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INTRODUCTIONINTRODUCTION
 Blood makes upBlood makes up 6- 8 of the body weight(60cc/kg6- 8 of the body weight(60cc/kg
body weight.body weight.
 blood formation commences 3blood formation commences 3rdrd
week ofweek of
intrauterine life.intrauterine life.
 In newborn blood volume is 300cc.In newborn blood volume is 300cc.
 This is doubled at one year.ten fold increasedThis is doubled at one year.ten fold increased
in 10-12 year old(25 kg body wt)3,000ccin 10-12 year old(25 kg body wt)3,000cc
DEFINITION OF ANEMIA;DEFINITION OF ANEMIA;
Anemia is defined as a numberAnemia is defined as a number
of the red bloods cell (RBCs) orof the red bloods cell (RBCs) or
hemoglobin concentration is reducedhemoglobin concentration is reduced
below normal value for age.below normal value for age.
- Wong’s(2008)- Wong’s(2008)
WHO proposed statisticsWHO proposed statistics
cut off point of Hbcut off point of Hb
Children 6months to 6 years =11g/dlChildren 6months to 6 years =11g/dl
Children 6 years to 14 years 12g/dlChildren 6 years to 14 years 12g/dl
Above 14 years male - 13g/dl, femaleAbove 14 years male - 13g/dl, female
-12g/dl-12g/dl
Grading of anemiaGrading of anemia
Mild anemia: 10-10.9g/dlMild anemia: 10-10.9g/dl
Moderate anemia: 7-9.9g/dlModerate anemia: 7-9.9g/dl
Severe anemia: <7g/dlSevere anemia: <7g/dl
Clinical grading of anemiaClinical grading of anemia
Pale conjunctiva, and mucous membrane-Pale conjunctiva, and mucous membrane-
mild anemiamild anemia
Pale skin- moderate anemiaPale skin- moderate anemia
Pale palm with skin and mucusPale palm with skin and mucus
membrane-severe anemiamembrane-severe anemia
Causes of anemiaCauses of anemia
Impaired RBC productionImpaired RBC production
Increased destruction of RBCIncreased destruction of RBC
Increased blood lossIncreased blood loss
Decreased RBC productionDecreased RBC production
CLASSIFICATIONCLASSIFICATION
1.Dereased red blood cell production1.Dereased red blood cell production
Pallor ,tachycardia/headache
Fatigue/shortness of breath
Nutritional deficiency
Iron,folate,B12,copper,
chronic blood loss,
Chronic disease.
Bone marrow failure
Aplastic anemia,
Red cell aplasia,
Malignancy,
Neuroblastoma
2.Increased red blood cell loss2.Increased red blood cell loss
Pallor ,Fatigue/headache ,muscle weaknessPallor ,Fatigue/headache ,muscle weakness
Cold skin ,Tachycardia ,Low blood presureCold skin ,Tachycardia ,Low blood presure
Acute blood loss
Epistaxis,
Hemophilia
Hypersplenism
ITP,DIC
INCREASED RED BLOOD DESTRUCTION
Intracorpuscular
Sickle cell anemia
Thalasemia
Enzymopathies(G6PP)
Membrane defects
Extracorpuscular
Drugs,
Toxic substances
Infection
Morphology charecteristics of RBCsMorphology charecteristics of RBCs
size(cell size)size(cell size)
Variation in RBCs size (Anisocytosis)Variation in RBCs size (Anisocytosis)
NormocytesNormocytes
MicrocytesMicrocytes
MacrocytesMacrocytes
Shape (irregular shape)Shape (irregular shape)
Spherocytes (globular cells)Spherocytes (globular cells)
DrepanocytesDrepanocytes
Morphology charecteristics of RBCsMorphology charecteristics of RBCs
contd…contd…
Color (staining characteristics);Color (staining characteristics);
NormochromicNormochromic
HypochromicHypochromic
HyperchromicHyperchromic
Anemia in newbornAnemia in newborn
Definition;Definition;
Anemia inAnemia in newbornnewborn is definedis defined
as venous hemoglobin less than 13gm/dlas venous hemoglobin less than 13gm/dl
in the first two weeks in a term baby andin the first two weeks in a term baby and
less than 12gm/dl in premature baby.less than 12gm/dl in premature baby.
Normal hemoglobin levels &Normal hemoglobin levels &
change after birthchange after birth
Cord blood range 13.7-20.1 gm/dlCord blood range 13.7-20.1 gm/dl
Hb concentration increased 6gm/dlHb concentration increased 6gm/dl
depending on amount of placentaldepending on amount of placental
transfusiontransfusion
Term infant by 7-9 wks “physiologicalTerm infant by 7-9 wks “physiological
anemia” occuranemia” occur
Erythoid activity starts 3-4 wksErythoid activity starts 3-4 wks
Preterm infant Hb fall earlier 7.8 - 9.6gm/dlPreterm infant Hb fall earlier 7.8 - 9.6gm/dl
Causes of pathological anemiaCauses of pathological anemia
Hemorrhage;Hemorrhage;
a.Obsteric accidentsa.Obsteric accidents
 placenta previaplacenta previa
 Abruptio placentaeAbruptio placentae
 Umblical cord ruptureUmblical cord rupture
b. Occult hemorrhage prior to birthb. Occult hemorrhage prior to birth
 Fetoplacental;Fetoplacental;
 Tight muchal cord , cesarean sectionTight muchal cord , cesarean section
 Fetomaternal;Fetomaternal;
 Traumatic amniocentesis ,twin to twinTraumatic amniocentesis ,twin to twin
transfusiontransfusion
Causes of pathological anemiaCauses of pathological anemia
contd…contd…
CC.Internal hemorrhages.Internal hemorrhages
 Cephal hematomaCephal hematoma
 Rupture of liver ,spleenRupture of liver ,spleen
HemolysisHemolysis
a. Immunea. Immune
 Rh ,ABO or minor group incompabilityRh ,ABO or minor group incompability
b. Hereditary disordersb. Hereditary disorders
 SpherocytosisSpherocytosis
Causes of pathological anemiaCauses of pathological anemia
contd…contd…C .InfectionC .Infection
 TORCH infectionsTORCH infections
 Bacterial sepsisBacterial sepsis
 MalariaMalaria
d. Red cell enzyme deficiencyd. Red cell enzyme deficiency
 G-6-PD deficiencyG-6-PD deficiency
e. Disseminated intravascular coagulatione. Disseminated intravascular coagulation
f. Alpha & gamma thalassemia syndromef. Alpha & gamma thalassemia syndrome
Causes of pathological anemiaCauses of pathological anemia
contd…contd…
Impaired red cell productionImpaired red cell production
 Congenital hypolastic anemiaCongenital hypolastic anemia
 Transcobalamine deficiencyTranscobalamine deficiency
 Congenital dyserythropoitic anemiaCongenital dyserythropoitic anemia
Clinical manifestationsClinical manifestations
AsphyxiaAsphyxia
Shock at birthShock at birth
PallorPallor
JaundiceJaundice
HepatomeglyHepatomegly
CHFCHF
SplenomegalySplenomegaly
Lab InvestigationsLab Investigations
Obstetric history- traumatic deliveryObstetric history- traumatic delivery
Birth history-twin ,precipitated deliveryBirth history-twin ,precipitated delivery
Maternal history-drug ingestionMaternal history-drug ingestion
Hemoglobin , Retic countHemoglobin , Retic count
Peripheral smearPeripheral smear
Bone marrow aspiration-decreased productionBone marrow aspiration-decreased production
Blood cultureBlood culture
Radiology- metaphysitisRadiology- metaphysitis
ManagementManagement
Anemia with shock;Anemia with shock;
Blood transfusionBlood transfusion
15-20cc/kg of o-ve blood or type specific15-20cc/kg of o-ve blood or type specific
blood should be infused 1-2 hrsblood should be infused 1-2 hrs
Reassure the baby additional transfusionReassure the baby additional transfusion
given if necessarygiven if necessary
If blood is not available initiate IV fluidsIf blood is not available initiate IV fluids
0.9% saline or RL 20-30 mts is given0.9% saline or RL 20-30 mts is given
Management contd…Management contd…
Anemia with CHFAnemia with CHF
Furesemide 1-2mg/kgFuresemide 1-2mg/kg
Packed cell transfusion 10-15cc/kgPacked cell transfusion 10-15cc/kg
6ml/kg of whole blood raised the Hb by6ml/kg of whole blood raised the Hb by
1gm/dl1gm/dl
Anemia due to hemolysisAnemia due to hemolysis
Double volume exchange transfusionDouble volume exchange transfusion
using semi packed cells to correct bothusing semi packed cells to correct both
hyperbilirubinemiahyperbilirubinemia
Alternative therapyAlternative therapy
Erythropoietin in combination with oral ironErythropoietin in combination with oral iron
Therapy with erythropoietin ranging 25-Therapy with erythropoietin ranging 25-
100u/kg/sc/alternative days ,start end of100u/kg/sc/alternative days ,start end of
first weekfirst week
Oral iron 2mg/kg/day/4 weeks,Oral iron 2mg/kg/day/4 weeks,
If reduced the need for blood transfusionIf reduced the need for blood transfusion
after 2after 2ndnd
week of lifeweek of life
Anemia in childrenAnemia in children
Pathophysiology;Pathophysiology;
Impaired production of red blood cell or bloodImpaired production of red blood cell or blood
lossloss
Circulating hemoglobin is reducedCirculating hemoglobin is reduced
Oxygen carrying capacity of the blood isOxygen carrying capacity of the blood is
decreased (falls bellow 7-8gm/dl)decreased (falls bellow 7-8gm/dl)
Tachycardia, increased cardiac out put occurTachycardia, increased cardiac out put occur
to increased blood flowto increased blood flow
Pathophysiology contd…Pathophysiology contd…
Blood flow diverts towards the vital organs &Blood flow diverts towards the vital organs &
tissue of the bodytissue of the body
Body demand for an increased in circulationBody demand for an increased in circulation
Cardiac failureCardiac failure
Clinical manifestationsClinical manifestations
Tachycardia ,tachypeniaTachycardia ,tachypenia
PalpitationPalpitation
Dyspenia ,Shortness of breathDyspenia ,Shortness of breath
Diaphoresis ,cyanosisDiaphoresis ,cyanosis
Child looks fatigued (sagging ,limp posture; slowChild looks fatigued (sagging ,limp posture; slow
,stained movements),stained movements)
Difficult to sucking in infantDifficult to sucking in infant
Growth retardationGrowth retardation
Delayed sexual maturationDelayed sexual maturation
CNS- Apathy ,dizziness ,irritability ,depressionCNS- Apathy ,dizziness ,irritability ,depression
Diagnostic evaluationDiagnostic evaluation
Age/sex group Hb (g/dl)Age/sex group Hb (g/dl)
Children 6mon-6 yr less than 11Children 6mon-6 yr less than 11
Children 6-14 yr less than 12Children 6-14 yr less than 12
Adult males less than 13Adult males less than 13
Adult females (non less than 12Adult females (non less than 12
Pregnant)Pregnant)
Adult female less than 11Adult female less than 11
(pregnant)(pregnant)
Therapeutic ManagementTherapeutic Management
Treat underlying causesTreat underlying causes
Blood transfusionBlood transfusion
Supportive medical careSupportive medical care
Oxygen therapyOxygen therapy
Bed restBed rest
Replacement of intravascular volume withReplacement of intravascular volume with
IV fluidsIV fluids
Nursing managementNursing management
Physical assessmentPhysical assessment
Preparation for laboratory studiesPreparation for laboratory studies
Reduction of oxygen needReduction of oxygen need
Administer oxygen therapyAdminister oxygen therapy
Administer transfusion therapyAdminister transfusion therapy
monitor vital signs 10-20 mts interval of 1monitor vital signs 10-20 mts interval of 1stst
30 mts30 mts
Inspect blood color carefullyInspect blood color carefully
Blood stored at 4deg C in refriginatorBlood stored at 4deg C in refriginator
Education of the parentsEducation of the parents
ComplicationComplication
Heart failureHeart failure
ParenthesisParenthesis
ConfusionConfusion
Specific type of anemia associated withSpecific type of anemia associated with
complicationscomplications
OutcomeOutcome
IronIron deficiency anemia or acute blooddeficiency anemia or acute blood
loss may have a favorable outcomeloss may have a favorable outcome
Iron deficiency anemiaIron deficiency anemia
Definition ;Definition ;
Iron deficiency anemia causedIron deficiency anemia caused
by a lack of sufficient iron for the synthesisby a lack of sufficient iron for the synthesis
of hemoglobin is the most prevalentof hemoglobin is the most prevalent
nutritional & hematological disorders.nutritional & hematological disorders.
