oxygenation in pediatrics by prof. unn hidle updated spring 2010

OXYGENATION OXYGENATION in Pediatricsin Pediatrics

ByByProf. Unn HidleProf. Unn Hidle

Updated Spring 2010Updated Spring 2010

Congenital Heart DiseaseCongenital Heart Disease

Incidence 5-8/1000 live birthsIncidence 5-8/1000 live births >35 well-recognized defects, but >35 well-recognized defects, but

most common is Ventricular Septal most common is Ventricular Septal Defect (VSD)Defect (VSD)

Etiology in 90% of CHD is unknownEtiology in 90% of CHD is unknown Multifactorial: Genetic and Multifactorial: Genetic and

environmentalenvironmental

Acyanotic VS CyanoticAcyanotic VS Cyanotic Cyanosis may occur as a later sign in Cyanosis may occur as a later sign in

“acyanotic” conditions. “acyanotic” conditions. Cyanotic conditions may appear Cyanotic conditions may appear

“pink” or of normal skin color“pink” or of normal skin color Classification system based on Classification system based on

hemodynamic characteristics is better hemodynamic characteristics is better (blood flow patterns):(blood flow patterns):– Increased pulmonary blood flowIncreased pulmonary blood flow– Decreased pulmonary blood flowDecreased pulmonary blood flow– Obstruction to blood flow out of the heartObstruction to blood flow out of the heart– Mixed blood flow (saturated and Mixed blood flow (saturated and

desaturated)desaturated)

““Acyanotic”Acyanotic”

Increased pulmonary blood flow OR Increased pulmonary blood flow OR obstruction of blood flow from obstruction of blood flow from ventriclesventricles

LEFT to RIGHT cardiac shuntingLEFT to RIGHT cardiac shunting (except when there is an obstruction)(except when there is an obstruction)

Often leads to CHF because excess Often leads to CHF because excess blood is going to the pulmonary blood is going to the pulmonary circulationcirculation

Murmurs are commonly auscultatedMurmurs are commonly auscultated

LEFT-TO-RIGHT CARDIAC LEFT-TO-RIGHT CARDIAC SHUNTSSHUNTS

DescriptionDescription– Blood is shunted to the right side of Blood is shunted to the right side of

the heart because the left side is the heart because the left side is normally functioning under a higher normally functioning under a higher pressure than the rightpressure than the right

– Oxygenated and unoxygenated Oxygenated and unoxygenated blood mixblood mix, which results in , which results in increased pulmonary bloodincreased pulmonary blood flow flow because the opening sends more because the opening sends more blood to the right side of the heart blood to the right side of the heart than normalthan normal

Types of L-to-R shuntingTypes of L-to-R shunting

Atrial-Septal Defect (ASD)Atrial-Septal Defect (ASD) Ventricular Septal Defect (VSD)Ventricular Septal Defect (VSD) Patent Ductus Arteriosus (PDA)Patent Ductus Arteriosus (PDA) Atrioventricular Canal Defect (AVCD)Atrioventricular Canal Defect (AVCD)

Obstructive/stenotic lesionsObstructive/stenotic lesions

Narrowing or constriction of an Narrowing or constriction of an opening in a valve or vessel that opening in a valve or vessel that results in obstruction of blood flow results in obstruction of blood flow through the areathrough the area

TYPES:TYPES: Aortic StenosisAortic Stenosis Pulmonary StenosisPulmonary Stenosis Coarctation of the AortaCoarctation of the Aorta

Overall Assessment: Overall Assessment: L-to-R or ObstructiveL-to-R or Obstructive

May be asymptomaticMay be asymptomatic May show signs and symptoms of CHFMay show signs and symptoms of CHF May exhibit failure to thriveMay exhibit failure to thrive Growth retardationGrowth retardation DiaphoresisDiaphoresis FatigueFatigue TachypneaTachypnea Poor eatingPoor eating DyspneaDyspnea HypoxemiaHypoxemia

ImplementationImplementation Assess respiratory status for the presence of Assess respiratory status for the presence of

nasal flaring and use of accessory musclenasal flaring and use of accessory muscle Auscultate lungs for the presence of crackles Auscultate lungs for the presence of crackles

and rhonchiand rhonchi Assess for signs of CHF such as fluid retention Assess for signs of CHF such as fluid retention

in the eyes, hands, feet, and chestin the eyes, hands, feet, and chest Assess for diuresisAssess for diuresis Assess urine output; weighing diapers is Assess urine output; weighing diapers is

necessarynecessary Assess calorie intakeAssess calorie intake Plan interventions to allow maximal rest for the Plan interventions to allow maximal rest for the

childchild Allow parent or child if appropriate to verbalize Allow parent or child if appropriate to verbalize

feelings and concerns regarding disorderfeelings and concerns regarding disorder

““Cyanotic”Cyanotic”

RIGHT to LEFT cardiac shuntRIGHT to LEFT cardiac shunt– Occur when blood is shunted to the left Occur when blood is shunted to the left

side of the heart because one of the right side of the heart because one of the right heart chambers has a higher pressureheart chambers has a higher pressure

– Oxygenated blood mixes with Oxygenated blood mixes with unoxygenated blood; cyanosis occursunoxygenated blood; cyanosis occurs

– Decreased pulmonary blood flow or mixed Decreased pulmonary blood flow or mixed blood flowblood flow

– Deoxygenated blood is circulating where Deoxygenated blood is circulating where oxygenated blood should oxygenated blood should

Usually does not lead to CHFUsually does not lead to CHF

Types of R- to – L shunting Types of R- to – L shunting

Tetralogy of Fallot (both)Tetralogy of Fallot (both) Transposition of the Great ArteriesTransposition of the Great Arteries Truncus ArteriosusTruncus Arteriosus Pulmonary Atresia Pulmonary Atresia Tricuspid AtresiaTricuspid Atresia Hypoplastic Left Heart SyndromeHypoplastic Left Heart Syndrome

AssessmentAssessment– Symptoms occur in the first week of lifeSymptoms occur in the first week of life– Dyspnea after feeding, crying, or other Dyspnea after feeding, crying, or other

activitiesactivities– Hypercyanotic or “tet spells” (blue spells) Hypercyanotic or “tet spells” (blue spells)

characterized by increased respiratory characterized by increased respiratory rate and depth and increased hypoxemia rate and depth and increased hypoxemia with TOFwith TOF

– Squatting episodesSquatting episodes with tetralogy of Fallot with tetralogy of Fallot– Signs of CHFSigns of CHF– Respiratory distressRespiratory distress– Clubbing of digitsClubbing of digits– Poor growthPoor growth– TachycardiaTachycardia

ImplementationImplementation Monitor vital signsMonitor vital signs Monitor respiratory status notifying physician Monitor respiratory status notifying physician

if any changes occurif any changes occur Auscultate breath sounds for crackles or ralesAuscultate breath sounds for crackles or rales Keep child as stress free as possibleKeep child as stress free as possible If respiratory effort is increased, place child in If respiratory effort is increased, place child in

reverse Trendelenburg (elevate head and reverse Trendelenburg (elevate head and upper body) to decrease the work of upper body) to decrease the work of breathingbreathing

Monitor for hypercyanosis and place child in Monitor for hypercyanosis and place child in knee-chest position and notify physicianknee-chest position and notify physician

Administer humidified oxygen as prescribedAdminister humidified oxygen as prescribed

Provide endotracheal tube and Provide endotracheal tube and ventilator care as prescribed and ventilator care as prescribed and restrain hands of intubated childrestrain hands of intubated child

Monitor for signs of CHFMonitor for signs of CHF Monitor body weight (daily weight)Monitor body weight (daily weight) Monitor I&O and notify physician if a Monitor I&O and notify physician if a

decrease in urine output occursdecrease in urine output occurs Palpate liver noting enlargement, Palpate liver noting enlargement,

which is an indication of right-sided which is an indication of right-sided heart failureheart failure

Administer diuretics as prescribedAdminister diuretics as prescribed

CHF – What do you think?CHF – What do you think?

When children develop congestive When children develop congestive heart failure from a congenital heart heart failure from a congenital heart defect, the failure is usually:defect, the failure is usually:– Right-sided onlyRight-sided only– Left-sided onlyLeft-sided only– Cor pulmonaleCor pulmonale– Both Right- and Left-sidedBoth Right- and Left-sided

CONGESTIVE HEART CONGESTIVE HEART FAILUREFAILURE

DescriptionDescription– Inability of the heart to pump sufficiently to Inability of the heart to pump sufficiently to

meet the metabolic needs of the bodymeet the metabolic needs of the body– Increased right ventricular workload leading to Increased right ventricular workload leading to

right sided heart failureright sided heart failure– In infants and children, inadequate cardiac In infants and children, inadequate cardiac

output is most commonly caused by congenital output is most commonly caused by congenital heart defects that produce an excessive heart defects that produce an excessive volume or pressure load on the myocardiumvolume or pressure load on the myocardium

– In children a In children a combination of both left-sided combination of both left-sided and right-sided heart failureand right-sided heart failure is usually present is usually present

Congestive Heart FailureCongestive Heart Failure

Common causesCommon causes– Volume overload – especially in L-to-R Volume overload – especially in L-to-R

shuntingshunting– Pressure overload – obstructive lesion Pressure overload – obstructive lesion

(i.e. coarctation of the aorta)(i.e. coarctation of the aorta)– Decreased contractility – cardiac Decreased contractility – cardiac

myopathy or myocardial ischemiamyopathy or myocardial ischemia– High cardiac output demandsHigh cardiac output demands

Mostly seen in L-to-R shuntingMostly seen in L-to-R shunting– ASD, VSD, PDA ASD, VSD, PDA

Congestive Heart FailureCongestive Heart Failure Assessment of Early and Late SymptomsAssessment of Early and Late Symptoms

– TachycardiaTachycardia– Arrhythmia – gallop (S3 and S4)Arrhythmia – gallop (S3 and S4)– Poor perfusionPoor perfusion– Mild cyanosisMild cyanosis– Tachypnea leading to dyspnea during feedingTachypnea leading to dyspnea during feeding– Poor feedingPoor feeding– Poor weight gain due to increased metabolic Poor weight gain due to increased metabolic

rate – FIRST SIGN!rate – FIRST SIGN!– Activity intoleranceActivity intolerance– Diaphoresis during feedingDiaphoresis during feeding

Congestive Heart FailureCongestive Heart Failure– RetractionsRetractions– WheezingWheezing– Cough/hoarseness (pressure on the laryngeal Cough/hoarseness (pressure on the laryngeal

nerves)nerves)– OrthopneaOrthopnea– Hepatosplenomegaly due to pooling of blood in Hepatosplenomegaly due to pooling of blood in

the portal circulation and accumulation in the the portal circulation and accumulation in the hepatic tissuehepatic tissue

– Edema / weight gain from Na and H2O retentionEdema / weight gain from Na and H2O retention– AscitisAscitis– Pleaural effusionPleaural effusion– Distended neck and peripheral veins from Distended neck and peripheral veins from

consistent increased CVPconsistent increased CVP– Prolonged CHF leads to developmental delaysProlonged CHF leads to developmental delays


Commonly used term for CHF is Commonly used term for CHF is cor cor pulmonalepulmonale resulting from obstructive resulting from obstructive lung disease (i.e. Cystic fibrosis or lung disease (i.e. Cystic fibrosis or bronchopulmonary dysplasia)bronchopulmonary dysplasia)

DiagnosisDiagnosis– Based on clinical S/SBased on clinical S/S– CXR: cardiomegaly and increased CXR: cardiomegaly and increased

pulmonary vascular markingpulmonary vascular marking– EKG: ventrcular hypertrophy (arrhythmias)EKG: ventrcular hypertrophy (arrhythmias)– ECHOECHO


Implementation goals:Implementation goals:– To improve cardiac functionTo improve cardiac function– To remove fluid and NaTo remove fluid and Na– To decrease cardiac demandsTo decrease cardiac demands– To improve tissue oxygenation and To improve tissue oxygenation and

decrease oxygen consumptiondecrease oxygen consumption


ImplementationImplementation– Elevate the head of the bedElevate the head of the bed– Administer oxygen as prescribed during Administer oxygen as prescribed during

stressful periods such as bouts of crying stressful periods such as bouts of crying or invasive proceduresor invasive procedures

– Feed in a relaxed environmentFeed in a relaxed environment– Provide small frequent feedings, which Provide small frequent feedings, which

will be less tiringwill be less tiring– Monitor Monitor STRICT I&OSTRICT I&O and and DAILY WEIGHTDAILY WEIGHT to to

assess for fluid retentionassess for fluid retention– Weigh diapersWeigh diapers


Implementation (cont’d)Implementation (cont’d)– Monitor for facial or peripheral Monitor for facial or peripheral

edema, auscultate lung sounds, and edema, auscultate lung sounds, and report weight gain to physicianreport weight gain to physician

– Monitor electrolyte levelsMonitor electrolyte levels– Treat existing infectionsTreat existing infections– Instruct parents regarding diagnosis Instruct parents regarding diagnosis

and administration of medicationsand administration of medications– Instruct parents CPRInstruct parents CPR

Medications used in CHFMedications used in CHF

DIGOXINDIGOXIN: : Know your Dig!Know your Dig!– Cardiac glycoside = improves function; Cardiac glycoside = improves function;

strengthens heart muscle; slows down strengthens heart muscle; slows down heart rateheart rate

– Therapeutic serum level: Therapeutic serum level: 0.8 – 2.0 ng/ml0.8 – 2.0 ng/ml– Monitor EKG rhythm for desired effect Monitor EKG rhythm for desired effect

(i.e. if prolonged P-R interval = hold and (i.e. if prolonged P-R interval = hold and notify MD)notify MD)

– Monitor AHR (i.e. bradycardia = hold Monitor AHR (i.e. bradycardia = hold dose) – know your child’s normal HR dose) – know your child’s normal HR rangerange

Medications cont.Medications cont. Angiotensin-converting enzyme (ACE Angiotensin-converting enzyme (ACE

inhibitors)inhibitors)– Inhibits the normal function of the renin-Inhibits the normal function of the renin-

angiotensin system in the kidneysangiotensin system in the kidneys– Blocks angiotensin II so instead of Blocks angiotensin II so instead of

vasoconstriction, vasodilatation occurs and vasoconstriction, vasodilatation occurs and arteries opens (decreased BP; improved arteries opens (decreased BP; improved systemic circulation; decreased afterload systemic circulation; decreased afterload and decreased R-L arterial pressure)and decreased R-L arterial pressure)

– Examples Captopril (Capoten); Enalapril Examples Captopril (Capoten); Enalapril (Vasotec) and (Vasotec) and Lisinopril (Zestil)Lisinopril (Zestil) = most = most common (QD)common (QD)

Medications cont.Medications cont.

