Ch.

12: Health Promotion of the Child and Family

cognitive development: sensorimotor and preoperational phase (piaget)
Growth and development during the toddler years
Ch. 13: Health Promotion of the Preschooler and Family
Social development
coping with concerns related to normal growth and development
Ch. 14: Health Problems of Toddlers and Preschoolers
Communicable diseases
selected poisonings in children
lead poisoning
physical abuse
Nursing care of the Maltreated child
CH 25: Pediatric Cardio

<---- normal heart

Cardiovascular dysfunction
Congenital Heart Diseases- defects present at birth
Acquired Cardiac Disorders-disease processes or abnormalities occurring after birth.
H & P examKids with Heart problems may have the following:
failure to thrive or they may be skinny kids d/t poor wt gain
cyanosis
chest deformities
visible pulsations of neck veins
respiratory excursion (tachypnea, dyspnea, expiratory grunt)
clubbed fingers
Nurse may find the following upon palpation, percussion and auscultation
hepatomegaly and/or splenomegaly
irregular heart rates, rhythms or sounds
Diagnostic eval (see p. 821 for complete list)
Most common are X-Ray, Electrocardiogram, Echocardiography, Cardiac
Catheterization, Cardiac MRI, Exercise stress test.
ECG-non-invasive and painless
Echo-used frequently

non-invasive and painless

possible to confirm dx of heart malformation with echo
kid has to be quiet otherwise errors or omissions may occur
Cardiac Catheterization-surgical intervention
may be outpatient
done prior to school-age
Nursing care for Cardiac cath.
Preop
diaper rash-this may be a reason to cancel the procedure
npo 4-6hr before procedure
prepare both kid and family-geared toward childs developmental level
mark pulses distal to insertion site
document baseline O2 sat
Postop
cardiac monitor and O2 for few hours after sx
recovery time-keep limb immobilized and straight
4-6hr for venous cath
6-8hr for arterial cath
Nurse monitor for
pulses-esp distal to site
temp/color of affected limb
VS
BS levels
Fluid intake
infants and kids w/polycythemia risk for dehydration/hypoglycemia
Dressing:
1. remove pressure dressing (usu doc) day after sx
2. cover with band-aid
3. keep site clean and dry-no tubs but can shower
4. observe for bleeding/fever-notify physician if occurs
5. avoid strenuous activity for a few days- but go to school
6. asa or ibuprofen for pain
Bleeding?
-apply direct pressure 1 inch above insertion site
Cardiac changes at birth
Fetus- Baby transitions in circulation and breathingclamping cord systemic BP lung expansion decreases pulmonary vascular resistance
(and increases pulmonary perfusion)
increase in O2 and decreased prostaglandin causes vasoconstriction of PDA
o increased pressure on L side closes foramen ovale
Foramen ovale- pathway from right atrium to left atrium through this tunnel
Ductus arteriosus- blood is pumped through the pulmonary artery where a major portion
is shunted to the descending aorta via this hole.
Classification of defects:

Acyanotic
1) Increased Pulmonary Blood Flow
blood flows in a L-to-R direction
ex: ASD, VSD, Patent Ductus Arteriosus, Atrioventricular canal
2) Obstruction of blood flow from ventricles
impedes blood flow out of the ventricles
Left sided obstruction-HF
Right sided obstruction- cyanosis
ex: Coarctation of aorta, Aortic stenosis, pulmonic stenosis

Cyanotic
3) Decreased pulmonary blood flow
ex:Tetralogy of Fallot, tricuspid atresia
4) Mixed blood flow
ex:Transposition of great arteries, total anomalous pulm. venous return, truncus
arteriosus, hypoplastic left heart syndrome

Congenital Cardiovascular Dysfunction: Increased Pulmonary blood flow- 4 types

1. Atrial Septal Defect (ASD)

Acyanotic disorder
Abnormal opening btwn R and L atriao blood moves from higher conc (L) to lower conc (R).
o Pulmonary HTN/reduced blood volume in systemic circulation
3 types
o Ostium Primum (ASD1)- opening @ lower end of septum-poss. mitral
valve abnormalities
o Ostium Secundum (ASD2)- opening near center of septum
o Sinus Venosus Defect- opening near junction of SVC and R atriumpossible partial anomalous pulmonary venous connection
S/S
o asymptomatic
o may develop HF
o ***Systolic murmur***w/ fixed split 2nd heart sound
o may have diastolic murmur
o @ risk for
Atrial dysrhythmias
pulmonary HTN
emboli/strokes later in life
Treatment

