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 in Pediatrics
    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
 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):
    –   Increased pulmonary blood flow
    –   Decreased pulmonary blood flow
    –   Obstruction to blood flow out of the heart
    –   Mixed blood flow (saturated and desaturated)
 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
   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

 Aortic Stenosis
 Pulmonary Stenosis
 Coarctation of the Aorta
         Overall Assessment:
           L-to-R or Obstructive
   May be asymptomatic
   May show signs and symptoms of CHF
   May exhibit failure to thrive
   Growth retardation
   Diaphoresis
   Fatigue
   Tachypnea
   Poor eating
   Dyspnea
   Hypoxemia
 Assess respiratory status for the presence of
  nasal flaring and use of accessory muscle
 Auscultate lungs for the presence of crackles and
 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
 Assess calorie intake
 Plan interventions to allow maximal rest for the
 Allow parent or child if appropriate to verbalize
  feelings and concerns regarding disorder
   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
– Symptoms occur in the first week of life
– Dyspnea after feeding, crying, or other
– 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
 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
   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
   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
    – 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
   Assessment of Early and Late Symptoms
    – 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
–   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
   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
   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
   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
    – 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.
   Angiotensin-converting enzyme (ACE inhibitors)
     – Inhibits the normal function of the renin-
       angiotensin 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.
    – 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
   PDA (Patent Ductus Arteriosis)
    –   Indomethacin (Indocin)
    –   Prostaglandin E1
    –   Coil embolization
    –   VATS = Video-assisted throacoscopic surgery
   ASD (Atrial Septal Defect)
    – Cardiac catheterization
    – Laparoscopic techniques
    – Sternotomy (“open heart”)
Cardiac Catheterization
Video Assisted Thorascopic Surgery
   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
   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
Resection & End-to-End
   Atroventricular Canal (AVC) defect
    – Complete surgical repair in infancy
    – Palliative if older
   TOF (Tetrology of Fallot)
    – Palliative surgery (Blalock-Taussig
    – Corrective open-heart surgery /
   Complete Transposition of Great Vessel
    – NO communication b/t systemic and
      pulmonary circulation
    – Repair WITHIN HOURS! Not conductive with
    – Palliative procedure = pulmonary artery
    – Corrective surgery = arterial switch procedure
   Truncus Arteriosus
    – EXTENSIVE surgical treatment
   Pulmonary Atresia
    – EXTENSIVE surgical treatment
   Tricuspid Atresia
    – Palliative and EXTENSIVE surgical treatment
   Hypoplastic Left Heart Syndrome (HLHS)
    – Multiple stage surgery
    – Heart transplantation
   Endocardial Cushing
    – Total mixing of blood = EXTENSIVE surgical
           What do you think?
   Chronic hypoxemia is clinically manifested
    by which of the following signs?
    – Squatting
    – Polycythemia
    – Clubbing
    – All of the above
              Cardiac Surgery
   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
   Implementation POST-OPERATIVELY
    – 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
   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
   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
           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
   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
   Follow a no added salt diet if prescribed
   Do not add any new foods to the infant’s eating
 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
 Instruct parents about the importance
   Avoid immunizations, invasive procedures,
    and dental visits for 2 months

   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)

   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?

    –   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

   Pre-procedure
    – History including allergies
    – Ht / Wt
    – NPO X 8 hours
    – Mark pulse location
    – Prepare for administration of analgesics
      / sedatives / anxiolytics
   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
   An inflammatory “autoimmune disease” that
    affects the connective tissues of the heart,
    joints, subcutaneous tissues, and/or blood
    vessels of the CNS

   Presents 2 to 6 weeks following an untreated or
    partially treated group A beta-hemolytic
    streptococcal infection of the upper respiratory