DIURETICSDIURETICS– Furosemide (Lasix)Furosemide (Lasix) = potassium = potassium

wastingwasting– Spironolactone (Aldactone)Spironolactone (Aldactone) = =

potassium sparing --- Be careful potassium sparing --- Be careful with IV additives!with IV additives!

What do you think?What do you think?

The two main angiotensin-converting The two main angiotensin-converting enzyme (ACE) inhibitors most enzyme (ACE) inhibitors most commonly used for children with CHF commonly used for children with CHF are:are:– Digoxin and captoprilDigoxin and captopril– Enalapril and CaptoprilEnalapril and Captopril– Enalapril and furosemideEnalapril and furosemide– Spironlolactone and captoprilSpironlolactone and captopril


The calories are usually increased for The calories are usually increased for an infant with CHF by:an infant with CHF by:– Increasing the frequency of feedingsIncreasing the frequency of feedings– Introducing solids into the dietIntroducing solids into the diet– Increasing the density of the formulaIncreasing the density of the formula– Gastrostomy feedingsGastrostomy feedings

Treatment Options For Various Treatment Options For Various ConditionsConditions

PDA (Patent Ductus Arteriosis)PDA (Patent Ductus Arteriosis)– Indomethacin (Indocin)Indomethacin (Indocin)– Prostaglandin E1Prostaglandin E1– Coil embolizationCoil embolization– VATS = Video-assisted throacoscopic VATS = Video-assisted throacoscopic

surgerysurgery ASD (Atrial Septal Defect)ASD (Atrial Septal Defect)

– Cardiac catheterizationCardiac catheterization– Laparoscopic techniques Laparoscopic techniques – Sternotomy (“open heart”)Sternotomy (“open heart”)

Cardiac CatheterizationCardiac Catheterization

VATS: VATS: Video Assisted Thorascopic Video Assisted Thorascopic

SurgerySurgery

SternotomySternotomy

VSD (Ventricular Septal Defect)VSD (Ventricular Septal Defect)– Some close on their ownSome close on their own– DiureticsDiuretics– Cadiotonics: Ca Channel Blockers Cadiotonics: Ca Channel Blockers

(Nifedipine or Procardia)(Nifedipine or Procardia)– If surgery, then similar to ASDIf surgery, then similar to ASD

Aortic and Pulmonic StenosisAortic and Pulmonic Stenosis– Balloon angioplastyBalloon angioplasty

Balloon AngioplastyBalloon Angioplasty


Coarctation of the aorta should be Coarctation of the aorta should be suspected when:suspected when:– The BP in the arms is different from the BP The BP in the arms is different from the BP

in the legsin the legs– The BP in the R-arm is different from the The BP in the R-arm is different from the

BP in the L-armBP in the L-arm– Apical pulse is greater than the radial Apical pulse is greater than the radial

pulsepulse– Point of maximum impulse is shifted to Point of maximum impulse is shifted to

the leftthe left

Coarctation of the AortaCoarctation of the Aorta– Specific S/S: Increased BP and Specific S/S: Increased BP and

bounding pulses in upper bounding pulses in upper extremities and decreased BP and extremities and decreased BP and weak/absent pulses (femoral) in weak/absent pulses (femoral) in lower extremitieslower extremities

– Balloon angioplastyBalloon angioplasty– Surgical intervention = end-to-end Surgical intervention = end-to-end

anastomosisanastomosis

Resection & End-to-End Resection & End-to-End AnastomosisAnastomosis

Atroventricular Canal (AVC) defectAtroventricular Canal (AVC) defect– Complete surgical repair in infancyComplete surgical repair in infancy– Palliative if olderPalliative if older

TOF (Tetrology of Fallot)TOF (Tetrology of Fallot)– Palliative surgery (Blalock-Taussig Palliative surgery (Blalock-Taussig

Shunt)Shunt)– Corrective open-heart surgery / Corrective open-heart surgery /

sternotomysternotomy

Complete Transposition of Great Complete Transposition of Great VesselVessel– NO communication b/t systemic and NO communication b/t systemic and

pulmonary circulationpulmonary circulation– Repair WITHIN HOURS! Not conductive Repair WITHIN HOURS! Not conductive

with lifewith life– Palliative procedure = pulmonary artery Palliative procedure = pulmonary artery

bandingbanding– Corrective surgery = arterial switch Corrective surgery = arterial switch

procedureprocedure Truncus ArteriosusTruncus Arteriosus

– EXTENSIVE surgical treatmentEXTENSIVE surgical treatment

Pulmonary AtresiaPulmonary Atresia– EXTENSIVE surgical treatmentEXTENSIVE surgical treatment

Tricuspid AtresiaTricuspid Atresia– Palliative and EXTENSIVE surgical treatmentPalliative and EXTENSIVE surgical treatment

Hypoplastic Left Heart Syndrome Hypoplastic Left Heart Syndrome (HLHS)(HLHS)– Multiple stage surgeryMultiple stage surgery– Heart transplantationHeart transplantation

Endocardial CushingEndocardial Cushing– Total mixing of blood = EXTENSIVE surgical Total mixing of blood = EXTENSIVE surgical

repairrepair


Chronic hypoxemia is clinically Chronic hypoxemia is clinically manifested by which of the following manifested by which of the following signs?signs?– SquattingSquatting– PolycythemiaPolycythemia– ClubbingClubbing– All of the aboveAll of the above

Cardiac SurgeryCardiac Surgery

Implementation Implementation PRE-OPERATIVELYPRE-OPERATIVELY– Similar as with CHFSimilar as with CHF

Remember HOB elevated / high Fowlers with Remember HOB elevated / high Fowlers with acyanotic conditions and knee-chest position acyanotic conditions and knee-chest position (squatting) with cyanotic conditions – (squatting) with cyanotic conditions – GENERALLY!GENERALLY!

– Prepare for procedurePrepare for procedure Developmentally appropriate teachingDevelopmentally appropriate teaching Preparing what is to be expected post-operativelyPreparing what is to be expected post-operatively

– EquipmentEquipment– Pulmonary exercisesPulmonary exercises


The MOST painful part of cardiac The MOST painful part of cardiac surgery for the child is usually thesurgery for the child is usually the– Thoracotomy incision siteThoracotomy incision site– Graft site on the legGraft site on the leg– Sternotomy incision siteSternotomy incision site– Intravenous insertion sitesIntravenous insertion sites

CARDIAC SURGERYCARDIAC SURGERY

Implementation Implementation POST-OPERATIVELYPOST-OPERATIVELY – NICU/PICUNICU/PICU– Monitor vital signs frequently (as per Monitor vital signs frequently (as per

policy)policy)– Monitor temperature – report ANY fevers! Monitor temperature – report ANY fevers!

** ** Usually low-grade fevers are accepted in 1st Usually low-grade fevers are accepted in 1st 24 hours (i.e. 101-101.5F)24 hours (i.e. 101-101.5F)

– Maintain aseptic techniqueMaintain aseptic technique– Monitor for signs of sepsis such as fever, Monitor for signs of sepsis such as fever,

chills, diaphoresis, lethargy, and altered chills, diaphoresis, lethargy, and altered levels of consciousnesslevels of consciousness

CARDIAC SURGERYCARDIAC SURGERY

Postoperatively Postoperatively (cont’d)(cont’d)– Monitor equipmentMonitor equipment::

Cardiac monitorCardiac monitor IV lines (CVL, PIVL, CVP, A-lines)IV lines (CVL, PIVL, CVP, A-lines) ET tubeET tube Chest tube **** see X-ray ****Chest tube **** see X-ray **** DressingsDressings NGTNGT FoleyFoley RestraintsRestraints

Thoracotomy: Chest tubeThoracotomy: Chest tube

Postoperatively (cont’d)Postoperatively (cont’d)– Assess for signs of discomfort such as Assess for signs of discomfort such as

irritability, changes in heart rate, irritability, changes in heart rate, respiratory rate and blood pressure, and respiratory rate and blood pressure, and inability to sleepinability to sleep

– SedationSedation– Pain Pain – Antibiotics and antipyretics as prescribedAntibiotics and antipyretics as prescribed– Encourage rest periodsEncourage rest periods– Facilitate parent-child contact Facilitate parent-child contact as soon a as soon a

possiblepossible


An infant who weighs 7 kg has just An infant who weighs 7 kg has just returned to the ICU following cardiac returned to the ICU following cardiac surgery. The chest tube has drained surgery. The chest tube has drained 30 ml in the past hour. In this 30 ml in the past hour. In this situation, what is the FIRST action for situation, what is the FIRST action for the nurse to take?the nurse to take?– Notify the surgeonNotify the surgeon– Identify any other signs of hemorrhageIdentify any other signs of hemorrhage– Suction the patientSuction the patient– Identify any other signs of renal failureIdentify any other signs of renal failure

HOME CARE POSTOPERATIVE HOME CARE POSTOPERATIVE CARDIAC SURGERYCARDIAC SURGERY

MOST IMPORTANTMOST IMPORTANT: Follow individualized : Follow individualized plan!plan!

Omit play outside for “several weeks”Omit play outside for “several weeks” Avoid activities where the child could fall, Avoid activities where the child could fall,

such as bike riding, for a period of timesuch as bike riding, for a period of time Avoid crowds in the immediate time after Avoid crowds in the immediate time after

dischargedischarge Follow a no added salt diet Follow a no added salt diet if prescribedif prescribed Do not add any new foods to the infant’s Do not add any new foods to the infant’s

eating scheduleeating schedule

Do not place creams, lotions, or powders Do not place creams, lotions, or powders on incision until completely healedon incision until completely healed

Child may return to school 2-3 weeks Child may return to school 2-3 weeks after discharge starting (after discharge starting (depending on depending on type of procedure and “individualized plantype of procedure and “individualized plan”)”)

No physical education until cleared by No physical education until cleared by MDMD

Instruct parents to discipline the child Instruct parents to discipline the child normallynormally

Instruct parents about the importance Instruct parents about the importance follow-upfollow-up

Avoid immunizations, invasive Avoid immunizations, invasive procedures, and dental visits for 2 procedures, and dental visits for 2 monthsmonths

Advise parents regarding the Advise parents regarding the importance of a dental visit every 6 importance of a dental visit every 6 months after age 3 and to inform the months after age 3 and to inform the dentist of the cardiac problem so that dentist of the cardiac problem so that antibiotics can be prescribed antibiotics can be prescribed if if necessarynecessary ( (new guidelines regarding new guidelines regarding prophylactic antibiotics)prophylactic antibiotics)

Inform parents to call the physician Inform parents to call the physician when coughing, tachypnea, cyanosis, when coughing, tachypnea, cyanosis, vomiting, diarrhea, anorexia, pain, vomiting, diarrhea, anorexia, pain, fever, or any swelling, redness, or fever, or any swelling, redness, or drainage occurs at the site of the drainage occurs at the site of the incisionincision

Critical ThinkingCritical Thinking Tommy, a 4-year old with TOF has just Tommy, a 4-year old with TOF has just

returned from the catheterization returned from the catheterization laboratory. He has vomited, and his mother laboratory. He has vomited, and his mother calls you to the bedside to tell you that he calls you to the bedside to tell you that he is bleeding. You arrive to find Tommy crying is bleeding. You arrive to find Tommy crying and sitting up in a puddle of blood. What is and sitting up in a puddle of blood. What is the the FIRSTFIRST thing you should do? thing you should do?

– Increase the rate of his IV fluidsIncrease the rate of his IV fluids– Give an antiemetic and keep Tommy NPOGive an antiemetic and keep Tommy NPO– Call the cardiologistCall the cardiologist– Lie Tommy down and apply direct pressure above the Lie Tommy down and apply direct pressure above the

catheterization sitecatheterization site

Cardiac CatheterizationCardiac Catheterization

Pre-procedurePre-procedure– History including allergiesHistory including allergies– Ht / WtHt / Wt– NPO X 8 hoursNPO X 8 hours– Mark pulse locationMark pulse location– Prepare for administration of Prepare for administration of

analgesics / sedatives / anxiolyticsanalgesics / sedatives / anxiolytics

Post-procedurePost-procedure– Monitor distal pulses and VS (BP)Monitor distal pulses and VS (BP)– Monitor perfusion: temperature and Monitor perfusion: temperature and

color of affected extremity and color of affected extremity and comparecompare

– Dressing for bleedingDressing for bleeding– Leg extended straight for 4-6 hoursLeg extended straight for 4-6 hours– Strict I&OStrict I&O– Encourage voiding to remove dyeEncourage voiding to remove dye– AnalgesicsAnalgesics

RHEUMATIC FEVERRHEUMATIC FEVER

RHEUMATIC FEVERRHEUMATIC FEVER An inflammatory “autoimmune disease” that An inflammatory “autoimmune disease” that

affects the connective tissues of the heart, affects the connective tissues of the heart, joints, subcutaneous tissues, and/or blood joints, subcutaneous tissues, and/or blood vessels of the CNSvessels of the CNS

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

Serious complication is Serious complication is rheumatic heart rheumatic heart diseasedisease, which affects the cardiac valves, which affects the cardiac valves

RHEUMATIC FEVERRHEUMATIC FEVER

Clinical ManifestationClinical Manifestation– Starts with URIStarts with URI– Inflammatory hemorrhagic bullous lesions Inflammatory hemorrhagic bullous lesions

called called Aschoff bodiesAschoff bodies are formed, which are formed, which causes swelling, fragmentation and causes swelling, fragmentation and alteration in the connective tissues alteration in the connective tissues (circulatory system, brain, pleura and (circulatory system, brain, pleura and joints)joints)

– Fibrous tissue forms causing valve stenosis Fibrous tissue forms causing valve stenosis and occlusion of blood flow = murmurand occlusion of blood flow = murmur

– Carditis is the end resultCarditis is the end result


One of the most common findings on One of the most common findings on physical examination of the child physical examination of the child with acute rheumatic heart disease is with acute rheumatic heart disease is – A Systolic murmurA Systolic murmur– Pleural friction rubPleural friction rub– An ejection clickAn ejection click– A split S2A split S2

Rheumatic FeverRheumatic Fever AssessmentAssessment

– Edema, inflammation of large jointsEdema, inflammation of large joints– Joint painJoint pain– FeverFever– Pallor and weaknessPallor and weakness– Anorexia, weight lossAnorexia, weight loss– Erythematous macular rash on trunk and extremitiesErythematous macular rash on trunk and extremities– ChoreaChorea– Subcutaneous nodules in the joints, scalp, and spineSubcutaneous nodules in the joints, scalp, and spine– ArthritisArthritis– EpistaxisEpistaxis– Abdominal painAbdominal pain– Systolic murmur, tachycardiaSystolic murmur, tachycardia– Pericardial rubPericardial rub

c/o chest pain (pericarditis)c/o chest pain (pericarditis) SOBSOB

Diagnostic EvaluationDiagnostic Evaluation No single S/S or lab test: No single S/S or lab test:

– Based on diagnostic guidelines set by the Based on diagnostic guidelines set by the American Heart Association (Jones criteria): American Heart Association (Jones criteria):

– Increased Anti-Streptolysin-O titers (ASLO)Increased Anti-Streptolysin-O titers (ASLO) Measure of antibodies formed against streptolysin-O Measure of antibodies formed against streptolysin-O

(streptococcal extracellular product which produces lysis of (streptococcal extracellular product which produces lysis of RBC). RBC).

