Transcript ASSESSMENT

OXYGENATION
in Pediatrics
By
Prof. Unn Hidle
Updated Spring 2010
Congenital Heart Disease
Incidence 5-8/1000 live births
 >35 well-recognized defects, but most
common is Ventricular Septal Defect
(VSD)
 Etiology in 90% of CHD is unknown
 Multifactorial: Genetic and environmental

Acyanotic VS Cyanotic
Cyanosis may occur as a later sign in
“acyanotic” conditions.
 Cyanotic conditions may appear “pink” or
of normal skin color
 Classification system based on
hemodynamic characteristics is better
(blood flow patterns):
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Increased pulmonary blood flow
Decreased pulmonary blood flow
Obstruction to blood flow out of the heart
Mixed blood flow (saturated and desaturated)
“Acyanotic”
Increased pulmonary blood flow OR
obstruction of blood flow from ventricles
 LEFT to RIGHT cardiac shunting (except
when there is an obstruction)
 Often leads to CHF because excess blood
is going to the pulmonary circulation
 Murmurs are commonly auscultated

LEFT-TO-RIGHT CARDIAC SHUNTS

Description
– Blood is shunted to the right side of the
heart because the left side is normally
functioning under a higher pressure
than the right
– Oxygenated and unoxygenated blood
mix, which results in increased
pulmonary blood flow because the
opening sends more blood to the right
side of the heart than normal
Types of L-to-R shunting
Atrial-Septal Defect (ASD)
 Ventricular Septal Defect (VSD)
 Patent Ductus Arteriosus (PDA)
 Atrioventricular Canal Defect (AVCD)

Obstructive/stenotic lesions

Narrowing or constriction of an opening in
a valve or vessel that results in obstruction
of blood flow through the area
TYPES:
 Aortic Stenosis
 Pulmonary Stenosis
 Coarctation of the Aorta
Overall Assessment:
L-to-R or Obstructive
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May be asymptomatic
May show signs and symptoms of CHF
May exhibit failure to thrive
Growth retardation
Diaphoresis
Fatigue
Tachypnea
Poor eating
Dyspnea
Hypoxemia
Implementation
Assess respiratory status for the presence of
nasal flaring and use of accessory muscle
 Auscultate lungs for the presence of crackles and
rhonchi
 Assess for signs of CHF such as fluid retention in
the eyes, hands, feet, and chest
 Assess for diuresis
 Assess urine output; weighing diapers is
necessary
 Assess calorie intake
 Plan interventions to allow maximal rest for the
child
 Allow parent or child if appropriate to verbalize
feelings and concerns regarding disorder

“Cyanotic”

RIGHT to LEFT cardiac shunt
– Occur when blood is shunted to the left side of
the heart because one of the right heart
chambers has a higher pressure
– Oxygenated blood mixes with unoxygenated
blood; cyanosis occurs
– Decreased pulmonary blood flow or mixed
blood flow
– Deoxygenated blood is circulating where
oxygenated blood should

Usually does not lead to CHF
Types of R- to – L shunting
Tetralogy of Fallot (both)
 Transposition of the Great Arteries
 Truncus Arteriosus
 Pulmonary Atresia
 Tricuspid Atresia
 Hypoplastic Left Heart Syndrome

Assessment
– Symptoms occur in the first week of life
– Dyspnea after feeding, crying, or other
activities
– Hypercyanotic or “tet spells” (blue spells)
characterized by increased respiratory rate and
depth and increased hypoxemia with TOF
– Squatting episodes with tetralogy of Fallot
– Signs of CHF
– Respiratory distress
– Clubbing of digits
– Poor growth
– Tachycardia
Implementation
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Monitor vital signs
Monitor respiratory status notifying physician if
any changes occur
Auscultate breath sounds for crackles or rales
Keep child as stress free as possible
If respiratory effort is increased, place child in
reverse Trendelenburg (elevate head and upper
body) to decrease the work of breathing
Monitor for hypercyanosis and place child in
knee-chest position and notify physician
Administer humidified oxygen as prescribed
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Provide endotracheal tube and ventilator
care as prescribed and restrain hands of
intubated child
Monitor for signs of CHF
Monitor body weight (daily weight)
Monitor I&O and notify physician if a
decrease in urine output occurs
Palpate liver noting enlargement, which is
an indication of right-sided heart failure
Administer diuretics as prescribed
CHF – What do you think?

When children develop congestive heart
failure from a congenital heart defect, the
failure is usually:
– Right-sided only
– Left-sided only
– Cor pulmonale
– Both Right- and Left-sided
CONGESTIVE HEART FAILURE

Description
– Inability of the heart to pump sufficiently to
meet the metabolic needs of the body
– Increased right ventricular workload leading to
right sided heart failure
– In infants and children, inadequate cardiac
output is most commonly caused by congenital
heart defects that produce an excessive
volume or pressure load on the myocardium
– In children a combination of both left-sided
and right-sided heart failure is usually present
Congestive Heart Failure

Common causes
– Volume overload – especially in L-to-R
shunting
– Pressure overload – obstructive lesion (i.e.
coarctation of the aorta)
– Decreased contractility – cardiac myopathy or
myocardial ischemia
– High cardiac output demands

Mostly seen in L-to-R shunting
– ASD, VSD, PDA
Congestive Heart Failure
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Assessment of Early and Late Symptoms
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Tachycardia
Arrhythmia – gallop (S3 and S4)
Poor perfusion
Mild cyanosis
Tachypnea leading to dyspnea during feeding
Poor feeding
Poor weight gain due to increased metabolic
rate – FIRST SIGN!
– Activity intolerance
– Diaphoresis during feeding
Congestive Heart Failure
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Retractions
Wheezing
Cough/hoarseness (pressure on the laryngeal nerves)
Orthopnea
Hepatosplenomegaly due to pooling of blood in the
portal circulation and accumulation in the hepatic tissue
Edema / weight gain from Na and H2O retention
Ascitis
Pleaural effusion
Distended neck and peripheral veins from consistent
increased CVP
Prolonged CHF leads to developmental delays
Congestive Heart Failure
Commonly used term for CHF is cor
pulmonale resulting from obstructive lung
disease (i.e. Cystic fibrosis or
bronchopulmonary dysplasia)
 Diagnosis

– Based on clinical S/S
– CXR: cardiomegaly and increased pulmonary
vascular marking
– EKG: ventrcular hypertrophy (arrhythmias)
– ECHO
CONGESTIVE HEART FAILURE

Implementation goals:
– To improve cardiac function
– To remove fluid and Na
– To decrease cardiac demands
– To improve tissue oxygenation and
decrease oxygen consumption
Congestive Heart Failure
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Implementation
– Elevate the head of the bed
– Administer oxygen as prescribed during
stressful periods such as bouts of crying or
invasive procedures
– Feed in a relaxed environment
– Provide small frequent feedings, which will be
less tiring
– Monitor STRICT I&O and DAILY WEIGHT to
assess for fluid retention
– Weigh diapers
CONGESTIVE HEART FAILURE
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Implementation (cont’d)
– Monitor for facial or peripheral edema,
auscultate lung sounds, and report
weight gain to physician
– Monitor electrolyte levels
– Treat existing infections
– Instruct parents regarding diagnosis
and administration of medications
– Instruct parents CPR
Medications used in CHF

DIGOXIN: Know your Dig!
– Cardiac glycoside = improves function;
strengthens heart muscle; slows down heart
rate
– Therapeutic serum level: 0.8 – 2.0 ng/ml
– Monitor EKG rhythm for desired effect (i.e. if
prolonged P-R interval = hold and notify MD)
– Monitor AHR (i.e. bradycardia = hold dose) –
know your child’s normal HR range
Medications cont.
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Angiotensin-converting enzyme (ACE inhibitors)
– Inhibits the normal function of the reninangiotensin system in the kidneys
– Blocks angiotensin II so instead of
vasoconstriction, vasodilatation occurs and
arteries opens (decreased BP; improved
systemic circulation; decreased afterload and
decreased R-L arterial pressure)
– Examples Captopril (Capoten); Enalapril
(Vasotec) and Lisinopril (Zestil) = most
common (QD)
Medications cont.