Incidence;Incidence;
More than 1.5 billion people are affectedMore than 1.5 billion people are affected
globallyglobally
Premature infants ,child 6month-3 yrs riskPremature infants ,child 6month-3 yrs risk
Adolescents 11-17 yrsAdolescents 11-17 yrs
ClassificationClassification
Exclusive Breast FeedingExclusive Breast Feeding
First 4-6 months of life protects child fromFirst 4-6 months of life protects child from
iron deficiency anemiairon deficiency anemia
Better bioavailability of iron from the breastBetter bioavailability of iron from the breast
milkmilk
Transfer iron from mother to childTransfer iron from mother to child
transplacentally 3transplacentally 3rdrd
trimester of pregnancytrimester of pregnancy
Premature babies have poor iron storePremature babies have poor iron store
Classification contd…Classification contd…
Tran placental blood transfusionTran placental blood transfusion
Immediate after birthImmediate after birth
Placental blood amount 75-125mlPlacental blood amount 75-125ml
11stst
15 sec , end of minute15 sec , end of minute
Half of the placental blood is transfusedHalf of the placental blood is transfused
from placenta to newbornfrom placenta to newborn
Dietary ironDietary iron
Non dietary ironNon dietary iron
CausesCauses
Decreased iron storedDecreased iron stored
 PretermPreterm
 Small for dateSmall for date
 TwinsTwins
Decreased intakeDecreased intake
 Delayed weaningDelayed weaning
 MalnutritionMalnutrition
 Iron poor dietIron poor diet
 Chronic diarrheaChronic diarrhea
 GI surgeryGI surgery
 Malabsorption syndromeMalabsorption syndrome
Causes contd…Causes contd…
Increased demandIncreased demand
PrematurityPrematurity
LBWLBW
InfantsInfants
Recovery from PEMRecovery from PEM
AdolescentsAdolescents
Increased lossesIncreased losses
Hook worm infestationsHook worm infestations
GI bleeding ,Meckle diverticulitisGI bleeding ,Meckle diverticulitis
Peptic ulcer ,Bleeding diathesisPeptic ulcer ,Bleeding diathesis
StagesStages
First stage;First stage;
o It consist of depletion of iron storageIt consist of depletion of iron storage
o Iron deficiency stateIron deficiency state
Lack of ironLack of iron
Restrict production of hemoglobinRestrict production of hemoglobin
Deficient iron storeDeficient iron store
Decreased concentration of sr.ferritinDecreased concentration of sr.ferritin
Stages contd…Stages contd…
Second stage;Second stage;
Decreased iron storeDecreased iron store
Low serum iron concentration & transferingLow serum iron concentration & transfering
saturationsaturation
Increased in total binding capacity but noIncreased in total binding capacity but no
anemiaanemia
Hb concentration low in the normalHb concentration low in the normal
Stages contd…Stages contd…
More severe form of iron deficiencyMore severe form of iron deficiency
Hemoglobin concentration fallenHemoglobin concentration fallen
 low sr .Iron concentrationlow sr .Iron concentration
 Low transferrin saturationLow transferrin saturation
 Low Hb & Hematocrit valueLow Hb & Hematocrit value
Increased free erythrocyte protoporphyrin ,Increased free erythrocyte protoporphyrin ,
Hypochromia , MycrocytosisHypochromia , Mycrocytosis
Clinical manifestationsClinical manifestations
Fall hemoglobinFall hemoglobin
Hyper dynamic circulationHyper dynamic circulation
 PalpitationPalpitation
 FatigueFatigue
 AnorexiaAnorexia
 Shortness of breathShortness of breath
 Decreased exercise toleranceDecreased exercise tolerance
 Congestive heart failureCongestive heart failure
Initial ;Initial ;
 Pallor ,Anorexia ,IrritabilityPallor ,Anorexia ,Irritability
Symptoms In school childrenSymptoms In school children
Impaired IQImpaired IQ
Short attention spanShort attention span
Impaired mental & motor DevelopmentImpaired mental & motor Development
DisabilityDisability
School dropoutsSchool dropouts
Pica 70-80%Pica 70-80%
Pedal edema- Impaired Renal functionPedal edema- Impaired Renal function
Papille edema-increased intracranialPapille edema-increased intracranial
tensiontension
CVS- tachycardia, CHF, Shortness ofCVS- tachycardia, CHF, Shortness of
breathbreath
InvestigationsInvestigations
Age in yrsAge in yrs Sr. ferritinSr. ferritin
(ng / dl)(ng / dl)
TransferritinTransferritin
SaturationSaturation
(%)(%)
RBCFreeRBCFree
erythrocyteerythrocyte
prothrombinprothrombin
(mg/dl)(mg/dl)
0.5 – 40.5 – 4
5 -105 -10
11 – 1411 – 14
>15>15
<10<10
<10<10
<10<10
<12<12
<12<12
<14<14
<16<16
<16<16
>80>80
>70>70
>70>70
>70>70
ManagementManagement
Drugs;Drugs;
 Ferrous sulfate-6mg/kg/2hr(3 doses)Ferrous sulfate-6mg/kg/2hr(3 doses)
 Iron Dextran (Inferon)- 9-50/kg;50mg/24hr/IMIron Dextran (Inferon)- 9-50/kg;50mg/24hr/IM
50mg;100mg/24hr/IV/IM50mg;100mg/24hr/IV/IM
 Folic acid - ( under 4 yrs) 0.4mg/dFolic acid - ( under 4 yrs) 0.4mg/d
 Vitamin B12 – 30-100ug/IM,SC (8 days)Vitamin B12 – 30-100ug/IM,SC (8 days)
Parenteral iron therapy;Parenteral iron therapy;
 Packed RBCs 2-3cc/kgPacked RBCs 2-3cc/kg
 Iron 50 mg dextran complexIron 50 mg dextran complex
Nursing managementNursing management
Instruct the parents about administer theInstruct the parents about administer the
iron medicationiron medication
Oral iron should give 2 divided dose withOral iron should give 2 divided dose with
citrus juicescitrus juices
Iron supplement should not administerIron supplement should not administer
with the milk substanceswith the milk substances
Encourage mother exclusively breast –fedEncourage mother exclusively breast –fed
infant by 4-6 months of ageinfant by 4-6 months of age
IM injection using Z- track methodIM injection using Z- track method
Iron medication dropper placed back ofIron medication dropper placed back of
the mouththe mouth
Out comeOut come
 Oral iron therapy is successful inOral iron therapy is successful in
the treatment of iron deficiency anemia.the treatment of iron deficiency anemia.
PreventionPrevention
 Exclusive Breast Feeding 4-6Exclusive Breast Feeding 4-6
monthsmonths
SICKLE CELLSICKLE CELL
ANEMIAANEMIA
SICKLE CELL ANEMIASICKLE CELL ANEMIA
INTRODUCTIONINTRODUCTION
Sickle cell anemia is one of a group ofSickle cell anemia is one of a group of
disease collectively termed hemoglobinopathies, indisease collectively termed hemoglobinopathies, in
which normal adult hemoglobin is partly or completelywhich normal adult hemoglobin is partly or completely
replaced by abnormal sickle hemoglobin sickle cellreplaced by abnormal sickle hemoglobin sickle cell
disease includes all pathologic feature are related todisease includes all pathologic feature are related to
the presence of Hemoglobin S.the presence of Hemoglobin S.
DEFINITION OF SICKLE CELL ANEMIADEFINITION OF SICKLE CELL ANEMIA::
Sickle cell anemia is the homozygous form of a group ofSickle cell anemia is the homozygous form of a group of
inherited diseases in which the normal adult hemoglobininherited diseases in which the normal adult hemoglobin
(hemoglobin A) is replaced by a variant form (hemoglobin(hemoglobin A) is replaced by a variant form (hemoglobin
S)S)
MODE OF TRANSMISSION OF SICKLE CELL ANEMIA;MODE OF TRANSMISSION OF SICKLE CELL ANEMIA;
Autosomol recessive disorder –Autosomol recessive disorder –both parents areboth parents are
carriers of the sickle trait. Each pregnancy has a one incarriers of the sickle trait. Each pregnancy has a one in
four chance or producing a child with sickle cell anemia.four chance or producing a child with sickle cell anemia.
PATHOPHYSIOLOGYPATHOPHYSIOLOGY
In hemoglobin S the defect is a substitution ofIn hemoglobin S the defect is a substitution of
valine for glutamine on the beta polypeptidevaline for glutamine on the beta polypeptide
chain of the globinchain of the globin
Sickled RBCs are crescent – shaped , haveSickled RBCs are crescent – shaped , have
reduced oxygen carrying stasis ,and decreasedreduced oxygen carrying stasis ,and decreased
life span.life span.
Sickled RBCs are rigid ; they cause trapping andSickled RBCs are rigid ; they cause trapping and
increased blood viscosity ,capillary stasis andincreased blood viscosity ,capillary stasis and
thrombosis ; eventually , tissue ischemia andthrombosis ; eventually , tissue ischemia and
necrosis result.necrosis result.
PATHOPHYSIOLOGY OF SICKEL CELL ANEMIAPATHOPHYSIOLOGY OF SICKEL CELL ANEMIA
Dehydration,
Acidosis, Fever,
Hypoxia
Heart failure
Hemodilution
Anemia
Hemolysis
Retinopathy
BlindnessChest
syndrome
Pain
Death
Paralysis
CVA
Diuresis,
enuresis
Heamaturia
GI tract
EyesLungBrainkidney
Chroniculcers
Osteomylitieis
Pain
Extremities
Infraction
Tissue ischemia
Local hypoxia
Vaso- occlusion
Splenomegaly
Hepatomegaly
Gallstones
Acute
Abdomen pain
Sickled RBC
CLINICAL MANIFESTATIONS OF SICKELCLINICAL MANIFESTATIONS OF SICKEL
CELL ANEMIACELL ANEMIA
The most acute symptoms of the diseaseThe most acute symptoms of the disease
occur during periods of exacerbation calledoccur during periods of exacerbation called
crisescrises
Three major types of sickle cell crisesThree major types of sickle cell crises ::
Vaso – occlusionVaso – occlusion
Acute splenic sequestrationAcute splenic sequestration
AplasticAplastic
VASO – OCCLUSION CRISISVASO – OCCLUSION CRISIS
Painful episodes marked by vessel occlusionPainful episodes marked by vessel occlusion
ischemia ,and necrosis caused by pooling of bloodischemia ,and necrosis caused by pooling of blood
and clumping of cellsand clumping of cells
 ExtremitiesExtremities – painful swelling of hands and feet,painful– painful swelling of hands and feet,painful
jointjoint
 AbdomenAbdomen – severe pain– severe pain
 CerebrumCerebrum – stroke , visual disturbances– stroke , visual disturbances
 LiverLiver – obstructive jaundice ,hepatic coma– obstructive jaundice ,hepatic coma
 KidneyKidney – hematuria– hematuria
 GenitalGenital – priapism– priapism
SPLENICSPLENIC SEQUESTRATION CRISISSEQUESTRATION CRISIS
Large quantities of blood are pooled inLarge quantities of blood are pooled in
the liver and spleenthe liver and spleen
 HepatomegalyHepatomegaly
 SplenomegalySplenomegaly
 Circulatory collapseCirculatory collapse
APLASTIC CRISISAPLASTIC CRISIS
Decreased RBC production, usually resultsDecreased RBC production, usually results
from a virus ( human parvovirus).from a virus ( human parvovirus).
HYPERHEMOLYTIC CRISISHYPERHEMOLYTIC CRISIS
Increased rate of RBC destruction characterizedIncreased rate of RBC destruction characterized
by anemia , jaundice and reticulocytosis.by anemia , jaundice and reticulocytosis.
 Acute chest syndromeAcute chest syndrome
Sickling occur in small vessels in the lungs.Sickling occur in small vessels in the lungs.
 Chest painChest pain
 FeverFever
 CoughCough
 TachypneaTachypnea
 WheezingWheezing
 HypoxiaHypoxia
GENERALGENERAL
 Growth retardationGrowth retardation
 Chronic anemiaChronic anemia
 Delayed sexual maturationDelayed sexual maturation
 susceptibility to sepsissusceptibility to sepsis
DIAGNOSTIC EVALUATIONS OF SICKEL CELLDIAGNOSTIC EVALUATIONS OF SICKEL CELL
ANEMIAANEMIA
 Hemoglobin electrophoresisHemoglobin electrophoresis
 Routine hematologic testRoutine hematologic test
 Sickle –turbidity testSickle –turbidity test
 Blood smearBlood smear
 Screening test for hemoglobinsScreening test for hemoglobins
 Antenatal screening –possibleAntenatal screening –possible
through amniocentesis.through amniocentesis.
PEDIATRIC COMPLICATIONS OF SICKLEPEDIATRIC COMPLICATIONS OF SICKLE
CELL ANEMIACELL ANEMIA
Delayed growth and developmentDelayed growth and development
Delayed onset of pubertyDelayed onset of puberty
Impaired fertility.Impaired fertility.
PriapismPriapism
 Enuresis especially at nightEnuresis especially at night
THERAPEUTIC MANAGEMENT OFTHERAPEUTIC MANAGEMENT OF
SICKEL CELL ANEMIASICKEL CELL ANEMIA
Aims :Aims :
 Prevent condition that enhancing sicklingPrevent condition that enhancing sickling
phenomenaphenomena
 Treat the medical emergencies of sickleTreat the medical emergencies of sickle
cell crisis.cell crisis.
 Hematopoietic stem cell transplantationHematopoietic stem cell transplantation
with stabilization of prior organ damage iswith stabilization of prior organ damage is
a possible cure fora possible cure for sickel cell anemiasickel cell anemia
MEDICAL MANAGEMENTMEDICAL MANAGEMENT
It is directed at supportive and symptomatic treatment.It is directed at supportive and symptomatic treatment.
 Rest to minimize energy expenditure and oxygen useRest to minimize energy expenditure and oxygen use
 Hydration through oral and IV therapyHydration through oral and IV therapy
 Electrolyte replacementElectrolyte replacement
 AnalgesicsAnalgesics
 Blood replacement to treat anemiaBlood replacement to treat anemia
 Antibiotics to treat infectionAntibiotics to treat infection
 Administration of pneumococcal and meningococcalAdministration of pneumococcal and meningococcal
vaccinesvaccines
 Oral penicillin prophylaxis is also recommended by 2Oral penicillin prophylaxis is also recommended by 2
months of agemonths of age
 Exchanged transfusionExchanged transfusion
 SplenectomySplenectomy
 Painful priapism may be treated by aspiration of thePainful priapism may be treated by aspiration of the
corpora cavernosum.corpora cavernosum.