Surgery- patch closure for moderate to large holes use Dacron or

pericardial patch
o Non-surgery- septal occlusion during cardiac catheterization
o repair completed before school age
o No activity restrictions after repair
Medications/prognosis
o Low dose ASA for 6 months after repair
o

2) Ventricular Septal Defect (VSD)

Acyanotic disorder
Most Common Abnormal opening btwn L and R ventricles- blood moves from
high pressure (L) to low pressure side of heart
classified according to location on septum
vary in size- most small holes close w/in first year of life
frequently occur with other problems
o pulmonary stenosis
o transposition of great vessel
o Patent Ductus Arteriosis
o atrial defects
o coarctation of aorta
S/S
o HF
o Loud murmur heard on L sternal border
o At risk for BE (?) and Pulmonary Vascular Obstructive Disease
Treatment
o Non surgical repair done during cardiac cath
o complete repair is tx of choice
small defects fixed with sutures
large defects fixed with knitted dacron patch
CardioPulmonary Bypass machine used in both cases
Complication of sx:

conduction issues
residual VSD
Medications/Prognosis
good, depending on location/size of hole
multiple holes are associated with higher risks

o
o
o

3) Atrioventricular Canal Defect

Acyanotic Disorder
Incomplete fusion of endocardial cushions
o low ASD w/ high VSD creating a large central AV valve
o allows blood to flow btwn all four chambers
direction of flow is determined by
o pulmonary/systemic resistance
o L/R vent. pressures
o compliance (stretch) of each chamber
common defect seen in Down Syndrome kids
S/S
o moderate to severe HF
o loud systolic murmur
o mild cyanosis that increases with crying
o high risk for pulmonary vascular obstructive disease
Tx
o Complete repair
most commonly done in infancy
patch closure of septal defect
reconstruction of AV valve tissues
o Palliative
pulmonary artery banding done in small infants with severe symptoms
o Post op complications
Heart block
HF
Mitral regurgitation
dystrhytmias
pulmonary HTN

Medications/Prognosis
o good may have mitral valve replacement later in life due to mitral regurgitation

4) Patent Ductus Arteriosus (PDA)

Acyanotic Disorder
failure of closure of artery connecting high pressure Aorta to low pressure
Pulmonary Artery within first few weeks of life
Continued patency of this vessel causes a L-R shunt
S/S
o Asymptomatic
o sometimes HF is seen
o characteristic machine like murmur heard
o widened and bounding pulse pressures
o @risk for BE (?) and Pulmonary Vascular Obstructive disease
Tx
o Non-Sx
coils to occlude PDA placed via cardiac cath
preterm or small infants may require sx
o Sx
surgical diversion or ligation via left thoracotomy
(new) video thoracoscopic sx---> 3 incisions to left chest, clip
placed on the ductus has proven to have speedier recovery
Medications/Prognosis
o Indomethacin (prostaglandin inhibitor) used in preterm infants and some
newborns has proven successful in closing this PDA
o Good outcomes with these babies
Congenital Cardiovascular Dysfunction: Obstructive Disorders (3 types)

1. Coarctation of the Aorta (COA)

o Acyanotic
o narrowing near the ductus arteriosus of aorta
o results in increased pressure to head and arms/decreased pressure to trunk and
legs
o S/S
HBP
bounding pulses in arms
weak/absent pulses in legs
cool to touch legs
signs of HF in infants
critical coarctation in infants can deteriorate quickly when acidosis and
hypotension occur
mechanical ventilation and inotropic support are needed before surgery
older kids with coarctation may experience
dizziness, HA, fainting, epistaxis (nosebleeds) form HTN
@ risk for HTN, ruptured aorta, aortic aneurysm, and stroke
o TX:
Non-sx: infants and little kids get balloon angioplasty
adolescents get stents
Sx: want to do before 6mo of age
indicated if stenosis is long or other complex anatomy are present
repair is by resection of coarcted porton w/ end-to-end
anastomosis of the aorta with enlarged portion of constricted area
via use of graft of left subclavian artery
o Medications/prognosis
to prevent post-op HTN:
Sodium Nitroprusside
Esmolol
Mirinone
followed by oral
beta blockers
ace inhibitors