Formed within 7 days and peaks at 4-6 weeks. Formed within 7 days and peaks at 4-6 weeks. Reliable test in evidence of recent streptococcal infectionReliable test in evidence of recent streptococcal infection Normal value is 0-120 TODD units. Value >333 = RFNormal value is 0-120 TODD units. Value >333 = RF 2 tests are required to confirm diagnosis2 tests are required to confirm diagnosis

– Increased C-Reactive Protein (CRP)Increased C-Reactive Protein (CRP)– LeukocytosisLeukocytosis– Anemia Anemia – Increased Erythrocyte Sedimentation Rate (ESR)Increased Erythrocyte Sedimentation Rate (ESR)– Prolonged P-R intervalProlonged P-R interval

RHEUMATIC FEVERRHEUMATIC FEVER ImplementationImplementation

– PREVENTION!!!!!!!!!!!PREVENTION!!!!!!!!!!!– Antibiotics for Strep (PCN or Erythromycin)Antibiotics for Strep (PCN or Erythromycin)– Antibiotics prophylactically (teaching)Antibiotics prophylactically (teaching)– Bedrest until afebrileBedrest until afebrile– Salicylates (Aspirin) is used for the INFLAMMATORY Salicylates (Aspirin) is used for the INFLAMMATORY

PROCESS to decrease fever and comfort (** Pediatrics + PROCESS to decrease fever and comfort (** Pediatrics + Aspirin = Reye’s syndrome – toxic encephalopathy) – This Aspirin = Reye’s syndrome – toxic encephalopathy) – This is an exception!!! is an exception!!!

– Steroids (i.e. prednisone) to decrease inflammationSteroids (i.e. prednisone) to decrease inflammation– Administer anti-inflammatory agents as prescribed and if Administer anti-inflammatory agents as prescribed and if

aspirin is prescribed, it should not be given to a child who aspirin is prescribed, it should not be given to a child who has chicken pox or other viral infectionshas chicken pox or other viral infections

– Initiate seizure precautions if the child is experiencing Initiate seizure precautions if the child is experiencing chorea chorea

Complications of Rheumatic Complications of Rheumatic FeverFever

PolyarthritisPolyarthritis– Edema, inflammation and effusion of joint tissueEdema, inflammation and effusion of joint tissue– Lat approx. 2 days after feverLat approx. 2 days after fever

Erythema marginatumErythema marginatum– Erythematous macules (clear center)Erythematous macules (clear center)

Subcutaneous nodulesSubcutaneous nodules– 0.5-1cm non-tender swelling nodules that resolves0.5-1cm non-tender swelling nodules that resolves

CarditisCarditis– Endocardium, pericardium and myocardiumEndocardium, pericardium and myocardium

CNS involvementCNS involvement– CNS irritability = CNS irritability = Sydenham’s Chorea (St Vitus DanceSydenham’s Chorea (St Vitus Dance): ):

Sudden, aimless movements of extremities, facial Sudden, aimless movements of extremities, facial grimacing and contortions.grimacing and contortions.

KAWASAKI DISEASEKAWASAKI DISEASE

An acquired heart disease with An acquired heart disease with progressive inflammation of the progressive inflammation of the vessels and damage to their wallsvessels and damage to their walls

Believed to be caused by a Believed to be caused by a noncontagious infectionnoncontagious infection

Without treatment, 20-25% develop Without treatment, 20-25% develop cardiac sequelacardiac sequela

The leading cause of heart disease in The leading cause of heart disease in the US and Japanthe US and Japan


The peak age for the incidence of The peak age for the incidence of Kawasaki disease isin theKawasaki disease isin the– Infant age groupInfant age group– Toddler age GroupToddler age Group– School age groupSchool age group– Adolescent age groupAdolescent age group

Diagnosis of Kawasaki Diagnosis of Kawasaki DiseaseDisease

No specific diagnostic testNo specific diagnostic test Dx is based on clinical signs & symptomsDx is based on clinical signs & symptoms

– Fever for > 5 daysFever for > 5 days– Bilateral conjunctival injection (inflammation) Bilateral conjunctival injection (inflammation)

without exudationwithout exudation– ““Strawberry tongue” Strawberry tongue” – Change in extremities: peripheral edema, erythema Change in extremities: peripheral edema, erythema

of palms and soles; desquamation of handsof palms and soles; desquamation of hands– Polymorphous rashPolymorphous rash– Cervical lymphadenopathy (one lymph node Cervical lymphadenopathy (one lymph node

>1.5cm)>1.5cm)

3 Phases of Kawasaki3 Phases of Kawasaki ACUTE PHASE ACUTE PHASE (1-2 weeks)(1-2 weeks)

– Abrupt onset of high fever >5 daysAbrupt onset of high fever >5 days– No response to antipyretics or antibioticsNo response to antipyretics or antibiotics– Bulbar conjunctiva with erythema and tearingBulbar conjunctiva with erythema and tearing– Inflamed pharynxInflamed pharynx– Red, cracked lipsRed, cracked lips– ““Strawberry tongue”Strawberry tongue”– Desquamation of hands and feetDesquamation of hands and feet– Cervical lymphadenopathyCervical lymphadenopathy– VERY irritable and inconsolableVERY irritable and inconsolable

ComplicationsComplications– Myocarditis, CHF and temporary arthritisMyocarditis, CHF and temporary arthritis– EKG changes (ischemia)EKG changes (ischemia)

Kawasaki DiseaseKawasaki Disease

SUBACUTE PHASE SUBACUTE PHASE (2 to 6-8 weeks)(2 to 6-8 weeks)– Fever resolves and s/s begins to disappearFever resolves and s/s begins to disappear– Still irritableStill irritable– Risk of developing coronary artery aneurysmRisk of developing coronary artery aneurysm– Thrombocytosis and hypercoagulability = Thrombocytosis and hypercoagulability =

coronary thrombosiscoronary thrombosis– Arthritis continuesArthritis continues

Lab resultsLab results– CBC: anemia and leukocytosisCBC: anemia and leukocytosis– Increased ESRIncreased ESR– Transient elevated LFTsTransient elevated LFTs

Kawasaki DiseaseKawasaki Disease

CONVALESCENT PHASECONVALESCENT PHASE– All clinical s/s are gone All clinical s/s are gone – This phase is completed when blood This phase is completed when blood

values return to normal; usually values return to normal; usually 6-8 6-8 weeks after onsetweeks after onset

– There is always a There is always a high risk of MIhigh risk of MI that that remainsremains

Treatment of KawasakiTreatment of Kawasaki High dose IV immune globulin (IVIG)High dose IV immune globulin (IVIG)

given within 10 days of infection. One given within 10 days of infection. One infusion of 2 Gm/Kg over 10 hours.infusion of 2 Gm/Kg over 10 hours.

High dose aspirinHigh dose aspirin for the first 2 weeks for the first 2 weeks– Decreases fever, edema and inflammationDecreases fever, edema and inflammation

This combination tends to show good This combination tends to show good results within 24 hoursresults within 24 hours

After 2 weeks of high dose aspirin, change After 2 weeks of high dose aspirin, change to low dose for the next 4-6 weeks to low dose for the next 4-6 weeks (prevent clots)(prevent clots)

Anticoagulation therapy (Coumadine) may Anticoagulation therapy (Coumadine) may be indicatedbe indicated


Because of the drug used for long-Because of the drug used for long-term therapy, children with Kawasaki term therapy, children with Kawasaki disease are at risk fordisease are at risk for– Chicken poxChicken pox– InfluenzaInfluenza– Reye syndromeReye syndrome– Myocardial infarctionMyocardial infarction

Kawasaki - NursingKawasaki - Nursing Teach to restrict physical activity due to increased Teach to restrict physical activity due to increased

bleedingbleeding Monitor cardiac status (CHF)Monitor cardiac status (CHF) Symptom reliefSymptom relief

– Fever reductionFever reduction– Mouth careMouth care

Discharge teachingDischarge teaching– Irritability may last up to 2 monthsIrritability may last up to 2 months– No live immunizations until about 5 months (11 months for No live immunizations until about 5 months (11 months for

Varicella and MMR)Varicella and MMR)– PROM exercisesPROM exercises– Assess for aspirin toxicity (tinnitus, H/A, dizziness and Assess for aspirin toxicity (tinnitus, H/A, dizziness and

confusion)confusion)– CPRCPR

PrognosisPrognosis– Usually full recoveryUsually full recovery

Hematologic DisordersHematologic Disorders

Sickle Cell AnemiaSickle Cell Anemia


Normal adult hemoglobin (HgbA) is Normal adult hemoglobin (HgbA) is partly or completely replaced by partly or completely replaced by abnormal sickl hemoglobin (HgbS)abnormal sickl hemoglobin (HgbS)

Sickle cell trait is the heterozygous Sickle cell trait is the heterozygous form form

Primarily seen in the African American Primarily seen in the African American groupgroup

The RBCs are “sickled” and cannot The RBCs are “sickled” and cannot carry enough Hgbcarry enough Hgb

They are destroyed earlier than normalThey are destroyed earlier than normal


Two main occurrences:Two main occurrences:– OBSTRUCTIONOBSTRUCTION from sickled RBCs from sickled RBCs– DESTRUCTIONDESTRUCTION of RBC of RBC

Sickled cells causes vaso-occlusion = Sickled cells causes vaso-occlusion = vaso-occlusive crisis (“sickle cell crisis”)vaso-occlusive crisis (“sickle cell crisis”)

Results in local hypoxia leading to tissue Results in local hypoxia leading to tissue ischemia and infarction (cellular death)ischemia and infarction (cellular death)

Can be life threateningCan be life threatening

S/S of Sickle Cell AnemiaS/S of Sickle Cell Anemia SPLEENSPLEEN

– Splenic sequestial crisis from engorgement Splenic sequestial crisis from engorgement with sickled cells (enlarged and no function)cells (enlarged and no function)

– By age 5 years, functional cells are replaced by fibrotic By age 5 years, functional cells are replaced by fibrotic tissue = tissue = functional ASPLENIAfunctional ASPLENIA

– Prone to infectionProne to infection HEPATICHEPATIC

– Hepatomegaly from extensive lysis of RBCHepatomegaly from extensive lysis of RBC– Development of gallstones (obstruction)Development of gallstones (obstruction)

RENALRENAL– Kidney ischemia leads to hematuria and decreased renal Kidney ischemia leads to hematuria and decreased renal

functionfunction– Nephrotic syndromeNephrotic syndrome

BONESBONES– Bone changes due to bone marrow’s hyperplastic Bone changes due to bone marrow’s hyperplastic

production of RBC to compensate for destruction of sickled production of RBC to compensate for destruction of sickled cellscells

– Bone marrow crisis (aplastic crisis) – may lead to Bone marrow crisis (aplastic crisis) – may lead to osteoporosisosteoporosis

– Lordosis and kyphosisLordosis and kyphosis– Osteomylitis secondary to chronic hypoxiapOsteomylitis secondary to chronic hypoxiap

CNSCNS– CVA due to occlusion, ischemia and infarctionCVA due to occlusion, ischemia and infarction

HEARTHEART– Cardiomegaly – from chronic anemiaCardiomegaly – from chronic anemia– Systolic flow murmurSystolic flow murmur– May lead to MIMay lead to MI

4 Types of Sickle Cell Crisis4 Types of Sickle Cell Crisis

Vaso-occlusive crisisVaso-occlusive crisis– Most common and non-life threateningMost common and non-life threatening– PAIN (joints; abdomen)PAIN (joints; abdomen)– PriapismPriapism

Spenic Sequestration crisisSpenic Sequestration crisis– More severeMore severe– Blood pools in spleen and causes quick Blood pools in spleen and causes quick

drop in blood volume = drop in blood volume = hypotensionhypotension and and hypovolemic shockhypovolemic shock

Other manifestationsOther manifestations

Exercise intoleranceExercise intolerance AnorexiaAnorexia Jaundice / icteric scleraJaundice / icteric sclera GallstonesGallstones Leg ulcersLeg ulcers Growth retardations (height and Growth retardations (height and

weight)weight) Delayed sexual maturationDelayed sexual maturation Decreased fertilityDecreased fertility

Aplastic crisisAplastic crisis– BM crisis: decreased RBC production BM crisis: decreased RBC production – Triggered by viral infection: human parvovirusTriggered by viral infection: human parvovirus– Increased destruction of RBCs leads to anemia Increased destruction of RBCs leads to anemia

(megaloblastic anemia resulting from folic acid (megaloblastic anemia resulting from folic acid and Vit. B deficiency)and Vit. B deficiency)

– PRBC transfusion usually requiredPRBC transfusion usually required

Hyperhemolytic crisisHyperhemolytic crisis– Very rareVery rare– Decreased production of non-sickled RBC & Decreased production of non-sickled RBC &

overall hyperplastic productionoverall hyperplastic production– Accelerated rate of RBC destruction and BM Accelerated rate of RBC destruction and BM

tries to replace dead RBCtries to replace dead RBC

Diagnosis of SCADiagnosis of SCA

Blood smearBlood smear– View sickled cellsView sickled cells– Hgb electrophoresis = “fingerprints” which Hgb electrophoresis = “fingerprints” which

detects different types of Hgb (A=Adult; detects different types of Hgb (A=Adult; F=Fetal; S=sickled)F=Fetal; S=sickled)

CBCCBC– Increased WBC, Platelets and IronIncreased WBC, Platelets and Iron

GeneticsGenetics– Perinatally through amniocentesis and CVSPerinatally through amniocentesis and CVS

