions
 Intracranial hematoma
 Diffuse injury
Subsequent Complications of
Head Injuries




Hypoxic brain injury
Increased ICP
Infection
Cerebral edema
Concussion




Transient and reversible
Results from trauma to the head
Instantaneous loss of awareness and
responsiveness lasting for minutes to hours
Generally followed by amnesia and confusion
Contusion and Laceration



Terms used to describe visible bruising and
tearing of cerebral tissue
Coup: bruising at the point of impact
Contrecoup: bruising at a site far removed from
the point of impact
Skull Fractures in Children


Great deal of force is required to produce a
skull fracture in an infant
Fracture on the underside of skull can tear the
meningeal artery, causing severe hemorrhage
with hypovolemic hypotension
Types of Skull Fractures





Linear
Depressed
Compound
Basilar
Diastatic
Complications of Head Trauma



Epidural hemorrhage
Subdural hemorrhage
Cerebral edema
Hematomas
Diagnostic Evaluation




Assessment of ABCs
Vital signs
Neurologic exam
Special tests: CT, MRI, behavioral assessment
Posttraumatic Syndromes




Postconcussion syndrome
Posttraumatic seizures
Structural complications such as hydrocephalu
True mental retardation occurs only after
severe injuries
Therapeutic Management




Care in hospital if severe injuries, loss of
consciousness for several minutes, prolonged
or continued seizures
NPO initially
Possible surgical interventions
Prognosis
Nursing Considerations






Frequent assessment: vital sign and
neurologic checks
Provide analgesia and sedation
Careful observation and recording
Family support
Rehabilitation
Prevention
Near Drowning




Drowning is the second leading cause of
accidental death in children
Death occurs from asphyxia while submerged
Can occur with even small quantity of water
(even as little as a pail of water)
Near-drowning: survived at least 24 hours
after submersion
Drowning – 2nd Leading Cause
of Death
Pathophysiology of Drowning



Hypoxia
Aspiration
Hypothermia
Therapeutic Management




Emergency resuscitative efforts at the scene
Management is based on degree of cerebral
insult
Aspiration is frequent complication
Prognosis
Nursing Considerations




Care depends on condition of the child
Helping parents cope with feelings of guilt
Parental anxiety related to prognosis
PREVENTION OF DROWNING
CNS INFECTIONS




CNS has limited response to injury
Difficult to distinguish etiology by looking at
clinical manifestations
Lab studies required to identify causative
agent
Inflammation can affect the meninges, brain,
or spinal cord
Bacterial Meningitis



Acute inflammation of the CNS
Decreased incidence following use of Hib
vaccine
Can be caused by various bacterial agents



Streptococcus pneumoniae
Group β streptococci
Escherichia coli
Transmission of
Bacterial Meningitis



Droplet infection from nasopharyngeal
secretions
Appears as extension of other bacterial
infection through vascular dissemination
Organisms then spread through CSF
Bacterial Meningitis



Diagnostics: LP is the definitive diagnostic test
Therapeutic management
Nursing considerations
Nonbacterial Meningitis
(Aseptic Meningitis)


Causative agents are principally viruses
Frequently associated with other diseases





Measles, mumps, herpes, leukemia
Onset abrupt or gradual
Manifestations: headache, fever, malaise
Diagnosis and treatment
Prognosis
Tuberculosis (TB) Meningitis




More likely to occur in very young children or
in immunosuppressed children
Ischemic infarction may occur
Fever, altered LOC, seizures
Common complication of this type of
meningitis: hydrocephalus
Brain Abscess




Pyogenic organisms gain access to neural
tissue by way of bloodstream from foci of
infection or from direct inoculation
Predisposing factors
Most common sites
Seizure disorders may be long-term
complication
Encephalitis



Inflammatory process of CNS with altered
function of brain and spinal cord
Variety of causative organisms—viral most
frequent
Vector reservoir in U.S.: mosquitoes and ticks
Clinical Manifestations of
Encephalitis: Onset Sudden or
Gradual