DIURETICS
– Furosemide (Lasix) = potassium wasting
– Spironolactone (Aldactone) = potassium
sparing --- Be careful with IV additives!
What do you think?

The two main angiotensin-converting
enzyme (ACE) inhibitors most commonly
used for children with CHF are:
– Digoxin and captopril
– Enalapril and Captopril
– Enalapril and furosemide
– Spironlolactone and captopril
What do you think?

The calories are usually increased for an
infant with CHF by:
– Increasing the frequency of feedings
– Introducing solids into the diet
– Increasing the density of the formula
– Gastrostomy feedings
Treatment Options For Various
Conditions
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PDA (Patent Ductus Arteriosis)
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Indomethacin (Indocin)
Prostaglandin E1
Coil embolization
VATS = Video-assisted throacoscopic surgery
ASD (Atrial Septal Defect)
– Cardiac catheterization
– Laparoscopic techniques
– Sternotomy (“open heart”)
Cardiac Catheterization
VATS:
Video Assisted Thorascopic Surgery
Sternotomy

VSD (Ventricular Septal Defect)
– Some close on their own
– Diuretics
– Cadiotonics: Ca Channel Blockers
(Nifedipine or Procardia)
– If surgery, then similar to ASD

Aortic and Pulmonic Stenosis
– Balloon angioplasty
Balloon Angioplasty
What do you think?

Coarctation of the aorta should be
suspected when:
– The BP in the arms is different from the BP in
the legs
– The BP in the R-arm is different from the BP
in the L-arm
– Apical pulse is greater than the radial pulse
– Point of maximum impulse is shifted to the
left
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Coarctation of the Aorta
– Specific S/S: Increased BP and bounding
pulses in upper extremities and
decreased BP and weak/absent pulses
(femoral) in lower extremities
– Balloon angioplasty
– Surgical intervention = end-to-end
anastomosis
Resection & End-to-End
Anastomosis
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Atroventricular Canal (AVC) defect
– Complete surgical repair in infancy
– Palliative if older
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TOF (Tetrology of Fallot)
– Palliative surgery (Blalock-Taussig
Shunt)
– Corrective open-heart surgery /
sternotomy

Complete Transposition of Great Vessel
– NO communication b/t systemic and
pulmonary circulation
– Repair WITHIN HOURS! Not conductive with
life
– Palliative procedure = pulmonary artery
banding
– Corrective surgery = arterial switch procedure

Truncus Arteriosus
– EXTENSIVE surgical treatment
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Pulmonary Atresia
– EXTENSIVE surgical treatment
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Tricuspid Atresia
– Palliative and EXTENSIVE surgical treatment
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Hypoplastic Left Heart Syndrome (HLHS)
– Multiple stage surgery
– Heart transplantation
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Endocardial Cushing
– Total mixing of blood = EXTENSIVE surgical
repair
What do you think?

Chronic hypoxemia is clinically manifested
by which of the following signs?
– Squatting
– Polycythemia
– Clubbing
– All of the above
Cardiac Surgery
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Implementation PRE-OPERATIVELY
– Similar as with CHF
 Remember HOB elevated / high Fowlers with
acyanotic conditions and knee-chest position
(squatting) with cyanotic conditions – GENERALLY!
– Prepare for procedure
 Developmentally appropriate teaching
 Preparing what is to be expected post-operatively
– Equipment
– Pulmonary exercises
What do you think?

The MOST painful part of cardiac surgery
for the child is usually the
– Thoracotomy incision site
– Graft site on the leg
– Sternotomy incision site
– Intravenous insertion sites
CARDIAC SURGERY
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Implementation POST-OPERATIVELY
– NICU/PICU
– Monitor vital signs frequently (as per policy)
– Monitor temperature – report ANY fevers! **
Usually low-grade fevers are accepted in 1st 24 hours
(i.e. 101-101.5F)
– Maintain aseptic technique
– Monitor for signs of sepsis such as fever, chills,
diaphoresis, lethargy, and altered levels of
consciousness
CARDIAC SURGERY
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Postoperatively (cont’d)
– Monitor equipment:
 Cardiac monitor
 IV lines (CVL, PIVL, CVP, A-lines)
 ET tube
 Chest tube **** see X-ray ****
 Dressings
 NGT
 Foley
 Restraints
Thoracotomy: Chest tube
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Postoperatively (cont’d)
– Assess for signs of discomfort such as
irritability, changes in heart rate, respiratory
rate and blood pressure, and inability to sleep
– Sedation
– Pain
– Antibiotics and antipyretics as prescribed
– Encourage rest periods
– Facilitate parent-child contact as soon a
possible
What do you think?

An infant who weighs 7 kg has just
returned to the ICU following cardiac
surgery. The chest tube has drained 30 ml
in the past hour. In this situation, what is
the FIRST action for the nurse to take?
– Notify the surgeon
– Identify any other signs of hemorrhage
– Suction the patient
– Identify any other signs of renal failure
HOME CARE POSTOPERATIVE
CARDIAC SURGERY
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MOST IMPORTANT: Follow individualized plan!
Omit play outside for “several weeks”
Avoid activities where the child could fall, such
as bike riding, for a period of time
Avoid crowds in the immediate time after
discharge
Follow a no added salt diet if prescribed
Do not add any new foods to the infant’s eating
schedule
Do not place creams, lotions, or powders
on incision until completely healed
 Child may return to school 2-3 weeks after
discharge starting (depending on type of
procedure and “individualized plan”)
 No physical education until cleared by MD
 Instruct parents to discipline the child
normally
 Instruct parents about the importance
follow-up
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Avoid immunizations, invasive procedures,
and dental visits for 2 months
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Advise parents regarding the importance
of a dental visit every 6 months after age 3
and to inform the dentist of the cardiac
problem so that antibiotics can be
prescribed if necessary (new guidelines
regarding prophylactic antibiotics)
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Inform parents to call the physician when
coughing, tachypnea, cyanosis, vomiting,
diarrhea, anorexia, pain, fever, or any
swelling, redness, or drainage occurs at
the site of the incision
Critical Thinking

Tommy, a 4-year old with TOF has just returned
from the catheterization laboratory. He has
vomited, and his mother calls you to the bedside
to tell you that he is bleeding. You arrive to find
Tommy crying and sitting up in a puddle of blood.
What is the FIRST thing you should do?
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Increase the rate of his IV fluids
Give an antiemetic and keep Tommy NPO
Call the cardiologist
Lie Tommy down and apply direct pressure above the
catheterization site
Cardiac Catheterization
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Pre-procedure
– History including allergies
– Ht / Wt
– NPO X 8 hours
– Mark pulse location
– Prepare for administration of analgesics
/ sedatives / anxiolytics
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Post-procedure
– Monitor distal pulses and VS (BP)
– Monitor perfusion: temperature and
color of affected extremity and compare
– Dressing for bleeding
– Leg extended straight for 4-6 hours
– Strict I&O
– Encourage voiding to remove dye
– Analgesics
RHEUMATIC FEVER
RHEUMATIC FEVER

An inflammatory “autoimmune disease” that
affects the connective tissues of the heart,
joints, subcutaneous tissues, and/or blood
vessels of the CNS
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Presents 2 to 6 weeks following an untreated or
partially treated group A beta-hemolytic
streptococcal infection of the upper respiratory
tract
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Serious complication is rheumatic heart disease,
which affects the cardiac valves
RHEUMATIC FEVER
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Clinical Manifestation
– Starts with URI
– Inflammatory hemorrhagic bullous lesions
called Aschoff bodies are formed, which
causes swelling, fragmentation and alteration
in the connective tissues (circulatory system,
brain, pleura and joints)
– Fibrous tissue forms causing valve stenosis
and occlusion of blood flow = murmur
– Carditis is the end result
What do you think?