2. Valvular Aortic Stenosis

o Acyanotic
o Narrowing or stricture of aortic valve causing
resistance to blood flow in left ventricle
decreased cardiac output
pulmonary vascular congestion
***Left Ventricular Hypertrophy
Primary manifestation
eventually leads to increased End Diastolic pressure resulting in
Pulmonary venous/arterial HTN
may also interfere w/ coronary artery blood flow resulting in MI
considered a serious defect b/c
obstruction is progressive
sudden episodes of myocardial ischemia occur
surgical repair rarely results in a normal valve
S/S
Infants present with
decreased CO
faint pulses
hypotension
tachycardia
poor feeding
kids present with
exercise intolerance
dizziness when standing
chest pain
may or may not have systolic murmur
@ risk for BE (?), coronary insufficiency, ventricular dysfunction
TX:
Sx: balloon dilation done in cath lab
Aortic valve replacement--->may temp. fix problem
surgical complications
aortic insufficiency, valvular regurgitation, tearing of valve leaflets,
loss of pulse in catheterized limb
Medications/Prognosis
Meh- will be recurrent and require additional surgeries throughout life.

3) Pulmonary Valve stenosis

Acyanotic
narrowing at entrance to Pulmonary artery
causes sided ventricular hypertrophy and decreased artery blood flow
Pulmonary atresia is rare and very serious
no pulmonary valve- is completely closed and there is no blood flow to lungs
S/S
o Asymptomatic
o Mild cyanosis or HF
o progressive narrowing causes increased symptoms
o loud systolic ejection murmur heard at (L) sternal border
o In severely ill kids, murmur may be muffled due to decreased CO and shunting of
blood
o @ risk for BE (?)
TX:
o Non-sx: Balloon angioplasty in Cardiac Cath lab-tx of choice
o sx: transventricular (closed) valvotomy- rare procedure
Medications/Prognosis
o Good! but have have valve incompetencies or valve restenosis
Congenital Cardiovascular Dysfunction: Defects with Decreased Pulmonary Blood Flow (2
types)

1. Tetralogy of Fallot (TOF)

o Cyanotic
o 4 characteristic defects

VSD
Pulmonic stenosis
Right Vent. Hypertrophy
Overriding Aorta
aorta lies over ventricular defect
Shunt direction depends on difference between systemic/pulmonary vascular
resistance
S/S
Moderate systolic murmur
Mild cyanosis at birth-progresses as pulmonic stenosis worsens
**Blue/Tet spells**
acyanotic spells occur when O2 demand exceeds blood supply
often occurs w/ feeding/crying
Child may assume squatting position to relieve symptoms
@ risk for emobli, sz, loc, sudden death after an acyanotic spell
Tx:
Complete repair
elective surgery done within 1st year of life, indicated when tet
spells increase and if hypercyanotic spells develop
Palliative shunt
indicated when infant cannot undergo complete repair
Blalock-Taussig shunt operation-tube graft providing blood flow to
pulmonary artery from L or R subclavian artery
last resort b/c shunts may distort pulm. artery
Medications/prognosis
Good! lifelong follow up visits w/ Cardiologist
through improved sx techniques, lower incidences of dysrhythmias and
sudden death are rare. but are still a post op risk

o
o

2. Tricuspid Atresia
o Cyanotic

tricuspid valve fails to develop

blood has to flow through an ASD or to a patent foramen ovale to left side of
heart and through a VSD to the ventricle and out to lungs.
o complete mixing of un-O2 + O2 blood on (L) side results in systemic desaturation
and pulmonary obstruction- Decreasing pulmon. blood flow.
o S/S
Infants
cyanosis, tachycardia, dyspnea
Children
chronic hypoxemia w/ clubbing
TX:
Palliative
pulmonary to systemic artery shunt-first surgery
Glenn Shunt
bidirectional shunt performed at 4-9 months as 2nd stage
surgery
Modified Fontan Procedure
separates O2 and un-O2 blood inside heart and eliminates
excessive volume load on ventricle but does not restore
anatomy or hemodynamics.
Medications/Prognosis
o Neonates w/dependent pulmonary blood flow through
ductus arteriosus need continuous infusion of
Prostaglandin E1- 0.1mcg/kg/min until surgery can be
performed
o Fairly good outcome
post-op complications: dysrhythmias, systemic venous HTN,
Pleural/pericardial effusions, Ventricular dysrhythmias.
Long term concerns: Development of protein losing enteropathy,
late ventricular dysfunction, developmental delays.
Congenital Cardiovascular Dysfunction: Mixed Defects (4 types)
o
o

1)Transposition of the great arteries or great vessels (TGA)