Signs and symptomsSigns and symptoms– Pain: joints, back and abdomenPain: joints, back and abdomen

Intervention for Sickle Cell Intervention for Sickle Cell CrisisCrisis

HydrationHydration to to preventprevent sickling of cells sickling of cells Prevent acidosis: Prevent acidosis: O2 supplementationO2 supplementation Supplement with Folic acidSupplement with Folic acid Bedrest to decrease energy expenditureBedrest to decrease energy expenditure PROMPROM PRBC transfusion prn (maintain Hgb PRBC transfusion prn (maintain Hgb

>10mg/dl)>10mg/dl) Analgesics / OpioidsAnalgesics / Opioids Heat for relief of joint painHeat for relief of joint pain Strict I&O and monitor weightStrict I&O and monitor weight Regular eye exam: damage to the retinaRegular eye exam: damage to the retina


Infants are often not diagnosed with sickle Infants are often not diagnosed with sickle cell anemia until they are 1 year of age. cell anemia until they are 1 year of age. Why?Why?– Usually there are no symptoms until after age Usually there are no symptoms until after age

1 year1 year– High intake of fluids from formulas prevents High intake of fluids from formulas prevents

sickle cell crises during this age.sickle cell crises during this age.– Fetal Hemoglobin is present during the first Fetal Hemoglobin is present during the first

year of lifeyear of life– Increased hemoglobin and hematocrit amounts Increased hemoglobin and hematocrit amounts

compensate during this periodcompensate during this period

““Treatment” Treatment” SplenectomySplenectomy

– In children < 5 years old (remember, they have In children < 5 years old (remember, they have functional asplenia > 5 years of age)functional asplenia > 5 years of age)

– May be life saving! Spleen is the major site of May be life saving! Spleen is the major site of sickling and destruction of RBCsickling and destruction of RBC

Prophylactic PO PenicillinProphylactic PO Penicillin– Reduce the chance of pneumococcal sepsisReduce the chance of pneumococcal sepsis

VaccinesVaccines– HIB (Haemophilus influenza type B)HIB (Haemophilus influenza type B)– MeningococcalMeningococcal

Bone Marrow TransplantBone Marrow Transplant

Complications of SCAComplications of SCA

STROKESTROKE– Sickled cells block major vessels to the Sickled cells block major vessels to the

brainbrain CHEST SYNDROMECHEST SYNDROME

– Resembles pneumoniaResembles pneumonia– May lead to restrictive lung disease and May lead to restrictive lung disease and

pulmonary hypertensionpulmonary hypertension OVERWHELMING INFECTIONSOVERWHELMING INFECTIONS

– Due to defective splenic functionDue to defective splenic function– May cause death in children <5 yearsMay cause death in children <5 years


Pat is a 4 year old being admitted Pat is a 4 year old being admitted because of diminished RBC because of diminished RBC production triggered by a viral production triggered by a viral infection. What type of sickle cell infection. What type of sickle cell crisis is she crisis is she most likelymost likely experiencing? experiencing?– Vasoocclusive crisisVasoocclusive crisis– Splenic sequestration crisisSplenic sequestration crisis– Aplastic CrisisAplastic Crisis– Hyperhemolytic crisisHyperhemolytic crisis


Therapeutic management of sickle Therapeutic management of sickle cell crisis generally includes which cell crisis generally includes which one of the following?one of the following?– Long-term oxygen use to enable the Long-term oxygen use to enable the

oxygen to reach the sickled RBCsoxygen to reach the sickled RBCs– Decrease in fluids to increase Decrease in fluids to increase

hemoconcentrationhemoconcentration– Diet high in iron to decrease anemiaDiet high in iron to decrease anemia– Bed rest to minimize energy ExpenditureBed rest to minimize energy Expenditure

HEMOPHILIAHEMOPHILIA

A group of bleeding disorders A group of bleeding disorders resulting from a congenital deficiency resulting from a congenital deficiency of specific coagulation proteinsof specific coagulation proteins

Excessive uncontrollable bleeding!Excessive uncontrollable bleeding! X-linked recessive trait: MOTHER X-linked recessive trait: MOTHER

passes it on to SON passes it on to SON – Boys (XY): Hemophilia is transmitted on Boys (XY): Hemophilia is transmitted on

X chromosomeX chromosome– Girls (XX): Inherits the carrier status Girls (XX): Inherits the carrier status

ONLYONLY

Two types of HemophiliaTwo types of Hemophilia

Hemophilia AHemophilia A– Classic hemophiliaClassic hemophilia– Most common – 75%Most common – 75%– Deficiency in clotting Factor VIIIDeficiency in clotting Factor VIII

Hemophilia BHemophilia B– Also called “Christmas disease”Also called “Christmas disease”– Deficiency of clotting Factor IXDeficiency of clotting Factor IX

Diagnosis of HemophiliaDiagnosis of Hemophilia

Based on history of bleedingBased on history of bleeding Abnormal PTT Abnormal PTT

– Prolonged > 40 seconds -- (Normal = 30-Prolonged > 40 seconds -- (Normal = 30-40 seconds)40 seconds)

Platelet functionPlatelet function– NormalNormal

Specific tests for factor VIII and IX Specific tests for factor VIII and IX assayassay

DNA testingDNA testing

Signs and symptomsSigns and symptoms InfantInfant

– Excessive bleeding at umbilical or circumcision Excessive bleeding at umbilical or circumcision sitesite

ChildChild– Excessive bleeding anywhere in the bodyExcessive bleeding anywhere in the body– HematuriaHematuria– Melena (black, tarry feces)Melena (black, tarry feces)– EpistaxisEpistaxis– Hemarthrosis = joint bleedingHemarthrosis = joint bleeding

Very painfulVery painful Dangerous = blood lossDangerous = blood loss Bone changes and cripling deformities over yearsBone changes and cripling deformities over years

HemophiliaHemophilia

Treatment of HemophiliaTreatment of Hemophilia Main focus: Main focus: replacement of missing clotting replacement of missing clotting

factorsfactors Blood transfusion for Factors VIII or IXBlood transfusion for Factors VIII or IX Cryoprecipitate (plasma derivative with factor VIII): Cryoprecipitate (plasma derivative with factor VIII):

NO LONGER RECOMMENDED! – cannot guarantee NO LONGER RECOMMENDED! – cannot guarantee safe elimination of Hepatitis or HIVsafe elimination of Hepatitis or HIV

CorticosteroidsCorticosteroids DDAVP (I-deamino-8-D-arginine vasopressin): IV or DDAVP (I-deamino-8-D-arginine vasopressin): IV or

intranasalintranasal– Causes vasoconstrictionCauses vasoconstriction– Increases plasma factor VIIIIncreases plasma factor VIII– Plasminogen activatorPlasminogen activator

Analgesics, but Analgesics, but NO aspirinNO aspirin

TEACHINGTEACHING Goal is to prevent bleeding!Goal is to prevent bleeding! Dental care: soft toothbrushDental care: soft toothbrush No contact sportsNo contact sports Avoid aspirinAvoid aspirin Diet: avoid obesity (stress on joints); iron rich foodsDiet: avoid obesity (stress on joints); iron rich foods Genetic counselingGenetic counseling Support groupsSupport groups Teach s/s of bleedingTeach s/s of bleeding If bleeding occurs: If bleeding occurs: RICERICE

– R = RestR = Rest– I = IceI = Ice– C = CompressionC = Compression– E = Elevation E = Elevation

Complications from Complications from HemophiliaHemophilia

Bone changes = crippling deformitiesBone changes = crippling deformities GI hemorrhageGI hemorrhage Intracranial bleedingIntracranial bleeding Bruising over spinal cord = paralysisBruising over spinal cord = paralysis Airway obstruction if bleeding in Airway obstruction if bleeding in

throat/pharynxthroat/pharynx


Which one of the following is the Which one of the following is the mostmost frequent form of internal frequent form of internal bleeding in the child with bleeding in the child with hemophilia?hemophilia?– HemarthrosisHemarthrosis– EpistaxisEpistaxis– Intracranial hemorrhageIntracranial hemorrhage– Gastrointestinal tract hemorrhageGastrointestinal tract hemorrhage

Beta (B) Thalassemia MajorBeta (B) Thalassemia Major Also called Also called Cooley’s anemiaCooley’s anemia Group of disorders characterized by Group of disorders characterized by reduced reduced

production of the globin chains in the synthesis production of the globin chains in the synthesis of hemoglobin (B-chain affected) of hemoglobin (B-chain affected)

Cultures such as Italians, Greeks and Syrian Cultures such as Italians, Greeks and Syrian living near the Mediterranean Sea are affectedliving near the Mediterranean Sea are affected

Another group is the Alpha, affecting groups Another group is the Alpha, affecting groups such as Chinese, Thai and Africans such as Chinese, Thai and Africans

Autosomal recessive trait: both males and Autosomal recessive trait: both males and females must be carriers in order to pass it onfemales must be carriers in order to pass it on

Types of ThalassemiaTypes of Thalassemia Thalassemia majorThalassemia major

– Cooley’s anemiaCooley’s anemia– HomozygousHomozygous– Those who have the diseasesThose who have the diseases– Severe anemia and not compatible with life without Severe anemia and not compatible with life without

transfusion supporttransfusion support Thalassemia minorThalassemia minor

– HeterozygousHeterozygous– Have trait onlyHave trait only– Asymptomatic or mild microcytic anemiaAsymptomatic or mild microcytic anemia

Thalassemia intermediaThalassemia intermedia– HeterozygousHeterozygous– Moderate to sever anemia and spenomegalyModerate to sever anemia and spenomegaly

DiagnosisDiagnosis

Hemoglobin electrophoresis (analysis Hemoglobin electrophoresis (analysis of protein mixtures) confirms the of protein mixtures) confirms the diagnosis and distinguishes the type diagnosis and distinguishes the type and severityand severity

There is no cure!There is no cure! Children usually die in late adolescentChildren usually die in late adolescent Must cope with frequent blood Must cope with frequent blood

transfusions in the late stages transfusions in the late stages Genetic counseling in all types Genetic counseling in all types

Signs and symptomsSigns and symptoms

PallorPallor Fatigue r/t hypoxiaFatigue r/t hypoxia Poor feedingPoor feeding HepatosplenomegalyHepatosplenomegaly Neurological impairment r/t hypoxiaNeurological impairment r/t hypoxia Bone and joint painBone and joint pain AnorexiaAnorexia Exercise intoleranceExercise intolerance Growth retardationGrowth retardation

Long term complicationsLong term complications Splenomegaly (splenectomy)Splenomegaly (splenectomy) Hepatomegaly: cirrhosis with jaundice (Hepatomegaly: cirrhosis with jaundice (yellow from yellow from

bilirubin mixed with retained ironbilirubin mixed with retained iron) = Bronze skin color) = Bronze skin color Weak bones due to osteoporosis = spontaneous fracturesWeak bones due to osteoporosis = spontaneous fractures Skeletal changesSkeletal changes

– Thick cranial bonesThick cranial bones– Enlarged maxillaEnlarged maxilla– Prominent facial bonesProminent facial bones– Bossing (but not from hydrocephalus, from bone changes)Bossing (but not from hydrocephalus, from bone changes)– ““Frankenstein appearance”Frankenstein appearance”

““Mongoloid” appearanceMongoloid” appearance Teeth:Teeth:

– Malocclusion (malposition of mandibular and maxillary teeth)Malocclusion (malposition of mandibular and maxillary teeth)– ““Buck teeth” = crowded and crooked teathBuck teeth” = crowded and crooked teath

Cardiac abnormalitiesCardiac abnormalities– DysrhythmiasDysrhythmias– CHFCHF– Fibrotic cardiac muscleFibrotic cardiac muscle

Gall bladder diseaseGall bladder disease– cholecystectomycholecystectomy

Growth retardationGrowth retardation Endocrine problem (DM) due to iron Endocrine problem (DM) due to iron

deposit’s effect on pancreasedeposit’s effect on pancrease Delayed sexual maturationDelayed sexual maturation

– Small genitaliaSmall genitalia– Decreased pubic hairDecreased pubic hair

TreatmentTreatment SUPPORTIVE!SUPPORTIVE! TransfusionsTransfusions are the foundation of medical are the foundation of medical

management (keep Hgb >9.5)management (keep Hgb >9.5) Iron Chelation theraphyIron Chelation theraphy = Deferoxamine = Deferoxamine

– Due to multiple transfusion, Due to multiple transfusion, iron stores increasesiron stores increases– Helps pull iron from tissues and eliminate FeHelps pull iron from tissues and eliminate Fe– Give Give withwith oral Vitamin C (to draw iron out of oral Vitamin C (to draw iron out of

tissues)tissues)– It is given either of 2 ways:It is given either of 2 ways:

SQ – over 8-10 hours 5-7 days/week (during sleep)SQ – over 8-10 hours 5-7 days/week (during sleep) IV – over 4 hoursIV – over 4 hours Can also be given as deep IM injectionCan also be given as deep IM injection

Prophylactic AntibioticsProphylactic Antibiotics

Folic acid supplements (Vitamin B9) – Folic acid supplements (Vitamin B9) – stimulates production of RBCsstimulates production of RBCs

Avoid injury, rest, good hygiene and Avoid injury, rest, good hygiene and nutritionnutrition

Avoid infectious personsAvoid infectious persons Avoid contact sportsAvoid contact sports Support groupsSupport groups Genetic counselingGenetic counseling

What do you think?What do you think? Norma, age 2 years, is to begin therapy for Norma, age 2 years, is to begin therapy for

B-thalassemia. Which one of the following B-thalassemia. Which one of the following would be appropriate for the nurse to include would be appropriate for the nurse to include in the educational session held with the in the educational session held with the parents?parents?– Norma will need frequent blood transfusions to Norma will need frequent blood transfusions to

keep her Hgb level above 12g/dlkeep her Hgb level above 12g/dl– Large doses of vitamin C will be needed Large doses of vitamin C will be needed

throughout the diseasethroughout the disease– Chelation therapy is delayed until after 6 years of Chelation therapy is delayed until after 6 years of

age to promote normal physical developmentage to promote normal physical development– To minimize the effect of iron overload, To minimize the effect of iron overload,

deferoxamine (Desferal), an Iron-Chelating agent, deferoxamine (Desferal), an Iron-Chelating agent, will be given IV or SQwill be given IV or SQ

DIC = Disseminated DIC = Disseminated Intravascular Intravascular CoagulationCoagulation

ANY QUESTIONS?????ANY QUESTIONS?????