One of the most common findings on
physical examination of the child with
acute rheumatic heart disease is
– A Systolic murmur
– Pleural friction rub
– An ejection click
– A split S2
Rheumatic Fever
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Assessment
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Edema, inflammation of large joints
Joint pain
Fever
Pallor and weakness
Anorexia, weight loss
Erythematous macular rash on trunk and extremities
Chorea
Subcutaneous nodules in the joints, scalp, and spine
Arthritis
Epistaxis
Abdominal pain
Systolic murmur, tachycardia
Pericardial rub
 c/o chest pain (pericarditis)
 SOB
Diagnostic Evaluation
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No single S/S or lab test:
– Based on diagnostic guidelines set by the American
Heart Association (Jones criteria):
– Increased Anti-Streptolysin-O titers (ASLO)
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 Measure of antibodies formed against streptolysin-O (streptococcal
extracellular product which produces lysis of RBC).
 Formed within 7 days and peaks at 4-6 weeks.
 Reliable test in evidence of recent streptococcal infection
 Normal value is 0-120 TODD units. Value >333 = RF
 2 tests are required to confirm diagnosis
Increased C-Reactive Protein (CRP)
Leukocytosis
Anemia
Increased Erythrocyte Sedimentation Rate (ESR)
Prolonged P-R interval
RHEUMATIC FEVER
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Implementation
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PREVENTION!!!!!!!!!!!
Antibiotics for Strep (PCN or Erythromycin)
Antibiotics prophylactically (teaching)
Bedrest until afebrile
Salicylates (Aspirin) is used for the INFLAMMATORY PROCESS to
decrease fever and comfort (** Pediatrics + Aspirin = Reye’s
syndrome – toxic encephalopathy) – This is an exception!!!
– Steroids (i.e. prednisone) to decrease inflammation
– Administer anti-inflammatory agents as prescribed and if aspirin
is prescribed, it should not be given to a child who has chicken
pox or other viral infections
– Initiate seizure precautions if the child is experiencing chorea
Complications of Rheumatic Fever
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Polyarthritis
– Edema, inflammation and effusion of joint tissue
– Lat approx. 2 days after fever
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Erythema marginatum
– Erythematous macules (clear center)
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Subcutaneous nodules
– 0.5-1cm non-tender swelling nodules that resolves
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Carditis
– Endocardium, pericardium and myocardium
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CNS involvement
– CNS irritability = Sydenham’s Chorea (St Vitus Dance): Sudden,
aimless movements of extremities, facial grimacing and
contortions.
KAWASAKI DISEASE
An acquired heart disease with progressive
inflammation of the vessels and damage
to their walls
 Believed to be caused by a noncontagious
infection
 Without treatment, 20-25% develop
cardiac sequela
 The leading cause of heart disease in the
US and Japan
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What do you think?

The peak age for the incidence of
Kawasaki disease isin the
– Infant age group
– Toddler age Group
– School age group
– Adolescent age group
Diagnosis of Kawasaki Disease
No specific diagnostic test
 Dx is based on clinical signs & symptoms
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– Fever for > 5 days
– Bilateral conjunctival injection (inflammation) without
exudation
– “Strawberry tongue”
– Change in extremities: peripheral edema, erythema of
palms and soles; desquamation of hands
– Polymorphous rash
– Cervical lymphadenopathy (one lymph node >1.5cm)
3 Phases of Kawasaki
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ACUTE PHASE (1-2 weeks)
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Abrupt onset of high fever >5 days
No response to antipyretics or antibiotics
Bulbar conjunctiva with erythema and tearing
Inflamed pharynx
Red, cracked lips
“Strawberry tongue”
Desquamation of hands and feet
Cervical lymphadenopathy
VERY irritable and inconsolable
Complications
– Myocarditis, CHF and temporary arthritis
– EKG changes (ischemia)
Kawasaki Disease
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SUBACUTE PHASE (2 to 6-8 weeks)
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Fever resolves and s/s begins to disappear
Still irritable
Risk of developing coronary artery aneurysm
Thrombocytosis and hypercoagulability = coronary
thrombosis
– Arthritis continues
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Lab results
– CBC: anemia and leukocytosis
– Increased ESR
– Transient elevated LFTs
Kawasaki Disease
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CONVALESCENT PHASE
– All clinical s/s are gone
– This phase is completed when blood values
return to normal; usually 6-8 weeks after
onset
– There is always a high risk of MI that remains
Treatment of Kawasaki
High dose IV immune globulin (IVIG) given
within 10 days of infection. One infusion of 2
Gm/Kg over 10 hours.
 High dose aspirin for the first 2 weeks

– Decreases fever, edema and inflammation
This combination tends to show good results
within 24 hours
 After 2 weeks of high dose aspirin, change to
low dose for the next 4-6 weeks (prevent clots)
 Anticoagulation therapy (Coumadine) may be
indicated

What do you think?

Because of the drug used for long-term
therapy, children with Kawasaki disease
are at risk for
– Chicken pox
– Influenza
– Reye syndrome
– Myocardial infarction
Kawasaki - Nursing
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Teach to restrict physical activity due to increased bleeding
Monitor cardiac status (CHF)
Symptom relief
– Fever reduction
– Mouth care
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Discharge teaching
– Irritability may last up to 2 months
– No live immunizations until about 5 months (11 months for Varicella and
MMR)
– PROM exercises
– Assess for aspirin toxicity (tinnitus, H/A, dizziness and confusion)
– CPR
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Prognosis
– Usually full recovery
Hematologic Disorders
Sickle Cell Anemia
Sickle Cell Anemia
Normal adult hemoglobin (HgbA) is partly
or completely replaced by abnormal sickl
hemoglobin (HgbS)
 Sickle cell trait is the heterozygous form
 Primarily seen in the African American
group
 The RBCs are “sickled” and cannot carry
enough Hgb
 They are destroyed earlier than normal

Sickle Cell Anemia

Two main occurrences:
– OBSTRUCTION from sickled RBCs
– DESTRUCTION of RBC
Sickled cells causes vaso-occlusion = vasoocclusive crisis (“sickle cell crisis”)
 Results in local hypoxia leading to tissue
ischemia and infarction (cellular death)
 Can be life threatening

S/S of Sickle Cell Anemia

SPLEEN
– Splenic sequestial crisis from engorgement with sickled cells
(enlarged and no function)
– By age 5 years, functional cells are replaced by fibrotic tissue =
functional ASPLENIA
– Prone to infection

HEPATIC
– Hepatomegaly from extensive lysis of RBC
– Development of gallstones (obstruction)
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RENAL
– Kidney ischemia leads to hematuria and decreased renal function
– Nephrotic syndrome

BONES
– Bone changes due to bone marrow’s hyperplastic production of
RBC to compensate for destruction of sickled cells
– Bone marrow crisis (aplastic crisis) – may lead to osteoporosis
– Lordosis and kyphosis
– Osteomylitis secondary to chronic hypoxiap

CNS
– CVA due to occlusion, ischemia and infarction
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HEART
– Cardiomegaly – from chronic anemia
– Systolic flow murmur
– May lead to MI
Sickle Cell Anemia
4 Types of Sickle Cell Crisis
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Vaso-occlusive crisis
– Most common and non-life threatening
– PAIN (joints; abdomen)
– Priapism

Spenic Sequestration crisis
– More severe
– Blood pools in spleen and causes quick drop
in blood volume = hypotension and
hypovolemic shock
Other manifestations
Exercise intolerance
 Anorexia
 Jaundice / icteric sclera
 Gallstones
 Leg ulcers
 Growth retardations (height and weight)
 Delayed sexual maturation
 Decreased fertility


Aplastic crisis
– BM crisis: decreased RBC production
– Triggered by viral infection: human parvovirus
– Increased destruction of RBCs leads to anemia
(megaloblastic anemia resulting from folic acid and
Vit. B deficiency)
– PRBC transfusion usually required

Hyperhemolytic crisis
– Very rare
– Decreased production of non-sickled RBC & overall
hyperplastic production
– Accelerated rate of RBC destruction and BM tries to
replace dead RBC
Diagnosis of SCA

Blood smear
– View sickled cells
– Hgb electrophoresis = “fingerprints” which detects
different types of Hgb (A=Adult; F=Fetal; S=sickled)

CBC
– Increased WBC, Platelets and Iron

Genetics
– Perinatally through amniocentesis and CVS

Signs and symptoms
– Pain: joints, back and abdomen
Intervention for Sickle Cell Crisis
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Hydration to prevent sickling of cells
Prevent acidosis: O2 supplementation
Supplement with Folic acid
Bedrest to decrease energy expenditure
PROM
PRBC transfusion prn (maintain Hgb >10mg/dl)
Analgesics / Opioids
Heat for relief of joint pain
Strict I&O and monitor weight
Regular eye exam: damage to the retina
What do you think?

