WEEK 9

DISTURBANCES IN CIRCULATION Laboratory/ Diagnostic Exams:

 2D Echo: reveals enlarged right side of the heart
HEART CONDUCTION SYSTEM
and ↑ pulmonary circulation
Sinoatrial Node (SA node)
 Cardiac catheterization: demonstrates separation
 Primary pacemaker of the heart
of RA and the ↑ 02 saturation in the RA
 Inherent firing rate of 60 to 100 impulses per minute
Atrioventricular Node (AV node)
Surgical treatment: Surgical Dacron patch closure
 Located in the right atrial wall near the tricuspid
 Open repair with C-P bypass during school age
valve
Non-surgical: may be closed using devices during cardiac
 Coordinates the incoming electrical impulses from
catheterization
the atria and after a slight delay relays the impulse
to ventricles
Nursing Management:
 40 to 60 impulses per minute
 Explain to parents the purpose of tests and
Bundle of His
procedures
 Specialized conducting tissue
 Teach parents ways to support nutrition, reduce
 Right bundle branch - conducting impulses to the
stress on heart, promote rest, and support growth
right ventricle
and development during preoperative period
 Left bundle branch - conducting impulses to the
 Teach parents signs of congestive heart failure
left ventricle
and infection
○ Divides into the left anterior and posterior
 Prepare parents and child for surgery by visiting
bundle branches
intensive care unit, explaining equipment and
Purkinje fibers/ cells
sounds
 Terminal point in the conduction system
 Prepare older child for post-operative experience,
 Specialized to rapidly conduct impulses through
including coughing and deep breathing and
the thick walls of the ventricles
need for movement
 30 to 40 impulses per minute
 Teach need for antibiotic prophylaxis to prevent
subacute bacterial endocarditis
CONGENITAL HEART DEFECTS
 Incidence: 1% of or about 40,000 births per year
VENTRICULAR SEPTAL DEFECT (VSD)
 Most common anomaly is VSD
 Defect in ventricular septum - error in early fetal
 25% of babies with CHD are critical and generally
development
needs surgery or other procedures in their 1st year
 Can occur anywhere in muscle or membranous
of life (CDC)
ventricular septum
 15% of CHD are associated with genetic
 20-25% of all CHDs are VSD
conditions
 Pressure LV→ RV and systemic arterial circulation
 28% of kids with CHD have another recognized
resistance → pulmonary circulation, blood flows
anomaly (trisomy 21)
through the defect and into the pulmonary artery
 RV becomes enlarged (Hypertrophied), over time
the RA may also become distended

Symptoms:
 Tachypnea, dyspnea
 Poor growth, reduced fluid intake
 Palpable thrills
 Systolic murmur at left lower sternal border
 May develop CHF

Treatments:
 Medications:
○ Furosemide: a diuretic which removes
excess fluid out of the body
ATRIAL SEPTAL DEFECT (ASD)
○ Digoxin: helps the heart pump more
 Abnormal opening between atria → blood from
forcefully
higher pressure (LA) to flow into lower pressure
○ Angiotensin-converting enzyme (ACE)
(RA).
inhibitor: relaxes blood vessels and help
 Increase 02 blood into R side of heart
heart to pump more easily
 RA & RV enlargement
 Surgical repair with bypass (procedure of choice)
 Cardiac failure is unusual in uncomplicated ASD
 Pulmonary artery banding (if not too large) or
 May be asymptomatic if small defect
patch
 Dyspnea
 Fatigue and poor growth
PATENT DUCTUS ARTERIOSUS (PDA)
 Soft systolic murmur in pulmonic area (splitting S2)
 Ductus SHOULD close by about age 15 hours after
 May develop CHF
birth
  Some shunting of blood may occur up to 24 hours
of life
 DUCTUS closes because increase in arterial
oxygen concentration that follows initiation of
pulmonary function
 Prostaglandin E leads to closure of PDA
 Allows blood to flow from left to right and
pulmonary blood flow
 Small PDA: asymptomatic
 Bounding peripheral pulses
 Widened pulse pressure (>25)
 Loud machine-like murmur at upper left sternal
border (Left intraclavicular area)
 Complication for Large PDA: CHF with tachypnea,
dyspnea, and hoarse cry
Definitive diagnosis: ECHO
Medical:
 (Premature) INDOMETHACIN to close PDA's;
surgical ligation if meds fail
 Prophylactic antibiotics to prevent bacterial
endocarditis
Surgery >> between age 1-2 years
OBSTRUCTIVE DEFECTS
 Blood flow in heart meets an area of anatomic
narrowing (stenosis) → obstruction to blood flow
 Pressure in ventricle & great arteries before
obstruction is increased; pressure in area beyond
obstruction is decreased
 Location of narrowing near the valve:
○ Valvular: site of valve itself
○ Subvalvular: narrowing in vent below
valve (ventricular outflow tract)
○ Supravalvular: narrowing in great art
above valve
 (+) pressure load on ventricle- decrease CO
 s/s CHF
 Mild obstruction: asymptomatic
 Severe stenosis: hypoxemia (rare)
COARCTATION OF AORTA
 The aorta is narrowed near the insertion of the
ductus arteriosus
 Increased pressure proximal to the defect
 Causes high BP & bounding pulses in arms; weak
or absent femoral pulses, and cool lower
extremities with low BP
Signs of CHF in infants:
 Condition can deteriorate rapidly
 Older kids may complain of dizziness, headache,
fainting and epistaxis from hypertension
 Patient at risk for ruptured aorta, aortic aneurysm, or
stroke
Treatment:
 Non-Surgical: balloon angioplasty. Usually
effective
 Surgical: does not require bypass since defect is
outside pericardium
 Post-op complication is hypertension
 Usually done before age 2 yrs.