Cyanotic
basically a switching of Aorta and Pulmonic Artery
Blood flow

O2 poor blood enters atrium, flows through ventricle and back to body via
aorta
o O2 rich blood eters (L) atrium, flows through (L) ventricle and back to lungs via
pulmonary arteries
2 separate closed systems **unless ASD or PDA is present. if these are present, at least
*some o2 rich blood can enter body.
S/S
o cyanotic at birth
unless PDA/ASD is present then they appear less cyanotic but have
symptoms of HF
o heart sounds vary
o cardiomegaly seen w/in 1st few weeks after birth
TX:
o Arterial switch procedure
transection and reanastomosis of ALL vessels in heart-->really detailed
sx...refer to p. 832 if you want more info.
Medications/Prognosis
o good!
o potential long term probs: suprapulmonic stenosis, neo-aortal dilation and
regurgitation
o

2)Total anomalous pulmonary venous connection (TAVPC)

Cyanotic
Pulmonary veins do not connect normally to L atrium
Pulmonary Veins return O2 rich blood to Superior Vena Cava via connecting vein
No O2 rich blood is going to body
ASD is advantageous to O2 rich blood delivery to body

when o2 delivery occurs, most likely ASD is present and is considered

unobstructed TAPVC

o
o
o

cyanosis early in life

infant rapidly deteriorated w/o immediate intervention
may be asymptomatic
vascular resistance decreases in infancy causing increase in pulmonary
blood flow resulting in S/S of HF
cyanosis worsens when obstruction occurs (no asd present)

S/S

TX:
pulmonary vein moved to back of Left atrium and ASD closed
connecting vein removed
post-op complications: reobstruction, bleeding, dysrhythmias, heart block,
pulmonary artery HTN, persistent HF
Medications/Prognosis
o <10% mortality when intervention is swift
o
o
o

3) Truncus Arteriosus
failure of normal separation of aorta and pulmonary artery
development of single vessel
blood mixes and leads to desaturation and hypoxemia
3 types
o type 1- single pulmonary trunk-->divides into L/R pulm. art.
o type 2- L/R pulmonary arteries arise separately but from same location
o type 3- pulmonary arteries arise independently
S/S
o Severe HF
o variable cyanosis
o poor growth
o activity intolerance
o holosystolic murmur @ left sternal border w/diastolic murmur is truncal
regurgitation is present

Tx:
Repair performed w/in 1st month of life
close VSD
Cut pulmonary artery from aorta and attach to ventricle
use of homograft when attaching Pulm. Art to Vent.
homograft is cadaver material and is not living/will not grow with child/will
have to be replaced
Medications/Prognosis
o Good!
o Future Sx will be necessary to replace cadaver conduit.
o
o
o

4) Hypoplastic Left Ventricular Syndrome (HLVS)

underdeveloped left side of the heart
results in small sized left ventricle and aortic atresia
blood is shunted to atrium via Patent foramen ovale/Atrial septal defect
Descending aorta receives blood via Patent Ductus Arteriosus supplying systemic blood
flow
S/S
o Signs of HF until PDA closes----> then progressive deterioration occurs
w/cyanosis and decreased CO. leading to cardiac collapse
o Fatal w/o intervention
TX:
o multiple stage approach
Norwood procedure- right ventricle becomes the systemic ventricle
pumping blood to body
Glenn Procedure- 3-6mo.
Modified Fontan procedure done at 2-3 years
o transplant
Medications/Prognosis
o Fatal without intervention

o
o

neonate will need ventilator and cardiac support until surgery

Prostaglandin E1 will be continuously infused to ensure adequate blood flow

Acquired Heart Diseases

Bacterial Endocarditis
o c/b bacteria entering blood stream causing infection to inner lining of heart,
generally involving valves
o Increased risk for kids with valvular abnormalities, prosthetic valves, shunts,
recent cardiac surgery, rheumatic heart disease w/ valve involvement
o S/S
slow onset, low intermittent fever, anorexia, malaise, wt. loss, cardiac
dysrhythmias, new or change in murmur, extracardiac emboli (splinter
hemorrhages on nails, osler nodes, janeway lesions, petechiae on oral
mucous membranes), vegetations seen on echo and may have HF
o TX:
ABX and Prevention in high risk kids
-cillins and mycins prove effective abx tx
in serious cases iv abx indicated
Nurse needs to educate parents on S/S of anorexia, wt. loss and malaise.
Rheumatic Fever
o inflammatory disorder-self limiting involvement of joints, skin, brain, serous
surfaces and heart
heart most serious complication-mitral valves compromised
o Diagnosis based on Jones criteria-suggest the presence of two major and one
major and two minor symptoms w/ recent strep infxn-high prob. of RF
o TX:
Kill strep infxn
prevent permanent damage
alleviate other sxs
prevent recurrences of RF w/ penicillin drugs and/or salicylates for joint
inflammation
o Nursing care
encourage medication compliance
facilitate recovery
provide emotional support
prevent disease
o Chorea is side effect and may develop weeks to months after infxn
is sudden and uncontrollable movements
can be frustrating for child
Kawasakis Disease
o acute systemic vasculitis of unknown cause
o not spread from person to person
o occurs in 3 phases:
Acute: abrupt onset w/ edema, redness of palms/soles and high fever
unrelieved by abx or antipyretics.