Infants are often not diagnosed with sickle cell
anemia until they are 1 year of age. Why?
– Usually there are no symptoms until after age 1 year
– High intake of fluids from formulas prevents sickle cell
crises during this age.
– Fetal Hemoglobin is present during the first year of
life
– Increased hemoglobin and hematocrit amounts
compensate during this period
“Treatment”

Splenectomy
– In children < 5 years old (remember, they have
functional asplenia > 5 years of age)
– May be life saving! Spleen is the major site of sickling
and destruction of RBC

Prophylactic PO Penicillin
– Reduce the chance of pneumococcal sepsis

Vaccines
– HIB (Haemophilus influenza type B)
– Meningococcal

Bone Marrow Transplant
Complications of SCA

STROKE
– Sickled cells block major vessels to the brain

CHEST SYNDROME
– Resembles pneumonia
– May lead to restrictive lung disease and
pulmonary hypertension

OVERWHELMING INFECTIONS
– Due to defective splenic function
– May cause death in children <5 years
What do you think?

Pat is a 4 year old being admitted because
of diminished RBC production triggered by
a viral infection. What type of sickle cell
crisis is she most likely experiencing?
– Vasoocclusive crisis
– Splenic sequestration crisis
– Aplastic Crisis
– Hyperhemolytic crisis
What do you think?

Therapeutic management of sickle cell
crisis generally includes which one of the
following?
– Long-term oxygen use to enable the oxygen
to reach the sickled RBCs
– Decrease in fluids to increase
hemoconcentration
– Diet high in iron to decrease anemia
– Bed rest to minimize energy Expenditure
HEMOPHILIA
A group of bleeding disorders resulting
from a congenital deficiency of specific
coagulation proteins
 Excessive uncontrollable bleeding!
 X-linked recessive trait: MOTHER passes it
on to SON

– Boys (XY): Hemophilia is transmitted on X
chromosome
– Girls (XX): Inherits the carrier status ONLY
Two types of Hemophilia

Hemophilia A
– Classic hemophilia
– Most common – 75%
– Deficiency in clotting Factor VIII

Hemophilia B
– Also called “Christmas disease”
– Deficiency of clotting Factor IX
Diagnosis of Hemophilia
Based on history of bleeding
 Abnormal PTT

– Prolonged > 40 seconds -- (Normal = 30-40
seconds)

Platelet function
– Normal
Specific tests for factor VIII and IX assay
 DNA testing

Signs and symptoms

Infant
– Excessive bleeding at umbilical or circumcision site

Child
–
–
–
–
–
Excessive bleeding anywhere in the body
Hematuria
Melena (black, tarry feces)
Epistaxis
Hemarthrosis = joint bleeding
 Very painful
 Dangerous = blood loss
 Bone changes and cripling deformities over years
Hemophilia
Treatment of Hemophilia
Main focus: replacement of missing clotting factors
 Blood transfusion for Factors VIII or IX
 Cryoprecipitate (plasma derivative with factor VIII): NO
LONGER RECOMMENDED! – cannot guarantee safe
elimination of Hepatitis or HIV
 Corticosteroids
 DDAVP (I-deamino-8-D-arginine vasopressin): IV or
intranasal

– Causes vasoconstriction
– Increases plasma factor VIII
– Plasminogen activator

Analgesics, but NO aspirin
TEACHING









Goal is to prevent bleeding!
Dental care: soft toothbrush
No contact sports
Avoid aspirin
Diet: avoid obesity (stress on joints); iron rich foods
Genetic counseling
Support groups
Teach s/s of bleeding
If bleeding occurs: RICE
–
–
–
–
R = Rest
I = Ice
C = Compression
E = Elevation
Complications from Hemophilia
Bone changes = crippling deformities
 GI hemorrhage
 Intracranial bleeding
 Bruising over spinal cord = paralysis
 Airway obstruction if bleeding in
throat/pharynx

What do you think?

Which one of the following is the most
frequent form of internal bleeding in the
child with hemophilia?
– Hemarthrosis
– Epistaxis
– Intracranial hemorrhage
– Gastrointestinal tract hemorrhage
Beta (B) Thalassemia Major
Also called Cooley’s anemia
 Group of disorders characterized by reduced

production of the globin chains in the synthesis
of hemoglobin (B-chain affected)
Cultures such as Italians, Greeks and Syrian
living near the Mediterranean Sea are affected
 Another group is the Alpha, affecting groups
such as Chinese, Thai and Africans
 Autosomal recessive trait: both males and
females must be carriers in order to pass it on

Types of Thalassemia

Thalassemia major
–
–
–
–

Cooley’s anemia
Homozygous
Those who have the diseases
Severe anemia and not compatible with life without transfusion
support
Thalassemia minor
– Heterozygous
– Have trait only
– Asymptomatic or mild microcytic anemia

Thalassemia intermedia
– Heterozygous
– Moderate to sever anemia and spenomegaly
Diagnosis
Hemoglobin electrophoresis (analysis of
protein mixtures) confirms the diagnosis
and distinguishes the type and severity
 There is no cure!
 Children usually die in late adolescent
 Must cope with frequent blood
transfusions in the late stages
 Genetic counseling in all types

Signs and symptoms









Pallor
Fatigue r/t hypoxia
Poor feeding
Hepatosplenomegaly
Neurological impairment r/t hypoxia
Bone and joint pain
Anorexia
Exercise intolerance
Growth retardation
Long term complications




Splenomegaly (splenectomy)
Hepatomegaly: cirrhosis with jaundice (yellow from bilirubin
mixed with retained iron) = Bronze skin color
Weak bones due to osteoporosis = spontaneous fractures
Skeletal changes
–
–
–
–
–


Thick cranial bones
Enlarged maxilla
Prominent facial bones
Bossing (but not from hydrocephalus, from bone changes)
“Frankenstein appearance”
“Mongoloid” appearance
Teeth:
– Malocclusion (malposition of mandibular and maxillary teeth)
– “Buck teeth” = crowded and crooked teath

Cardiac abnormalities
– Dysrhythmias
– CHF
– Fibrotic cardiac muscle

Gall bladder disease
– cholecystectomy
Growth retardation
 Endocrine problem (DM) due to iron deposit’s
effect on pancrease
 Delayed sexual maturation

– Small genitalia
– Decreased pubic hair
Treatment
SUPPORTIVE!
Transfusions are the foundation of medical
management (keep Hgb >9.5)
 Iron Chelation theraphy = Deferoxamine


–
–
–
–

Due to multiple transfusion, iron stores increases
Helps pull iron from tissues and eliminate Fe
Give with oral Vitamin C (to draw iron out of tissues)
It is given either of 2 ways:
 SQ – over 8-10 hours 5-7 days/week (during sleep)
 IV – over 4 hours
 Can also be given as deep IM injection
Prophylactic Antibiotics
Folic acid supplements (Vitamin B9) –
stimulates production of RBCs
 Avoid injury, rest, good hygiene and
nutrition
 Avoid infectious persons
 Avoid contact sports
 Support groups
 Genetic counseling

What do you think?