   Serious complication is rheumatic heart disease,
    which affects the cardiac valves
   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
   Assessment
    –   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
   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)
          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
   Implementation
    – 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
   Polyarthritis
    – Edema, inflammation and effusion of joint tissue
    – Lat approx. 2 days after fever
   Erythema marginatum
    – Erythematous macules (clear center)
   Subcutaneous nodules
    – 0.5-1cm non-tender swelling nodules that resolves
   Carditis
    – Endocardium, pericardium and myocardium
   CNS involvement
    – CNS irritability = Sydenham’s Chorea (St Vitus Dance): Sudden,
      aimless movements of extremities, facial grimacing and
 An acquired heart disease with
  progressive inflammation of the vessels
  and damage to their walls
 Believed to be caused by a noncontagious
 Without treatment, 20-25% develop
  cardiac sequela
 The leading cause of heart disease in the
  US and Japan
          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
    – Fever for > 5 days
    – Bilateral conjunctival injection (inflammation) without
    – “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
   ACUTE PHASE (1-2 weeks)
    –   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
   SUBACUTE PHASE (2 to 6-8 weeks)
    – Fever resolves and s/s begins to disappear
    – Still irritable
    – Risk of developing coronary artery aneurysm
    – Thrombocytosis and hypercoagulability = coronary
    – Arthritis continues
   Lab results
    – CBC: anemia and leukocytosis
    – Increased ESR
    – Transient elevated LFTs
             Kawasaki Disease
    – All clinical s/s are gone
    – This phase is completed when blood values
      return to normal; usually 6-8 weeks after
    – 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
           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
   Teach to restrict physical activity due to increased bleeding
   Monitor cardiac status (CHF)
   Symptom relief
    – Fever reduction
    – Mouth care
   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
   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
 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
 Sickled cells causes vaso-occlusion =
  vaso-occlusive 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
    – 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
    – Hepatomegaly from extensive lysis of RBC
    – Development of gallstones (obstruction)
    – Kidney ischemia leads to hematuria and decreased renal function
    – Nephrotic syndrome
    – 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
    – Cardiomegaly – from chronic anemia
    – Systolic flow murmur
    – May lead to MI
Sickle Cell Anemia
     4 Types of Sickle Cell Crisis
   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
   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
    – Increased hemoglobin and hematocrit amounts
      compensate during this period
   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
    – Sickled cells block major vessels to the brain
    – Resembles pneumonia
    – May lead to restrictive lung disease and
      pulmonary hypertension
    – 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
    – Long-term oxygen use to enable the oxygen
      to reach the sickled RBCs
    – Decrease in fluids to increase
    – Diet high in iron to decrease anemia
    – Bed rest to minimize energy Expenditure
 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
    – 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
   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
        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
    – Causes vasoconstriction
    – Increases plasma factor VIII
    – Plasminogen activator
   Analgesics, but NO aspirin
   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
           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
   Thalassemia minor
    – Heterozygous
    – Have trait only
    – Asymptomatic or mild microcytic anemia
   Thalassemia intermedia
    – Heterozygous
    – Moderate to sever anemia and spenomegaly
 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
 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
 Avoid infectious persons
 Avoid contact sports
 Support groups
 Genetic counseling
            What do you think?
   Norma, age 2 years, is to begin therapy for B-
    thalassemia. 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
        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
   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
          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
 Often diagnosed with clinical symptoms
 Thrombocytopenia
    – Platelet count <20,000 m3/dl
    – On blood smear, the platelets are large
      (megathrombocytes) = increased marrow
   Usually occurs around time of puberty
    – Danger of bleeding with menses
 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
   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
    – 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
    – 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.
   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
   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
    – Decreased Hgb/Hct = ANEMIA
    – Decreased platelets = thrombocytopenia
   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
    – Acute Myelogenous Leukemia (AML)
          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
   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
    – 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
   Infection
    – A major cause of death in the
      immunosuppressed child
    – Can occur through autocontamination (i.e.
      Staph aureus on the skin) or cross
    – Most common sites of infection are the skin,
      respiratory tract, and GI tract
Neutropenic Diet
Reverse Isolation
   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
    – 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
   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