Subacute: begins when fever resides, and lasts until all clinical signs have
resolved.
feet and hands often peel at this stage
kid is @ high risk for developing cardiac complications (MI,
Coronary artery aneurysms) at this stage
Convalescent: all clinical signs are gone but blood values are still
abnormal.
**Long term complications are aortic aneurysms w/ potential for MI
s/s of aortic aneurysm: abd pain, vomiting, restlessness,
inconsolable crying, pallor, shock and chest pain/pressure
medications:
High dose IV Gamma Globulins and Salicylate therapy given in
hospital
Low dose ASA given out of hosp. esp. when cardiac involvement
is suspected.
Nursing care:
nurses must know irritability is hallmark of KD and most
challenging problem
Majority of nursing care is directed on symptomatic relief
During acute subacute phase- watch carefully! kid is at high risk
for cardiac complications!
Patient education
tell parents to prolong vax 11 months due to current unknown
immune compromise
will need lifelong medical follow up depending on amount of
cardiac involvement

Chapter 26
Anemia
describes a condition in which the number of RBCs or hemoglobin concentration
is reduced below normal values for age. Thus diminishing the oxygen carrying
capacity. *Most common hematologic d/o in infancy/childhood
Classification
Etiology: manifested by erythrocyte or Hgb depletion
Morphology: characteristic changes in RBC size, shape or color
Classifications of anemia

Consequences of Anemia: basic physiologic defect caused by anemia is

decrease in the oxygen-carrying capacity of blood and decreased amount of
oxygen available to tissues.
Effects of anemia on the circulatory system
Hemodilution: decreased peripheral resistance, greater quantity of blood
return to the heart
Increased cardiac circulation and turbulence: may have a murmur, may
lead to cardiac failure
cyanosis is not typical
growth retardation: delayed sexual maturation
Diagnostic Evaluation:
History and physical examination: lack of energy, easy fatigability, and
pallor
CBC: decreased RBC, decreased Hgb/Hct. Hgb: less than 10 or 11g/dL
other tests can be done to determine the particular type of anemia.
Management of Anemia:
Treat the underlying cause: transfusion after hemorrhage if needed;
nutritional intervention for deficiency anemias
Supportive Care: IV fluids to replace intravascular volume, oxygen, bed
rest, diet, family education
Nursing Considerations: prepare the child and family for lab tests, decrease
oxygen demands, prevent complications, support and educate family.
Iron Deficiency Anemia

based on diet-inadequate supply of dietary iron.

predictable development periods
Premature infants: at risk b/c reduced fetal iron supply
infants: at risk as a result of primarily cow milk intake and not eating an
adequate amount of iron-containing formula/food
adolescents: due to rapid growth weight combined with poor eating habits,
menses, obesity or strenuous activities
Symptoms: pale skin, irritability, tachycardia, fatigue, glossitis (inflammation of
the tongue), Koilonychia (concave or spoon fingernails)
Therapeutic management:
Infants: iron-fortified formula, do not give fresh cow milk,
Children: addition of iron-rich foods, oral iron supplements are prescribed
and must be given with vit C because it facilitates absorption of iron.
Nursing considerations: oral iron should be given as prescribed in two divided
doses btwn meals, always give with citrus juice never milk, turns stool tarry
green, use straw so iron doesnt stain teeth, N/V may occur, supplementation.
*primary nursing objective is to prevent nutritional anemia through family
education
Sickle Cell Anemia
is one group of diseases collectively termed hemoglobinopathies in which normal
adult Hgb is partily or completely replaced by abnormal sickle Hgb
Hereditary d/o whose clinical, hematologic and pathologic features are related to
the presence of HbS
occurs primarily in AA, occasionally ppl of mediterranean descent, south
american, arabian and east indian descent
Etiology:
Autosomal recessive d/o: one in 12 AA is a carrier, if both parents have
trait each child with have a 1 in 4 likelihood of having dz
Patho: leads to tissue damage throughout body
Diagnostic Evaluation
newborn screening is mandatory in most of the US so infants can be
identified before symptoms occur. early diagnosis before 3mo enables
initiation of appropriate interventions to minimize cx
test cord blood in newborns, genetic testing to identify carriers and
children who have the dz
sickle turbidity test: fingerstick, yields accurate results in 3 min in children
over 6mo of age
hemoglobin electrophoresis: fingerprinting of the protein. accurate, rapid
and specific test for detecting the homozygous and heterozygous forms of
dz.
Therapeutic Management:
Goals: prevent sickling phenomena and tx medical emergencies
analgesics for pain