Norma, age 2 years, is to begin therapy for Bthalassemia. Which one of the following would
be appropriate for the nurse to include in the
educational session held with the parents?
– Norma will need frequent blood transfusions to keep
her Hgb level above 12g/dl
– Large doses of vitamin C will be needed throughout
the disease
– Chelation therapy is delayed until after 6 years of age
to promote normal physical development
– To minimize the effect of iron overload, deferoxamine
(Desferal), an Iron-Chelating agent, will be given IV
or SQ
DIC = Disseminated
Intravascular Coagulation
ANY QUESTIONS?????
Idopathic Thrombocytopenic
Purpura (ITP)

Aquired hemorrhagic disorder:
–
–
–
–

Excessive destruction of platelets (thrombocytopenia)
Purpura (petechia), mucocutaneous bleeding
Occasional bleeding into tissues
Normal bone marrow with unusual increase in large, young
platelets
Acute or transient
– Acute self limiting: “comes and goes”
– Chronic: “comes and goes” into remission (>6 month duration)
– Acute following viral illnesses such as measles, varicella, mumps
and URI (onset usually 1-4 weeks after the viral illness)
– Suspected that an immune mechanism is the basis for the
thrombocytopenia
Signs and symptoms








Easy bruising especially over bony areas
Bleeding from gums / mucous membranes
Epistaxis
Menorrhalgia (painful menstruation)
Hematemesis
Hematomas
Hemarthrosis
Intracranial hemorrhage is a rare (<1% of
cases), but serious outcome
Diagnosis
Often diagnosed with clinical symptoms
 Thrombocytopenia

– Platelet count <20,000 m3/dl
– On blood smear, the platelets are large
(megathrombocytes) = increased marrow
production

Usually occurs around time of puberty
– Danger of bleeding with menses
ITP
Treatment
Excellent prognosis (75% recover)
 Sometimes no medical interventions, self-limiting
 Restrict activity! Prevention from trauma and bleeding
 Corticosteroids: Prednisone; Decadron (Dexamethasone)

– Increases platelet count temporarily
– Avoid long term therapy (usually <3 weeks) in order to decrease side effects:
 Bone marrow suppression
 Cushingoid changes
 Growth failure

Blood transfusion = Only transient benefits
– Given in life threatening situations

Platelet transfusion = Only transient benefits
– Platelets has a short survival

Splenectomy
– If unresponsive to treatment
– Usually only with chronic ITP
– Decreased risk for hemorrhage
Treatment

Chemotherapy agents = Not commonly used
– Vincristine
– Severe side effects

Danazol (danocrine)
– Decreases estrogen and halt menses

IVIG (Gamma Globulin)
– IV administration of antibodies to spare the removal of antibody coated
platelets in the spleen
– Sustained rises of platelet count
– Large doses may induce remission

Rituxan
– Monoclonal antibody
– New and investigational

Sandimmune (cyclosporin)
– Used to prevent organ rejection and inhibit WBC growth factors

Excorim System (Protein A Immunoabsorption)
– Antibody removal system, used mostly in Europe
– Plasmapharesis: Plasma is removed and filtered until desired lowered
immunoglobulin level is achieved:
 Plasma is removed and “rinsed” before being re-mixed with blood in the cell separator
and returned to the patient.
 The process is performed on-line and continuous until the desired amount of plasma is
processed.
– Very noisy, LIJ has it

Anti-D antibody
– Temporary and sometimes long-term elevation of the platelet counts
– Works by coating the RBC and blocking the spleen’s destruction of certain
platelets.
– By doing so, there is an increase in platelet count within 1-3 days with a peak in
counts 8 days after infusion
– The elevation in platelets last approximately 1 month – longer than IVIG
– Advantage over IVIG in that it is given IV push and IT IS CHEAPER!
 Pre-medicate:
– Decrease risk of reaction
– Decrease pain
What do you think?

Which one of the following does the nurse
recognize as true when administering
anti-D antibody for ITP?
– The platelet count will increase immediately
after administration
– Eligible patients include those with lupus
– Bone marrow examination to first rule out
leukemia is necessary before administration
– Pre-medicate the patient with Acetaminophen
before medication is infused.
LEUKEMIA
LEUKEMIA

Description
– Malignant exacerbation in the number of leukocytes,
usually at an immature stage, in the bone marrow
– Affects the bone marrow causing anemia, leukopenia,
the production of immature cells, thrombocytopenia,
and a decline in immunity
– The cause is unknown and appears to involve gene
damage of cells leading to the transformation of cells
from a normal state to a malignant state
LEUKEMIA

Description (cont’d)
– The most common form of childhood cancer
– Peak incidence is age 2-6 years for acute lymphocytic
leukemia (ALL)
– Dramatic improvements in survival rates, 80% (>5
years) due to the extensive research
– Risk factors include genetic, viral, immunologic, and
environmental factors and exposure to radiation,
chemicals, and medications
– Risk factors in children include those with Down’s
syndrome or a twin of a child who has had leukemia
What do you think?

Of the following assessment findings, the
one that would most likely be seen in a
child with leukemia is:
– Weakness of the eye muscle.
– Bruising, nosebleeds, paleness, and fatigue.
– Wheezing and shortness of breath.
– Abdominal swelling
Affects bone marrow = anemia + bleeding
 Diagnosis is based on % of blasts
(immature WBC) in the blood
 CBC will show

– May have an increased WBC, BUT they are
really leukopenic because the WBC are
IMMATURE
– Decreased Hgb/Hct = ANEMIA
– Decreased platelets = thrombocytopenia
LEUKEMIA

Classification of Leukemia
– Acute Lymphocytic Leukemia (ALL)





Mostly lymphoblasts present in bone marrow
Age of onset is less than 15 years
Usually higher success in treating
3 subtypes (“markers” on cell surface antigens)
84% of the leukemias





Mostly myeloblasts present in bone marrow
Age of onset is between 15 and 39 years
Survival rate is not as positive
8 subtypes
Approximately 20% of the leukemias
– Acute Myelogenous Leukemia (AML)
LEUKEMIA

Assessment: COMMON PRESENTATION!!!
– Most of the signs/symptoms are a result of bone
marrow infiltrate
– Anorexia, fatigue, weakness, weight loss
– Pallor and anemia
– Bruising and bleeding from the nose or gums, rectal
bleeding, hematuria
– Prolonged bleeding after minor abrasions or
lacerations
– Hemorrhage and Petechiae
– Elevated temperature
– Lymphadenopathy, splenomegaly, hepatomegaly from
marked infiltration. May lead to fibrosis
– Palpitations and tachycardia
– Dyspnea on exertion
– Vague abdominal pain caused by inflammation from
normal flora invading intestinal tract
– Wasting of major organs due to infiltration of
leukemic cells
– Increased ICP if meninges are infiltrated
– CNS: severe headache, vomiting, papilledema (edema
& inflammation of the optic nerve which may result in
blindness), irritability, lethargy and eventually coma
– Muscle wasting, weight loss, anorexia and fatigue
LEUKEMIA
LEUKEMIA

Infection
– A major cause of death in the
immunosuppressed child
– Can occur through autocontamination (i.e.
Staph aureus on the skin) or cross
contamination
– Most common sites of infection are the skin,
respiratory tract, and GI tract
Infection:
Neutropenic Diet
Reverse Isolation
LEUKEMIA

Protecting the Child from Infection
–
–
–
–
–
Initiate protective isolation procedures
Maintain child in a private room
Frequent and thorough handwashing
Strict aseptic technique for all nursing procedures
Limit the number of caregivers entering the child’s room and
ensure that anyone entering the child’s room is wearing a mask
= REVERSE ISOLOATION
– Keep supplies for child separate from supplies for other children
– Reduce exposure to environmental organisms by eliminating raw
fruits and vegetables from the diet, and keeping fresh flowers
and standing water out of the child’s room * Neutropenic diet
What do you think?