   Fatigue and Nutrition
    – Assist the child in selecting a well-balanced
    – Provide small meals that require little chewing
    – Assist the child in self-care and mobility
    – NORMALIZE their life as much as possible!
   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
    – Monitor for nausea, vomiting, and diarrhea
    – Assess oral mucous membranes for stomatitis
    – Monitor for renal, hepatic, and cardiac toxicity
   Chemotherapy
    – Inform parents that hair loss may occur from
    – 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
    – Introduce family to other families of children
      with cancer
    Treatment (i.e. NYII protocol)
    – 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
    – Also called consolidation
    – Serves to maintain the remission phase
    – Duration may be 2 1/2 to 3 years
    – Continue corticosteroids, methotrexate and
       Side effects to treatment
   Chemotherapy
    – Nausea and vomiting
    – Alopescia (temporary)
    – Certain agents, i.e cytarabine (cytosin) will cause
      peripheral neuropathy with decreased sensation and
   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
 “Cured” if 5 years in remission after
 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”
Infections of the upper
  and middle airways
 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
   – form antibodies
 Children usually have larger tonsils than adolescents or
 Adenoids are located above the tonsils
 Tonsillitis usually occurs with pharyngitis
    – 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)
    – Based of visual inspection and clinical manifestation
    – Throat culture
     – 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)
    – 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
– 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
   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
   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
   Respiratory system:
     – Bronchi, bronchio-pneumonia & bronchial emphysema (barrel
     – Increased secretion requiring intervention
     – Classic cough secondary to increased mucus
     – Atelectasis
     – Secondary respiratory infections = increased morbidity and
   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
    – 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)
            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
            Wheezing, rales, dyspnea, non-productive dry cough
            May develop atelectasis
            Clubbing
            May have ear, nose and throat problem more than average kids.
      Long-term complications
    Corpulmonal - Right sided enlargement
    CHF = Pulmonary blood flow is obstructed by the mucous
    Esophageal varisies = enlarged vein - may bleed
    Jaundice
    Spleenomegaly
    ducts blocked, tubes blocked, mucous in vaginal tract
    vas deferens blocked
                CYSTIC FIBROSIS
     – Respiratory:
         Cupping, postural drainage, deep breathing,
          bronchodilators (Q4H before meals) – percussion/vibriation
         Aerosol treatments (bronchodilators)
     – Nutrition:
         Increase SALT, especially with exercise
         Salt tablets in warm weather
         Pancreatic enzymes pills taken with meals (non-fat & non-
          protein 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
   Chronic inflammatory disorder of the airway with airway
    obstruction that can be partially or completely reversed
   Chronic condition with acute exacerbations or persistent
   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:
     – Bronchospasm
     – Increased mucous secretion
     – Usually results from allergic hyper response. Before puberty –
     – 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
    – Respiratory difficulties of an asthma attack result from
      inflammation that contributes to airway obstruction
    – Mucus formation , mucosal swelling and airway muscle
    – 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)
   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
    – 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
    – Used in combination with inhaled corticosteroids with persistent
    – 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:
     – 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.)


    – 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
– 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)
    – 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.
    – 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
 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
    – 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.
    – Rhinorrhea
    – Pharyngitis
    – Coughing
    – Sneezing
    – Breath sounds: wheezing, diffuse crackles/rhonchi,
    – 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.
    – Poor feeding, slight lethargy, irritability, possibly low
      grade fever
    – Tests done on nasal or nasopharyngeal secretions
      (swab or aspirate) using IFA or ELISA techniques for
      RSV antigen detection.

    – 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
    – IV fluids are preferred until crisis of the disease has passed
    – Blood gas values guide the therapy (if available)
     – Bronchodilators, corticosteroids, cough suppressants and
       antibiotics have not proved to be effective.
     – Bronchodilators may be used for symptomatic wheezing
     – For intubated and vetilated patients:
         An anti-viral agent is the only specific therapy available for
         HOWEVER, studies have NOT confirmed its effectiveness
           and it is therefore reserved for life-threatening cases (i.e.
           premature infants, underlying conditions, etc)
          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
             – Administered via mist in hood or tent
   High risk infants and children:
    – RespiGam (RSV immune globulin) can be used as
      preventative treatment for RSV as well as Synagis
    – Hand washing and not touching nasal mucosa or
    – 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.

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