Idopathic Thrombocytopenic Idopathic Thrombocytopenic Purpura (ITP)Purpura (ITP)

Aquired hemorrhagic disorderAquired hemorrhagic disorder::– Excessive destruction of platelets (thrombocytopenia)Excessive destruction of platelets (thrombocytopenia)– Purpura (petechia), mucocutaneous bleedingPurpura (petechia), mucocutaneous bleeding– Occasional bleeding into tissuesOccasional bleeding into tissues– Normal bone marrowNormal bone marrow with unusual increase in large, young with unusual increase in large, young

plateletsplatelets Acute or transientAcute or transient

– Acute self limiting: “comes and goes”Acute self limiting: “comes and goes”– Chronic: “comes and goes” into remission (>6 month Chronic: “comes and goes” into remission (>6 month

duration)duration)– Acute following viral illnesses such as measles, varicella, Acute following viral illnesses such as measles, varicella,

mumps and URI (onset usually 1-4 weeks after the viral mumps and URI (onset usually 1-4 weeks after the viral illness)illness)

– Suspected that an immune mechanism is the basis for the Suspected that an immune mechanism is the basis for the thrombocytopeniathrombocytopenia

Signs and symptomsSigns and symptoms

Easy bruising especially over bony areasEasy bruising especially over bony areas Bleeding from gums / mucous membranesBleeding from gums / mucous membranes EpistaxisEpistaxis Menorrhalgia (painful menstruation)Menorrhalgia (painful menstruation) HematemesisHematemesis HematomasHematomas HemarthrosisHemarthrosis Intracranial hemorrhage is a rare (<1% of Intracranial hemorrhage is a rare (<1% of

cases), but serious outcomecases), but serious outcome

DiagnosisDiagnosis

Often diagnosed with clinical symptomsOften diagnosed with clinical symptoms ThrombocytopeniaThrombocytopenia

– Platelet count <20,000 m3/dl Platelet count <20,000 m3/dl – On blood smear, the platelets are large On blood smear, the platelets are large

(megathrombocytes) = increased marrow (megathrombocytes) = increased marrow productionproduction

Usually occurs around time of pubertyUsually occurs around time of puberty– Danger of bleeding with mensesDanger of bleeding with menses

ITPITP

Treatment Treatment Excellent prognosis (75% recover)Excellent prognosis (75% recover) Sometimes no medical interventions, self-limitingSometimes no medical interventions, self-limiting Restrict activity! Prevention from trauma and bleedingRestrict activity! Prevention from trauma and bleeding CorticosteroidsCorticosteroids: Prednisone; Decadron (Dexamethasone) : Prednisone; Decadron (Dexamethasone)

– Increases platelet count temporarilyIncreases platelet count temporarily– Avoid long term therapy (usually <3 weeks) in order to decrease side Avoid long term therapy (usually <3 weeks) in order to decrease side

effects:effects: Bone marrow suppressionBone marrow suppression Cushingoid changesCushingoid changes Growth failureGrowth failure

Blood transfusionBlood transfusion = Only transient benefits = Only transient benefits– Given in life threatening situationsGiven in life threatening situations

Platelet transfusionPlatelet transfusion = Only transient benefits = Only transient benefits– Platelets has a short survivalPlatelets has a short survival

SplenectomySplenectomy – If unresponsive to treatmentIf unresponsive to treatment– Usually only with chronic ITPUsually only with chronic ITP– Decreased risk for hemorrhageDecreased risk for hemorrhage

TreatmentTreatment Chemotherapy agentsChemotherapy agents = Not commonly used = Not commonly used

– VincristineVincristine– Severe side effectsSevere side effects

Danazol (danocrine)Danazol (danocrine)– Decreases estrogen and halt mensesDecreases estrogen and halt menses

IVIG (Gamma Globulin)IVIG (Gamma Globulin) – IV administration of antibodies to spare the removal of IV administration of antibodies to spare the removal of

antibody coated platelets in the spleenantibody coated platelets in the spleen– Sustained rises of platelet countSustained rises of platelet count– Large doses may induce remissionLarge doses may induce remission

RituxanRituxan– Monoclonal antibodyMonoclonal antibody– New and investigational New and investigational

Sandimmune (cyclosporinSandimmune (cyclosporin))– Used to prevent organ rejection and inhibit WBC growth factorsUsed to prevent organ rejection and inhibit WBC growth factors

Excorim System (Protein A Immunoabsorption)Excorim System (Protein A Immunoabsorption)– Antibody removal systemAntibody removal system, used mostly in Europe, used mostly in Europe– PlasmapharesisPlasmapharesis: Plasma is removed and filtered until desired : Plasma is removed and filtered until desired lowered lowered

immunoglobulin levelimmunoglobulin level is achieved: is achieved: Plasma is removed and “rinsed” before being re-mixed with blood in the cell Plasma is removed and “rinsed” before being re-mixed with blood in the cell

separator and returned to the patient. separator and returned to the patient. The process is performed on-line and continuous until the desired amount of The process is performed on-line and continuous until the desired amount of

plasma is processed.plasma is processed.– Very noisy, LIJ has itVery noisy, LIJ has it

Anti-D antibodyAnti-D antibody– Temporary and sometimes long-term elevation of the platelet counts Temporary and sometimes long-term elevation of the platelet counts – Works by coating the RBC and blocking the spleen’s Works by coating the RBC and blocking the spleen’s destruction of destruction of

certain platelets.certain platelets.– By doing so, there is an increase in platelet count within 1-3 days with By doing so, there is an increase in platelet count within 1-3 days with

a peak in counts 8 days after infusion a peak in counts 8 days after infusion – The elevation in platelets last approximately 1 month – longer than The elevation in platelets last approximately 1 month – longer than

IVIGIVIG– Advantage over IVIG in that it is given IV push and Advantage over IVIG in that it is given IV push and IT IS CHEAPERIT IS CHEAPER! !

Pre-medicate: Pre-medicate: – Decrease risk of reactionDecrease risk of reaction– Decrease painDecrease pain


Which one of the following does the Which one of the following does the nurse recognize as true when nurse recognize as true when administering anti-D antibody for ITP?administering anti-D antibody for ITP?– The platelet count will increase immediately The platelet count will increase immediately

after administrationafter administration– Eligible patients include those with lupusEligible patients include those with lupus– Bone marrow examination to first rule out Bone marrow examination to first rule out

leukemia is necessary before administrationleukemia is necessary before administration– Pre-medicate the patient with Pre-medicate the patient with

Acetaminophen before medication is infused.Acetaminophen before medication is infused.

LEUKEMIALEUKEMIA

LEUKEMIALEUKEMIA

DescriptionDescription– Malignant exacerbation in the number of Malignant exacerbation in the number of

leukocytesleukocytes, usually at an , usually at an immature stageimmature stage, in , in the bone marrowthe bone marrow

– Affects the bone marrow causing anemia, Affects the bone marrow causing anemia, leukopenia, the production of immature cells, leukopenia, the production of immature cells, thrombocytopenia, and a decline in immunitythrombocytopenia, and a decline in immunity

– The cause is unknown and appears to involve The cause is unknown and appears to involve gene damage of cells leading to the gene damage of cells leading to the transformation of cells from a normal state to a transformation of cells from a normal state to a malignant statemalignant state

LEUKEMIALEUKEMIA

Description (cont’d)Description (cont’d)– The most common form of childhood cancerThe most common form of childhood cancer– Peak incidence is age 2-6 years for acute Peak incidence is age 2-6 years for acute

lymphocytic leukemia (ALL)lymphocytic leukemia (ALL)– Dramatic improvements in survival rates, 80% (>5 Dramatic improvements in survival rates, 80% (>5

years) due to the extensive researchyears) due to the extensive research– Risk factors include genetic, viral, immunologic, and Risk factors include genetic, viral, immunologic, and

environmental factors and exposure to radiation, environmental factors and exposure to radiation, chemicals, and medicationschemicals, and medications

– Risk factors in children include those with Down’s Risk factors in children include those with Down’s syndrome or a twin of a child who has had leukemiasyndrome or a twin of a child who has had leukemia


Of the following assessment findings, Of the following assessment findings, the one that would most likely be the one that would most likely be seen in a child with leukemia is:seen in a child with leukemia is:– Weakness of the eye muscle.Weakness of the eye muscle.– Bruising, nosebleeds, paleness, and Bruising, nosebleeds, paleness, and

fatigue.fatigue.– Wheezing and shortness of breath.Wheezing and shortness of breath.– Abdominal swellingAbdominal swelling

Affects bone marrow = anemia + Affects bone marrow = anemia + bleedingbleeding

Diagnosis is based on % of blasts Diagnosis is based on % of blasts (immature WBC) in the blood(immature WBC) in the blood

CBC will showCBC will show– May have an increased WBC, May have an increased WBC, BUTBUT they they

are really leukopenic because the WBC are really leukopenic because the WBC are are IMMATUREIMMATURE

– Decreased Hgb/Hct = ANEMIADecreased Hgb/Hct = ANEMIA– Decreased platelets = thrombocytopeniaDecreased platelets = thrombocytopenia

LEUKEMIALEUKEMIA Classification of LeukemiaClassification of Leukemia

– Acute Lymphocytic Leukemia (ALL)Acute Lymphocytic Leukemia (ALL) Mostly lymphoblasts present in bone marrow Mostly lymphoblasts present in bone marrow Age of onset is less than 15 yearsAge of onset is less than 15 years Usually higher success in treatingUsually higher success in treating 3 subtypes (“markers” on cell surface antigens)3 subtypes (“markers” on cell surface antigens) 84% of the leukemias 84% of the leukemias

– Acute Myelogenous Leukemia (AML)Acute Myelogenous Leukemia (AML) Mostly myeloblasts present in bone marrow Mostly myeloblasts present in bone marrow Age of onset is between 15 and 39 yearsAge of onset is between 15 and 39 years Survival rate is not as positiveSurvival rate is not as positive 8 subtypes8 subtypes Approximately 20% of the leukemiasApproximately 20% of the leukemias

LEUKEMIALEUKEMIA

Assessment: Assessment: COMMON COMMON PRESENTATION!!!PRESENTATION!!!– Most of the signs/symptoms are a result of bone Most of the signs/symptoms are a result of bone

marrow infiltratemarrow infiltrate– Anorexia, fatigue, weakness, weight lossAnorexia, fatigue, weakness, weight loss– Pallor and anemiaPallor and anemia– Bruising and bleeding from the nose or gums, Bruising and bleeding from the nose or gums,

rectal bleeding, hematuriarectal bleeding, hematuria– Prolonged bleeding after minor abrasions or Prolonged bleeding after minor abrasions or

lacerationslacerations– Hemorrhage and PetechiaeHemorrhage and Petechiae– Elevated temperatureElevated temperature

– Lymphadenopathy, splenomegaly, hepatomegaly Lymphadenopathy, splenomegaly, hepatomegaly from marked infiltration. May lead to fibrosisfrom marked infiltration. May lead to fibrosis

– Palpitations and tachycardiaPalpitations and tachycardia

– Dyspnea on exertionDyspnea on exertion

– Vague abdominal pain caused by inflammation from Vague abdominal pain caused by inflammation from normal flora invading intestinal tractnormal flora invading intestinal tract

– Wasting of major organs due to infiltration of Wasting of major organs due to infiltration of leukemic cellsleukemic cells

– Increased ICP if meninges are infiltratedIncreased ICP if meninges are infiltrated

– CNS: severe headache, vomiting, papilledema CNS: severe headache, vomiting, papilledema (edema & inflammation of the optic nerve which may (edema & inflammation of the optic nerve which may result in blindness), irritability, lethargy and result in blindness), irritability, lethargy and eventually comaeventually coma

– Muscle wasting, weight loss, anorexia and fatigueMuscle wasting, weight loss, anorexia and fatigue

LEUKEMIALEUKEMIA

LEUKEMIALEUKEMIA

InfectionInfection– A major cause of death in the A major cause of death in the

immunosuppressed childimmunosuppressed child– Can occur through autocontamination Can occur through autocontamination

(i.e. Staph aureus on the skin) or cross (i.e. Staph aureus on the skin) or cross contaminationcontamination

– Most common sites of infection are the Most common sites of infection are the skin, respiratory tract, and GI tractskin, respiratory tract, and GI tract

Infection:Infection:Neutropenic DietNeutropenic Diet

Reverse IsolationReverse Isolation

LEUKEMIALEUKEMIA

Protecting the Child from Protecting the Child from InfectionInfection– Initiate protective isolation proceduresInitiate protective isolation procedures– Maintain child in a private room Maintain child in a private room – Frequent and thorough handwashingFrequent and thorough handwashing– Strict aseptic technique for all nursing proceduresStrict aseptic technique for all nursing procedures– Limit the number of caregivers entering the child’s room Limit the number of caregivers entering the child’s room

and ensure that anyone entering the child’s room is and ensure that anyone entering the child’s room is wearing a mask = REVERSE ISOLOATIONwearing a mask = REVERSE ISOLOATION

– Keep supplies for child separate from supplies for other Keep supplies for child separate from supplies for other childrenchildren

– Reduce exposure to environmental organisms by Reduce exposure to environmental organisms by eliminating raw fruits and vegetables from the diet, and eliminating raw fruits and vegetables from the diet, and keeping fresh flowers and standing water out of the keeping fresh flowers and standing water out of the child’s room * child’s room * Neutropenic dietNeutropenic diet


The severe cellular damage that is The severe cellular damage that is caused by chemotherapy drugs caused by chemotherapy drugs infiltrating into surrounding tissue infiltrating into surrounding tissue occurs when the chemotherapeutic occurs when the chemotherapeutic agent is a(n)agent is a(n)– HormoneHormone– SteroidSteroid– VesicantVesicant– antimetaboliteantimetabolite

LEUKEMIALEUKEMIA

BleedingBleeding– During the period of greatest bone During the period of greatest bone

marrow suppression (the nadir), the marrow suppression (the nadir), the platelet count may be extremely lowplatelet count may be extremely low

– Children with platelet counts below Children with platelet counts below 20,000/mm20,000/mm33 may need a platelet may need a platelet transfusiontransfusion

– For children with severe blood loss, For children with severe blood loss, packed red blood cells may be prescribed packed red blood cells may be prescribed (for Hgb <8.0)(for Hgb <8.0)

LEUKEMIALEUKEMIA

Fatigue and NutritionFatigue and Nutrition– Assist the child in selecting a well-Assist the child in selecting a well-

balanced dietbalanced diet– Provide small meals that require little Provide small meals that require little

chewingchewing– Assist the child in self-care and mobility Assist the child in self-care and mobility

activitiesactivities– NORMALIZENORMALIZE their life as much as their life as much as

possible!possible!