 Risk of recurrence
AORTIC STENOSIS
 Narrowing of aortic valve usually malformed in Bl-
rather than TRI- cuspid valve
 Causes increased resistance in left ventricle,
dcreased CO, L ventricular hypertrophy and
pulmonary vascular congestion
 L ventricular wall is hypertrophied>> increased
pulmonary vascular resistance & pulmonary
hypertension
 LVH >> decrease coronary artery perfusion &
increase risk of MI
Clinical manifestations:
 Signs of decreased CO: faint pulses, hypotension,
poor feeding, tachycardia
 Murmur; exercise intolerance
 Chest pain, dizziness with standing
Treatment:
 Balloon angioplasty to dilate the valve;
Surgery:
 Konne procedure (valve replacement)
 May require repeat procedures
PULMONARY STENOSIS
 Pulmonary valve is stenosed
 Narrowing at entrance to pulmonary artery >> R
ventricular hypertrophy and decreased
pulmonary blood flow
 Extreme form of PS: Pulmonary atresia (total fusion
of the commisures and no blood flow to lungs)
 PS >> RVH, R ventricular failure >> R atrial pressure
increases and may reopen foramen ovale
 Shunts unoxygenated blood to L atrium >>
systemic cyanosis
 May lead to CHF
 Often have PDA as well
 Cardiomegaly on CXR
Treatment:
 Balloon angioplasty to dilate the valve
Surgical treatment:
 Breck procedure (Bypass to do valvotomy)
 Usually can repair with catheterization
DEFECTS OF DECREASED PULMONARY BLOOD FLOW
 Obstruction of pulmonary blood flow + anatomic
defect (ASD/VSD) between R & L side of heart
 Difficulty of blood exiting R heart via pulmonary
artery → increase R side pressure > L pressure →
desaturated blood shunt R to L→ desaturated
blood in systemic circulation
 Hypoxemia, usually cyanotic
TETRALOGY OF FALLOT
Involves four heart defects:
1. Ventricular Septal Defect
2. Pulmonary stenosis
3. Right ventricular hypertrophy
4. Overriding aorta
 Hemodynamics vary widely
 Depends on extent of pulmonic valve stenosis &
size of VSD
 If VSD is large, pressures are equal in R and L
ventricles. Blood is shunted in the direction of the
least resistance (pulmonary or systemic vascular
resistance)
 PVR is > than systemic vascular resistance, shunt will be
R to L
Clinical manifestations:
 "TET SPELLS" or "blue spells" with acute episodes of
cyanosis and hypoxia
 Anoxic after feeding or with crying. RISK of emboli,
LOC, sudden death, seizures
 Repairs: usually indicated when Tet spells and
hypercyanotic spells increase
o Stage 1: Blalock or modified Blalock shunt
>>blood to pulmonary arteries from Lor R
subclavian artery
o Complete repair: usually in 1st year of life.
Repair of VSD, resect stenosed area, and
patch R ventricular outflow
TRICUSPID ATRESIA
 Failure of tricuspid valve to develop
 No communication from R atrium to R ventricle
 Blood flows thru an ASD or a patent FO to L side of
the heart thru a VSD to R ventricle to lungs
 Often associated with PS and TGS
 Complete mixing unO2 and O2 blood
 in L side of the heart → systemic desaturation,
pulmonary obstruction → decrease pulmonary
blood flow
 At birth: presence of patent FO (or ASD) is required
to permit blood flow across septum into Latrium
o PDA allows blood flow to pulmonary
artery for oxygenation
Manifestations: cyanosis, tachycardia, dyspnea,
hypoxemia, clubbing
 At risk for bacterial endocarditis, brain abcess, stroke
Treatment: NB - pulmonary blood flow depends on PDA
 Continuous infusion of PGE 1 until Sx
 Palliative: shunt (pulmonary-to-systemic art
anastamosis)
 Pulmonary artery banding
 Modified Fontan procedure
MIXED DEFECTS
 Survival on postnatal period depends on mixing of
blood from pulmonary systemic circulation within
cardiac chambers
 Blood is mixed from pulmonary and systemic
circulations within the heart chambers >> Relative
desaturation of blood in systemic blood flow
 Cardiac output decreases because of volume
load on ventricle
 Signs of desaturation, cyanosis, and CHF, but
variable depending on anatomy
 Degree of cyanosis not always visible & signs of
CHF
o TGA: severe cyanosis in 1st day of life →
CHF (later)
o Truncus arteriosus: severe CHF 1st few
weeks of life and mild desaturation
HYPOPLASTIC LEFT HEART SYNDROME
 Left side of the heart is underdeveloped
 Left ventricle is small and aortic atresia
 Most blood flows across patent foramen ovale to
R atrium - to R ventricle and out the pulmonary
 Descending aorta receives blood from the
 PDA to supply the systemic circulation
 PDA closure >> rapid deterioration and CHF.
Treatment: Keep ductus open with Prostaglandin E infusion
Surgical Treatment:
 Norwood procedure to create a new aorta using
the main pulmonary artery and creation of large
ASD
 Bidirectional Glenn Shunt at 6-9 months age to
reduce volume load on the R ventricle
 Modified Fontan procedure, similar to Tricuspid
atresia repair
 Transplant may be an option for some parts.