Malaise
Fever
Headache/dizziness
Stiff neck
Nausea/vomiting
Ataxia
Speech difficulties
Clinical Manifestation of Severe
Encephalitis





High fever
Disorientation/stupor/coma
Seizures/spasticity
Ocular palsies
Paralysis
Encephalitis



Diagnostic evaluation
Therapeutic management
Nursing considerations
Rabies




An acute infection of the CNS
Caused by virus transmitted by saliva of
infected animal
Virus multiplies in muscles and fatal if
untreated
Highest incidence in humans is in children
younger than 15 years
Rabies (cont.)

12% of cases come from domestic animals,
especially cats

Infected wild animals are most commonly
raccoons, skunks, foxes, and bats

Unprovoked attack is more likely to indicate
a rabid animal than a provoked attack
Therapeutic Management of
Rabies





Inactivated rabies vaccines
Globulins
HRIG: human rabies immune globulin ASAP
after exposure
HDCV: human diploid cell rabies vaccine
Guidelines for use from World Health
Organization
Nursing Considerations

Support and reassurance for child and family

Analgesia with EMLA cream to injection sites
Reye Syndrome (RS)


A disorder defined as toxic encephalopathy
associated with other characteristic organ
involvement
Characterized by fever, profoundly impaired
consciousness, and disordered hepatic
function
Reye Syndrome (cont.)



Etiology is obscure
Most cases follow a common viral illness
Potential association between aspirin therapy
for fever and development of RS
Reye Syndrome (cont.)





Pathophysiology
Diagnostic evaluation
Therapeutic management
Prognosis
Nursing considerations
Seizures




Caused by malfunctions of brain’s electrical
system
Determined by site of origin
Most common neurologic dysfunction in kids
Occur with wide variety of CNS conditions
Seizures: Signs and Symptoms




Change in LOC
Involuntary movements
Posturing
Changes in perception, behaviors, or
sensations
Types of Seizures

Epileptic

Nonepileptic


Most seizures are idiopathic
May result from acute medical illness
Etiology of Seizures



Idiopathic (no known cause)
Genetic factors –may alter sz threshold
Acquired
After brain injury
Hypoxia
Infections
Toxins
Incidence and Occurrence


2.3 million Americans affected
Especially children and elderly

More seizures in children younger than
2 years than any other age group
Epilepsy



Definition: ≥2 “unprovoked” seizures
Idiopathic epilepsy: cause unknown
Seizures are an indispensable characteristic
of epilepsy
Seizures


A single seizure not generally classified as
epileptic
Single seizure not generally treated with LT
Rx
Major Causes of Seizures in
Children



Birth injuries (anoxia) or congenital defects of
CNS
Acute infections in late infancy and early
childhood
In children older than 3 years, usually is
idiopathic
Absence Seizures




Formerly called petit mal or “lapses”
Brief loss of consciousness
Minimal or no change in muscle tone
Almost always appear in childhood
(4 to 12 years)
Absence Seizures (cont.)






Sudden onset of 20+ events per day
No warning/no aura
Duration 5 to 10 seconds
Motor: lip smacking, twitching of eyes, face,
slight hand movements
May drop object; child rarely falls
No incontinence
Absence Seizures (cont.)

Often misdiagnosed




Inattention/daydreaming
ADD/ADHDSchool issues
Behavioral management
School issues
Atonic Seizures





Sudden momentary loss of muscle tone
Onset usually ages 2 to 5 years
May or may not have loss of consciousness
Sudden fall to ground, often on face
Less severe—head droops forward several
times
Myoclonic Seizures






Sudden brief contractions of muscle group
May be single or repetitive
No loss of consciousness
Often occur when falling asleep
May be nonspecific symptom in many CNS
disorders
May be mistaken as exaggerated startle
reflex
Status Epilepticus


Definition: seizure lasting more than
30 minutes or series of seizures without
regaining premorbid LOC
Treatment:



Maintain airway
Establish IV access
Medications
Status Epilepticus (cont.)