The severe cellular damage that is caused
by chemotherapy drugs infiltrating into
surrounding tissue occurs when the
chemotherapeutic agent is a(n)
– Hormone
– Steroid
– Vesicant
– antimetabolite
LEUKEMIA

Bleeding
– During the period of greatest bone marrow
suppression (the nadir), the platelet count
may be extremely low
– Children with platelet counts below
20,000/mm3 may need a platelet transfusion
– For children with severe blood loss, packed
red blood cells may be prescribed (for Hgb
<8.0)
LEUKEMIA

Fatigue and Nutrition
– Assist the child in selecting a well-balanced
diet
– Provide small meals that require little chewing
– Assist the child in self-care and mobility
activities
– NORMALIZE their life as much as possible!
LEUKEMIA

Chemotherapy
– Monitor for severe bone marrow suppression
– Monitor for infection and bleeding
– Protect child from life-threatening infections
for at least 2 to 3 weeks following
chemotherapy
– Monitor for nausea, vomiting, and diarrhea
– Assess oral mucous membranes for stomatitis
– Monitor for renal, hepatic, and cardiac toxicity
LEUKEMIA

Chemotherapy
– Inform parents that hair loss may occur from
chemotherapy
– Instruct parents about the care of central
venous access devices as necessary
– Listen and encourage child and family to
verbalize their feelings and express their
concerns
– Introduce family to other families of children
with cancer
Treatment (i.e. NYII protocol)

INDUCTION THERAPY
– 4-6 weeks in length
– Goal is to achieve complete remission
 Corticosteroid = decrease inflammation
 Vincristine and L-asparaginase with or without Doxorubicin
 CNS prophylactic therapy: IT (not IV due to the blood-brain
barrier) Methotrexate – given directly into the CSF via LP. It
protects against CNS invasion of leukemic cells. Radiation
used to be done but changed due to long term side effects.
– Goal is met if <5% blasts in BM
– If CNS disease is present, give Sanctuary therapy
= combination of chemotherapy agents, more intense
and therefore need to watch for neurotoxicity

Always concerned about tumor lysis syndrome during
induction therapy
– Caused by sudden, rapid death of cells in response to treatment
and that in terms causes electrolyte and metabolic disturbances

To prevent tumor lysis syndrome:
–
–
–
–

Alkalanization = 2X IV maintenance therapy with NaHCO3
Allopurinol to decrease uric acid
Amphojel to decrease phosphate
Strict I&O maintaining urine pH >7.5
Tumor lysis syndrome would be a concern with:
–
–
–
–
Increased Phosphate
Increased Uric Acid
Increased Potassium
Decreased Calcium

MAINTENANCE
– Also called consolidation
– Serves to maintain the remission phase
– Duration may be 2 1/2 to 3 years
– Continue corticosteroids, methotrexate and
vincristine
Side effects to treatment

Chemotherapy
– Nausea and vomiting
– Alopescia (temporary)
– Certain agents, i.e cytarabine (cytosin) will cause
peripheral neuropathy with decreased sensation and
reflexes

Interthecal chemotherapy – Ommaya
resorvoir or LP
– Spinal headache
– Keep flat or Trendelenberg for at least 1 hour after
administration to prevent headache and distribute
drug throughout the body
Bone Marrow Transplant






Not recommended for children with ALL during
first remission because of the excellent result
with chemotherapy during second remission
Complete reverse isolation
Pt is typed and crossed with donor marrow
Sibling is the #1 choice for HLA match
BM donor banks – difficult for certain ethnicities
BM aspirate – local anesthesia
Outcome
“Cured” if 5 years in remission after
treatment
 Adjunct goal: Pt has no assessment of
disease but cancer cells are evident
through tests
 Palliative goal: Make them “comfortable”
and control s/s until death occurs (i.e.
Hospice or home)

– “To Live Among Lions”
IMPAIRED GAS
EXCHANGE
&/OR INEFFECTIVE
BREATHING PATTERNS
Infections of the upper
and middle airways
TONSILLITIS







An infection or inflammation (hypertrophy) of the
palatine tonsils
Most children with pharyngitis have infected tonsils, but
NOT necessarily tonsillitis (may “just” have phyaryngitis)
Viral or bacterial
Tonsils are lymphoid tissues:
– filter and protect respiration tract from invasion of
microorganisms
– form antibodies
Children usually have larger tonsils than adolescents or
adults
Adenoids are located above the tonsils
Tonsillitis usually occurs with pharyngitis
TONSILLITIS

ASSESSMENT:
– Enlarged and edematous tonsils
– “Kissing tonsils” = they may meet at midline and obstruct
passage of food or air
– Frequent throat infections
– Cervical lymphadenopathy
– Difficulty swallowing or breathing
– Adenoids enlarge and cause difficulty for air to pass from nose
to pharynx.
– Usually snoring when adenoids are affected
– Mouth breathing
– Drying of mucous membranes secondary to mouth breathing
– Otitis media and possibly decreased hearing may result
(adenoids close to eusthasian tubes)

DIAGNOSIS:
– Based of visual inspection and clinical manifestation
– Throat culture
TONSILLITIS

MANAGEMENT:
– Symptomatic treatment:
 Provide comfort
 Acetaminophen (may be given PR) or ibuprofen to decrease
inflammation and throat pain
 Cool, non-acidic fluids and soft foods; throat lozenges
 Ice chips or frozen juice pops (increase hydration)
 Humidification / vaporiziation (cool mist)
 Gargle with warm salt water (soothing)
– Antibiotics based on C/S (Throat culture: pharyngeal – tonsil swab).
First line antibiotic is usually penicillin base (i.e. amoxicillin)
– Surgery: Tonsillectomy &/or adenoidectomy (T&A)
 Recommended if recurrent throat infections (documented Strep
throat: >3 in 6 months or >5/ in one year)
 Chronic tonsillitis
 Obstructive sleep apnea
 Nasal speech
 After the age of 3 years (it can stimulate growth of other lymphoid
tissue in the nasopharynx)
TONSILLITIS

NURSING PRE-OP and POST-OP:
–
–
–
–
–
Teaching is very important
Baseline VS
History of bleeding pattern
Side-lying following surgery to drain secretions
No coughing frequently, clearing throat and blowing nose - may
aggravate operative site
– Assess for hemorrhage:
 Most common within the first 24 hours or 7-10 days after tonsillectomy (scar
is forming during that time)
 Use penlight
 Inspect secretion and vomit for evidence of fresh blood
 Pallor
 Increased pulse (>120, but depends on age-group – refer to G&D chart)
 If bleeding is suspected, call MD immediately
– Diet: Cool water, crushed ice, ice pops, dilute fruit juice (avoid citrus acidic - irritating to site), soft foods - cooked fruits, jello, soup, etc
TONSILLITIS
– Airway obstruction may occur due to edema or accumulated secretions
– Assessment of respiration distress:




Stridor
Drooling
Restlessness
Increased respiration rate
– Suction & 02 should be available
– Assess for infection (most common first 7-8 days):
 White coating in back of throat
 Odor
 Low grade fever (report temp >102) (38.8C)
– Discharge teaching:








Avoid highly seasoned food
Avoid gargling
Avoid vigorous tooth-brushing
Avoid Ibuprofen the first post-op week. Use acetaminophen
Discourage coughing and throat clearing
Use mild analgesics
Limit activity to decrease potential for hemorrhage
Persistent sever earache, fever or cough required MD evaluation.
What do you think?

Adenoidectomy would be contraindicated
in a child
– with recurrent otitis media.
– with malignancy.
– with Thrombocytopenia.
– over the age of 3 years.
What do you think?

Which of the following food(s) is/are the
MOST appropriate to offer first to an alert
child who is in the post-operative period
following a tonsillectomy?
– Ice cream
– Red gelatin
– Flavored Ice Pops
– All of the above
CROUP SYNDROME
Term applied to a broad classification of upper
airway illnesses that result from swelling of the
epiglottis and larynx, often extending into the
trachea and bronchi
 Boys > girls
 Seasonal:

– Late autumn and early winter = more frequent
episodes

Classification system of croup includes viral
syndromes such as (the “big three” of pediatric
respiratory illness = affects the greatest number
of children across all age groups in both sexes):
– 1) Spasmodic laryngitis (spasmodic croup)
CROUP SYNDROME
– 2) Laryngotracheobronchitis (LBT) = most common!