 Opioids such as morphine, oxycodoneOpioids such as morphine, oxycodone
 Bone marrow transplantation.Bone marrow transplantation.
NURSING MANAGEMENT OF SICKLE CELLNURSING MANAGEMENT OF SICKLE CELL
ANEMIAANEMIA
Sickle cell anemia cannot be cured.Sickle cell anemia cannot be cured.
 Teach parents the optimal home care for theirTeach parents the optimal home care for their
children.children.
The nurses emphasizes the importance ofThe nurses emphasizes the importance of
adequate hydration to prevent sickling and toadequate hydration to prevent sickling and to
delay the stasis –thrombosis ischemia cycle in adelay the stasis –thrombosis ischemia cycle in a
crisis.crisis.
Give instruction on how many daily glasses orGive instruction on how many daily glasses or
bottles of fluid are required.bottles of fluid are required.
Many foods are also source of fluid:Many foods are also source of fluid: SoupsSoups
Increased fluids combined with impaired kidneyIncreased fluids combined with impaired kidney
function result in the problem of enuresis.function result in the problem of enuresis.
To alleviate parents anxiety ,the parents are taughtTo alleviate parents anxiety ,the parents are taught
the supportive and symptomatic care the childthe supportive and symptomatic care the child
receives in the hospitalreceives in the hospital
 Care of during crisisCare of during crisis
The general care of during a crisisThe general care of during a crisis ::
1.1. Minimization of energy expenditure and use ofMinimization of energy expenditure and use of
oxygenoxygen
2.2. Promotion of hydrationPromotion of hydration
3.3. Replacement of electrolytes and bloodReplacement of electrolytes and blood
4.4. Administration of antibioticsAdministration of antibiotics
5.5. Management by surgeryManagement by surgery
6.6. Provide skin careProvide skin care
7.7. Protect from injuryProtect from injury
1.Minimization of energy expenditure and use1.Minimization of energy expenditure and use
of oxygenof oxygen
Bed rest to minimize oxygen conception.Bed rest to minimize oxygen conception.
Passive range of motion exercise can promote circulation.Passive range of motion exercise can promote circulation.
The child needs for oxygen ,especially signs of cardiac failure.The child needs for oxygen ,especially signs of cardiac failure.
Short term oxygen therapy can prevent sickling ,but it dose notShort term oxygen therapy can prevent sickling ,but it dose not
usually reverse the sickling that already occur.usually reverse the sickling that already occur.
Prolonged oxygen therapy is not given because it can depressProlonged oxygen therapy is not given because it can depress
bone marrow activity, increased anemia .bone marrow activity, increased anemia .
2.Promotion of hydration2.Promotion of hydration
Assess the signs of dehydrationAssess the signs of dehydration
Oral and intravenous fluid therapy is necessary toOral and intravenous fluid therapy is necessary to
dilute the blood and sickle.dilute the blood and sickle.
Monitor Intake and out put chart.Monitor Intake and out put chart.
The nurse should observe the sings ofThe nurse should observe the sings of
hypokalemia and any abnormal electrolytes value.hypokalemia and any abnormal electrolytes value.
3.Replacement of electrolytes and blood.3.Replacement of electrolytes and blood.
During blood transfusion ,the nurse has theDuring blood transfusion ,the nurse has the
responsibility of observing for signs of transfusionresponsibility of observing for signs of transfusion
reaction .reaction .
Hypervolemia from rapid transfusing can increaseHypervolemia from rapid transfusing can increase
the workload of the heart.the workload of the heart.
The risk of hepatitis ,transfusion reaction andThe risk of hepatitis ,transfusion reaction and
hemosiderosis must considered when multiplehemosiderosis must considered when multiple
transfusion are planned.transfusion are planned.
4.Relief of pain4.Relief of pain
Assess for location severity ,Assess for location severity ,
duration and quality of painduration and quality of pain
Assess intensity of pain withAssess intensity of pain with
the use of an age –appropriatethe use of an age –appropriate
pain rating scale.pain rating scale.
provide comfort position .provide comfort position .
Gently handle painful jointsGently handle painful joints
and extremities provide supportand extremities provide support
with and maintain body alignment.with and maintain body alignment.
Apply warmth to the affected area.Apply warmth to the affected area.
Provide massage over theProvide massage over the
affected area.affected area.
Provide diversional therapyProvide diversional therapy
(music , play therapy)(music , play therapy)
Administer analgesic as per doctors orderAdminister analgesic as per doctors order
Encourage relaxation techniquesEncourage relaxation techniques
like deep breathing exercise.like deep breathing exercise.
Avoid the use of ice ,cold compressesAvoid the use of ice ,cold compresses
which may cause vasoconstriction.which may cause vasoconstriction.
5.Administration of antibiotics5.Administration of antibiotics
Infection are a frequent precipitator of cellInfection are a frequent precipitator of cell
crisis.crisis.
Administer antibiotics:Administer antibiotics:
Ex: Penicillin ,CephalosporinsEx: Penicillin ,Cephalosporins
6.Management by surgical intervention6.Management by surgical intervention::
Splenectomy may become necessary.Splenectomy may become necessary.
Determine whether the spleen is continuingDetermine whether the spleen is continuing
to enlarge.to enlarge.
Monitor the vital signs and blood pressure toMonitor the vital signs and blood pressure to
determine the presence of shock .determine the presence of shock .
7.Provide skin care7.Provide skin care ;;
Assess the for signs of hypoxia , dehydration,Assess the for signs of hypoxia , dehydration,
cyanosis.cyanosis.
Check the vital signsCheck the vital signs
Monitor intake and output chart.Monitor intake and output chart.
Encourage the child to drink fluid every 2Encourage the child to drink fluid every 2
hours.hours.
Cut short the nails.Cut short the nails.
Provide adequate rest to decrease oxygenProvide adequate rest to decrease oxygen
expenditure.expenditure.
Perform passive range of motion exercise.Perform passive range of motion exercise.
Apply moister cream.Apply moister cream.
Provide safe environment to protect fromProvide safe environment to protect from
injury.injury.
8.Protect from injury8.Protect from injury;;
Assess the child condition.Assess the child condition.
Child should be closely supervised.Child should be closely supervised.
Child should not be left alone.Child should not be left alone.
Provide safety precautions remove sharp objectsProvide safety precautions remove sharp objects
from child.from child.
Provide safe environment to protect from injuryProvide safe environment to protect from injury
OutcomeOutcome
The prognosis for individuals who haveThe prognosis for individuals who have
sickle cell trait is generally good. With bettersickle cell trait is generally good. With better
nutrition and more adequate prevention andnutrition and more adequate prevention and
treatment of infection , increasing numbers oftreatment of infection , increasing numbers of
individuals are surviving into adult life.individuals are surviving into adult life.
PreventionPrevention
Public educationPublic education
ScreeningScreening
Genetic counsellingGenetic counselling
THALASSEMIATHALASSEMIA
The thalassemia syndromes are aThe thalassemia syndromes are a
heterogeneous group of usually inheritedheterogeneous group of usually inherited
chronic disorder that are characterized bychronic disorder that are characterized by
an absence or decreased synthesis of onean absence or decreased synthesis of one
of the normal globin chain of hemoglobin.of the normal globin chain of hemoglobin.
CAUSES OF THALASSEMIACAUSES OF THALASSEMIA
Alpha or beta chainAlpha or beta chain
PATHOPHYSIOLOGY OF THALASSEMIAPATHOPHYSIOLOGY OF THALASSEMIA
Normal postnatal Hgb is composed of 2 alpha and 2Normal postnatal Hgb is composed of 2 alpha and 2
beta polypeptide chain.beta polypeptide chain.
In beta thalassemia – partial or complete deficiencyIn beta thalassemia – partial or complete deficiency
In the synthesis of beta chainsIn the synthesis of beta chains
Increased beta synthesis of alpha chain and gammaIncreased beta synthesis of alpha chain and gamma
chain productionchain production
Defective Hemoglobin formationDefective Hemoglobin formation
Unbalanced polypeptide unitUnbalanced polypeptide unit
Damage of RBCDamage of RBC
Severe anemiaSevere anemia
TYPES OF THALASSEMIATYPES OF THALASSEMIA
The two forms of beta thalassemiaThe two forms of beta thalassemia
Thalassemia majorThalassemia major
Thalassemia minorThalassemia minor
Thalassemia minor:Thalassemia minor:
Thalassemia minor is associated withThalassemia minor is associated with
decreased beta chain synthesis and isdecreased beta chain synthesis and is
the heterozygous form.the heterozygous form.
It produces little effect on the child on theIt produces little effect on the child on the
child expect mild anemia andchild expect mild anemia and
hypochromiahypochromia
Thalassemia majorThalassemia major (( Cooley's anemia orCooley's anemia or
Mediterranean anemiaMediterranean anemia))
It is associated with absent beta – chainIt is associated with absent beta – chain
synthesis and is the homozygous form.synthesis and is the homozygous form.
It produces sever anemiaIt produces sever anemia
CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS OFOF THALASSEMIATHALASSEMIA
Absent or defective synthesis of Hemoglobin AAbsent or defective synthesis of Hemoglobin A
Inadequate Structured RBCsInadequate Structured RBCs
Decreased life span of the RBCsDecreased life span of the RBCs
FeverFever
AnorexiaAnorexia
Progressive severe anemiaProgressive severe anemia
Enlarge d spleenEnlarge d spleen
HemosiderinHemosiderin
HemochromatosisHemochromatosis
HypoxiaHypoxia
HeadacheHeadache
Precordial painPrecordial pain
SkinSkin
Greenish –brown ,bronzed skin with fineGreenish –brown ,bronzed skin with fine
frecklesfreckles
HeartHeart
Increased cardiac overload,Increased cardiac overload,
cardiac failurecardiac failure
SpleenSpleen
Enlargement of spleenEnlargement of spleen
Abdominal distensionAbdominal distension
LiverLiver
CirrhosisCirrhosis
Lymph nodesLymph nodes
Lymph nodes in the abdomen enlarged.Lymph nodes in the abdomen enlarged.
skeletal systemskeletal system
The expanded marrow of the enlarged skull andThe expanded marrow of the enlarged skull and
face produces a typical facies:face produces a typical facies:
Frontal and parietal bossingFrontal and parietal bossing
Slanting or mongoloid eyesSlanting or mongoloid eyes
A depressed nasal bridgeA depressed nasal bridge
Prominent malar eminence or cheek boneProminent malar eminence or cheek bone
Enlargement of the maxillaEnlargement of the maxilla
DIAGNOSTIC EVALUATIONDIAGNOSTIC EVALUATION OFOF
THALASSEMIATHALASSEMIA
Hypochromia and microcytosisHypochromia and microcytosis
Hemoglobin level may fall to less thanHemoglobin level may fall to less than
5 gm/dl5 gm/dl
Unconjugated serum bilirubin is elevatedUnconjugated serum bilirubin is elevated
X –ray – over growth of maxilla and vertical trabeculaeX –ray – over growth of maxilla and vertical trabeculae
Total erythrocyte counts are low –Total erythrocyte counts are low –
2to3 million /mm32to3 million /mm3
Mean Corpuscular hemoglobin ,mean corpuscular volume,Mean Corpuscular hemoglobin ,mean corpuscular volume,
mean corpuscular hemoglobin concentration are low.mean corpuscular hemoglobin concentration are low.
COMPLICATIONS OF THALASSEMIACOMPLICATIONS OF THALASSEMIA
Transfusion reactionTransfusion reaction
HemosiderosisHemosiderosis
CirrhosisCirrhosis
InfectionInfection
Growth failureGrowth failure
Cardiac diseaseCardiac disease
THERAPEUTIC MANAGEMENT OFTHERAPEUTIC MANAGEMENT OF THALASSEMIATHALASSEMIA
Thalassemia cannot be cured, supportive therapyThalassemia cannot be cured, supportive therapy
is essential to prevent complicationsis essential to prevent complications
The major therapeutic management are:The major therapeutic management are:
Transfusion therapy;Transfusion therapy;
Transfusion therapy in thalassemia has two majorTransfusion therapy in thalassemia has two major
goalsgoals::
Prevent anemiaPrevent anemia
Suppress endogenous erythropoises to avoid ineffectiveSuppress endogenous erythropoises to avoid ineffective
erythropoiesis.erythropoiesis.
The objectives of transfusion therapy areThe objectives of transfusion therapy are::
To maintain Hemoglobin level of 10.5 g / dlTo maintain Hemoglobin level of 10.5 g / dl
To prevent hypoxiaTo prevent hypoxia
CHELATION THERAPYCHELATION THERAPY
The goal of chelationThe goal of chelation is to iron overloadis to iron overload
and subsequently maintain ferritin level belowand subsequently maintain ferritin level below
1000 ng / ml .1000 ng / ml .
Desferrioxamine:Desferrioxamine:
The dose is 20 to 40 mg/kg/day given subcutaneously over 8The dose is 20 to 40 mg/kg/day given subcutaneously over 8
to 10 hour for 6 night a week.to 10 hour for 6 night a week.
Intravenous desferalIntravenous desferal ::
It can be given particularly in those with very high ironIt can be given particularly in those with very high iron
overload through port – a- caths (central line)overload through port – a- caths (central line)
High dose desferal given in severe hemosiderosis toHigh dose desferal given in severe hemosiderosis to
prevent/reverse cardiac toxicity of iron overload.prevent/reverse cardiac toxicity of iron overload.
Deferiprone:Deferiprone:
Dose:75 to 100 mg/kg body weight/day in three to four dividedDose:75 to 100 mg/kg body weight/day in three to four divided
doses.doses.