bed rest with mild range of motion

hydration to thin blood
minimize infections with antibiotics
hydroxurea: disease modifying drug
blood transfusion: replacement of sickle cells with normal RBCs,
successful, rapid method of reducing the number of circulating sickle cells
thus slowing down the hypoxia
SICKLE CELL CRISIS: acute exacerbations that vary in severity and frequency
Precipitating factors: anything that increases the bodys need for oxygen or alters
the transport of oxygen, trauma, fever/infxn, physical and emotional stress,
increased blood viscosity due to dehydration, hypoxia (results from high altitude,
poorly pressurized airplanes, hypoventilation, vasoconstriction due to
hypothermia).
Types:
o Vaso-occlusive (VOC): most common and painful. characterized by
ischemia causing mild to severe pain that may last from minutes to days.
Stasis of blood and clumping cuts off circulation in sm vessels. S/S: fever,
pain, tissue engorgement
o Splenic Sequestration: pooling of large amount of blood usually in the
spleen and infrequently in the liver that causes a decreased blood volume
and ultimately shock. life threatening, causes profound anemia and
hypovolemia
o Aplastic Crises: diminished RBC production usually caused by viral
infection that may result in profound anemia and pallor
o Acute Chest Syndrome: similar to pneumonia. presence of new pulmonary
infiltrate (sickling in the lungs), S/S: chest pain, fever, cough, tachypnea,
wheezing hypoxia
o Cerebrovascular Accident CVA: sudden and severe complication, often
with no related illnesses. sickled cells block the major blood vessles in the
brain resulting in cerebral infarction
TX: HOP (Hydration, Oxygen, Pain relief)
B-Thalassemia (cooleys anemia)
Inherited blood disorders of hemoglobin synthesis classified by the hemoglobin
chain affected and by the amount of effect. Autosomal recessive disorder with
varying expressivity. Both parents must be carriers to have offspring with the
major form of the disease. Occurs in Greeks, Italians and Syrians
Four Types:
o Thalassemia minor: Asymptomatic and silent, heterozygous
o Thalassemia trait: mild microcytic anemia, heterozygous
o Thalassemia intermediate: moderate to severe anemia plus splenomegaly
o Thalassemia major: (cooley anemia) severe anemia requiring transfusions
for survival. cardiac failure and death in early childhood w/o transfusions
Patho:

o anemia results from defective synthesis of hemoglobin, structurally

impaired RBCs and a shortened life of RBCs
o Chronic hypoxia: headache, irritability, precordial and bone pain, exercise
intolerance, anorexia, epistaxis (bleeding from the nose)
o Detected in infancy or toddlerhood: pallor, failure to thrive,
hepatosplenomegaly, severe anemia-Hgb less than 6g/dL
Medical management of thalassemia major
o blood transfusion to maintain normal Hgb levels
o side effect hemosiderosis: excess iron from hemolysis of supplemental
RBCs in transfusions and from the rapid destruction of defective cells is
stored in various organs
Tx: iron-chelating drugs such as deferoxamine (Desferal), binds
excess iron for excretion by the kidney
Aplastic Anemia
refers to bone marrow failure condition in which the formed elements of the blood
are simultaneously depressed. Pancytopenia: all formed elements of the blood
are depressed
Can be Congenital or Acquired: most are idiopathic
Common Causes: human parvovirus infxn, hepatitis or overwhelming infxn,
irradiation, immune disorders such as eosinophilic fasciitis and
hypoimmunoglobulinemia, drugs (chemo agents, anticonvulsants, and abx),
industrial or household chemicals, infiltration and replacement of myeloid
elements, idiopathic.
Manifestations: anemia, leukopenia, decreased platelet count
Dx: CBC, reticulocyte count, bone marrow aspiration
Tx: restore function to marrow, immunosuppressive therapy or bone marrow
transplant
Hemophilia
a group of hereditary bleeding disorders that result from deficiencies of specific
clotting factors. Typically an X-linked recessive pattern. Affects males only and
Females are carriers. Fibrin clot cannot form
Types
o Hemophilia A: only males get but transferred by mothers. classic
hemophilia (deficiency of clotting factor 8). Accounts for 80% of cases of
hemophilia.
o Hemophilia B: christmas disease deficiency in factor IX
o von Willebrand disease (vWD): deficiency, abnormality or absence o vWf
and factor 8. affects both males & females