3 months - 8 years
Viral invasion
Slow progression.
Specific symptoms:
– URL
– Stridor
– Degree of inspiratory stridor and may lead to respiration distress due to edema or
obstruction of airway
– Seal-like “barking” cough
– Resonant cough: “brassy” like in sound
– Hoarseness
– Dyspnea
– Restlessness
– Irritability
– Low grade fever
– Nontoxic
– Treatment:
 Humidity
 Racemic epinephrine.
CROUP SYNDROME
– 3) Bacterial syndromes (includes bacterial
trachitis and epiglottitis)





1-8 years old
Bacteria Invasion (Haemophilus influenzae)
Almost non-existent due to vaccination: Hib = PREVENTION!
Rapidly progresses
Specific symptoms
–
–
–
–
–
–
Dysphagia
Stridor aggravated when supine
Drooling
High fever
Toxic
Tachycardia and tachypnea
 Treatment:
– Antibiotic therapy
– Airway protection (intubate in the OR)
– Corticosteroids for edema
What do you think?

In the child who is suspected to have
epiglottitis the nurse should:
– Have Intubation equipment available
– Prepare to immunize the child for
Haemophilus influenzae
– Obtain a throat culture
– All of the above
CYSTIC FIBROSIS

Inherited autosomal recessive disorder (both parents) of the
exocrine glands that results in physiologic alterations in:
– Respiratory system:
–
–
–
–




 Abnormal accumulation of viscous, dehydrated mucus
 Inflammation and lung changes
Gastrointestinal system
Integumentary system
Musculoskeletal system
Reproductive system
All body organs with mucous ducts become obstructed and
damaged
Predominantly in white children
Gender is not a factor
Average life span is 30 years (may vary)
CYSTIC FIBROSIS





Blocked pancreatic ducts and resulting pancreatic damage causes a
STOP in the secretion of natural enzymes necessary to digest fats
and protein.
As a result, essential nutrients are excreted in the stool
ESSENTIAL CHANGES IN PATHOPHYSIOLOGY:
Respiratory system:
– Bronchi, bronchio-pneumonia & bronchial emphysema (barrel
chest)
– Increased secretion requiring intervention
– Classic cough secondary to increased mucus
– Atelectasis
– Secondary respiratory infections = increased morbidity and
mortality
Small intestine:
– Obstruction from mucous
– If newborn does not pass meconium you must R/O cystic fibrosis
CYSTIC FIBROSIS
Pancrease:
– Ducts blocked
– Unable to absorb & digest fat
– Missing pancreatic enzymes that break down fat
– Steathorrhea
– Frothy (bulky and large in quantity)
– Foul smelly
– Contain fat (greasy)
 Bile ducts:
– Obstructed
– May lead to cirrhosis
– Jaundice, portal HTN
 Sweat glands and salivary glands:
– Lose a lot of salt – electrolyte imbalances due to loss of Na and Cl
– SALTY KISSES!!!!!! – initial S/S
 Reproductive system:
– Male sterility due to blockage or absence of the vas deferens
– Females difficulty conceiving due to increased mucus secretions in the
reproductive tract interfering with sperm passage

CYSTIC FIBROSIS

DIAGNOSIS:
– Family history - genetic
– SWEAT TEST: patch on skin with measure of amount of Na absorbed
 Chloride concentration of 50-60 mEq/L is suspicious
 Chloride concentration >60mEq/L is diagnostic
– If positive: Pancreatic enzyme fast (eliminate) and check for steatorrhea
– CXR: to check for mucus
– CNS: Possibly hyperactive children (evident early in childhood)

ASSESSMENT:
– INTESTINAL & PANCREAS:




Steatorrhea (fat, frothy, foul smelling)
Eats large amount, but does not gain weights because can not absorb fat
Missing pancreatic enzymes - Trypsin, Lipase and Amylase.
Need vitamins because fat-soluble vitamins will not be absorbed through food
– RESPIRATORY:




Wheezing, rales, dyspnea, non-productive dry cough
May develop atelectasis
Clubbing
May have ear, nose and throat problem more than average kids.
CYSTIC FIBROSIS
Long-term complications
– HEART:
 Corpulmonal - Right sided enlargement
 CHF = Pulmonary blood flow is obstructed by the mucous
– ESOPHOGEAL:
 Esophageal varisies = enlarged vein - may bleed
– LIVER:
 Jaundice
– SPLEEN:
 Spleenomegaly
– REPRODUCTIVE SYSTEM:
 ducts blocked, tubes blocked, mucous in vaginal tract
 vas deferens blocked
CYSTIC FIBROSIS

TREATMENT:
– Respiratory:
 Cupping, postural drainage, deep breathing,
bronchodilators (Q4H before meals) – percussion/vibriation
vest
 Aerosol treatments (bronchodilators)
– Nutrition:
 Increase SALT, especially with exercise
 HIGH CALORIES AND PROTEINS
 Salt tablets in warm weather
 Pancreatic enzymes pills taken with meals (non-fat & nonprotein such as fruit, vegetables or carbohydrates).
Sprinkle on food, “mix” in well AS A “SANDWICH”. Don't
get on lips or skin = breakdown.
– NURSING:
 Teach about disease and how to explain the condition to
the child
 Becomes progressively worse
 Avoid infection
 Encourage activity, rest, ventilate feelings, support groups
 Genetic counseling
Percussion Vest
Postural Drainage
ASTHMA







Chronic inflammatory disorder of the airway with airway
obstruction that can be partially or completely reversed
Chronic condition with acute exacerbations or persistent
symptoms
Approximately 5 million children are affected in the United States
(school absenteeism)
Boys > girls until the age of 10 years and then it equals out
Most are diagnosed before the age of 5 years
Called Reactive Airway Disease (RAD) in infants….. Discussion:
RAD vs ASTHMA
ETIOLOGY:
– Bronchospasm
– Increased mucous secretion
– Usually results from allergic hyper response. Before puberty –
boys
– Air gets trapped in the lungs and cannot get out (wheeze)
– Major allergic component:
 Seasonal allergies
 Irritants: smoke
 Roach allergy
 Food allergies
 Skin sensitivity tests to determine causative agents
ASTHMA

ASSESSMENT:
– Respiratory difficulties of an asthma attack result from
inflammation that contributes to airway obstruction
(narrowing)
– Mucus formation , mucosal swelling and airway muscle
contraction
– A stimulus or TRIGGER initiates an asthmatic episode
– S/S visible includes:
 Air hunger
 Dyspnea
 Anxiety
 Coughing (productive vs non-productive)
 Fatigue
 Wheezing - inspiratory and/or expiratory
 Tachypnea
 Retractions
 Cyanosis, and diaphoresis --- late signs
 Barrel chest (chronic)
ASTHMA
TREATMENT OPTIONS

Long-term control medications
– Leukotriene modifiers
– Long-acting beta-2 agonists (LABAs)
– Theophylline

Quick-relief medications
– Short-acting beta-2 agonists
– Ipratropium (Atrovent)
– Oral and intravenous corticosteroids

Medications for allergy-induced asthma
– Immunotherapy
– Anti-IgE monoclonal antibodies
Long-term control medications
(Usually taken on a daily basis)

Inhaled corticosteroids:
– fluticasone (Flovent Diskus), budesonide (Pulmicort),
triamcinolone (Azmacort), flunisolide (Aerobid), beclomethasone
(Qvar) and others.
– Reduce airway inflammation and are the
– Most commonly used long-term asthma medication
– Low-risk for side effects as compared to systemic corticosteroids
– These inhaled medications work by opening airways, reducing
inflammation and decreasing mucus production.