SPLENECTOMYSPLENECTOMY
The child has already developed Splenomegaly and signsThe child has already developed Splenomegaly and signs
of hypersplenism are present, and is above 5 years ofof hypersplenism are present, and is above 5 years of
age,splenectomy should be done.age,splenectomy should be done.
Indications of splenectomyIndications of splenectomy::
Decrease in WBC and platelet count is a relatively lateDecrease in WBC and platelet count is a relatively late
manifestation of hypersplenism.manifestation of hypersplenism.
Child should receive pneumococcal vaccine, H.influenza type bChild should receive pneumococcal vaccine, H.influenza type b
vaccine ,vaccine ,and meningococcal vaccine 6 to 8 weeks prior to surgery.and meningococcal vaccine 6 to 8 weeks prior to surgery.
NURSING MANAGEMENTNURSING MANAGEMENT OF THALASSEMIAOF THALASSEMIA
The nurses role in the care of infants and childrenThe nurses role in the care of infants and children
includes;includes;
– Early assessmentEarly assessment
– Preparation for diagnostic procedurePreparation for diagnostic procedure
– Administration of transfusion therapyAdministration of transfusion therapy
– Care during surgical procedureCare during surgical procedure
– Prevention of infectionPrevention of infection
– Observe for ComplicationsObserve for Complications
– Education and support of the parents and child.Education and support of the parents and child.
Early AssessmentEarly Assessment
History collection--- ethnic background ,History collection--- ethnic background ,
sings and symptoms and early diagnosissings and symptoms and early diagnosis
Preparation for diagnostic studiesPreparation for diagnostic studies
The child and parent are prepared forThe child and parent are prepared for
X – ray ,laboratory test.X – ray ,laboratory test.
explain about benefits and needs of diagnosticexplain about benefits and needs of diagnostic
procedure.procedure.
explain to the parents and child about amount andexplain to the parents and child about amount and
duration of procedure.duration of procedure.
The nurse should consider the parents and childThe nurse should consider the parents and child
feelings and get co operation from parents and child.feelings and get co operation from parents and child.
Administration Of Transfusion TherapyAdministration Of Transfusion Therapy
The transfusion therapy is given to increase theThe transfusion therapy is given to increase the
circulation of hemoglobincirculation of hemoglobin
Transfusion therapy that maintain the hemoglobinTransfusion therapy that maintain the hemoglobin
level at 10.5 gm/dllevel at 10.5 gm/dl
The nurse can function as an advocate for the familyThe nurse can function as an advocate for the family
by helping arrange for blood transfusion and medicalby helping arrange for blood transfusion and medical
supervisionsupervision
The nurse in charge plans the transfusion therapy wellThe nurse in charge plans the transfusion therapy well
and give sufficient time for proper testing.and give sufficient time for proper testing.
The nurse is responsible for maintaining the vein – theThe nurse is responsible for maintaining the vein – the
lifeline of thalassemic child.lifeline of thalassemic child.
Complications of transfusionComplications of transfusion
Iron overloadIron overload
Non hemolytic febrile reaction.Non hemolytic febrile reaction.
InfectionsInfections
Care of surgical procedureCare of surgical procedure::
Splenectomy may be performed for the correction ofSplenectomy may be performed for the correction of
hypersplenismhypersplenism
The nurse prepare the family and child for surgeryThe nurse prepare the family and child for surgery
Preoperative teachingPreoperative teaching
Complication, the duration of the hospitalization andComplication, the duration of the hospitalization and
recovery time.recovery time.
Legal preparation of surgeryLegal preparation of surgery
Get the ConsentGet the Consent
Day of surgeryDay of surgery
To verify consentTo verify consent
Laboratory dataLaboratory data
Records of any consultationRecords of any consultation
Baseline vital signsBaseline vital signs
Preparation of skinPreparation of skin
Remove nail polish and not wear cosmeticRemove nail polish and not wear cosmetic
Preoperative medicationPreoperative medication
Post Operative CarePost Operative Care
Protection from infection.Protection from infection.
Vital signs and blood pressure are recordedVital signs and blood pressure are recorded
frequently until stable.frequently until stable.
Suctioning is intermittent and maintained for notSuctioning is intermittent and maintained for not
more than 5 secondsmore than 5 seconds
Intake and output chart maintainedIntake and output chart maintained
Provide adequate rest and ambulationProvide adequate rest and ambulation
Provide firm pillow and favorite stuffed animalProvide firm pillow and favorite stuffed animal
placed against the chest.placed against the chest.
Provide nonpharmacologic method to relive painProvide nonpharmacologic method to relive pain
Stay with child during procedureStay with child during procedure
Teach procedure to family membersTeach procedure to family members
The child is kept warm to prevent heat lossThe child is kept warm to prevent heat loss
Allow the parents to express the feelings.Allow the parents to express the feelings.
Clarify their doubtsClarify their doubts
Provide support as neededProvide support as needed
Provide information on resources availableProvide information on resources available
regarding respite care to provide short term careregarding respite care to provide short term care
The child is treated with oral broad –spectrumThe child is treated with oral broad –spectrum
antibiotics.antibiotics.
The nurse should observe signs of cholecystitisThe nurse should observe signs of cholecystitis
Prevention of infectionPrevention of infection
Observe sign of infectionObserve sign of infection
Maintain sterile procedureMaintain sterile procedure
Maintain aseptic environmentMaintain aseptic environment
Use sterile equipmentsUse sterile equipments
Maintain handwashing before and after the procedure.Maintain handwashing before and after the procedure.
Isolate child as indicated to prevent nosocomialIsolate child as indicated to prevent nosocomial
infectioninfection
Provide high protein and high caloric dietProvide high protein and high caloric diet
Teach child and family ,manifestation of illnessTeach child and family ,manifestation of illness
Appropriate antibiotic therapy.Appropriate antibiotic therapy.
Nursing management on child with AnemiaNursing management on child with Anemia
1.1.Impaired gas exchange related to diminishedImpaired gas exchange related to diminished
oxygen carrying capacity to the blood asoxygen carrying capacity to the blood as
evidenced by restlessness & shortness of breathevidenced by restlessness & shortness of breath
Interventions;Interventions;
Monitor vital signs &Blood gas analysisMonitor vital signs &Blood gas analysis
Administer the oxygen to the childAdminister the oxygen to the child
Providing rest to the childProviding rest to the child
Administer the blood transfusion to the childAdminister the blood transfusion to the child
Provide comfort position head elevated (Fowler’sProvide comfort position head elevated (Fowler’s
position) &back rest to the childposition) &back rest to the child
Explain the child reason for restrict the activity.Explain the child reason for restrict the activity.
2.Ineffective Breathing pattern related to2.Ineffective Breathing pattern related to
decreased hemoglobin & diminished oxygendecreased hemoglobin & diminished oxygen
carrying capacity as evidenced by dyspniacarrying capacity as evidenced by dyspnia
Intervention;Intervention;
 Monitor the respiratory rate ,Breathing sound, ABGMonitor the respiratory rate ,Breathing sound, ABG
analysis ,skin color.analysis ,skin color.
 Raised bed –give fowler’s position & cardiac tableRaised bed –give fowler’s position & cardiac table
 Administer the oxygen to the childAdminister the oxygen to the child
 Administer the blood transfusion to the childAdminister the blood transfusion to the child
 Restrict the child activitiesRestrict the child activities
 Provide psychological support to the childProvide psychological support to the child
 Provide child recreation therapy (watching TVProvide child recreation therapy (watching TV
,Telling stories),Telling stories)
3.Impaired tissue perfusion related to inadequate3.Impaired tissue perfusion related to inadequate
blood volume as evidenced by pale skin, cyanosedblood volume as evidenced by pale skin, cyanosed
Interventions;Interventions;
 Monitor Cardiac rate ,RhythmMonitor Cardiac rate ,Rhythm
 Administer IV fluid (Electrolyte) to the childAdminister IV fluid (Electrolyte) to the child
 Administer the oxygen to the childAdminister the oxygen to the child
 Administer the blood transfusion to the childAdminister the blood transfusion to the child
 Monitor vital signs every 10-20mts 1Monitor vital signs every 10-20mts 1stst
4hrs4hrs
 Maintain intake and output chart dailyMaintain intake and output chart daily
 provide skin careprovide skin care
 Administer antihypertensiveAdminister antihypertensive
4.Imbalance nutrition less than body requirement4.Imbalance nutrition less than body requirement
related torelated to
inadequate intake of essential nutritioninadequate intake of essential nutrition
Asses the nutritional status if the chilrd,24Asses the nutritional status if the chilrd,24
hrs recall for the child nutritional status.hrs recall for the child nutritional status.
Encourage the child intake iron ,protein.Encourage the child intake iron ,protein.
Vitamins ,rich diets.Vitamins ,rich diets.
Provide small & frequent dietProvide small & frequent diet
Reduced noxious environmental (sight,Reduced noxious environmental (sight,
smell) stimuli.smell) stimuli.
Allow favorite & special foodAllow favorite & special food
Provide balance diet in hygienicProvide balance diet in hygienic
preparationspreparations
Contd…Contd…
Measure total intake & out put chart dailyMeasure total intake & out put chart daily
Provide dietary supplements vitamins ,ironProvide dietary supplements vitamins ,iron
, folate ,protein, folate ,protein
Health education regarding Balance diet,Health education regarding Balance diet,
hygienic food practicehygienic food practice
5.Fatigue related to decreased hemoglobin &5.Fatigue related to decreased hemoglobin &
diminished oxygen carrying capacity of blooddiminished oxygen carrying capacity of blood
Interventions;Interventions;
Assess the child condition of fatigueAssess the child condition of fatigue
Provide all the articles near to the childProvide all the articles near to the child
bed sidebed side
Assist the child for personal hygiene.Assist the child for personal hygiene.
Provide complete rest to the child &Provide complete rest to the child &
restrict the child activitiesrestrict the child activities
Explain the child reason for restrict the activity.Explain the child reason for restrict the activity.
Provide side rails to the childProvide side rails to the child
6.Activity intolerance related to imbalance between6.Activity intolerance related to imbalance between
oxygen supply /demand as evidenced byoxygen supply /demand as evidenced by
increased pulseincreased pulse
Interventions;Interventions;
Assess the child condition of fatigueAssess the child condition of fatigue
Encourage alternative rest & activityEncourage alternative rest & activity
Restrict the play activities like running,Restrict the play activities like running,
jumping.jumping.
Assist the child for regular physical activityAssist the child for regular physical activity
(personal care ,ambulation ,transfers)(personal care ,ambulation ,transfers)
Limit the number of visitorsLimit the number of visitors
7.Impaired growth and development related to7.Impaired growth and development related to
poor intake of nutrition as evidenced by weightpoor intake of nutrition as evidenced by weight
lossloss
Assess the weight of the child according toAssess the weight of the child according to
the age,& development.the age,& development.
Provide dietary supplements vitamins ,iron ,Provide dietary supplements vitamins ,iron ,
folate ,proteinfolate ,protein
Provide small & frequent diet in attractiveProvide small & frequent diet in attractive
mannermanner
Reduced noxious environmental (sight,Reduced noxious environmental (sight,
smell) stimuli.smell) stimuli.
Involve the family while planning the careInvolve the family while planning the care
of childof child
8.Fear related to8.Fear related to painful diagnosticpainful diagnostic
procedureprocedure
Interventions;Interventions;
Asses the child and parents level of fearAsses the child and parents level of fear
Provide psychological support to theProvide psychological support to the
parents as well as childparents as well as child
Explain the every step of procedure,&Explain the every step of procedure,&
benefits of procedurebenefits of procedure
Maintain calm ,safe environmentMaintain calm ,safe environment
throughout the hospitalizationthroughout the hospitalization
Provide opportunity for decision makingProvide opportunity for decision making
regarding care ,allow to ask their doubts.regarding care ,allow to ask their doubts.
9.Interupted family process related to child9.Interupted family process related to child
in hospitalizationin hospitalization
Explain the every step of procedureExplain the every step of procedure
Explain the reason for the procedure andExplain the reason for the procedure and
benefits of procedurebenefits of procedure
Maintain good interpersonal relationship toMaintain good interpersonal relationship to
the child parentsthe child parents
Allow to ask their doubts.Allow to ask their doubts.
Provide psychological support to theProvide psychological support to the
parents as well as childparents as well as child
Every procedure allow the parents near toEvery procedure allow the parents near to
the child.the child.
10. Ineffective management of therapeutic10. Ineffective management of therapeutic
regimen related to lack of knowledgeregimen related to lack of knowledge
Assess the level of knowledge of the childAssess the level of knowledge of the child
parentsparents
Explain the every step of procedure,& benefits ofExplain the every step of procedure,& benefits of
procedureprocedure
Explain the disease condition,treatment, dietExplain the disease condition,treatment, diet
regimentregiment
Teach the parents hygienic food preparationTeach the parents hygienic food preparation
Allow to ask their doubts.Allow to ask their doubts.