Manifestations: bleeding tendencies range from mild to severe

o S/S: may not occur until 6 mo of age, history of bleeding, nosebleeds and
bruising. Prolonged bleeding,

o Hemarthrosis: bleeding into joint spaces of the knee, ankle or elbow, leads
to impaired mobility and eventually bony changes and disability. s/s:
warmth, pain, bruising, and decreased movement.
o spontaneous hematuria and hematoma

Medical management:
o replacement of missing clotting factors (factor 8 concentrates or synthetic
form of vasopressin 1-deamino8-d-arginine vasopressin DDAVP)
IV, increases factor 8 activity by two or four times used for mild
hemophilia. cant use for severe anemia
o transfusions: prompt intervention to reduce complications
o medications: corticosteroids
o exercise and physical therapy
o RICE (Rest, Ice, Compression, Elevate)
Leukemias
describes a broad group of malignant diseases of the bone marrow and
lymphatic system. Most common form of childhood cancer. Unrestricted
proliferation of immature WBCs in the blood-forming tissues of the body but lab
levels are low. Bone marrow suppression (myelosuppression) occurs.
Two forms are generally recognized
o Acute Lymphoid leukemia (ALL): children with trisomy 21 have a 20 times
greater risk for developing ALL
o Acute Nonlymphoid (Myelogenous) Leukemia (AML):
o liver and spleen are the most severely affected organs
Assessment of Leukemia: anemia (weakness/fatigue), neutropenia (infxn),
thrombocytopenia (bleeding), spleen and liver enlarged and painful, CNS
symptoms: N/V, lethargy, irritability, convulsions
o Late signs: fractures, bone pain, increased ICP
o 3 main consequences: anemia, infxn, bleeding
Diagnosis:
o usually suspected based on history and physical: fever, s/s of low blood
counts, lymph node enlargement, and enlarged liver/spleen
o peripheral blood smear: immature forms of leukocytes, frequently low
blood counts
o definitive diagnosis is based on bone marrow aspiration or biopsy
o lumbar puncture to evaluate CNS involvement
Management of Leukemia: chemotherapeutic agents and cranial irradiation
o Four Phases:
Induction therapy for 4-6 weeks, achieves complete remission or
less than 5% leukemic cells in the bone marrow
CNS prophylactic therapy (intrathecal chemo): prevents leukemic
cells from invading the CNS
Intensification (consolidation): eradicates residual leukemia cells
Maintenance therapy: serves to maintain the remission phase.

Nursing Management:
o Family-centered care, pain relief, Myelosuppression (infxn control,
bleeding, anemia), Chemotherapy, and Drug toxicity, provide emotional
support
Lymphomas
a group of neoplastic diseases that arise from the lymphoid and hematopoietic
systems, are divided into Hodgkin lymphoma (HL), and non-Hodgkin Lymphoma
(NHL)
Hodgkins disease
o originates in the lymphoid system and primarily involves the lymph nodes.
more prevalent in patients 15-19 years old
o metastasizes to non-nodal or extralymphatic sites: spleen, liver, bone
marrow, and lungs
o classified by lymphocytic predominance, nodular sclerosis, mixed
cellularity and lymphocytic depletion.
o Reed-sternberg cells present, centralized in one-node or around the neck
o S/S: fever, night-sweats, weight-loss
Non-Hodgkin disease:
o more prevalent in children younger than 14 years old
o the disease is usually diffuse rather than nodular, develops in peripheral
lymph nodes or throughout body
o the cell type is either undifferentiated or poorly differentiated,
dissemination occurs early, more often and rapidly, mediastinal
involvement and invasion of meninges are common
o symptoms related to pressure of enlarged lymph nodes
o TX: radiation and chemotherapy
ITP (Immune Thrombocytopenia, Idiopathic thrombocytopenic Purpura)
acquired hemorrhagic disorder characterized by:
o (1) thrombocytopenia: excessive destruction of platelets
o (2) absence or minimal signs of bleeding: easy bruising, mucosal
bleeding, petechiae
o (3) normal bone marrow with normal or increased number of immature
platelets (megakaryocytes) and eosinophils.
Occurs in 2 forms: Acute self-limiting (seen after upper respiratory tract
infections; MMR, chickenpox or infection with parvovirus. Chronic (lasting longer
than 12 months)
Clinical manifestations: easy bruising, bleeding from mucous membranes, and
internal hemorrhage. box 26-6
treatment: primarily supportive b/c the course of dz is self-limited, activity
restrictions (no contact sports), prednisone, IVIG (intravenous immune globulin),
Anti-D antibody (inexpensive, one dose, prolonged survival of platelets,
splenectomy (for severe cases and those not responsive to medications)