Mortality rate is very high (30%- 50%)
TRANSPOSITION OF THE GREAT VESSELS
 Pulmonary artery leaves the L ventricle and the
aorta exits from the R ventricle
 No communication between the systemic and
pulmonary circulations
 Must have PDA or Septal defect to permit blood
flow
 Surgical treatment of choice is Arterial switch
procedure to resect and reanastamose great
vessels
 Coronary arteries have to be reimplanted to
supply myocardial circulation
 Other procedures are possible - depending on the
defect
TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION
 Rare defect
o Furosemides, Thiazides, Spirinolactone
o Fluid restriction
o Sodium restriction
 Decrease cardiac demands
o Bed rest, treat infection
o Preserve body temperature; reduce effort of
breathing (semi-fowlers)
o Sedate an irritable child
 Improve tissue oxygenation & decrease O2
consumption
o 02 vasodilator
o Cool humidified 02
ACQUIRED CARDIOVASCULAR DISORDERS
CLINICAL CONSEQUENCES OF CONGENITAL HEART DISEASE
CONGESTIVE HEART FAILURE
 Inability of the heart to pump and adequate
amount of blood to the systemic circulation at
normal filling pressures to meet the metabolic
demands of the body
 Due to structural deformity in children (septal
defects) increased blood volume & pressure in the
heart → MYOCARDIAL FAILURE
 Can occur with cardiomyopathy, dysrythmia,
severe electrolyte imbalances
 Could also be due to excess demands on a
normal cardiac muscle (sepsis / severe anemia)
RIGHT SIDED HEART FAILURE
 RV unable to pump blood to Pulmonary Artery→
increased pressure in RA and in the systemic
venous circulation
 Systemic venous HPN → hepatosplenomegaly
LEFT SIDED HEART FAILURE
 LV unable to pump blood to the systemic
circulation→ increased LA pressure and
pulmonary vv→ congestion in lungs → increased
 pulmonary pressure→ pulmonary edema → →invade adjacent tissues (mitral/aortic valves)
pulmonary HPN →breaks off and embolize elsewhere (spleen,
kidney, CNS) → death
Signs and symptoms of CHF
 Each side of the heart depends on the adequate Diagnostics
function of the other  Based on clinical manifestations
 Failure of one chamber affects the opposite  Blood c/s: definitive diagnosis
chamber  ECG/CXR (cardiomegaly)
 If not corrected, it may lead to cardiac damage →  Increased ESR, increased WBC, anemia,
Inadequate CO → decreased supply to the kidneys → microscopic hematuria
Na and H2O resorption → hypervolemia, increased  2D-echo-vegetations, valve function
workload on the heart, pulmonary and systemic
congestion Clinical manifestation
 Insidious onset, unexplained fever (low grade,
CONGESTIVE HEART FAILURE in Children intermittent)
 Impaired myocardial function  Anorexia, malaise, weight loss
o Tachycardia, fatigue, weakness, restless,  Extracardiac emboli
pale, cool extremities, decreased BP,  Splinter h'ge-thin black lines under nails
decreased urine output  Osler nodes - red, painful, intradermal nodes on
 Pulmonary congestion pads of phalanges
o Tachypnea, dyspnea, respiratory distress,  Laneway lesions – painless h'ges on panes and
exercise intolerance cyanosis, wheezes soles
 Systemic venous congestion  Petechiae on oral mucous membrane
 Peripheral and periorbital edema, weight gain,  May be present: CHF, dysrythmia, new murmur
ascites, hepatomegaly, neck vein distention
Therapeutic management
CHF Treatment  High dose antibiotics: Penicillin, ampicillin,
Goals: methicillin, cloxacillin, streptomycin, or
1. Improved cardiac function gentamycin
 Digitalize - Digoxin  IV/IM x 4 weeks at least
o Increased CO, decreased size and venous  Amphoterecin or flucytosine for fungal infections
pressure, relieve edema  Treat 2-8 weeks. If antibiotics is unsuccessful >>
o Lanoxin (Pedia) - more rapid in onset - Oral/IV CHF develops, vulvular damage
doses x 24 hours followed by maintenance dose  Should be instituted immediately
(BID) to maintain blood levels (Digitalizing Dose)  Blood c/s periodically to evaluate response to
 ACE Inhibitors (Capoten / Enalapril) antibiotics
o (-) normal function of R-A system in kidney  Prophylaxis before dental procedures,
o Blocks conversion of Al to All (Vasodilator) bronchoscopy, T&A, surgeries, childbirth
o Captopril - can be given smaller doses  Prophylaxis: 1 hour before procedures (IV) or may
2. Remove accumulated Fluid and Sodium use PO in some cases
o Diuretics - mainstay of treatment to eliminate  Family dentist should be advised of existing heart
excess H20 and salt problems
o Bidirectional glenn
3. Keep hct and blood viscosity within acceptable limits RHEUMATIC HEART DISEASE (RHF)
(hydrate)
4. Monitor for anemia, Fe supplementation, blood Rheumatic Fever (RF)
transfusion  Inflammatory disease occurs after Group A Beta-
5. Respiratory infection or reduce pulmonary function can haemolytic streptococcal throat infection
worsen hypoxemia
o Aggressive pulmonary hygiene Self-limiting
o CPT, antibiotics  Affects joints, skin, brain, serious surfaces, and
o 02 heart

ENDOCARDITIS (Bacterial Infective Endocarditis) Risk factors:

 BE, IE or subacute bacterial endocarditis (SBE)  Age and sex: (5-15 years old) female
 Infection in valves and endocardium  Housing and socioeconomic status
 Sequelae of sepsis in child with cardiac disease of  Season: rainy season
congenital anomaly  Genetic predisposition
 Affects children with valvular abnormalities,
prosthetic valves, recent heart surgery with Clinical Manifestations:
invasive lines and RHD with valve involvement,  Acute febrile-like illness (2-3 weeks after
drug abuse streptococcal throat infection)
 Staph aureus, strep viridians (most common),  Non-specific
candida albicans, gram negative bacteria o Fever
 Enter blood system thru: dental (most common), o Joint pain
UTI, cardiac catheterization, surgery, etc. o Loss of appetite
 Organism in endocardium → vegetations o Muscle ache
(verrucae) → fibrin deposits→ platelet thrombi
 Specific
o Joints (swelling and pain of larger joints like knee,
ankle, elbow and wrist occurring in rapid
succession - "migratory arthritis")
o Heart (causes inflammation of the whole layers of
the heart- PANCARDITIS)-Palpitation, chest pain,
shortness of breathe, leg swelling, etc.
o Skin & subcutaneous tissues (non-itching macular
rash and painless mobile nodules over joints and
spines)
o Central Nervous System (a late manifestation) -
abnormal movements of the limbs with muscle
weakness and emotional labiality.