LEUKEMIALEUKEMIA

ChemotherapyChemotherapy– Monitor for severe bone marrow suppressionMonitor for severe bone marrow suppression– Monitor for infection and bleedingMonitor for infection and bleeding– Protect child from life-threatening infections for Protect child from life-threatening infections for

at least 2 to 3 weeks following chemotherapyat least 2 to 3 weeks following chemotherapy– Monitor for nausea, vomiting, and diarrheaMonitor for nausea, vomiting, and diarrhea– Assess oral mucous membranes for stomatitisAssess oral mucous membranes for stomatitis– Monitor for renal, hepatic, and cardiac toxicityMonitor for renal, hepatic, and cardiac toxicity

LEUKEMIALEUKEMIA

Chemotherapy Chemotherapy – Inform parents that hair loss may occur Inform parents that hair loss may occur

from chemotherapyfrom chemotherapy– Instruct parents about the care of central Instruct parents about the care of central

venous access devices as necessaryvenous access devices as necessary– Listen and encourage child and family to Listen and encourage child and family to

verbalize their feelings and express their verbalize their feelings and express their concernsconcerns

– Introduce family to other families of Introduce family to other families of children with cancerchildren with cancer

Treatment (i.e. NYII Treatment (i.e. NYII protocol)protocol)

INDUCTION THERAPYINDUCTION THERAPY– 4-6 weeks in length4-6 weeks in length– Goal is to achieve complete remissionGoal is to achieve complete remission

Corticosteroid = decrease inflammationCorticosteroid = decrease inflammation Vincristine and L-asparaginase with or without Vincristine and L-asparaginase with or without

DoxorubicinDoxorubicin CNS prophylactic therapy: IT (not IV due to the blood-CNS prophylactic therapy: IT (not IV due to the blood-

brain barrier) Methotrexate – given directly into the CSF brain barrier) Methotrexate – given directly into the CSF via LP. It protects against CNS invasion of leukemic cells. via LP. It protects against CNS invasion of leukemic cells. Radiation used to be done but changed due to long term Radiation used to be done but changed due to long term side effects. side effects.

– Goal is met if <5% blasts in BMGoal is met if <5% blasts in BM– If CNS disease is present, give If CNS disease is present, give Sanctuary Sanctuary

therapytherapy = combination of chemotherapy agents, = combination of chemotherapy agents, more intense and therefore need to watch for more intense and therefore need to watch for neurotoxicity neurotoxicity

Always concerned about Always concerned about tumor lysis syndrometumor lysis syndrome during induction therapyduring induction therapy– Caused by sudden, rapid death of cells in response to Caused by sudden, rapid death of cells in response to

treatment and that in terms causes electrolyte and treatment and that in terms causes electrolyte and metabolic disturbancesmetabolic disturbances

To prevent tumor lysis syndrome:To prevent tumor lysis syndrome:– Alkalanization = 2X IV maintenance therapy with Alkalanization = 2X IV maintenance therapy with

NaHCO3NaHCO3– Allopurinol to decrease uric acidAllopurinol to decrease uric acid– Amphojel to decrease phosphateAmphojel to decrease phosphate– Strict I&O maintaining urine pH >7.5Strict I&O maintaining urine pH >7.5

Tumor lysis syndrome would be a concern with:Tumor lysis syndrome would be a concern with:– Increased PhosphateIncreased Phosphate– Increased Uric AcidIncreased Uric Acid– Increased PotassiumIncreased Potassium– Decreased CalciumDecreased Calcium

MAINTENANCEMAINTENANCE– Also called consolidationAlso called consolidation– Serves to maintain the remission phaseServes to maintain the remission phase– Duration may be 2 1/2 to 3 yearsDuration may be 2 1/2 to 3 years– Continue corticosteroids, methotrexate Continue corticosteroids, methotrexate

and vincristine and vincristine

Side effects to treatmentSide effects to treatment ChemotherapyChemotherapy

– Nausea and vomitingNausea and vomiting– Alopescia (temporary)Alopescia (temporary)– Certain agents, i.e cytarabine (cytosin) will Certain agents, i.e cytarabine (cytosin) will

cause peripheral neuropathy with decreased cause peripheral neuropathy with decreased sensation and reflexessensation and reflexes

Interthecal chemotherapy – Ommaya Interthecal chemotherapy – Ommaya resorvoir or LPresorvoir or LP– Spinal headacheSpinal headache– Keep flat or Trendelenberg for at least 1 hour Keep flat or Trendelenberg for at least 1 hour

after administration to prevent headache and after administration to prevent headache and distribute drug throughout the bodydistribute drug throughout the body

Bone Marrow TransplantBone Marrow Transplant

Not recommended for children with ALL Not recommended for children with ALL during first remission because of the during first remission because of the excellent result with chemotherapy during excellent result with chemotherapy during second remissionsecond remission

Complete reverse isolationComplete reverse isolation Pt is typed and crossed with donor marrowPt is typed and crossed with donor marrow SiblingSibling is the #1 choice for HLA match is the #1 choice for HLA match BM donor banks – difficult for certain BM donor banks – difficult for certain

ethnicitiesethnicities BM aspirate – local anesthesiaBM aspirate – local anesthesia

OutcomeOutcome

““Cured” if 5 years in remission after Cured” if 5 years in remission after treatmenttreatment

Adjunct goalAdjunct goal: Pt has no assessment : Pt has no assessment of disease but cancer cells are of disease but cancer cells are evident through testsevident through tests

Palliative goalPalliative goal: Make them : Make them “comfortable” and control s/s until “comfortable” and control s/s until death occurs (i.e. Hospice or home)death occurs (i.e. Hospice or home)– ““To Live Among Lions”To Live Among Lions”

IMPAIRED GAS IMPAIRED GAS EXCHANGE EXCHANGE

&/OR INEFFECTIVE &/OR INEFFECTIVE BREATHING PATTERNSBREATHING PATTERNS

Infections of the Infections of the upper and middle upper and middle

airwaysairways

TONSILLITISTONSILLITIS An An infection or inflammationinfection or inflammation (hypertrophy) of the (hypertrophy) of the

palatine tonsilspalatine tonsils Most children with pharyngitis have infected tonsils, but Most children with pharyngitis have infected tonsils, but

NOT necessarily tonsillitis (may “just” have phyaryngitis)NOT necessarily tonsillitis (may “just” have phyaryngitis) Viral or bacterialViral or bacterial Tonsils are lymphoid tissues: Tonsils are lymphoid tissues:

– filter and protect respiration tract from invasion of filter and protect respiration tract from invasion of microorganismsmicroorganisms

– form antibodiesform antibodies Children usually have larger tonsils than adolescents or Children usually have larger tonsils than adolescents or

adults adults Adenoids are located above the tonsilsAdenoids are located above the tonsils Tonsillitis usually occurs with pharyngitisTonsillitis usually occurs with pharyngitis

TONSILLITISTONSILLITIS ASSESSMENTASSESSMENT::

– Enlarged and edematous tonsilsEnlarged and edematous tonsils– ““Kissing tonsils” = they may meet at midline and obstruct Kissing tonsils” = they may meet at midline and obstruct

passage of food or airpassage of food or air– Frequent throat infectionsFrequent throat infections– Cervical lymphadenopathyCervical lymphadenopathy– Difficulty swallowing or breathingDifficulty swallowing or breathing– Adenoids enlarge and cause difficulty for air to pass from Adenoids enlarge and cause difficulty for air to pass from

nose to pharynx. nose to pharynx. – Usually snoring when adenoids are affectedUsually snoring when adenoids are affected– Mouth breathingMouth breathing– Drying of mucous membranes secondary to mouth Drying of mucous membranes secondary to mouth

breathingbreathing– Otitis media and possibly decreased hearing may result Otitis media and possibly decreased hearing may result

(adenoids close to eusthasian tubes)(adenoids close to eusthasian tubes) DIAGNOSISDIAGNOSIS::

– Based of visual inspection and clinical Based of visual inspection and clinical manifestationmanifestation

– Throat cultureThroat culture

TONSILLITISTONSILLITIS MANAGEMENTMANAGEMENT::

– Symptomatic treatment:Symptomatic treatment: Provide comfortProvide comfort Acetaminophen (may be given PR) or ibuprofen to decrease Acetaminophen (may be given PR) or ibuprofen to decrease

inflammation and throat paininflammation and throat pain Cool, non-acidic fluids and soft foods; throat lozengesCool, non-acidic fluids and soft foods; throat lozenges Ice chips or frozen juice pops (increase hydration)Ice chips or frozen juice pops (increase hydration) Humidification / vaporiziation (cool mist)Humidification / vaporiziation (cool mist) Gargle with warm salt water (soothing)Gargle with warm salt water (soothing)

– Antibiotics based on C/S (Throat culture: pharyngeal – tonsil Antibiotics based on C/S (Throat culture: pharyngeal – tonsil swab). First line antibiotic is usually swab). First line antibiotic is usually penicillinpenicillin base (i.e. base (i.e. amoxicillin)amoxicillin)

– SurgerySurgery: Tonsillectomy &/or adenoidectomy (T&A): Tonsillectomy &/or adenoidectomy (T&A) Recommended if recurrent throat infections (Recommended if recurrent throat infections (documented documented

Strep throat: >3 in 6 months or >5/ in one yearStrep throat: >3 in 6 months or >5/ in one year)) Chronic tonsillitisChronic tonsillitis Obstructive sleep apneaObstructive sleep apnea Nasal speechNasal speech After the age of 3 years (it can stimulate growth of other After the age of 3 years (it can stimulate growth of other

lymphoid tissue in the nasopharynx) lymphoid tissue in the nasopharynx)

TONSILLITISTONSILLITIS NURSING PRE-OP and POST-OPNURSING PRE-OP and POST-OP::

– Teaching is very importantTeaching is very important– Baseline VSBaseline VS– History of bleeding patternHistory of bleeding pattern– Side-lying following surgery to drain secretionsSide-lying following surgery to drain secretions– No coughing frequently, clearing throat and blowing nose - No coughing frequently, clearing throat and blowing nose -

may aggravate operative sitemay aggravate operative site– Assess for hemorrhageAssess for hemorrhage::

Most common within the first 24 hours or 7-10 days after Most common within the first 24 hours or 7-10 days after tonsillectomy (scar is forming during that time)tonsillectomy (scar is forming during that time)

Use penlightUse penlight Inspect secretion and vomit for evidence of fresh bloodInspect secretion and vomit for evidence of fresh blood PallorPallor Increased pulse (>120, but depends on age-group – refer to G&D Increased pulse (>120, but depends on age-group – refer to G&D

chart)chart) If bleeding is suspected, call MD immediatelyIf bleeding is suspected, call MD immediately

– DietDiet: Cool water, crushed ice, ice pops, dilute fruit juice (avoid : Cool water, crushed ice, ice pops, dilute fruit juice (avoid citrus - acidic - irritating to site), soft foods - cooked fruits, citrus - acidic - irritating to site), soft foods - cooked fruits, jello, soup, etcjello, soup, etc

TONSILLITISTONSILLITIS– Airway obstruction may occur due to edema or accumulated secretions Airway obstruction may occur due to edema or accumulated secretions – Assessment of respiration distressAssessment of respiration distress: :

StridorStridor DroolingDrooling RestlessnessRestlessness Increased respiration rate Increased respiration rate

– Suction & 02 should be availableSuction & 02 should be available– Assess for infection Assess for infection (most common first 7-8 days):(most common first 7-8 days):

White coating in back of throatWhite coating in back of throat OdorOdor Low grade fever (report temp >102) (38.8C)Low grade fever (report temp >102) (38.8C)

– Discharge teaching:Discharge teaching: Avoid highly seasoned foodAvoid highly seasoned food Avoid garglingAvoid gargling Avoid vigorous tooth-brushingAvoid vigorous tooth-brushing Avoid Ibuprofen the first post-op week. Use acetaminophenAvoid Ibuprofen the first post-op week. Use acetaminophen Discourage coughing and throat clearingDiscourage coughing and throat clearing Use mild analgesicsUse mild analgesics Limit activity to decrease potential for hemorrhageLimit activity to decrease potential for hemorrhage Persistent sever earache, fever or cough required MD evaluation.Persistent sever earache, fever or cough required MD evaluation.


Adenoidectomy would be Adenoidectomy would be contraindicated in a child contraindicated in a child – with recurrent otitis media.with recurrent otitis media.– with malignancy.with malignancy.– with Thrombocytopenia.with Thrombocytopenia.– over the age of 3 years.over the age of 3 years.


Which of the following food(s) is/are Which of the following food(s) is/are the MOST appropriate to offer first to the MOST appropriate to offer first to an alert child who is in the post-an alert child who is in the post-operative period following a operative period following a tonsillectomy?tonsillectomy?– Ice creamIce cream– Red gelatinRed gelatin– Flavored Ice PopsFlavored Ice Pops– All of the aboveAll of the above

CROUP SYNDROMECROUP SYNDROME Term applied to a broad classification of Term applied to a broad classification of

upper airway illnesses that result from upper airway illnesses that result from swelling of the epiglottis and larynx, often swelling of the epiglottis and larynx, often extending into the trachea and bronchiextending into the trachea and bronchi

Boys > girlsBoys > girls SeasonalSeasonal: :

– Late autumn and early winter = more frequent Late autumn and early winter = more frequent episodesepisodes

Classification system of croup includes Classification system of croup includes viral syndromes such as (the viral syndromes such as (the ““big threebig three”” of pediatric respiratory illness = affects the of pediatric respiratory illness = affects the greatest number of children across all age greatest number of children across all age groups in both sexes):groups in both sexes):– 1) Spasmodic laryngitis (spasmodic croup)1) Spasmodic laryngitis (spasmodic croup)

CROUP SYNDROMECROUP SYNDROME– 2) 2) Laryngotracheobronchitis (LBT) = most Laryngotracheobronchitis (LBT) = most

common!common! 3 months - 8 years3 months - 8 years Viral invasionViral invasion Slow progression. Slow progression. Specific symptoms:Specific symptoms:

– URLURL– StridorStridor– Degree of inspiratory stridor and may lead to respiration distress due to Degree of inspiratory stridor and may lead to respiration distress due to

edema or obstruction of airway edema or obstruction of airway – Seal-like “barking” coughSeal-like “barking” cough– Resonant cough: “brassy” like in soundResonant cough: “brassy” like in sound– HoarsenessHoarseness– DyspneaDyspnea– RestlessnessRestlessness– IrritabilityIrritability– Low grade feverLow grade fever– NontoxicNontoxic– Treatment:Treatment:

HumidityHumidity Racemic epinephrine.Racemic epinephrine.