Chapter 27
I.

Urinary Tract Infection

A.
B.
C.
D.
E.
F.

II.
II.

opposite of respiratory infection (lower is better higher is worse)

Peak between 2-6 yrs of age
Females are at a higher risk
E.Coli is the most common pathogen
Recurrent may lead to transient vesicoureteral reflux (VUR)
Manifestations
1. strong smelling urine, abnormal stream, frequent or infrequent
voiding, persistent diaper rash
2. classic symptoms greater than 2 years of age
3. many are asymptomatic
G. Diagnostic Evaluation
1. examination of the urine- reveals WBC, presence of the bacterium
1. it is recommended to collect the urine via a bag
B. Therapeutic Management
a. eliminate infections, identify contributing factors, prevent urosepsis,
and preserve renal function
b. abx if indicated
c. antipyretics
d. cultures/UAs every month for 3 months
C. Nursing Care Management
a. Education, detect anatomic defects, girls at higher risk, education
on appropriate use of ABX
D. Prevention
a. most important goal
b. promote health maintenance (safe sex practices, good hygiene)
Defects of the Genitourinary Tract
A. didnt have much in the book, review slides of hydrocele and inguinal
hernia and such
Nephrotic Syndrome- most common presentation of glomerular injury in children
A. Types
a. Minimal Change Nephrotic Syndrome
1. Manifestations- proteinuria, edema, hypoalbuminemia, and
hyperlipidemia
2. most common type
3. Pathophysiology- nephrons become permeable to proteins
causing loss of albumin and then fluid shifts into interstitial
spaces causing edema and ascites
4. Diagnosis- Urinary analysis, blood test for protein levels,
mostly based on symptoms and history
5. Treatment- CORTICOSTEROID THERAPY (2mg/kg/day
divided into two doses), immunosuppressive therapy,
diuretics, regular diet (sodium restriction)
6. Nursing management- catheterize if necessary (strict sterile
technique), strict is&os, elevate parts of body with edema,
encourage fluids to dilute urine, avoid concentrated and

II.

II.

aggravating fluids, cultures following, parent teaching about

home care and diet
b. Secondary Nephrotic Syndrome
c. Congenital Nephrotic Syndrome
Acute Glomerulonephritis
A. Common symptoms- oliguria, edema, HTN and circulatory congestion,
hematuria, and proteinuria
B. Most common cause is strep infection, can also be caused by SLE, sickle
cell, or bacterial endocarditis
C. Primarily in school aged children, 6-7 years of age, uncommon in children
younger than 2 years
D. Pathophysiology
a. minor enlargement, glomerular capillary loops very swollen
b. retention of sodium, which causes retention of fluids
E. Clinical Course- edema usually persists for 4-10 days to 2-3 weeks
a. weight fluxuates, urine remains smoky brown, high BP
b. 1st sign of improvement is a small increase in urinary output
and decrease in body weight
F. Mostly manage symptoms- low sodium and protein, accurate Is & Os,
monitor daily weight, BP monitoring
G. Diagnosis- serologic testing, UA, cultures for Strep, Serum Complement
Level (C3) is the more important diagnostic criteria (decreases then
returns to normal 8-10 weeks after onset
a. can do x-ray, r in rare cases (atypical) renal biopsy
H. Nursing care- nutrition management, fluid monitoring, can stay at home if
no HTN
Wilms Tumor
A. More common in African Americans and males, diagnosed by 3 years
B. Malignant tumor, most often on left kidney
C. Diagnosis comes from family history of cancer, history and physical,
localized abdominal swelling
D. Treatment is surgical removal, chemotherapy, and radiation
E. KEY POINT: NEVER PALPATE THE TUMOR!!!!!!!!!!