Leukotriene modifiers:
– montelukast (Singulair), zafirlukast (Accolate) and zileuton (Zyflo
CR)
– Inhaled medications; work by opening airways, reducing
inflammation and mucus production
Long-term control medications
(Usually taken on a daily basis)

Cromolyn and nedocromil (Tilade)
– Inhaled medications reduce asthma signs and symptoms by decreasing
allergic reactions
– Considered a second choice to inhaled corticosteroids
– Given TID to QID

Long-acting beta-2 agonists (LABAs):
– salmeterol (Serevent Diskus) and formoterol (Foradil Aerolizer).
– Inhaled, long-acting bronchodilators, open the airways and reduce
inflammation
– Used in combination with inhaled corticosteroids with persistent
asthma
– Long-acting bronchodilators should not be used for quick relief of
asthma symptoms

Theophylline
– Daily tablet that opens your airways (bronchodilator)
– Relaxes the muscles around the airways – VERY RARELY USED
Quick-Relief Medications
(also called rescue medications)

Short-acting beta-2 agonists:
– Albuterol and Xopenex
– Inhaled bronchodilators, relax airway muscles
– Act within minutes, and effects last four to six hours

Ipratropium (Atrovent)
– Inhaled anticholinergic for the immediate relief of your
symptoms; relaxes the airway
– Mostly used for emphysema and chronic bronchitis

Oral and intravenous corticosteroids
–
–
–
–
Prednisone and methylprednisolone
In treatment of acute asthma attacks and severe asthma
May cause serious side effects when used long term.
Used short-term for asthma
Other Asthma Medications

Medications for allergy-induced asthma:
– To decrease the body's sensitivity to a particular
allergen or prevent the immune system from reacting
to allergens. Allergy treatments for asthma include:
 Immunotherapy:
– Allergy-desensitization shots (immunotherapy) are generally
given once a week for a few months, then once a month for a
period of three to five years. Over time, they gradually reduce
your immune system reaction to specific allergens.
 Anti-IgE monoclonal antibodies:
– omalizumab (Xolair)
– Reduces the immune system's reaction to allergens
– Xolair is delivered by injection every two to four weeks.
ASTHMA TREATMENT
Most commonly what we see:

TREATMENT:
– Bronchodilators: Beta2-agonists (short-acting: Proventil /
Albuterol) for ACUTE ATTACK.
 Proventil: teach about SE including tachycardia,
nervousness, tremors
– Inhaled steroids: Used as a prophylactic (i.e. before exercise),
during and after exacerbation of asthma for more effective
treatment (i.e. fluticasone (Flovent Diskus), budesonide
(Pulmicort), flunisolide (Aerobid), combination drug such as
Advair [Fluticasone and Salmeterol])
 Now considered MOST EFFECTVE treatment in asthma:
– Reduces asthma symptoms and flare-ups
– Improves lung function
– Reduces bronchial reactivity
– NOT ADDICTIVE!
 Increased risk of thrush
– Rinse mouth and/or brush teeth after treatment

Treatment (cont.)
– GIVE ALL MDI MEDICATIONS WITH SPACER
– Oral corticosteroids:
 If inhaled steroids are not used, systemic steroids with a 5
day course of Prednisone is usually given
 Due to the short course of treatment, it is very rare to see
any of the common side effects to corticosteroids:
– HTN, muscle wasting, adrenal suppression, Cushing's,
impaired immune system, hirshuism, anorexia
– Anti-inflammatory:
 Used for prophylaxis such as Cromolyn
 NOT USED during exacerbation of asthma
ASTHMA
– Epinephrine: Fast acting (arrhythmias, nervous,
restless, tremor, headache, insomnia). Used more if
asthma is caused by allergic reaction
– Aminophylline: VERY RARELY SEEN USED Must be
administered through IV Pump! (tachycardia,
nervous, n & v, anxiety, seizures)
– Theophyllin: VERY RARELY SEEN USED! Intoxication
can occur-monitor serum concentrations (seizures,
n/v, tachycardia, anxiety)
ASTHMA

NURSING:
– Know baseline PEAK FLOW METER:
–
–
–
–
–
–
–
–
–
–
–
 Best attempt out of three rapid expirations
 Consider the zone they are in (red, yellow, green)
High Fowler's position and bend forward in mild exacerbation
CPT: Always do aerosol therapy before CPT
Oxygen
Suctioning as needed
VS very important
Monitor PC02 (35-48) & P02 (80-100) pH (7.35-7.45) if
possible (ABG or VBG)
Pulse oximetry (keep PO2 >95%)
Extensive IV fluids to hydrate them and liquify secretions
Antibiotics prophylactically or specific for present infection
Promote normal activities
Prevent further episodes, support.

STATUS ASTHMATICUS:
– Acute, severe prolonged asthma attack.
– Does not respond to normal treatment.
– Person can die.
– Teach patient not to wait too long before
initiating treatment or seeing MD/NP
ASTHMA
ASTHMA
BRONCHIOLITIS
Lower respiratory tract illness caused by virus or bacteria which
causes inflammation and obstruction of the bronchioles
 Occurs mostly in in toddlers and preschoolers
 Most severe in infants under 6 months (if <2 months, very prone
and hospitalized)
 Most common cause: Respiratory Syncytial Virus (RSV):

– Viral infection mostly of bronchiolar level
– Primarily occurs in winter & spring
– The disease usually begins in the fall, reaches a peak in the winter and
decreases in the spring
– Bronchiole mucosa swells and fill with mucous and exudate
– Frequently sloughed epithelial cells obstruct the lumen, mostly on
expiration
– Hyperinflation could occur and cause air trapping
– The trapped air distal to the obstruction causes progressive
overinflation (emphysema)
– Transmitted via respiratory secretions, hand to eye, nose or other
mucous membrane.
BRONCHIOLITIS

ASSESSMENT:
–
–
–
–
–
Rhinorrhea
Pharyngitis
Coughing
Sneezing
Breath sounds: wheezing, diffuse crackles/rhonchi,
rales
– Intermittent fever.
– With progression of illness:
 Increase coughing and wheezing
 Air hunger, tachypnea, retractions, cyanosis
– Severe illness:
 Tachypnea >70/min., listlessness, apneic spells, poor air
exchange, poor breath sounds.
 OM and conjunctivitis may also be present.
– Once the lower airway is involved, classic
manifestations include:
 Signs of altered air exchange, such as wheezing, retractions,
crackles, dyspnea, tachypnea and diminished breath sounds.

INFANTS' ASSESSMENTS:
– Poor feeding, slight lethargy, irritability, possibly low
grade fever

DIAGNOSIS:
– Tests done on nasal or nasopharyngeal secretions
(swab or aspirate) using IFA or ELISA techniques for
RSV antigen detection.
BRONCHIOLITIS

TREATMENT:
– Based on the symptoms
– PREVENTION: RSV Medication: SYNAGIS (palivizumab)
 Monthly injection in the winter-months which provides antibodies
 Used for premis and other high-risk groups
– High humidity
– Adequate fluid intake
– Rest
– Medications depending on symptoms and cause
– Hospitalization:
 Usually recommended for children with complicating conditions,
such as underlying lung or heart disease.
 The child who is tachypneic, has marked retractions, seems
listless, history of poor fluid Intake should also be admitted.
 Any infant born premature or SGA
– Mist therapy combined with oxygen to maintain adequate O2
saturations
– IV fluids are preferred until crisis of the disease has passed
– Blood gas values guide the therapy (if available)
BRONCHIOLITIS

MEDICATIONS:
– Bronchodilators, corticosteroids, cough suppressants and
antibiotics have not proved to be effective.
– Bronchodilators may be used for symptomatic wheezing
– For intubated and vetilated patients:
 FUROSEMIDE, THEOPHYLLINE AND CORTICOSTEROIDS
– RIBAVIRIN
 An anti-viral agent is the only specific therapy available for
RSV.
 HOWEVER, studies have NOT confirmed its effectiveness
and it is therefore reserved for life-threatening cases (i.e.
premature infants, underlying conditions, etc)
 VERY RARELY SEEN USED!
 Ribavirin has many precautions:
– Visitors and nurses must know about the potential
harm to themselves if exposed to the drug
 Teratogenic: Pregnant women should not be
exposed to medication
 Cannot use contact lenses with administration as it
calcifies
– Administered via mist in hood or tent
BRONCHIOLITIS

High risk infants and children:
– RespiGam (RSV immune globulin) can be used as
preventative treatment for RSV as well as Synagis

NURSING:
– Hand washing and not touching nasal mucosa or
conjunctiva
– Diminish the number of personnel, visitors and
uninfected patients in contact with the child
– Nurses try to avoid taking other patients who are
considered high risk for catching RSV.
THE END!