Medications:

Diastat (prefilled rectal syringe)
 Versed (intranasal)
 IV Ativan or valproic acid
 IV loading with phenytoin for ongoing
management
Emergency Management



High-dose sedatives
Maintain patent airway
Prepare for respiratory support
Pediatric Diagnosis of
Seizure Disorders

Ascertain type of seizure


History, observation
Determine the cause of the event

Diagnostics
• EEG, MRI
• Labs: glucose, electrolytes, BUN, Ca++
• LP
Common Pediatric
Seizure Triggers





Changes in dark-light patterns (camera flashes,
headlights, rotating fan blades, reflections off
snow or water)
Sudden loud noises
Extreme temperature changes
Dehydration
Fatigue
Febrile Seizures





Transient disorder of childhood
Affect approximately 3% of children
Usually occur between ages 6 months and
3 years
Rare after age 5
Twice as frequent in males
Febrile Seizures (cont.)




Cause ??????
Usually in temperatures higher than 101.8°
Seizure occurs when temperature is rising,
not after
Seizure usually over before arriving in
emergency department
Febrile Seizure Treatment



Fever reduction
Evaluate history (episodic and family)
Seizure control if ongoing

Diazepam (rectal)
Febrile Seizures


95% to 98% of children with febrile seizures
do not have epilepsy or neurologic damage
Management:

Avoid tepid baths—usually ineffective
 Vigorous use of antipyretics
 Protect child from injury during seizure
 Call 911 if seizure lasts more than 5 minutes
Febrile Seizure Treatment

Prophylaxis with medications

If focal or prolonged seizures
 If neurologic anomalies
 If first-degree relative has seizure history
 In child less than 1 year old
 If multiple seizures in 2-hour period
RX
Phenobarbital



Febrile seizures, neonatal seizures
Also for other seizures: front-line IV choice if
patient does not respond to diazepam
High dosage may require respiratory support
Phenytoin (Dilantin)




PO or slow IV push (<50 mg/min)
Precipitates when mixed with glucose
Side effects: Gingival hyperplasia, ataxia,
rashes, acne, hirsutism, osteoporosis
Onset 5 to 30 minutes; duration
12 to 24 hours
Fosphenytoin



May be given with saline or glucose
Rate up to 150 mg/min
IV or IM
Valproic Acid




Trade names: Depakote, Depakene
IV or PO
IV for status epilepticus
S.E.: Hepatotoxicity!
Diazepam





Trade names: Valium IV, Diastat (rectal gel)
Medication of choice for status epilepticus
Rectal gel for home or prehospital
management
Onset 3 to 10 minutes; short duration
(minutes)
Concurrent loading with phenytoin for
sustained control of seizures
Ativan


Alternative to diazepam
May be preferable to diazepam


Longer duration of action
Less respiratory distress in children older than
2 years
Midazolam (Versed)



Intranasal route
For acute epileptic seizures
Onset 3 to 5 minutes; duration
Pharmacologic Management



Monotherapy is treatment of choice for
pediatrics
Gradual increase of dose until seizure control
or signs of toxicity
Polypharmacy if uncontrolled with one drug
Pharmacologic Management
(cont.)




Monitor therapeutic levels
Increase dosage as child grows
Monitor for known side effects
Avoid abrupt discontinuation—gradual
dose reduction
When to Discontinue




When seizure free for 2 years
Normal EEG
Avoid DC during puberty or when subject to
frequent infections
Recurrence possible within first year
Nursing Interventions

Observe and document episode

Protect from injury
 Stay calm
 Remain with child
 Privacy if possible
Home Management of Seizures




CPR training for family members
Rectal diazepam available for intractable
seizures
Activity restriction on individual basis
Safety devices—helmets, no swimming
alone, awareness of school, other caregivers
Migraine Headache






Autosomal dominant disorder
Cause unknown
Precipitating factors
Symptoms
Symptomatic pain relief
Pharmacologic options