CROUP SYNDROMECROUP SYNDROME– 3) Bacterial syndromes (includes bacterial 3) Bacterial syndromes (includes bacterial

trachitis and trachitis and epiglottitisepiglottitis)) 1-8 years old1-8 years old Bacteria Invasion (Bacteria Invasion (Haemophilus influenzaeHaemophilus influenzae)) Almost non-existent due to vaccination: Hib = PREVENTION!Almost non-existent due to vaccination: Hib = PREVENTION! Rapidly progresses Rapidly progresses Specific symptomsSpecific symptoms

– DysphagiaDysphagia– Stridor aggravated when supine Stridor aggravated when supine – DroolingDrooling– High feverHigh fever– ToxicToxic– Tachycardia and tachypneaTachycardia and tachypnea

Treatment:Treatment: – Antibiotic therapyAntibiotic therapy– Airway protection (intubate in the OR)Airway protection (intubate in the OR)– Corticosteroids for edemaCorticosteroids for edema


In the child who is suspected to have In the child who is suspected to have epiglottitis the nurse should:epiglottitis the nurse should:– Have Intubation equipment availableHave Intubation equipment available– Prepare to immunize the child for Prepare to immunize the child for

Haemophilus influenzaeHaemophilus influenzae– Obtain a throat cultureObtain a throat culture– All of the aboveAll of the above

CYSTIC FIBROSISCYSTIC FIBROSIS Inherited Inherited autosomal recessive disorderautosomal recessive disorder (both parents) of (both parents) of

the the exocrine glandsexocrine glands that results in physiologic alterations that results in physiologic alterations in: in: – Respiratory system:Respiratory system:

Abnormal accumulation of viscous, dehydrated mucusAbnormal accumulation of viscous, dehydrated mucus Inflammation and lung changesInflammation and lung changes

– Gastrointestinal systemGastrointestinal system– Integumentary systemIntegumentary system– Musculoskeletal system Musculoskeletal system – Reproductive systemReproductive system

All body organs with mucous ducts become obstructed and All body organs with mucous ducts become obstructed and damageddamaged

Predominantly in white childrenPredominantly in white children Gender is not a factorGender is not a factor Average life span is 30 years (may vary)Average life span is 30 years (may vary)

CYSTIC FIBROSISCYSTIC FIBROSIS Blocked pancreatic ducts and resulting pancreatic damage causes a Blocked pancreatic ducts and resulting pancreatic damage causes a

STOPSTOP in the secretion of natural enzymes in the secretion of natural enzymes necessary to digest necessary to digest fats fats and proteinand protein..

As a result, essential nutrients are excreted in the stoolAs a result, essential nutrients are excreted in the stool ESSENTIAL CHANGES IN PATHOPHYSIOLOGY:ESSENTIAL CHANGES IN PATHOPHYSIOLOGY: Respiratory systemRespiratory system::

– Bronchi, bronchio-pneumonia & bronchial emphysema (barrel Bronchi, bronchio-pneumonia & bronchial emphysema (barrel chest)chest)

– Increased secretion requiring interventionIncreased secretion requiring intervention– Classic cough secondary to increased mucusClassic cough secondary to increased mucus– AtelectasisAtelectasis– Secondary respiratory infections = increased morbidity and Secondary respiratory infections = increased morbidity and

mortalitymortality Small intestineSmall intestine::

– Obstruction from mucousObstruction from mucous– If newborn does not pass meconium you must R/O cystic fibrosisIf newborn does not pass meconium you must R/O cystic fibrosis

CYSTIC FIBROSISCYSTIC FIBROSIS Pancrease:Pancrease:

– Ducts blockedDucts blocked– Unable to absorb & digest fat Unable to absorb & digest fat – Missing pancreatic enzymes that break down fatMissing pancreatic enzymes that break down fat– SteathorrheaSteathorrhea– Frothy (bulky and large in quantity)Frothy (bulky and large in quantity)– Foul smelly Foul smelly – Contain fat (greasy)Contain fat (greasy)

Bile ducts:Bile ducts:– ObstructedObstructed– May lead to cirrhosisMay lead to cirrhosis– Jaundice, portal HTNJaundice, portal HTN

Sweat glands and salivary glands:Sweat glands and salivary glands:– Lose a lot of salt – electrolyte imbalances due to loss of Na and Lose a lot of salt – electrolyte imbalances due to loss of Na and

ClCl– SALTY KISSES!!!!!! – initial S/SSALTY KISSES!!!!!! – initial S/S

Reproductive system:Reproductive system:– Male sterility due to blockage or absence of the vas deferensMale sterility due to blockage or absence of the vas deferens– Females difficulty conceiving due to increased mucus Females difficulty conceiving due to increased mucus

secretions in the reproductive tract interfering with sperm secretions in the reproductive tract interfering with sperm passagepassage

CYSTIC FIBROSISCYSTIC FIBROSIS DIAGNOSIS:DIAGNOSIS:

– Family history - genetic Family history - genetic – SWEAT TESTSWEAT TEST: patch on skin with measure of amount of Na absorbed: patch on skin with measure of amount of Na absorbed

Chloride concentration of 50-60 mEq/L is suspiciousChloride concentration of 50-60 mEq/L is suspicious Chloride concentration >60mEq/L is diagnosticChloride concentration >60mEq/L is diagnostic

– If positive: Pancreatic enzyme fast (eliminate) and check for steatorrheaIf positive: Pancreatic enzyme fast (eliminate) and check for steatorrhea– CXR: to check for mucusCXR: to check for mucus– CNS: Possibly hyperactive children (evident early in childhood)CNS: Possibly hyperactive children (evident early in childhood)

ASSESSMENT:ASSESSMENT: – INTESTINAL & PANCREAS:INTESTINAL & PANCREAS:

Steatorrhea (fat, frothy, foul smelling) Steatorrhea (fat, frothy, foul smelling) Eats large amount, but does not gain weights because can not absorb fatEats large amount, but does not gain weights because can not absorb fat Missing pancreatic enzymes - Trypsin, Lipase and Amylase. Missing pancreatic enzymes - Trypsin, Lipase and Amylase. Need vitamins because fat-soluble vitamins will not be absorbed through foodNeed vitamins because fat-soluble vitamins will not be absorbed through food

– RESPIRATORYRESPIRATORY:: Wheezing, rales, dyspnea, non-productive dry coughWheezing, rales, dyspnea, non-productive dry cough May develop atelectasis May develop atelectasis ClubbingClubbing May have ear, nose and throat problem more than average kids.May have ear, nose and throat problem more than average kids.

CYSTIC FIBROSISCYSTIC FIBROSISLong-term complicationsLong-term complications

– HEART:HEART: Corpulmonal - Right sided enlargementCorpulmonal - Right sided enlargement CHF = Pulmonary blood flow is obstructed by the CHF = Pulmonary blood flow is obstructed by the

mucousmucous

– ESOPHOGEAL:ESOPHOGEAL: Esophageal varisies = enlarged vein - may bleedEsophageal varisies = enlarged vein - may bleed

– LIVER: LIVER: Jaundice Jaundice

– SPLEEN: SPLEEN: Spleenomegaly Spleenomegaly

– REPRODUCTIVE SYSTEM:REPRODUCTIVE SYSTEM: ducts blocked, tubes blocked, mucous in vaginal tractducts blocked, tubes blocked, mucous in vaginal tract vas deferens blocked vas deferens blocked

CYSTIC FIBROSISCYSTIC FIBROSIS TREATMENT:TREATMENT:

– RespiratoryRespiratory: : Cupping, postural drainage, deep breathing, bronchodilators Cupping, postural drainage, deep breathing, bronchodilators

(Q4H before meals) – percussion/vibriation vest(Q4H before meals) – percussion/vibriation vest Aerosol treatments (bronchodilators)Aerosol treatments (bronchodilators)

– NutritionNutrition: : Increase SALT, especially with exerciseIncrease SALT, especially with exercise HIGH CALORIES AND PROTEINSHIGH CALORIES AND PROTEINS Salt tablets in warm weatherSalt tablets in warm weather Pancreatic enzymes pills taken with meals (non-fat & non-Pancreatic enzymes pills taken with meals (non-fat & non-

protein such as fruit, vegetables or carbohydrates). Sprinkle protein such as fruit, vegetables or carbohydrates). Sprinkle on food, “mix” in well AS A “SANDWICH”on food, “mix” in well AS A “SANDWICH”. . Don't get on lips or Don't get on lips or skin = breakdown. skin = breakdown.

– NURSINGNURSING:: Teach about disease and how to explain the condition to the Teach about disease and how to explain the condition to the

childchild Becomes progressively worseBecomes progressively worse Avoid infectionAvoid infection Encourage activity, rest, ventilate feelings, support groups Encourage activity, rest, ventilate feelings, support groups Genetic counselingGenetic counseling

Percussion VestPercussion Vest

Postural DrainagePostural Drainage

ASTHMAASTHMA Chronic Chronic inflammatory disorderinflammatory disorder of the airway with airway of the airway with airway

obstruction that can be partially or completely reversedobstruction that can be partially or completely reversed Chronic condition with acute exacerbations or persistent Chronic condition with acute exacerbations or persistent

symptomssymptoms Approximately 5 million children are affected in the United Approximately 5 million children are affected in the United

States (school absenteeism)States (school absenteeism) Boys > girls until the age of 10 years and then it equals outBoys > girls until the age of 10 years and then it equals out Most are diagnosed before the age of 5 yearsMost are diagnosed before the age of 5 years Called Called Reactive Airway Disease (RAD) in infantsReactive Airway Disease (RAD) in infants….. …..

Discussion: RAD vs ASTHMADiscussion: RAD vs ASTHMA ETIOLOGY:ETIOLOGY:

– BronchospasmBronchospasm– Increased mucous secretionIncreased mucous secretion– Usually results from allergic hyper response. Before Usually results from allergic hyper response. Before

puberty – boyspuberty – boys– Air gets trapped in the lungs and cannot get out Air gets trapped in the lungs and cannot get out

(wheeze)(wheeze)– Major allergic component: Major allergic component:

Seasonal allergiesSeasonal allergies Irritants: smokeIrritants: smoke Roach allergyRoach allergy Food allergiesFood allergies Skin sensitivity testsSkin sensitivity tests to determine causative agents to determine causative agents

ASTHMAASTHMA ASSESSMENT:ASSESSMENT:

– Respiratory difficulties of an asthma attack result from Respiratory difficulties of an asthma attack result from inflammation that contributes to airway obstruction (narrowing)inflammation that contributes to airway obstruction (narrowing)

– Mucus formation , mucosal swelling and airway muscle Mucus formation , mucosal swelling and airway muscle contractioncontraction

– A stimulus orA stimulus or TRIGGER TRIGGER initiates an asthmatic episode initiates an asthmatic episode– S/S visible includesS/S visible includes: :

Air hungerAir hunger DyspneaDyspnea AnxietyAnxiety Coughing (productive vs non-productive)Coughing (productive vs non-productive) FatigueFatigue Wheezing - inspiratory and/or expiratoryWheezing - inspiratory and/or expiratory TachypneaTachypnea RetractionsRetractions Cyanosis, and diaphoresis --- late signsCyanosis, and diaphoresis --- late signs Barrel chest (chronic)Barrel chest (chronic)

ASTHMAASTHMA

TREATMENT OPTIONSTREATMENT OPTIONS Long-term control medicationsLong-term control medications

– Leukotriene modifiersLeukotriene modifiers– Long-acting beta-2 agonists (LABAs)Long-acting beta-2 agonists (LABAs)– TheophyllineTheophylline

Quick-relief medicationsQuick-relief medications – Short-acting beta-2 agonistsShort-acting beta-2 agonists– Ipratropium (Atrovent)Ipratropium (Atrovent)– Oral and intravenous corticosteroids Oral and intravenous corticosteroids

Medications for allergy-induced Medications for allergy-induced asthmaasthma– Immunotherapy Immunotherapy – Anti-IgE monoclonal antibodiesAnti-IgE monoclonal antibodies

Long-term control medicationsLong-term control medications (Usually taken on a daily basis)(Usually taken on a daily basis)

Inhaled corticosteroidsInhaled corticosteroids: : – fluticasone (fluticasone (Flovent DiskusFlovent Diskus), budesonide (), budesonide (PulmicortPulmicort), ),

triamcinolone (Azmacort), flunisolide (Aerobid), triamcinolone (Azmacort), flunisolide (Aerobid), beclomethasone (Qvar) and others. beclomethasone (Qvar) and others.

– Reduce airway inflammation and are the Reduce airway inflammation and are the – Most commonly used long-term asthma medicationMost commonly used long-term asthma medication– Low-risk for side effects as compared to systemic Low-risk for side effects as compared to systemic

corticosteroidscorticosteroids– These inhaled medications work by opening airways, These inhaled medications work by opening airways,

reducing inflammation and decreasing mucus production. reducing inflammation and decreasing mucus production.