Explain the parents source of iron rich foodsExplain the parents source of iron rich foods
11.Risk for infection related to depressed11.Risk for infection related to depressed
body defensesbody defenses
Interventions;Interventions;
Place the child private roomPlace the child private room
Maintain aseptic precautions all staffs andMaintain aseptic precautions all staffs and
parentsparents
Screen all visitors as much as possibleScreen all visitors as much as possible
Monitor temperatureMonitor temperature
Evaluate the child potential site of infectionEvaluate the child potential site of infection
provide complete nutritious diet to theprovide complete nutritious diet to the
childchild
Administer vaccine up to the age
12.Potential complication related to heart failure12.Potential complication related to heart failure
Interventions;Interventions;
Asses the signs & symptoms of heartAsses the signs & symptoms of heart
failure ,numbness ,tingling on bottom offailure ,numbness ,tingling on bottom of
footfoot
Check the body weight daily & vitalCheck the body weight daily & vital
signs ,every 15-20 mtssigns ,every 15-20 mts
Avoid or limited the activitiesAvoid or limited the activities
Provide adequate rest & comfortProvide adequate rest & comfort
measuresmeasures
Administer diureticsAdminister diuretics
Maintain I/O CHART every 24 hrsMaintain I/O CHART every 24 hrs
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Anemia

  • 1. INTRODUCTIONINTRODUCTION  Blood makes upBlood makes up 6- 8 of the body weight(60cc/kg6- 8 of the body weight(60cc/kg body weight.body weight.  blood formation commences 3blood formation commences 3rdrd week ofweek of intrauterine life.intrauterine life.  In newborn blood volume is 300cc.In newborn blood volume is 300cc.  This is doubled at one year.ten fold increasedThis is doubled at one year.ten fold increased in 10-12 year old(25 kg body wt)3,000ccin 10-12 year old(25 kg body wt)3,000cc
  • 2.
  • 3. DEFINITION OF ANEMIA;DEFINITION OF ANEMIA; Anemia is defined as a numberAnemia is defined as a number of the red bloods cell (RBCs) orof the red bloods cell (RBCs) or hemoglobin concentration is reducedhemoglobin concentration is reduced below normal value for age.below normal value for age. - Wong’s(2008)- Wong’s(2008)
  • 4. WHO proposed statisticsWHO proposed statistics cut off point of Hbcut off point of Hb Children 6months to 6 years =11g/dlChildren 6months to 6 years =11g/dl Children 6 years to 14 years 12g/dlChildren 6 years to 14 years 12g/dl Above 14 years male - 13g/dl, femaleAbove 14 years male - 13g/dl, female -12g/dl-12g/dl Grading of anemiaGrading of anemia Mild anemia: 10-10.9g/dlMild anemia: 10-10.9g/dl Moderate anemia: 7-9.9g/dlModerate anemia: 7-9.9g/dl Severe anemia: <7g/dlSevere anemia: <7g/dl
  • 5. Clinical grading of anemiaClinical grading of anemia Pale conjunctiva, and mucous membrane-Pale conjunctiva, and mucous membrane- mild anemiamild anemia Pale skin- moderate anemiaPale skin- moderate anemia Pale palm with skin and mucusPale palm with skin and mucus membrane-severe anemiamembrane-severe anemia
  • 6. Causes of anemiaCauses of anemia Impaired RBC productionImpaired RBC production Increased destruction of RBCIncreased destruction of RBC Increased blood lossIncreased blood loss Decreased RBC productionDecreased RBC production
  • 7. CLASSIFICATIONCLASSIFICATION 1.Dereased red blood cell production1.Dereased red blood cell production Pallor ,tachycardia/headache Fatigue/shortness of breath Nutritional deficiency Iron,folate,B12,copper, chronic blood loss, Chronic disease. Bone marrow failure Aplastic anemia, Red cell aplasia, Malignancy, Neuroblastoma
  • 8. 2.Increased red blood cell loss2.Increased red blood cell loss Pallor ,Fatigue/headache ,muscle weaknessPallor ,Fatigue/headache ,muscle weakness Cold skin ,Tachycardia ,Low blood presureCold skin ,Tachycardia ,Low blood presure Acute blood loss Epistaxis, Hemophilia Hypersplenism ITP,DIC
  • 9. INCREASED RED BLOOD DESTRUCTION Intracorpuscular Sickle cell anemia Thalasemia Enzymopathies(G6PP) Membrane defects Extracorpuscular Drugs, Toxic substances Infection
  • 10. Morphology charecteristics of RBCsMorphology charecteristics of RBCs size(cell size)size(cell size) Variation in RBCs size (Anisocytosis)Variation in RBCs size (Anisocytosis) NormocytesNormocytes MicrocytesMicrocytes MacrocytesMacrocytes Shape (irregular shape)Shape (irregular shape) Spherocytes (globular cells)Spherocytes (globular cells) DrepanocytesDrepanocytes
  • 11. Morphology charecteristics of RBCsMorphology charecteristics of RBCs contd…contd… Color (staining characteristics);Color (staining characteristics); NormochromicNormochromic HypochromicHypochromic HyperchromicHyperchromic
  • 12. Anemia in newbornAnemia in newborn Definition;Definition; Anemia inAnemia in newbornnewborn is definedis defined as venous hemoglobin less than 13gm/dlas venous hemoglobin less than 13gm/dl in the first two weeks in a term baby andin the first two weeks in a term baby and less than 12gm/dl in premature baby.less than 12gm/dl in premature baby.
  • 13. Normal hemoglobin levels &Normal hemoglobin levels & change after birthchange after birth Cord blood range 13.7-20.1 gm/dlCord blood range 13.7-20.1 gm/dl Hb concentration increased 6gm/dlHb concentration increased 6gm/dl depending on amount of placentaldepending on amount of placental transfusiontransfusion Term infant by 7-9 wks “physiologicalTerm infant by 7-9 wks “physiological anemia” occuranemia” occur Erythoid activity starts 3-4 wksErythoid activity starts 3-4 wks Preterm infant Hb fall earlier 7.8 - 9.6gm/dlPreterm infant Hb fall earlier 7.8 - 9.6gm/dl
  • 14. Causes of pathological anemiaCauses of pathological anemia Hemorrhage;Hemorrhage; a.Obsteric accidentsa.Obsteric accidents  placenta previaplacenta previa  Abruptio placentaeAbruptio placentae  Umblical cord ruptureUmblical cord rupture b. Occult hemorrhage prior to birthb. Occult hemorrhage prior to birth  Fetoplacental;Fetoplacental;  Tight muchal cord , cesarean sectionTight muchal cord , cesarean section  Fetomaternal;Fetomaternal;  Traumatic amniocentesis ,twin to twinTraumatic amniocentesis ,twin to twin transfusiontransfusion
  • 15. Causes of pathological anemiaCauses of pathological anemia contd…contd… CC.Internal hemorrhages.Internal hemorrhages  Cephal hematomaCephal hematoma  Rupture of liver ,spleenRupture of liver ,spleen HemolysisHemolysis a. Immunea. Immune  Rh ,ABO or minor group incompabilityRh ,ABO or minor group incompability b. Hereditary disordersb. Hereditary disorders  SpherocytosisSpherocytosis
  • 16. Causes of pathological anemiaCauses of pathological anemia contd…contd…C .InfectionC .Infection  TORCH infectionsTORCH infections  Bacterial sepsisBacterial sepsis  MalariaMalaria d. Red cell enzyme deficiencyd. Red cell enzyme deficiency  G-6-PD deficiencyG-6-PD deficiency e. Disseminated intravascular coagulatione. Disseminated intravascular coagulation f. Alpha & gamma thalassemia syndromef. Alpha & gamma thalassemia syndrome
  • 17. Causes of pathological anemiaCauses of pathological anemia contd…contd… Impaired red cell productionImpaired red cell production  Congenital hypolastic anemiaCongenital hypolastic anemia  Transcobalamine deficiencyTranscobalamine deficiency  Congenital dyserythropoitic anemiaCongenital dyserythropoitic anemia
  • 18. Clinical manifestationsClinical manifestations AsphyxiaAsphyxia Shock at birthShock at birth PallorPallor JaundiceJaundice HepatomeglyHepatomegly CHFCHF SplenomegalySplenomegaly
  • 19. Lab InvestigationsLab Investigations Obstetric history- traumatic deliveryObstetric history- traumatic delivery Birth history-twin ,precipitated deliveryBirth history-twin ,precipitated delivery Maternal history-drug ingestionMaternal history-drug ingestion Hemoglobin , Retic countHemoglobin , Retic count Peripheral smearPeripheral smear Bone marrow aspiration-decreased productionBone marrow aspiration-decreased production Blood cultureBlood culture Radiology- metaphysitisRadiology- metaphysitis
  • 20. ManagementManagement Anemia with shock;Anemia with shock; Blood transfusionBlood transfusion 15-20cc/kg of o-ve blood or type specific15-20cc/kg of o-ve blood or type specific blood should be infused 1-2 hrsblood should be infused 1-2 hrs Reassure the baby additional transfusionReassure the baby additional transfusion given if necessarygiven if necessary If blood is not available initiate IV fluidsIf blood is not available initiate IV fluids 0.9% saline or RL 20-30 mts is given0.9% saline or RL 20-30 mts is given
  • 21. Management contd…Management contd… Anemia with CHFAnemia with CHF Furesemide 1-2mg/kgFuresemide 1-2mg/kg Packed cell transfusion 10-15cc/kgPacked cell transfusion 10-15cc/kg 6ml/kg of whole blood raised the Hb by6ml/kg of whole blood raised the Hb by 1gm/dl1gm/dl Anemia due to hemolysisAnemia due to hemolysis Double volume exchange transfusionDouble volume exchange transfusion using semi packed cells to correct bothusing semi packed cells to correct both hyperbilirubinemiahyperbilirubinemia
  • 22. Alternative therapyAlternative therapy Erythropoietin in combination with oral ironErythropoietin in combination with oral iron Therapy with erythropoietin ranging 25-Therapy with erythropoietin ranging 25- 100u/kg/sc/alternative days ,start end of100u/kg/sc/alternative days ,start end of first weekfirst week Oral iron 2mg/kg/day/4 weeks,Oral iron 2mg/kg/day/4 weeks, If reduced the need for blood transfusionIf reduced the need for blood transfusion after 2after 2ndnd week of lifeweek of life
  • 23. Anemia in childrenAnemia in children Pathophysiology;Pathophysiology; Impaired production of red blood cell or bloodImpaired production of red blood cell or blood lossloss Circulating hemoglobin is reducedCirculating hemoglobin is reduced Oxygen carrying capacity of the blood isOxygen carrying capacity of the blood is decreased (falls bellow 7-8gm/dl)decreased (falls bellow 7-8gm/dl) Tachycardia, increased cardiac out put occurTachycardia, increased cardiac out put occur to increased blood flowto increased blood flow
  • 24. Pathophysiology contd…Pathophysiology contd… Blood flow diverts towards the vital organs &Blood flow diverts towards the vital organs & tissue of the bodytissue of the body Body demand for an increased in circulationBody demand for an increased in circulation Cardiac failureCardiac failure
  • 25. Clinical manifestationsClinical manifestations Tachycardia ,tachypeniaTachycardia ,tachypenia PalpitationPalpitation Dyspenia ,Shortness of breathDyspenia ,Shortness of breath Diaphoresis ,cyanosisDiaphoresis ,cyanosis Child looks fatigued (sagging ,limp posture; slowChild looks fatigued (sagging ,limp posture; slow ,stained movements),stained movements) Difficult to sucking in infantDifficult to sucking in infant Growth retardationGrowth retardation Delayed sexual maturationDelayed sexual maturation CNS- Apathy ,dizziness ,irritability ,depressionCNS- Apathy ,dizziness ,irritability ,depression
  • 26. Diagnostic evaluationDiagnostic evaluation Age/sex group Hb (g/dl)Age/sex group Hb (g/dl) Children 6mon-6 yr less than 11Children 6mon-6 yr less than 11 Children 6-14 yr less than 12Children 6-14 yr less than 12 Adult males less than 13Adult males less than 13 Adult females (non less than 12Adult females (non less than 12 Pregnant)Pregnant) Adult female less than 11Adult female less than 11 (pregnant)(pregnant)
  • 27. Therapeutic ManagementTherapeutic Management Treat underlying causesTreat underlying causes Blood transfusionBlood transfusion Supportive medical careSupportive medical care Oxygen therapyOxygen therapy Bed restBed rest Replacement of intravascular volume withReplacement of intravascular volume with IV fluidsIV fluids
  • 28. Nursing managementNursing management Physical assessmentPhysical assessment Preparation for laboratory studiesPreparation for laboratory studies Reduction of oxygen needReduction of oxygen need Administer oxygen therapyAdminister oxygen therapy Administer transfusion therapyAdminister transfusion therapy monitor vital signs 10-20 mts interval of 1monitor vital signs 10-20 mts interval of 1stst 30 mts30 mts Inspect blood color carefullyInspect blood color carefully Blood stored at 4deg C in refriginatorBlood stored at 4deg C in refriginator Education of the parentsEducation of the parents
  • 29. ComplicationComplication Heart failureHeart failure ParenthesisParenthesis ConfusionConfusion Specific type of anemia associated withSpecific type of anemia associated with complicationscomplications OutcomeOutcome IronIron deficiency anemia or acute blooddeficiency anemia or acute blood loss may have a favorable outcomeloss may have a favorable outcome
  • 30. Iron deficiency anemiaIron deficiency anemia Definition ;Definition ; Iron deficiency anemia causedIron deficiency anemia caused by a lack of sufficient iron for the synthesisby a lack of sufficient iron for the synthesis of hemoglobin is the most prevalentof hemoglobin is the most prevalent nutritional & hematological disorders.nutritional & hematological disorders. Incidence;Incidence; More than 1.5 billion people are affectedMore than 1.5 billion people are affected globallyglobally Premature infants ,child 6month-3 yrs riskPremature infants ,child 6month-3 yrs risk Adolescents 11-17 yrsAdolescents 11-17 yrs