Diagnostic approach:
Modified Jones Criteria
 2 major or 1 major + 2 minor manifestations + strep
infection
MAJOR
 Carditis - tachycardia out of proportion to degree of
fever
o Cardiomegaly, murmur, muffled heart sounds
o Pericardial friction rub, pericardial pain, changes
in ECG
o Involves endocardium, pericardium,
myocardium
o Most commonly the mitral valve
 Polyarthritis
o Arthritis is reversible and migrates, especially in
large joints (knees, elbow, hips, shoulders, wrists)
o Swollen, hot, red, painful joints, after 1-2 days
affects different joints
 Erythma marginatum - rash
o Transitory, non pruritic
o Trunk and proximal portion of extremities
o Red macule with clear center wavy, well-
dermacated border
 Subcutaneous nodules
o Small nontender nodules
o Bony prominences - hands, feet, elbows, scalp,
scapulae, vertebrae
o Persistent indefinitely after onset of the disease
and resolve with no resulting damage
 St. Vitus Dance - The Fifth Manifestation (Sydenham's
Chorea)
o St. Vitus Dance(aka, chorea) reflects CNS
involvement
o Definition: Chorea refers to sudden, aimless
movements of extremities, involuntary facial
grimaces, speech disturbances, emotional lability
and muscle weakness
o Worse with anxiety and relieved by rest
MINOR
 Arthralgia
 Fever
LABORATORY
 Increased ESR, CRP
o Supporting evidence of antecedent group A
Strep Infection
 Throat c/s, rapid Ag test
 ASO titer (most reliable - 80% children)
 AntiDNAse. ESR, CRP
 ECG, CXR-evidence of heart involvement
TREATMENT
 Goals
o Eradication of haemolytic strep
o Prevention of permanent cardiac
damage - Palliation of other symptoms
o Prevention or recurrence of RF
 Prevention of RHD
o Treatment of streptococcal tonsillitis /
pharyngitis
 Penicillin G - IMX1
 Penicillin V- oral x 10 days
 Sulfa-oral x 10 days
 Erythromycin (if allergic to
above) - oral x 10 days
o Treatment of recurrent RF
 Same as above
 Salicylates (ASA) - control inflammatory process
esp. joints, dec fever and discomfort
 Bed rest - during febrile phase but need not be
strict
 Should be followed medically x 5 years at least
NURSING CONSIDERATIONS
 Encourage compliance with drug
o Encourage adherence to tx'c plan
o (+) poor compliance: monthly injections
 Facilitate recovery from illness
and  Provide emotional support
 Prevent disease
 Interventions during homecare
o Provide rest and adequate nutrition
o Once fever is over, resume moderate
activities, improve appetite
o Carditis: most disturbing and frustrating
manifestation
o Gradual
o Mistaken for nervousness, clumsiness,
inattentiveness
o Limits physical activity
o Stress parents and teachers: illness is
temporary and disappears in time
Rheumatic Heart Disease
 Most common complication of RF
 Damage to valves leading to stenosis or regurgitation
with resultant hemodynamic disturbance.
KAWASAKI DISEASE
 Mucocutaneous LN syndrome
 Acute systemic vasculitis
 <5 y/o (Peak: toddlers)
 Self limiting but 20% children without treatment
develop cardiac disease
 Etiology: unknown
C-conjunctivitis (pink eye)
R-rash
A-arthritis (joint pain)
S-strawberry tongue
H-hands (peeling skin)
Area involved: CVS
 Initially: Inflammation arterioles, venules,
capilliaries formation coronary artery aneurysm
 Death: result of coronary thrombosis or severe scar
formation & stenosis of main coronary artery
 Myocardial infarction from thrombosis
No specific diagnostic test
 IRRITABILITY - hallmark of Kawasaki Disease
 Persists in 2 weeks
  Help family adjust to the disorder
 Educate family
 Help family cope with effects of the disorder
 Prepare child and family for surgery
Surgical Interventions
 Open-Heart
 Closed heart procedures
 Staged procedures
 Prepare child and family for procedures
Postoperative Care of the Child
 Monitor vital signs and A/V pressures
 Intraarterial monitoring of BP
 Intracardiac monitoring
 Respiratory needs
 Rest, comfort and pain management
 Fluid management
 Progression of activity
Postoperative Complications
 CHF - due to excessive pulmonary blood flow or
fluid overload
 Hypoxia - inadequate pulmonary blood flow /
respiratory problems
 Dysrythmias
o Early post op period
o Due to electrolyte imbalance
(hypokalemia) & surgical intervention to
septum / myocardium
o ECG pattern, apical pulses x 1 minute
 Cardiac tamponade
o Compression of heart by blood and other
effusion (clots) in pericardial sac→
restricts normal heart movement
o Rising and equalizing RA and LA pressures,
narrowing pulse pressure, tachycardia,
dyspnea, apprehension, abrupt stop to
chest tube drainage from mediastinal
tubes
o 2-D echo to confirm the diagnosis
o Treatment: Pericardiocentesis
 Decreased cardiac output syndrome
 Signs of Shock: decreased BP, decreased pulse
pressure, cool extremities, metabolic acidosis,
oliguria
 Aggressively treated with IV inotropes (dopamine,
dobutamine, milrinone)
 Decreased peripheral perfusion
o Capillary refill time, skin color, warm/cold
extremities, pulses (strong/weak)
 Pulmonary changes
o Areas of atelectasis common after Sx
Tests of Cardiac Function
Echocardiography (2D Echo)
 Enables visualization and measurements of
turbulence, accentuation, direction, and
decreases/ increases in blood flow, along with
pressure gradients
 Uses high-frequency sound waves obtained by a
transducer to produce an image of cardiac
structure
 Most useful in diagnosis of CHD in critically ill
babies
Angiography
 Assess route of blood flow in heart & major blood
vessels
Chest X-ray
 Used to evaluate for Cardiomegaly, pulmonary
vascular marking, and pulmonary venous
congestion
 Also rules out pulmonary disease
Electrocardiogram (ECG/EKG)
 Electrical activity of the heart
 Not used for diagnosis but provide data on
abnormal electrical activity that warrants the