Leukotriene modifiersLeukotriene modifiers: : – montelukast (montelukast (SingulairSingulair), zafirlukast (Accolate) and zileuton ), zafirlukast (Accolate) and zileuton

(Zyflo CR) (Zyflo CR) – Inhaled medications; work by opening airways, reducing Inhaled medications; work by opening airways, reducing

inflammation and mucus productioninflammation and mucus production

Long-term control medicationsLong-term control medications (Usually taken on a daily basis)(Usually taken on a daily basis)

CromolynCromolyn and nedocromil (Tilade) and nedocromil (Tilade)– Inhaled medications reduce asthma signs and symptoms by Inhaled medications reduce asthma signs and symptoms by

decreasing allergic reactionsdecreasing allergic reactions– Considered a second choice to inhaled corticosteroidsConsidered a second choice to inhaled corticosteroids– Given TID to QIDGiven TID to QID

Long-acting beta-2 agonists (LABAs): Long-acting beta-2 agonists (LABAs): – salmeterol (Serevent Diskus) and formoterol (Foradil salmeterol (Serevent Diskus) and formoterol (Foradil

Aerolizer). Aerolizer). – Inhaled, long-acting bronchodilators, open the airways and Inhaled, long-acting bronchodilators, open the airways and

reduce inflammationreduce inflammation– Used in combination with inhaled corticosteroids with Used in combination with inhaled corticosteroids with

persistent asthmapersistent asthma– Long-acting bronchodilators should not be used for quick relief Long-acting bronchodilators should not be used for quick relief

of asthma symptomsof asthma symptoms

TheophyllineTheophylline– Daily tablet that opens your airways (bronchodilator)Daily tablet that opens your airways (bronchodilator)– Relaxes the muscles around the airways – Relaxes the muscles around the airways – VERY RARELY USEDVERY RARELY USED

Quick-Relief MedicationsQuick-Relief Medications(also called rescue medications)(also called rescue medications)

Short-acting beta-2 agonists:Short-acting beta-2 agonists:– Albuterol and XopenexAlbuterol and Xopenex– Inhaled bronchodilators, relax airway musclesInhaled bronchodilators, relax airway muscles– Act within minutes, and effects last four to six hoursAct within minutes, and effects last four to six hours

Ipratropium (Ipratropium (AtroventAtrovent))– Inhaled anticholinergic for the immediate relief of your Inhaled anticholinergic for the immediate relief of your

symptoms; relaxes the airwaysymptoms; relaxes the airway– Mostly used for emphysema and chronic bronchitisMostly used for emphysema and chronic bronchitis

Oral and intravenous corticosteroids Oral and intravenous corticosteroids – Prednisone and methylprednisolonePrednisone and methylprednisolone– In treatment of acute asthma attacks and severe In treatment of acute asthma attacks and severe

asthmaasthma– May cause serious side effects when used long term.May cause serious side effects when used long term.– Used short-term for asthmaUsed short-term for asthma

Other Asthma MedicationsOther Asthma Medications Medications for Medications for allergy-induced asthmaallergy-induced asthma::

– To decrease the body's sensitivity to a particular To decrease the body's sensitivity to a particular allergen or prevent the immune system from allergen or prevent the immune system from reacting to allergens. Allergy treatments for reacting to allergens. Allergy treatments for asthma include: asthma include: Immunotherapy:Immunotherapy:

– Allergy-desensitization shots (immunotherapy) are generally Allergy-desensitization shots (immunotherapy) are generally given once a week for a few months, then once a month for given once a week for a few months, then once a month for a period of three to five years. Over time, they gradually a period of three to five years. Over time, they gradually reduce your immune system reaction to specific allergens.reduce your immune system reaction to specific allergens.

Anti-IgE monoclonal antibodies:Anti-IgE monoclonal antibodies:– omalizumab (Xolair)omalizumab (Xolair)– Reduces the immune system's reaction to allergensReduces the immune system's reaction to allergens– Xolair is delivered by injection every two to four weeks.Xolair is delivered by injection every two to four weeks.

ASTHMA TREATMENTASTHMA TREATMENTMost commonly what we see:Most commonly what we see:

TREATMENT:TREATMENT:– BronchodilatorsBronchodilators: Beta2-agonists (short-acting: : Beta2-agonists (short-acting: Proventil / Proventil /

AlbuterolAlbuterol) for ACUTE ATTACK. ) for ACUTE ATTACK. Proventil: teach about SE including tachycardia, Proventil: teach about SE including tachycardia,

nervousness, tremorsnervousness, tremors– Inhaled steroidsInhaled steroids: Used as a prophylactic (i.e. before : Used as a prophylactic (i.e. before

exercise), during and after exacerbation of asthma for exercise), during and after exacerbation of asthma for more effective treatment (i.e. fluticasone (more effective treatment (i.e. fluticasone (Flovent DiskusFlovent Diskus), ), budesonide budesonide (Pulmicort(Pulmicort), flunisolide (), flunisolide (AerobidAerobid),), combination combination drug such as Advair [Fluticasone and Salmeterol])drug such as Advair [Fluticasone and Salmeterol]) Now considered MOST EFFECTVE treatment in asthma:Now considered MOST EFFECTVE treatment in asthma:

– Reduces asthma symptoms and flare-upsReduces asthma symptoms and flare-ups– Improves lung functionImproves lung function– Reduces bronchial reactivityReduces bronchial reactivity– NOT ADDICTIVE!NOT ADDICTIVE!

Increased risk of thrushIncreased risk of thrush– Rinse mouth and/or brush teeth after treatmentRinse mouth and/or brush teeth after treatment

Treatment (cont.)Treatment (cont.)

– GIVE ALL MDI MEDICATIONS WITH SPACERGIVE ALL MDI MEDICATIONS WITH SPACER

– Oral corticosteroidsOral corticosteroids: : If inhaled steroids are not used, systemic steroids If inhaled steroids are not used, systemic steroids

with a with a 5 day course of Prednisone5 day course of Prednisone is usually given 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-inflammatoryAnti-inflammatory: : Used for prophylaxis such as Used for prophylaxis such as CromolynCromolyn NOT USED during exacerbation of asthmaNOT USED during exacerbation of asthma

ASTHMAASTHMA

– Epinephrine:Epinephrine: Fast acting (arrhythmias, Fast acting (arrhythmias, nervous, restless, tremor, headache, nervous, restless, tremor, headache, insomnia). Used more if asthma is caused by insomnia). Used more if asthma is caused by allergic reactionallergic reaction

– Aminophylline:Aminophylline: VERY RARELY SEEN USEDVERY RARELY SEEN USED Must be administered through IV Pump! Must be administered through IV Pump! (tachycardia, nervous, n & v, anxiety, seizures) (tachycardia, nervous, n & v, anxiety, seizures)

– TheophyllinTheophyllin: : VERY RARELY SEEN USEDVERY RARELY SEEN USED! ! Intoxication can occur-monitor serum Intoxication can occur-monitor serum concentrations (seizures, n/v, tachycardia, concentrations (seizures, n/v, tachycardia, anxiety) anxiety)

ASTHMAASTHMA NURSING:NURSING:

– Know baseline Know baseline PEAK FLOW METERPEAK FLOW METER: : Best attempt out of three rapid expirationsBest attempt out of three rapid expirations Consider the zone they are in (red, yellow, green)Consider the zone they are in (red, yellow, green)

– High Fowler's position and bend forward in mild High Fowler's position and bend forward in mild exacerbationexacerbation

– CPT: Always do aerosol therapy before CPTCPT: Always do aerosol therapy before CPT– OxygenOxygen– Suctioning as neededSuctioning as needed– VS very importantVS very important– Monitor PC02 (35-48) & P02 (80-100) pH (7.35-7.45) if Monitor PC02 (35-48) & P02 (80-100) pH (7.35-7.45) if

possible (ABG or VBG)possible (ABG or VBG)– Pulse oximetry (keep PO2 >95%)Pulse oximetry (keep PO2 >95%)– Extensive IV fluids to hydrate them and liquify secretionsExtensive IV fluids to hydrate them and liquify secretions– Antibiotics prophylactically or specific for present Antibiotics prophylactically or specific for present

infectioninfection– Promote normal activitiesPromote normal activities– Prevent further episodes, support. Prevent further episodes, support.

STATUS ASTHMATICUS:STATUS ASTHMATICUS:– Acute, severe prolonged asthma attack.Acute, severe prolonged asthma attack.– Does not respond to normal treatment. Does not respond to normal treatment. – Person can die. Person can die. – Teach patient not to wait too long before Teach patient not to wait too long before

initiating treatment or seeing MD/NP initiating treatment or seeing MD/NP

ASTHMAASTHMA

BRONCHIOLITISBRONCHIOLITIS Lower respiratory tract illness caused by virus or bacteria Lower respiratory tract illness caused by virus or bacteria

which causes inflammation and obstruction of the which causes inflammation and obstruction of the bronchiolesbronchioles

Occurs mostly in in toddlers and preschoolersOccurs mostly in in toddlers and preschoolers Most severe in infants under 6 months (if <2 months, very Most severe in infants under 6 months (if <2 months, very

prone and hospitalized)prone and hospitalized) Most common cause: Most common cause: Respiratory Syncytial Virus (RSV)Respiratory Syncytial Virus (RSV): :

– Viral infection mostly of bronchiolar levelViral infection mostly of bronchiolar level– Primarily occurs in winter & springPrimarily occurs in winter & spring– The disease usually begins in the fall, reaches a peak in the The disease usually begins in the fall, reaches a peak in the

winter and decreases in the springwinter and decreases in the spring– Bronchiole mucosa swells and fill with mucous and exudateBronchiole mucosa swells and fill with mucous and exudate– Frequently sloughed epithelial cells obstruct the lumen, mostly Frequently sloughed epithelial cells obstruct the lumen, mostly

on expirationon expiration– Hyperinflation could occur and cause air trappingHyperinflation could occur and cause air trapping– The trapped air distal to the obstruction causes progressive The trapped air distal to the obstruction causes progressive

overinflation (emphysema)overinflation (emphysema)– Transmitted via respiratory secretions, hand to eye, nose or Transmitted via respiratory secretions, hand to eye, nose or

other mucous membrane.other mucous membrane.

BRONCHIOLITISBRONCHIOLITIS

ASSESSMENT:ASSESSMENT: – RhinorrheaRhinorrhea– PharyngitisPharyngitis– CoughingCoughing– SneezingSneezing– Breath sounds: wheezing, diffuse Breath sounds: wheezing, diffuse

crackles/rhonchi, ralescrackles/rhonchi, rales– Intermittent fever. Intermittent fever. – With progression of illness:With progression of illness:

Increase coughing and wheezingIncrease coughing and wheezing Air hunger, tachypnea, retractions, cyanosisAir hunger, tachypnea, retractions, cyanosis

– Severe illness:Severe illness: Tachypnea >70/min., listlessness, apneic spells, poor Tachypnea >70/min., listlessness, apneic spells, poor

air exchange, poor breath sounds.air exchange, poor breath sounds. OM and conjunctivitis may also be present. OM and conjunctivitis may also be present.

– Once the Once the lower airway is involvedlower airway is involved, classic , classic manifestations include: manifestations include: Signs of altered air exchange, such as wheezing, Signs of altered air exchange, such as wheezing,

retractions, crackles, dyspnea, tachypnea and retractions, crackles, dyspnea, tachypnea and diminished breath sounds.diminished breath sounds.

INFANTS' ASSESSMENTS:INFANTS' ASSESSMENTS: – Poor feeding, slight lethargy, irritability, Poor feeding, slight lethargy, irritability,

possibly low grade feverpossibly low grade fever DIAGNOSISDIAGNOSIS: :

– Tests done on nasal or nasopharyngeal Tests done on nasal or nasopharyngeal secretions (swab or aspirate) using IFA or ELISA secretions (swab or aspirate) using IFA or ELISA techniques for RSV antigen detection.techniques for RSV antigen detection.

BRONCHIOLITISBRONCHIOLITIS TREATMENTTREATMENT: :

– Based on the symptomsBased on the symptoms– PREVENTIONPREVENTION: RSV Medication: SYNAGIS (palivizumab): RSV Medication: SYNAGIS (palivizumab)

Monthly injection in the winter-months which provides Monthly injection in the winter-months which provides antibodiesantibodies

Used for premis and other high-risk groupsUsed for premis and other high-risk groups– High humidityHigh humidity– Adequate fluid intake Adequate fluid intake – Rest Rest – Medications depending on symptoms and causeMedications depending on symptoms and cause– Hospitalization: Hospitalization:

Usually recommended for children with complicating Usually recommended for children with complicating conditions, such as underlying lung or heart disease.conditions, such as underlying lung or heart disease.

The child who is tachypneic, has marked retractions, seems The child who is tachypneic, has marked retractions, seems listless, history of poor fluid Intake should also be listless, history of poor fluid Intake should also be admitted.admitted.

Any infant born premature or SGA Any infant born premature or SGA – Mist therapy combined with oxygen to maintain adequate O2 Mist therapy combined with oxygen to maintain adequate O2

saturationssaturations– IV fluids are preferred until crisis of the disease has passedIV fluids are preferred until crisis of the disease has passed– Blood gas values guide the therapy (if available)Blood gas values guide the therapy (if available)

BRONCHIOLITISBRONCHIOLITIS MEDICATIONS: MEDICATIONS:

– Bronchodilators, corticosteroids, cough suppressants and Bronchodilators, corticosteroids, cough suppressants and antibiotics have antibiotics have notnot proved to be effective. proved to be effective.

– Bronchodilators may be used for symptomatic wheezingBronchodilators may be used for symptomatic wheezing– For intubated and vetilated patients: For intubated and vetilated patients:

FUROSEMIDE, THEOPHYLLINE AND CORTICOSTEROIDSFUROSEMIDE, THEOPHYLLINE AND CORTICOSTEROIDS– RIBAVIRINRIBAVIRIN

An anti-viral agent is the only specific therapy available for An anti-viral agent is the only specific therapy available for RSV. RSV.

HOWEVER, studies have NOT confirmed its effectiveness and it HOWEVER, studies have NOT confirmed its effectiveness and it is therefore reserved for life-threatening cases (i.e. premature is therefore reserved for life-threatening cases (i.e. premature infants, underlying conditions, etc)infants, underlying conditions, etc)

VERY RARELY SEEN USED!VERY RARELY SEEN USED! Ribavirin has many precautions: Ribavirin has many precautions:

– Visitors and nurses must know about the potential harm to Visitors and nurses must know about the potential harm to themselves if exposed to the drugthemselves if exposed to the drug

TeratogenicTeratogenic: Pregnant women should not be exposed to : Pregnant women should not be exposed to medicationmedication

Cannot use contact lenses with administration as it Cannot use contact lenses with administration as it calcifiescalcifies

– Administered via mist in hood or tentAdministered via mist in hood or tent

BRONCHIOLITISBRONCHIOLITIS

High risk infants and children:High risk infants and children:– RespiGamRespiGam (RSV immune globulin) can be used (RSV immune globulin) can be used

as preventative treatment for RSV as well as as preventative treatment for RSV as well as SynagisSynagis

NURSING:NURSING: – Hand washing and not touching nasal mucosa Hand washing and not touching nasal mucosa

or conjunctivaor conjunctiva– Diminish the number of personnel, visitors and Diminish the number of personnel, visitors and

uninfected patients in contact with the childuninfected patients in contact with the child– Nurses try to avoid taking other patients who Nurses try to avoid taking other patients who

are considered high risk for catching RSV.are considered high risk for catching RSV.

THE END!THE END!

oxygenation in pediatrics by prof. unn hidle updated spring 2010

Documents

obstruction of blood

increased pulmonary

excess blood

better blood flow patterns

normal slide

unoxygenated blood mix

environmental slide

auscultated slide