  • 31. ClassificationClassification Exclusive Breast FeedingExclusive Breast Feeding First 4-6 months of life protects child fromFirst 4-6 months of life protects child from iron deficiency anemiairon deficiency anemia Better bioavailability of iron from the breastBetter bioavailability of iron from the breast milkmilk Transfer iron from mother to childTransfer iron from mother to child transplacentally 3transplacentally 3rdrd trimester of pregnancytrimester of pregnancy Premature babies have poor iron storePremature babies have poor iron store
  • 32. Classification contd…Classification contd… Tran placental blood transfusionTran placental blood transfusion Immediate after birthImmediate after birth Placental blood amount 75-125mlPlacental blood amount 75-125ml 11stst 15 sec , end of minute15 sec , end of minute Half of the placental blood is transfusedHalf of the placental blood is transfused from placenta to newbornfrom placenta to newborn Dietary ironDietary iron Non dietary ironNon dietary iron
  • 33. CausesCauses Decreased iron storedDecreased iron stored  PretermPreterm  Small for dateSmall for date  TwinsTwins Decreased intakeDecreased intake  Delayed weaningDelayed weaning  MalnutritionMalnutrition  Iron poor dietIron poor diet  Chronic diarrheaChronic diarrhea  GI surgeryGI surgery  Malabsorption syndromeMalabsorption syndrome
  • 34. Causes contd…Causes contd… Increased demandIncreased demand PrematurityPrematurity LBWLBW InfantsInfants Recovery from PEMRecovery from PEM AdolescentsAdolescents Increased lossesIncreased losses Hook worm infestationsHook worm infestations GI bleeding ,Meckle diverticulitisGI bleeding ,Meckle diverticulitis Peptic ulcer ,Bleeding diathesisPeptic ulcer ,Bleeding diathesis
  • 35. StagesStages First stage;First stage; o It consist of depletion of iron storageIt consist of depletion of iron storage o Iron deficiency stateIron deficiency state Lack of ironLack of iron Restrict production of hemoglobinRestrict production of hemoglobin Deficient iron storeDeficient iron store Decreased concentration of sr.ferritinDecreased concentration of sr.ferritin
  • 36. Stages contd…Stages contd… Second stage;Second stage; Decreased iron storeDecreased iron store Low serum iron concentration & transferingLow serum iron concentration & transfering saturationsaturation Increased in total binding capacity but noIncreased in total binding capacity but no anemiaanemia Hb concentration low in the normalHb concentration low in the normal
  • 37. Stages contd…Stages contd… More severe form of iron deficiencyMore severe form of iron deficiency Hemoglobin concentration fallenHemoglobin concentration fallen  low sr .Iron concentrationlow sr .Iron concentration  Low transferrin saturationLow transferrin saturation  Low Hb & Hematocrit valueLow Hb & Hematocrit value Increased free erythrocyte protoporphyrin ,Increased free erythrocyte protoporphyrin , Hypochromia , MycrocytosisHypochromia , Mycrocytosis
  • 38. Clinical manifestationsClinical manifestations Fall hemoglobinFall hemoglobin Hyper dynamic circulationHyper dynamic circulation  PalpitationPalpitation  FatigueFatigue  AnorexiaAnorexia  Shortness of breathShortness of breath  Decreased exercise toleranceDecreased exercise tolerance  Congestive heart failureCongestive heart failure Initial ;Initial ;  Pallor ,Anorexia ,IrritabilityPallor ,Anorexia ,Irritability
  • 39. Symptoms In school childrenSymptoms In school children Impaired IQImpaired IQ Short attention spanShort attention span Impaired mental & motor DevelopmentImpaired mental & motor Development DisabilityDisability School dropoutsSchool dropouts Pica 70-80%Pica 70-80% Pedal edema- Impaired Renal functionPedal edema- Impaired Renal function Papille edema-increased intracranialPapille edema-increased intracranial tensiontension CVS- tachycardia, CHF, Shortness ofCVS- tachycardia, CHF, Shortness of breathbreath
  • 40. InvestigationsInvestigations Age in yrsAge in yrs Sr. ferritinSr. ferritin (ng / dl)(ng / dl) TransferritinTransferritin SaturationSaturation (%)(%) RBCFreeRBCFree erythrocyteerythrocyte prothrombinprothrombin (mg/dl)(mg/dl) 0.5 – 40.5 – 4 5 -105 -10 11 – 1411 – 14 >15>15 <10<10 <10<10 <10<10 <12<12 <12<12 <14<14 <16<16 <16<16 >80>80 >70>70 >70>70 >70>70
  • 41. ManagementManagement Drugs;Drugs;  Ferrous sulfate-6mg/kg/2hr(3 doses)Ferrous sulfate-6mg/kg/2hr(3 doses)  Iron Dextran (Inferon)- 9-50/kg;50mg/24hr/IMIron Dextran (Inferon)- 9-50/kg;50mg/24hr/IM 50mg;100mg/24hr/IV/IM50mg;100mg/24hr/IV/IM  Folic acid - ( under 4 yrs) 0.4mg/dFolic acid - ( under 4 yrs) 0.4mg/d  Vitamin B12 – 30-100ug/IM,SC (8 days)Vitamin B12 – 30-100ug/IM,SC (8 days) Parenteral iron therapy;Parenteral iron therapy;  Packed RBCs 2-3cc/kgPacked RBCs 2-3cc/kg  Iron 50 mg dextran complexIron 50 mg dextran complex
  • 42. Nursing managementNursing management Instruct the parents about administer theInstruct the parents about administer the iron medicationiron medication Oral iron should give 2 divided dose withOral iron should give 2 divided dose with citrus juicescitrus juices Iron supplement should not administerIron supplement should not administer with the milk substanceswith the milk substances Encourage mother exclusively breast –fedEncourage mother exclusively breast –fed infant by 4-6 months of ageinfant by 4-6 months of age IM injection using Z- track methodIM injection using Z- track method Iron medication dropper placed back ofIron medication dropper placed back of the mouththe mouth
  • 43. Out comeOut come  Oral iron therapy is successful inOral iron therapy is successful in the treatment of iron deficiency anemia.the treatment of iron deficiency anemia. PreventionPrevention  Exclusive Breast Feeding 4-6Exclusive Breast Feeding 4-6 monthsmonths
  • 45. SICKLE CELL ANEMIASICKLE CELL ANEMIA INTRODUCTIONINTRODUCTION Sickle cell anemia is one of a group ofSickle cell anemia is one of a group of disease collectively termed hemoglobinopathies, indisease collectively termed hemoglobinopathies, in which normal adult hemoglobin is partly or completelywhich normal adult hemoglobin is partly or completely replaced by abnormal sickle hemoglobin sickle cellreplaced by abnormal sickle hemoglobin sickle cell disease includes all pathologic feature are related todisease includes all pathologic feature are related to the presence of Hemoglobin S.the presence of Hemoglobin S.
  • 46. DEFINITION OF SICKLE CELL ANEMIADEFINITION OF SICKLE CELL ANEMIA:: Sickle cell anemia is the homozygous form of a group ofSickle cell anemia is the homozygous form of a group of inherited diseases in which the normal adult hemoglobininherited diseases in which the normal adult hemoglobin (hemoglobin A) is replaced by a variant form (hemoglobin(hemoglobin A) is replaced by a variant form (hemoglobin S)S) MODE OF TRANSMISSION OF SICKLE CELL ANEMIA;MODE OF TRANSMISSION OF SICKLE CELL ANEMIA; Autosomol recessive disorder –Autosomol recessive disorder –both parents areboth parents are carriers of the sickle trait. Each pregnancy has a one incarriers of the sickle trait. Each pregnancy has a one in four chance or producing a child with sickle cell anemia.four chance or producing a child with sickle cell anemia.
  • 47. PATHOPHYSIOLOGYPATHOPHYSIOLOGY In hemoglobin S the defect is a substitution ofIn hemoglobin S the defect is a substitution of valine for glutamine on the beta polypeptidevaline for glutamine on the beta polypeptide chain of the globinchain of the globin Sickled RBCs are crescent – shaped , haveSickled RBCs are crescent – shaped , have reduced oxygen carrying stasis ,and decreasedreduced oxygen carrying stasis ,and decreased life span.life span. Sickled RBCs are rigid ; they cause trapping andSickled RBCs are rigid ; they cause trapping and increased blood viscosity ,capillary stasis andincreased blood viscosity ,capillary stasis and thrombosis ; eventually , tissue ischemia andthrombosis ; eventually , tissue ischemia and necrosis result.necrosis result.
  • 48. PATHOPHYSIOLOGY OF SICKEL CELL ANEMIAPATHOPHYSIOLOGY OF SICKEL CELL ANEMIA Dehydration, Acidosis, Fever, Hypoxia Heart failure Hemodilution Anemia Hemolysis Retinopathy BlindnessChest syndrome Pain Death Paralysis CVA Diuresis, enuresis Heamaturia GI tract EyesLungBrainkidney Chroniculcers Osteomylitieis Pain Extremities Infraction Tissue ischemia Local hypoxia Vaso- occlusion Splenomegaly Hepatomegaly Gallstones Acute Abdomen pain Sickled RBC
  • 49. CLINICAL MANIFESTATIONS OF SICKELCLINICAL MANIFESTATIONS OF SICKEL CELL ANEMIACELL ANEMIA The most acute symptoms of the diseaseThe most acute symptoms of the disease occur during periods of exacerbation calledoccur during periods of exacerbation called crisescrises Three major types of sickle cell crisesThree major types of sickle cell crises :: Vaso – occlusionVaso – occlusion Acute splenic sequestrationAcute splenic sequestration AplasticAplastic
  • 50. VASO – OCCLUSION CRISISVASO – OCCLUSION CRISIS Painful episodes marked by vessel occlusionPainful episodes marked by vessel occlusion ischemia ,and necrosis caused by pooling of bloodischemia ,and necrosis caused by pooling of blood and clumping of cellsand clumping of cells  ExtremitiesExtremities – painful swelling of hands and feet,painful– painful swelling of hands and feet,painful jointjoint  AbdomenAbdomen – severe pain– severe pain  CerebrumCerebrum – stroke , visual disturbances– stroke , visual disturbances  LiverLiver – obstructive jaundice ,hepatic coma– obstructive jaundice ,hepatic coma  KidneyKidney – hematuria– hematuria  GenitalGenital – priapism– priapism
  • 51. SPLENICSPLENIC SEQUESTRATION CRISISSEQUESTRATION CRISIS Large quantities of blood are pooled inLarge quantities of blood are pooled in the liver and spleenthe liver and spleen  HepatomegalyHepatomegaly  SplenomegalySplenomegaly  Circulatory collapseCirculatory collapse
  • 52. APLASTIC CRISISAPLASTIC CRISIS Decreased RBC production, usually resultsDecreased RBC production, usually results from a virus ( human parvovirus).from a virus ( human parvovirus). HYPERHEMOLYTIC CRISISHYPERHEMOLYTIC CRISIS Increased rate of RBC destruction characterizedIncreased rate of RBC destruction characterized by anemia , jaundice and reticulocytosis.by anemia , jaundice and reticulocytosis.  Acute chest syndromeAcute chest syndrome Sickling occur in small vessels in the lungs.Sickling occur in small vessels in the lungs.  Chest painChest pain  FeverFever  CoughCough  TachypneaTachypnea  WheezingWheezing  HypoxiaHypoxia
  • 53. GENERALGENERAL  Growth retardationGrowth retardation  Chronic anemiaChronic anemia  Delayed sexual maturationDelayed sexual maturation  susceptibility to sepsissusceptibility to sepsis DIAGNOSTIC EVALUATIONS OF SICKEL CELLDIAGNOSTIC EVALUATIONS OF SICKEL CELL ANEMIAANEMIA  Hemoglobin electrophoresisHemoglobin electrophoresis  Routine hematologic testRoutine hematologic test  Sickle –turbidity testSickle –turbidity test  Blood smearBlood smear  Screening test for hemoglobinsScreening test for hemoglobins  Antenatal screening –possibleAntenatal screening –possible through amniocentesis.through amniocentesis.
  • 54. PEDIATRIC COMPLICATIONS OF SICKLEPEDIATRIC COMPLICATIONS OF SICKLE CELL ANEMIACELL ANEMIA Delayed growth and developmentDelayed growth and development Delayed onset of pubertyDelayed onset of puberty Impaired fertility.Impaired fertility. PriapismPriapism  Enuresis especially at nightEnuresis especially at night
  • 55. THERAPEUTIC MANAGEMENT OFTHERAPEUTIC MANAGEMENT OF SICKEL CELL ANEMIASICKEL CELL ANEMIA Aims :Aims :  Prevent condition that enhancing sicklingPrevent condition that enhancing sickling phenomenaphenomena  Treat the medical emergencies of sickleTreat the medical emergencies of sickle cell crisis.cell crisis.  Hematopoietic stem cell transplantationHematopoietic stem cell transplantation with stabilization of prior organ damage iswith stabilization of prior organ damage is a possible cure fora possible cure for sickel cell anemiasickel cell anemia
  • 56. MEDICAL MANAGEMENTMEDICAL MANAGEMENT It is directed at supportive and symptomatic treatment.It is directed at supportive and symptomatic treatment.  Rest to minimize energy expenditure and oxygen useRest to minimize energy expenditure and oxygen use  Hydration through oral and IV therapyHydration through oral and IV therapy  Electrolyte replacementElectrolyte replacement  AnalgesicsAnalgesics  Blood replacement to treat anemiaBlood replacement to treat anemia  Antibiotics to treat infectionAntibiotics to treat infection
  • 57.  Administration of pneumococcal and meningococcalAdministration of pneumococcal and meningococcal vaccinesvaccines  Oral penicillin prophylaxis is also recommended by 2Oral penicillin prophylaxis is also recommended by 2 months of agemonths of age  Exchanged transfusionExchanged transfusion  SplenectomySplenectomy  Painful priapism may be treated by aspiration of thePainful priapism may be treated by aspiration of the corpora cavernosum.corpora cavernosum.  Opioids such as morphine, oxycodoneOpioids such as morphine, oxycodone  Bone marrow transplantation.Bone marrow transplantation.