need for additional evaluation
Arterial Blood Gasses (ABG)
 Helpful in differentiating between cardiac and
pulmonary pathologies
 Important when the child is at risk or complications
such as acidosis and shock
Cardiac Catheterization
 Most invasive diagnostic procedure
 Radio opaque catheter inserted through
peripheral blood vessel into the heart
 Performed to evaluate heart Valves, heart
function and blood supply, or heart abnormalities
 Used also for treatment (when combined with
angioplasty)
 Complications:
o Infective endocarditis
o bleeding/ bruising
o Changes in circulation on Cath side
 Pre-op care
o History and physical examination
o Laboratory works (ECG, 2D Echo, CBC)
o NPO
o Preprocedural teaching
 Post-op care
o Monitor VS
o Monitor extremity distal to the catheter
insertion site
o Leep leg immobilized
o Measure I + O
o Check for bleeding at insertion site
RED BLOOD CELL DISORDERS
Anemia
 The most common hematologic disorder of
childhood
 Decreases in number of RBCs and/ or hemoglobin
concentration below normal
 Decreased oxygen-carrying capacity of blood
 Not a disease itself but an indication or
manifestation of an underlying pathologic
process
Classification of Anemia
Etiology and physiology
 RBC and/ or Hgb depletion
 Provides direction for planning of nursing care
Morphology
 Characteristic changes in RBC size, shape, and/ or
color
 Most useful in terms of laboratory evaluation of
anemia
RBC Morphology
SIZE (CELL SIZE)
 Variation in RBC sizes(anisocytosis)
 Normocytes (normal cell size)
 Microcytes (smaller than normal cell size)
 Macrocytes (larger than normal cell size)
SHAPE (CELL SHAPE)
 Variation in RBC shapes (poikilocytosis)
 Spherocytes-globular cells
 Drepanocytes-sickle-shaped cells
 Numerous other irregularly shaped cells
COLOR (STAINING CHARACTERISTICS)
 Variation in Hgb concentrations in the RBC
 Normochromin - sufficient/ normal amount of
hgb/ rbc
 Hypochromic-reduced amount of hgb/rbc
 Hyperchromic-increase amount of hgb/rbc
Etiology/pathophysiology
 Excessive blood loss-acute/ chronic hemorrhage
 Normocytic, normochromic anemia as long as
there's enough iron stores for hgb synthesis
 Trauma, bleeding disorder, parasitism
Destruction (hemolysis)
 Intracorpuscular - sickle cell anemia
 Extracorpuscular - infections (malaria, immune
mechanisms sepsis), chemicals,
 Destruction outpace production
Decrease/ impaired production of rbc
 BM failure or nutritional deficiency
Morphology:
 Size: normocytes, microcytes, macrocytes,
anisocytosis
 Shape: poikilocytes, spherocytes, drepanocytes
 Staining characteristics
 Normo/ hypo/ hyperchromic
Consequences of anemia
 Basic physiologic defect:
o Decrease in oxygen-carrying capacity of blood
and decreased amount of oxygen available to
tissues
 When anemia develops slowly, child adapts
 Effects of Anemia on Circulatory System
 Hemodilution
 Decreased peripheral resistance
 Decrease oxygen-carrying capacity of blood →
compensatory increase in HR & Cardiac Output
 Increased cardiac circulation and turbulence
 May have murmur
 May lead to cardiac failure
 Cyanosis - not evident
 Hgb levels must exceed 5g/dl before cyanosis is
evident
 CNS manifestations:
o Headache, dizziness, light headedness, irritability,
slowed thought processes, decrease attention
span, apathy, depression
 Growth retardation - spurt
o Decrease cellular metabolism & to existing
anorexia
o Chronic severe anemia
 Delayed sexual maturation in older child
Diagnostic Evaluation
 History & PE: lack of energy, easy fatigability, pallor
 Unless anemia is severe, first clue to the disorder may
be alterations in CBC
 CBC
 Decreased RBCs
 Decreased Hgb (<10 or 11g/dl) and Hct
 Other tests for particular type of anemia
 Increase reticulocyte count; bone marrow aspiration
 (+) sickle cells in peripheral blood smears
Therapeutic Management
 Treat underlying cause
o Transfusion after hemorrhage if needed
o Nutritional intervention for deficiency anemias
 Supportive care
 o IV fluids to replace intravascular volume
o Oxygen
o Bed rest
Nursing Care Management
 History & PE
o Nutrition-lactose intolerant, inadequate intake of
iron
o Past history of chronic, recurrent infection
o Eating habits- pica (consumption of non-nutritive
subs-dirt, starch, lead-based paint chips, paper)
o Bowel habits, (+) frank blood in stools or black tarry
stools (due to chronic blood loss)
o Family history of hereditary diseases - thalassemia,
sickle cell disease
 Clues to possible iron deficiency
o Baby drinks too much milk
 Teenager on liquid/ vegetarian diet
Nursing considerations
 Prepare child and family for laboratory tests
o Several blood tests done sequentially multiple
finger/ heel punctures or venipunctures
o Explain significance of each tests
o Encourage parents/family support
o Allow child to play during procedure
 Suggested explanations
o RBC: carry 02 you breathe from your lungs to all
parts of the body
o WBC: help keep germs from causing infection
o Platelets: help make bleeding stop over the hurt
area
o Plasma: liquid portion of blood that has clotting
factors that make bleeding stop
 Decrease oxygen demands
o Assess energy level & minimize excess demands
o Diversional activities, prevent separation from
parents
o Signs of exertion: tachycardia, palpitations,
tachypnea, dyspnea, SOB, hyperpnea,
breathlessness, dizziness, light headedness,
diaphoresis, change in skin color
o Child look fatigued: sagging, limp posture, slow
strained movements, inability to tolerate
additional activity, difficulty sucking in infants
 Prevent complications
o Prevent tissue hypoxia - causes cellular
dysfunction & disturbed metabolic processes
weaken the host's defenses against foreign
agents
o Prevent exposure to infectious agents - worsens