  • 58. NURSING MANAGEMENT OF SICKLE CELLNURSING MANAGEMENT OF SICKLE CELL ANEMIAANEMIA Sickle cell anemia cannot be cured.Sickle cell anemia cannot be cured.  Teach parents the optimal home care for theirTeach parents the optimal home care for their children.children. The nurses emphasizes the importance ofThe nurses emphasizes the importance of adequate hydration to prevent sickling and toadequate hydration to prevent sickling and to delay the stasis –thrombosis ischemia cycle in adelay the stasis –thrombosis ischemia cycle in a crisis.crisis.
  • 59. Give instruction on how many daily glasses orGive instruction on how many daily glasses or bottles of fluid are required.bottles of fluid are required. Many foods are also source of fluid:Many foods are also source of fluid: SoupsSoups Increased fluids combined with impaired kidneyIncreased fluids combined with impaired kidney function result in the problem of enuresis.function result in the problem of enuresis. To alleviate parents anxiety ,the parents are taughtTo alleviate parents anxiety ,the parents are taught the supportive and symptomatic care the childthe supportive and symptomatic care the child receives in the hospitalreceives in the hospital  Care of during crisisCare of during crisis
  • 60. The general care of during a crisisThe general care of during a crisis :: 1.1. Minimization of energy expenditure and use ofMinimization of energy expenditure and use of oxygenoxygen 2.2. Promotion of hydrationPromotion of hydration 3.3. Replacement of electrolytes and bloodReplacement of electrolytes and blood 4.4. Administration of antibioticsAdministration of antibiotics 5.5. Management by surgeryManagement by surgery 6.6. Provide skin careProvide skin care 7.7. Protect from injuryProtect from injury
  • 61. 1.Minimization of energy expenditure and use1.Minimization of energy expenditure and use of oxygenof oxygen Bed rest to minimize oxygen conception.Bed rest to minimize oxygen conception. Passive range of motion exercise can promote circulation.Passive range of motion exercise can promote circulation. The child needs for oxygen ,especially signs of cardiac failure.The child needs for oxygen ,especially signs of cardiac failure. Short term oxygen therapy can prevent sickling ,but it dose notShort term oxygen therapy can prevent sickling ,but it dose not usually reverse the sickling that already occur.usually reverse the sickling that already occur. Prolonged oxygen therapy is not given because it can depressProlonged oxygen therapy is not given because it can depress bone marrow activity, increased anemia .bone marrow activity, increased anemia .
  • 62. 2.Promotion of hydration2.Promotion of hydration Assess the signs of dehydrationAssess the signs of dehydration Oral and intravenous fluid therapy is necessary toOral and intravenous fluid therapy is necessary to dilute the blood and sickle.dilute the blood and sickle. Monitor Intake and out put chart.Monitor Intake and out put chart. The nurse should observe the sings ofThe nurse should observe the sings of hypokalemia and any abnormal electrolytes value.hypokalemia and any abnormal electrolytes value.
  • 63. 3.Replacement of electrolytes and blood.3.Replacement of electrolytes and blood. During blood transfusion ,the nurse has theDuring blood transfusion ,the nurse has the responsibility of observing for signs of transfusionresponsibility of observing for signs of transfusion reaction .reaction . Hypervolemia from rapid transfusing can increaseHypervolemia from rapid transfusing can increase the workload of the heart.the workload of the heart. The risk of hepatitis ,transfusion reaction andThe risk of hepatitis ,transfusion reaction and hemosiderosis must considered when multiplehemosiderosis must considered when multiple transfusion are planned.transfusion are planned.
  • 64. 4.Relief of pain4.Relief of pain Assess for location severity ,Assess for location severity , duration and quality of painduration and quality of pain Assess intensity of pain withAssess intensity of pain with the use of an age –appropriatethe use of an age –appropriate pain rating scale.pain rating scale. provide comfort position .provide comfort position . Gently handle painful jointsGently handle painful joints and extremities provide supportand extremities provide support with and maintain body alignment.with and maintain body alignment. Apply warmth to the affected area.Apply warmth to the affected area. Provide massage over theProvide massage over the affected area.affected area. Provide diversional therapyProvide diversional therapy (music , play therapy)(music , play therapy) Administer analgesic as per doctors orderAdminister analgesic as per doctors order Encourage relaxation techniquesEncourage relaxation techniques like deep breathing exercise.like deep breathing exercise. Avoid the use of ice ,cold compressesAvoid the use of ice ,cold compresses which may cause vasoconstriction.which may cause vasoconstriction.
  • 65. 5.Administration of antibiotics5.Administration of antibiotics Infection are a frequent precipitator of cellInfection are a frequent precipitator of cell crisis.crisis. Administer antibiotics:Administer antibiotics: Ex: Penicillin ,CephalosporinsEx: Penicillin ,Cephalosporins 6.Management by surgical intervention6.Management by surgical intervention:: Splenectomy may become necessary.Splenectomy may become necessary. Determine whether the spleen is continuingDetermine whether the spleen is continuing to enlarge.to enlarge. Monitor the vital signs and blood pressure toMonitor the vital signs and blood pressure to determine the presence of shock .determine the presence of shock .
  • 66. 7.Provide skin care7.Provide skin care ;; Assess the for signs of hypoxia , dehydration,Assess the for signs of hypoxia , dehydration, cyanosis.cyanosis. Check the vital signsCheck the vital signs Monitor intake and output chart.Monitor intake and output chart. Encourage the child to drink fluid every 2Encourage the child to drink fluid every 2 hours.hours. Cut short the nails.Cut short the nails. Provide adequate rest to decrease oxygenProvide adequate rest to decrease oxygen expenditure.expenditure. Perform passive range of motion exercise.Perform passive range of motion exercise. Apply moister cream.Apply moister cream. Provide safe environment to protect fromProvide safe environment to protect from injury.injury.
  • 67. 8.Protect from injury8.Protect from injury;; Assess the child condition.Assess the child condition. Child should be closely supervised.Child should be closely supervised. Child should not be left alone.Child should not be left alone. Provide safety precautions remove sharp objectsProvide safety precautions remove sharp objects from child.from child. Provide safe environment to protect from injuryProvide safe environment to protect from injury
  • 68. OutcomeOutcome The prognosis for individuals who haveThe prognosis for individuals who have sickle cell trait is generally good. With bettersickle cell trait is generally good. With better nutrition and more adequate prevention andnutrition and more adequate prevention and treatment of infection , increasing numbers oftreatment of infection , increasing numbers of individuals are surviving into adult life.individuals are surviving into adult life. PreventionPrevention Public educationPublic education ScreeningScreening Genetic counsellingGenetic counselling
  • 69.
  • 70. THALASSEMIATHALASSEMIA The thalassemia syndromes are aThe thalassemia syndromes are a heterogeneous group of usually inheritedheterogeneous group of usually inherited chronic disorder that are characterized bychronic disorder that are characterized by an absence or decreased synthesis of onean absence or decreased synthesis of one of the normal globin chain of hemoglobin.of the normal globin chain of hemoglobin. CAUSES OF THALASSEMIACAUSES OF THALASSEMIA Alpha or beta chainAlpha or beta chain
  • 71. PATHOPHYSIOLOGY OF THALASSEMIAPATHOPHYSIOLOGY OF THALASSEMIA Normal postnatal Hgb is composed of 2 alpha and 2Normal postnatal Hgb is composed of 2 alpha and 2 beta polypeptide chain.beta polypeptide chain. In beta thalassemia – partial or complete deficiencyIn beta thalassemia – partial or complete deficiency In the synthesis of beta chainsIn the synthesis of beta chains Increased beta synthesis of alpha chain and gammaIncreased beta synthesis of alpha chain and gamma chain productionchain production Defective Hemoglobin formationDefective Hemoglobin formation Unbalanced polypeptide unitUnbalanced polypeptide unit Damage of RBCDamage of RBC Severe anemiaSevere anemia
  • 72. TYPES OF THALASSEMIATYPES OF THALASSEMIA The two forms of beta thalassemiaThe two forms of beta thalassemia Thalassemia majorThalassemia major Thalassemia minorThalassemia minor Thalassemia minor:Thalassemia minor: Thalassemia minor is associated withThalassemia minor is associated with decreased beta chain synthesis and isdecreased beta chain synthesis and is the heterozygous form.the heterozygous form. It produces little effect on the child on theIt produces little effect on the child on the child expect mild anemia andchild expect mild anemia and hypochromiahypochromia
  • 73. Thalassemia majorThalassemia major (( Cooley's anemia orCooley's anemia or Mediterranean anemiaMediterranean anemia)) It is associated with absent beta – chainIt is associated with absent beta – chain synthesis and is the homozygous form.synthesis and is the homozygous form. It produces sever anemiaIt produces sever anemia
  • 74. CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS OFOF THALASSEMIATHALASSEMIA Absent or defective synthesis of Hemoglobin AAbsent or defective synthesis of Hemoglobin A Inadequate Structured RBCsInadequate Structured RBCs Decreased life span of the RBCsDecreased life span of the RBCs FeverFever AnorexiaAnorexia Progressive severe anemiaProgressive severe anemia Enlarge d spleenEnlarge d spleen HemosiderinHemosiderin HemochromatosisHemochromatosis HypoxiaHypoxia HeadacheHeadache Precordial painPrecordial pain
  • 75. SkinSkin Greenish –brown ,bronzed skin with fineGreenish –brown ,bronzed skin with fine frecklesfreckles HeartHeart Increased cardiac overload,Increased cardiac overload, cardiac failurecardiac failure SpleenSpleen Enlargement of spleenEnlargement of spleen Abdominal distensionAbdominal distension
  • 76. LiverLiver CirrhosisCirrhosis Lymph nodesLymph nodes Lymph nodes in the abdomen enlarged.Lymph nodes in the abdomen enlarged. skeletal systemskeletal system The expanded marrow of the enlarged skull andThe expanded marrow of the enlarged skull and face produces a typical facies:face produces a typical facies: Frontal and parietal bossingFrontal and parietal bossing Slanting or mongoloid eyesSlanting or mongoloid eyes A depressed nasal bridgeA depressed nasal bridge Prominent malar eminence or cheek boneProminent malar eminence or cheek bone Enlargement of the maxillaEnlargement of the maxilla
  • 77. DIAGNOSTIC EVALUATIONDIAGNOSTIC EVALUATION OFOF THALASSEMIATHALASSEMIA Hypochromia and microcytosisHypochromia and microcytosis Hemoglobin level may fall to less thanHemoglobin level may fall to less than 5 gm/dl5 gm/dl Unconjugated serum bilirubin is elevatedUnconjugated serum bilirubin is elevated X –ray – over growth of maxilla and vertical trabeculaeX –ray – over growth of maxilla and vertical trabeculae Total erythrocyte counts are low –Total erythrocyte counts are low – 2to3 million /mm32to3 million /mm3 Mean Corpuscular hemoglobin ,mean corpuscular volume,Mean Corpuscular hemoglobin ,mean corpuscular volume, mean corpuscular hemoglobin concentration are low.mean corpuscular hemoglobin concentration are low.
  • 78. COMPLICATIONS OF THALASSEMIACOMPLICATIONS OF THALASSEMIA Transfusion reactionTransfusion reaction HemosiderosisHemosiderosis CirrhosisCirrhosis InfectionInfection Growth failureGrowth failure Cardiac diseaseCardiac disease
  • 79. THERAPEUTIC MANAGEMENT OFTHERAPEUTIC MANAGEMENT OF THALASSEMIATHALASSEMIA Thalassemia cannot be cured, supportive therapyThalassemia cannot be cured, supportive therapy is essential to prevent complicationsis essential to prevent complications The major therapeutic management are:The major therapeutic management are: Transfusion therapy;Transfusion therapy; Transfusion therapy in thalassemia has two majorTransfusion therapy in thalassemia has two major goalsgoals:: Prevent anemiaPrevent anemia Suppress endogenous erythropoises to avoid ineffectiveSuppress endogenous erythropoises to avoid ineffective erythropoiesis.erythropoiesis. The objectives of transfusion therapy areThe objectives of transfusion therapy are:: To maintain Hemoglobin level of 10.5 g / dlTo maintain Hemoglobin level of 10.5 g / dl To prevent hypoxiaTo prevent hypoxia
  • 80. CHELATION THERAPYCHELATION THERAPY The goal of chelationThe goal of chelation is to iron overloadis to iron overload and subsequently maintain ferritin level belowand subsequently maintain ferritin level below 1000 ng / ml .1000 ng / ml . Desferrioxamine:Desferrioxamine: The dose is 20 to 40 mg/kg/day given subcutaneously over 8The dose is 20 to 40 mg/kg/day given subcutaneously over 8 to 10 hour for 6 night a week.to 10 hour for 6 night a week. Intravenous desferalIntravenous desferal :: It can be given particularly in those with very high ironIt can be given particularly in those with very high iron overload through port – a- caths (central line)overload through port – a- caths (central line) High dose desferal given in severe hemosiderosis toHigh dose desferal given in severe hemosiderosis to prevent/reverse cardiac toxicity of iron overload.prevent/reverse cardiac toxicity of iron overload. Deferiprone:Deferiprone: Dose:75 to 100 mg/kg body weight/day in three to four dividedDose:75 to 100 mg/kg body weight/day in three to four divided doses.doses.