the anemia by increasing metabolic needs,
interferes with erythropoiesis & shortens survival
time of RBC
o Signs: fever, leukocytosis
o Main complication: cardiac decompensation
o Result from excessive demands on heart due
to increase metabolic needs or cardiac
overload
o Observe for signs and symptoms of heart
failure
o Minimize hypoxia, IVF monitoring
 Support family
IRON DEFICIENCY ANEMIA
 Caused by inadequate supply of dietary iron
 Most prevalent nutritional disorder in US & most
common mineral disturbance
 12-36 months: at risk due to cow's milk
 Generally is preventable
o Iron-fortified cereals and formulas for infants
o Special needs of premature infants
o Adolescents at risk due to rapid growth and poor
eating habits
Etiology
 Inadequate iron supply
o Lack of iron stores at birth: low BW, PT, Multiple
births
o Severe iron deficiency in mother (Hgb
<9g/dl)
o Fetal blood loss before/ at delivery
o Bleeding in infant
o Inadequate intake
o Rapid growth rate
o Excessive milk intake, delayed addition of
solid foods
o Poor general eating habits
o Exclusive BF of infant after 6 months of
age
 Impaired absorption
o Chronic diarrhea
o (+) iron inhibitors
o Phylates, phosphates, oxalates
o Malabsorption disorder
o Lactose intolerance; inflammatory bowel
disease
 Excess demands for iron required for growth: PT, LBW
babies, adolescents, pregnancy
 Blood loss-epistaxis, polyps, meckels, parasitism;
acute/ chronic hemorrhage
 At birth: FT infant's supply of iron is approximately
300mg or 75mg/kg BW
 Last trimester pregnancy → iron transferred from
mother to fetus
 Most iron stored in circulating erythrocytes of fetus,
remainder stored in fetal liver, spleen, BM iron stores
adequate x 1st 5-6 months in FT infant & 2-3 months in
PT or multiple births
 If dietary iron is not supplied to meet the infant's growth
demands after fetal iron stores are depleted -(+) iron
deficiency anemia
 Should not be confused with physiologic anemia
 Vegetarian diet popular among teenagers
o Infants & toddlers fed inappropriate with vegan
diet → severe protein-energy malnutrition; vitamin
B12, iron & vitamin D deficiency
 Most are underweight
 Overweight babies - excessive milk ingestion (milk
babies)
o Milk: poor source of iron, is given to exclusion of
solid foods
o 50% iron deficient infants fed on cow's milk have
increase fecal loss of blood
Pathophysiology
 Iron: required for production of hgb
 1 molecule hgb: protein (globin) + 4 molecules of
pigmented compound (heme)
 Each molecule of heme contains 1 atom of iron
 Iron stores deficient, production of hgb reduced
 Main effect: decrease hgb level & reduced
oxygen carrying capacity of blood
Signs/symptoms
 Fatigue, underweight
  Some overweight (milk babies) - chubby, pale,
poor muscle development, prone to infection,
skin: porcelain like
 Enhances plasma protein leakage in infants -
edema, retarded growth, decrease serum
concentration of proteins, albumin, Y-globulin,
transferring
 Inability to concentrate, irritability
 Palpitations, tachycardia
 Dyspnea on exertion
 Pica, blue sclera, sphenomegaly
 Dry brittle nails
 Koilonychia - concave/spoon-shaped - fingernails
Laboratory findings
 RBC - normal/ borderline/ moderately reduced
that is out of proportion to low hgb level
 CBC - low levels of hct, hgb
○ Microcytic, hypochromic
○ RBC: bull's eye target
 Decrease MCV, MCH, MCHC
 Low serum iron, low ferritin
 Reticulocyte count - normal/ slightly reduced
 Occult blood test - Guaiac test to r/o chronic
blood loss
 Bone marrow aspiration - MOST definitive
Medical Management
 Hematinics
 Blood transfusion
Therapeutic Management
 Primary goal: prevention through optimum nutrition &
appropriate iron supplementation
 Guidelines to prevent iron deficiency
o Use only BM or iron-fortified formula (12mg/dl iron)
for 1st 12 months
○ Begin to supplementation (in the form of iron-
fortified commercial formula or, in BF infants, iron-
fortified infant cereal) to provide 1mkd of iron by
4-6 months of age in FT infants & by 2 months of
age in PT infants
○ Administer iron drops (FeSO4) at dose of 2-3mkd,
maximum 15mg/day to BF PT infants after 2
months of age, & give iron-fortified infant cereal
when solid foods are introduced
○ Limit the amount of formula to no more than
1L/day to encourage intake of iron-rich solid foods
 Main treatment: correction of underlying problem
 Iron-fortified commercial formula & iron- fortified
cereal: best sources of supplemental iron in formula-
fed infants
o < 12 months: should not receive fresh cow's milk
o May increase risk of GI loss from milk allergy or
Gl mucosal damage from lack of cytochrome
iron
 Dietary addition iron-rich foods
o Inadequate as sole prescription of IDA → poorly
absorbed
o Red meat, organ meat, legumes, green
leafy.vegetables, raisins, iron-fortified cereals/
formula
o Must be supplemented with oral iron x 3months
o 3-6mg of elemental iron/kg/day BID/TID
o FeSO4: more rapidly absorbed than ferric iron
+ Vitamin C/ juices/ vit. C enriched foods
 If hgb level fails to rise after 1 month oral therapy,
assess for:
o Persistent bleeding
o Iron malabsorption
o Noncompliance
o Improper iron administration
o Other causes
 Parenteral (IV/IM) iron: safe and effective
o Painful, expensive, could cause regional
lymphadenopathy/ allergies
o Reserved for children who have iron
malabsorption or chronic hemoglobinuria
 Transfusions - severe anemia, serious infection,
cardiac dysfunction, surgical emergency when
anesthesia is required
o Packed RBC (2-3cc/kg)
 Supplemental oxygen- severe tissue hypoxia
Prognosis
 Very good
 Severe IDA & long standing:
o Cognitive, behavioral, motor impairment may
result
Nursing considerations
 Instruct parents in the administration of iron
o 2-3 divided doses between meals when presence
of free HCI is greatest
o More iron absorbed (1-2 hours before meals)
o Taken with citrus fruit/ juice- aids in absorption
o Cow's milk interferes with absorption
o Iron turns stools to tarry green color
o Absence of greenish black stool may be a clue to
poor administration of iron
o (+) vomiting/ diarrhea: can be given with meals,
reduce dose then gradually increase until
tolerated
 Liquid preparations: may stain teeth temporarily
o Taken thru straw/ syringe/ medicine dropper
o Brush teeth after intake
 Parenteral iron: iron dextran
o Z-track method; (not massaged after injection to
minimize skin staining & irritation
o No more than 1 ml given on 1 site
o No IV route
o Observe for anaphylaxis on IV administration
o Test dose before routine use
 Diet
o Thru family education
o Breast milk: poor iron source after 5 months of
lactation
o Nurse must reinforce the importance of iron
supplementation by 4-6 months of age
o Formula-fed: use iron-fortified formula
o Introduce solid at appropriate age: cereals
 Dispel popular myth that milk is a "perfect food"
o May equate weight gain with "healthy child" &
"good mothering"
 Stress that overweight is not synonymous with good
health
 Diet education of teenagers
o Effect of anemia on appearance (pale), & energy
level
Sickle Cell Anemia
 A hereditary hemoglobinopathy
Ethnicity
 Occurs primarily in African-Americans
o Occurrence 1 in 375 infants born in US
 o 1 in 12 have sickle cell trait
o Occasionally also in people of
Mediterranean descent
o Also seen in South American, Arabian,
and East Indian descent
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 trait)
o 1 in 12 African-Americans are carriers (have
sickle cell
o If both parents have trait, each offspring will
have 1 in 4 likelihood of having disease
Pathophysiology
 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
Clinical features result of:
 Obstruction caused by sickled RBCs
 Increased RBC destruction
Most complication can be traced to this process & its
impact on various organs of body
 Stasis with enlargement
 Infarction with ischemia & destruction
 Replacement with fibrous tissue (scarring)
Pathophysiology with complications
 Large tissue infarctions occur
 Damaged tissues in organs lead to impaired function
o Splenic sequestration
○ May require splenectomy at early age
○ Results in decreased immunity
Clinical manifestations
 General: possible growth retardation
o Chronic anemia (hgb 6-9g/dl)
o Possible delayed sexual maturation
o Marked susceptibility to sepsis
 Vaso-occlusive crisis:
o Pain in areas of involvement
o Manifestations related to ischemia:
o Extremities: painful swelling hands/feet (sickle
cell dactylitis/ "hand-foot syndrome"), painful
joints
o Abdominal pain: like surgical condition
o Cerebrum: stroke, visual disturbances - Chest
like PNM
o Liver: jaundice, hepatic coma
o Kidney: hematuria
o Genital: priapism (painful penile erection)
 Sequestration crisis: pooling large amounts of blood
o Hepatomegaly
o Splenomegaly
o Circulatory collapse
 Effects of chronic Vaso-occlusive Phenomena
o Heart: cardiomegaly, systolic murmurs
o Lungs: pulmonary infections/ insufficiency
o Kidneys: inability to concentrate urine,
progressive renal failure, enuresis
o Liver: hepatomegaly, cirrhosis, intrahepatic
cholestasis
o Eyes: visual disturbances, retinal detachment,
blindness
o Extremities: lordosis/ kyphosis, chronic leg ulcers,
osteomyelitis
o CNS: hemiparesis, seizures
SICKLE CELL CRISIS
Precipitating factors
 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
o From high altitude, poorly pressurized
airplanes, hypoventilation, vasoconstriction
due to hypothermia
 Acute exacerbations that vary in severity and
frequency
Types
 Vaso-occlusive (VOC) thrombotic
o Most common type of crisis - very painful
o Stasis of blood with clumping of cells in
microcirculation → ischemia infarction
o Signs: fever, pain, tissue engorgement
 Sequestration crisis:
o Pooling of blood in liver & spleen
o Decreased blood volume
o Shock
 Splenic sequestration
o Life threatening - death can occur within hours
o Blood pools in the spleen
o Signs:
○ Profound anemia, hypovolemia, shock
 Aplastic crises
o Diminished production and increased destruction
of RBCs
o Triggered by viral infection or depletion of folic
acid
o Signs include profound anemia, pallor
 Hyperhemolytic crisis:
o Accelerated rate RBC destruction
o Anemia, jaundice, reticulocytosis
o Other coexisting conditions: viral illness, G6PD
deficiency
Acute Chest Syndrome
 Similar to PNM
 New pulmonary infiltrate
 Severe chest, back, abdominal pain
 Fever > 38.5°C, very congested cough
 Tachypnea, dyspnea, wheezing, hypoxia
 Retractions
 Declining oxygen saturation
 Serious complication
Cerebrovascular accident (CVA)
 Stroke
 Sudden & severe complication
 No related illnesses
 Sickled cells block major blood vessels in brain →
cerebral infarction - neurologic impairment
  Repeat CVAS: greater brain damage
o 70% untreated children
 Severe, unrelieved headaches
 Severe vomiting
 Jerking/twitching of face, legs, arms
 Seizures
 Strange abnormal behavior
 Inability to move arm/leg
 Stagger or an unsteady walk
 Stutter/ slurred speech
 Weakness in hands/feet/ legs
 Changes in vision
Diagnosis of sickle cell
 Cord blood in newborns
 Newborn screening done in 43 states
 Genetic testing to identify carriers and children
who have disease
 Sickle turbidity test
o Quick screening purposes in children older
than 6 months
 Family is taught to administer prophylactic
antibiotics & identify early signs of infection to seek
medical therapy ASAP
 If not diagnosed early, symptoms manifest during
toddler & preschool years
 Occasionally 1st diagnosed during a crisis that