  • 81. SPLENECTOMYSPLENECTOMY The child has already developed Splenomegaly and signsThe child has already developed Splenomegaly and signs of hypersplenism are present, and is above 5 years ofof hypersplenism are present, and is above 5 years of age,splenectomy should be done.age,splenectomy should be done. Indications of splenectomyIndications of splenectomy:: Decrease in WBC and platelet count is a relatively lateDecrease in WBC and platelet count is a relatively late manifestation of hypersplenism.manifestation of hypersplenism. Child should receive pneumococcal vaccine, H.influenza type bChild should receive pneumococcal vaccine, H.influenza type b vaccine ,vaccine ,and meningococcal vaccine 6 to 8 weeks prior to surgery.and meningococcal vaccine 6 to 8 weeks prior to surgery.
  • 82. NURSING MANAGEMENTNURSING MANAGEMENT OF THALASSEMIAOF THALASSEMIA The nurses role in the care of infants and childrenThe nurses role in the care of infants and children includes;includes; – Early assessmentEarly assessment – Preparation for diagnostic procedurePreparation for diagnostic procedure – Administration of transfusion therapyAdministration of transfusion therapy – Care during surgical procedureCare during surgical procedure – Prevention of infectionPrevention of infection – Observe for ComplicationsObserve for Complications – Education and support of the parents and child.Education and support of the parents and child. Early AssessmentEarly Assessment History collection--- ethnic background ,History collection--- ethnic background , sings and symptoms and early diagnosissings and symptoms and early diagnosis
  • 83. Preparation for diagnostic studiesPreparation for diagnostic studies The child and parent are prepared forThe child and parent are prepared for X – ray ,laboratory test.X – ray ,laboratory test. explain about benefits and needs of diagnosticexplain about benefits and needs of diagnostic procedure.procedure. explain to the parents and child about amount andexplain to the parents and child about amount and duration of procedure.duration of procedure. The nurse should consider the parents and childThe nurse should consider the parents and child feelings and get co operation from parents and child.feelings and get co operation from parents and child.
  • 84. Administration Of Transfusion TherapyAdministration Of Transfusion Therapy The transfusion therapy is given to increase theThe transfusion therapy is given to increase the circulation of hemoglobincirculation of hemoglobin Transfusion therapy that maintain the hemoglobinTransfusion therapy that maintain the hemoglobin level at 10.5 gm/dllevel at 10.5 gm/dl The nurse can function as an advocate for the familyThe nurse can function as an advocate for the family by helping arrange for blood transfusion and medicalby helping arrange for blood transfusion and medical supervisionsupervision The nurse in charge plans the transfusion therapy wellThe nurse in charge plans the transfusion therapy well and give sufficient time for proper testing.and give sufficient time for proper testing. The nurse is responsible for maintaining the vein – theThe nurse is responsible for maintaining the vein – the lifeline of thalassemic child.lifeline of thalassemic child. Complications of transfusionComplications of transfusion Iron overloadIron overload Non hemolytic febrile reaction.Non hemolytic febrile reaction. InfectionsInfections
  • 85. Care of surgical procedureCare of surgical procedure:: Splenectomy may be performed for the correction ofSplenectomy may be performed for the correction of hypersplenismhypersplenism The nurse prepare the family and child for surgeryThe nurse prepare the family and child for surgery Preoperative teachingPreoperative teaching Complication, the duration of the hospitalization andComplication, the duration of the hospitalization and recovery time.recovery time. Legal preparation of surgeryLegal preparation of surgery Get the ConsentGet the Consent Day of surgeryDay of surgery To verify consentTo verify consent Laboratory dataLaboratory data Records of any consultationRecords of any consultation Baseline vital signsBaseline vital signs Preparation of skinPreparation of skin Remove nail polish and not wear cosmeticRemove nail polish and not wear cosmetic Preoperative medicationPreoperative medication
  • 86. Post Operative CarePost Operative Care Protection from infection.Protection from infection. Vital signs and blood pressure are recordedVital signs and blood pressure are recorded frequently until stable.frequently until stable. Suctioning is intermittent and maintained for notSuctioning is intermittent and maintained for not more than 5 secondsmore than 5 seconds Intake and output chart maintainedIntake and output chart maintained Provide adequate rest and ambulationProvide adequate rest and ambulation Provide firm pillow and favorite stuffed animalProvide firm pillow and favorite stuffed animal placed against the chest.placed against the chest. Provide nonpharmacologic method to relive painProvide nonpharmacologic method to relive pain Stay with child during procedureStay with child during procedure Teach procedure to family membersTeach procedure to family members
  • 87. The child is kept warm to prevent heat lossThe child is kept warm to prevent heat loss Allow the parents to express the feelings.Allow the parents to express the feelings. Clarify their doubtsClarify their doubts Provide support as neededProvide support as needed Provide information on resources availableProvide information on resources available regarding respite care to provide short term careregarding respite care to provide short term care The child is treated with oral broad –spectrumThe child is treated with oral broad –spectrum antibiotics.antibiotics. The nurse should observe signs of cholecystitisThe nurse should observe signs of cholecystitis
  • 88. Prevention of infectionPrevention of infection Observe sign of infectionObserve sign of infection Maintain sterile procedureMaintain sterile procedure Maintain aseptic environmentMaintain aseptic environment Use sterile equipmentsUse sterile equipments Maintain handwashing before and after the procedure.Maintain handwashing before and after the procedure. Isolate child as indicated to prevent nosocomialIsolate child as indicated to prevent nosocomial infectioninfection Provide high protein and high caloric dietProvide high protein and high caloric diet Teach child and family ,manifestation of illnessTeach child and family ,manifestation of illness Appropriate antibiotic therapy.Appropriate antibiotic therapy.
  • 89. Nursing management on child with AnemiaNursing management on child with Anemia 1.1.Impaired gas exchange related to diminishedImpaired gas exchange related to diminished oxygen carrying capacity to the blood asoxygen carrying capacity to the blood as evidenced by restlessness & shortness of breathevidenced by restlessness & shortness of breath Interventions;Interventions; Monitor vital signs &Blood gas analysisMonitor vital signs &Blood gas analysis Administer the oxygen to the childAdminister the oxygen to the child Providing rest to the childProviding rest to the child Administer the blood transfusion to the childAdminister the blood transfusion to the child Provide comfort position head elevated (Fowler’sProvide comfort position head elevated (Fowler’s position) &back rest to the childposition) &back rest to the child Explain the child reason for restrict the activity.Explain the child reason for restrict the activity.
  • 90. 2.Ineffective Breathing pattern related to2.Ineffective Breathing pattern related to decreased hemoglobin & diminished oxygendecreased hemoglobin & diminished oxygen carrying capacity as evidenced by dyspniacarrying capacity as evidenced by dyspnia Intervention;Intervention;  Monitor the respiratory rate ,Breathing sound, ABGMonitor the respiratory rate ,Breathing sound, ABG analysis ,skin color.analysis ,skin color.  Raised bed –give fowler’s position & cardiac tableRaised bed –give fowler’s position & cardiac table  Administer the oxygen to the childAdminister the oxygen to the child  Administer the blood transfusion to the childAdminister the blood transfusion to the child  Restrict the child activitiesRestrict the child activities  Provide psychological support to the childProvide psychological support to the child  Provide child recreation therapy (watching TVProvide child recreation therapy (watching TV ,Telling stories),Telling stories)
  • 91. 3.Impaired tissue perfusion related to inadequate3.Impaired tissue perfusion related to inadequate blood volume as evidenced by pale skin, cyanosedblood volume as evidenced by pale skin, cyanosed Interventions;Interventions;  Monitor Cardiac rate ,RhythmMonitor Cardiac rate ,Rhythm  Administer IV fluid (Electrolyte) to the childAdminister IV fluid (Electrolyte) to the child  Administer the oxygen to the childAdminister the oxygen to the child  Administer the blood transfusion to the childAdminister the blood transfusion to the child  Monitor vital signs every 10-20mts 1Monitor vital signs every 10-20mts 1stst 4hrs4hrs  Maintain intake and output chart dailyMaintain intake and output chart daily  provide skin careprovide skin care  Administer antihypertensiveAdminister antihypertensive
  • 92. 4.Imbalance nutrition less than body requirement4.Imbalance nutrition less than body requirement related torelated to inadequate intake of essential nutritioninadequate intake of essential nutrition Asses the nutritional status if the chilrd,24Asses the nutritional status if the chilrd,24 hrs recall for the child nutritional status.hrs recall for the child nutritional status. Encourage the child intake iron ,protein.Encourage the child intake iron ,protein. Vitamins ,rich diets.Vitamins ,rich diets. Provide small & frequent dietProvide small & frequent diet Reduced noxious environmental (sight,Reduced noxious environmental (sight, smell) stimuli.smell) stimuli. Allow favorite & special foodAllow favorite & special food Provide balance diet in hygienicProvide balance diet in hygienic preparationspreparations
  • 93. Contd…Contd… Measure total intake & out put chart dailyMeasure total intake & out put chart daily Provide dietary supplements vitamins ,ironProvide dietary supplements vitamins ,iron , folate ,protein, folate ,protein Health education regarding Balance diet,Health education regarding Balance diet, hygienic food practicehygienic food practice
  • 94. 5.Fatigue related to decreased hemoglobin &5.Fatigue related to decreased hemoglobin & diminished oxygen carrying capacity of blooddiminished oxygen carrying capacity of blood Interventions;Interventions; Assess the child condition of fatigueAssess the child condition of fatigue Provide all the articles near to the childProvide all the articles near to the child bed sidebed side Assist the child for personal hygiene.Assist the child for personal hygiene. Provide complete rest to the child &Provide complete rest to the child & restrict the child activitiesrestrict the child activities Explain the child reason for restrict the activity.Explain the child reason for restrict the activity. Provide side rails to the childProvide side rails to the child
  • 95. 6.Activity intolerance related to imbalance between6.Activity intolerance related to imbalance between oxygen supply /demand as evidenced byoxygen supply /demand as evidenced by increased pulseincreased pulse Interventions;Interventions; Assess the child condition of fatigueAssess the child condition of fatigue Encourage alternative rest & activityEncourage alternative rest & activity Restrict the play activities like running,Restrict the play activities like running, jumping.jumping. Assist the child for regular physical activityAssist the child for regular physical activity (personal care ,ambulation ,transfers)(personal care ,ambulation ,transfers) Limit the number of visitorsLimit the number of visitors
  • 96. 7.Impaired growth and development related to7.Impaired growth and development related to poor intake of nutrition as evidenced by weightpoor intake of nutrition as evidenced by weight lossloss Assess the weight of the child according toAssess the weight of the child according to the age,& development.the age,& development. Provide dietary supplements vitamins ,iron ,Provide dietary supplements vitamins ,iron , folate ,proteinfolate ,protein Provide small & frequent diet in attractiveProvide small & frequent diet in attractive mannermanner Reduced noxious environmental (sight,Reduced noxious environmental (sight, smell) stimuli.smell) stimuli. Involve the family while planning the careInvolve the family while planning the care of childof child
  • 97. 8.Fear related to8.Fear related to painful diagnosticpainful diagnostic procedureprocedure Interventions;Interventions; Asses the child and parents level of fearAsses the child and parents level of fear Provide psychological support to theProvide psychological support to the parents as well as childparents as well as child Explain the every step of procedure,&Explain the every step of procedure,& benefits of procedurebenefits of procedure Maintain calm ,safe environmentMaintain calm ,safe environment throughout the hospitalizationthroughout the hospitalization Provide opportunity for decision makingProvide opportunity for decision making regarding care ,allow to ask their doubts.regarding care ,allow to ask their doubts.
  • 98. 9.Interupted family process related to child9.Interupted family process related to child in hospitalizationin hospitalization Explain the every step of procedureExplain the every step of procedure Explain the reason for the procedure andExplain the reason for the procedure and benefits of procedurebenefits of procedure Maintain good interpersonal relationship toMaintain good interpersonal relationship to the child parentsthe child parents Allow to ask their doubts.Allow to ask their doubts. Provide psychological support to theProvide psychological support to the parents as well as childparents as well as child Every procedure allow the parents near toEvery procedure allow the parents near to the child.the child.
  • 99. 10. Ineffective management of therapeutic10. Ineffective management of therapeutic regimen related to lack of knowledgeregimen related to lack of knowledge Assess the level of knowledge of the childAssess the level of knowledge of the child parentsparents Explain the every step of procedure,& benefits ofExplain the every step of procedure,& benefits of procedureprocedure Explain the disease condition,treatment, dietExplain the disease condition,treatment, diet regimentregiment Teach the parents hygienic food preparationTeach the parents hygienic food preparation Allow to ask their doubts.Allow to ask their doubts. Explain the parents source of iron rich foodsExplain the parents source of iron rich foods
  • 100. 11.Risk for infection related to depressed11.Risk for infection related to depressed body defensesbody defenses Interventions;Interventions; Place the child private roomPlace the child private room Maintain aseptic precautions all staffs andMaintain aseptic precautions all staffs and parentsparents Screen all visitors as much as possibleScreen all visitors as much as possible Monitor temperatureMonitor temperature Evaluate the child potential site of infectionEvaluate the child potential site of infection provide complete nutritious diet to theprovide complete nutritious diet to the childchild Administer vaccine up to the age
  • 101. 12.Potential complication related to heart failure12.Potential complication related to heart failure Interventions;Interventions; Asses the signs & symptoms of heartAsses the signs & symptoms of heart failure ,numbness ,tingling on bottom offailure ,numbness ,tingling on bottom of footfoot Check the body weight daily & vitalCheck the body weight daily & vital signs ,every 15-20 mtssigns ,every 15-20 mts Avoid or limited the activitiesAvoid or limited the activities Provide adequate rest & comfortProvide adequate rest & comfort measuresmeasures Administer diureticsAdminister diuretics Maintain I/O CHART every 24 hrsMaintain I/O CHART every 24 hrs