follows URI or GI infections
Therapeutic management
Goals:
 Prevent conditions that enhance sickling
phenomena responsible for the pathologic
sequalae
 Treat medical emergencies of sickle cell crisis
Medical management
 Supportive and symptomatic
 Provide rest to minimize energy expenditure &
oxygen use
 Hydration oral & IVF
 Electrolyte replacement - hypoxia results in
metabolic acidosis → sickling
 Analgesics-severe pain from vaso occlusion
 Antibiotics to treat any existing infection
o Aggressive treatment of infection
o Possible prophylactic antibiotics from 2
months - 5 years
o Pneumococcal & meningococcal vaccines,
flu vaccines
o Oral penicillin prophylaxis: by 2 months of age
 Monitor reticulocyte count regularly to evaluate
bone marrow function
 Blood transfusion, if given early in crisis, may
reduce ischemia
o Exchange transfusion: reduces # circulating
sickle cells
 Frequent transfusion leads to hemosiderosis (iron in
tissues)
o Treat with iron-chelation such as feroxamine +
vitamin C to promote iron excretion
o Rx-hydronurea (cytotoxic) leads to
decreased production of abnormal blood
cells and less apin
o Splenectomy-splenic sequestration
○ Functional asplenia (spleen atrophies
on its own) routine splenectomy not
recommended
o Painful priapism: aspiration corpora
cavernosum
SICKLE CELL PAIN (Vaso-occlussive pain)
 Greatly affects development
 Multidisciplinary
 Mild to moderate pain: ibuprofen/
acetaminophen +/- codeine
 Severe: opioids
 Patient controlled analgesia
Nursing Management
 Keeping child well hydrated and oxygenated
 Avoid tight clothing that could impair circulation
 Keep wound clean and dry
 Provide bed rest to decrease energy expenditure
and oxygen use
 Correct metabolic acidosis
 Administer medications as ordered:
o Analgesics: Acetaminophen, meperidine,
morphine (avoid aspirin as it enhances
acidosis, which promotes sickling)
o Avoid anticoagulants (sludging is not due to
clotting)
o Antibiotics
 Administer blood transfusions as ordered
 Keep arms and legs from becoming cold
 Decrease emotional stress
 Provide good skin care, especially to legs
 Test siblings for presence of sickle cell trait/ disease
 Provide client teaching and discharge planning
concerning:
o Pre-op teaching for splenectomy if needed
o Genetic counseling
o Need to avoid activities that interfere with
oxygenation, such as mountain climbing,
flying in unpressurized planes
APLASTIC ANEMIA
 A serious condition in which the bone marrow
does not produce enough new blood cells.
 May be passed down from the parents or develop
sometime during childhood.
 Develop at any age.
 May occur suddenly, or it can occur slowly and
get worse over a long period of time.
 Symptoms include tiredness, paleness, frequent
infections, and easy bruising and bleeding.
With fewer blood cells:
 Less oxygen sent to organs, tissues and cells (from
too few red blood cells)
 Increased risk of infection (from too few white
blood cells)
 Increased risk of bleeding problems (from too few
platelets)
 Cause is unknown
 Inherited
Acquired Causes (Primary):
 Infection. (Hepatitis or liver infection, and other
different viral illnesses, such as Epstein-Barr virus
(EBV), cytomegalovirus (CMV), parvovirus B19, or
human immunodeficiency virus (HIV))
 Cancer. Some cancers affect the bone marrow.
 Autoimmune disease. For example, lupus and
rheumatoid arthritis
 Medications.
  Toxins. For example, heavy metals, pesticides, o Avoid intramuscular injections
benzene o Hematest urine and stool
 Radiation therapy and chemotherapy. For the o Observe for oozing from gums, petechiae, or
treatment of cancer. ecchymoses
 Provide client teaching and discharge planning
Secondary Causes: concerning
 Paroxysmal Nocturnal Hemoglobinuria (PNH) o Self-care regimen
o Causes RBC to break down too soon. o Identification of offending agent and
o Paroxysmal nocturnal hemoglobinuria can importance of avoiding it.
lead to aplastic anemia, or aplastic anemia  The child should avoid:
can evolve into paroxysmal nocturnal o Activities that increase the chance of
hemoglobinuria. infection or bleeding.
 Fanconi's anemia o Staying away from people who are sick
o A rare, inherited disease that leads to aplastic o Eating uncooked foods
anemia. o Contact sports (for example, football, hockey,
o Children born with it tend to be smaller than skiing, or rollerblading)
average and have birth defects, such as o Traveling to high altitudes (children with a low
underdeveloped limbs. red blood cell count will have increased
fatigue and need for oxygen in high altitudes)
Clinical Manifestations:
 From decreased red blood cells:
o Headache
o Dizziness
o Shortness of breath
 Lack of energy or tiring easily (fatigue)
o Pale skin
o Chest pain
o Irregular heart beat
o Enlarged heart
 From too few white blood cells:
o Fevers
o Mouth sores
o Infections
 From too few platelets:
o Easy bruising
o Nosebleeds
o Bleeding gums
o Blood in the stool
o Heavy bleeding with menstrual periods
 Other symptoms:
o Nausea
o Skin rashes

Blood tests
 Hemoglobin and hematocrit
 Complete blood count, or CBC (RBC, WBC,
Platelets, and Reticulocytes
 Peripheral smear
Bone marrow aspiration and/or biopsy

Medical Management
 Blood transfusions (until bone marrow begins to
produce blood cells)
 Aggressive treatment of infections
 Blood and Bone marrow Stem cell transplantation
 Drug therapy
o Corticosteroids and/ or androgens to
stimulate bone marrow function and to
increase capillary resistance.
o Estrogen and/or progesterone to prevent
amenorrhea in female clients
 Identification and withdrawal of offending agent
or drug

Nursing Management:
 Monitor for signs of bleeding and provide
measures to minimize risk
o Use a soft toothbrush