Cardiac

1
Cardiac

• Elisa A. Mancuso RNC, MS, FNS
• Professor of Nursing

2
Hemodynamics

• Preload-
• Venous return during diastole.
• Volume of blood Hydration status
• Afterload-
• Arterial flow during systole.
• L Ventricular pressure to open Aortic valve.
• Cardiac Output HR x SV
• Heart Rate X Stroke Volume

3
Murmurs

• Abnormal heart sounds
• Improper closing or opening of valves
• 80 of murmurs in kids are innocent
• Close proximity of heart to chest wall
• Stills Murmur
• Blood rushing out of aorta
• Anemia
• Fever

4
Fetal Circulation

• Oxygenated blood ? Inferior Vena Cava (IVC)
• IVC ? RA ? FO ? LA ? LV ? Aorta ? Head Arms
• ?? pressure in RA ? Foramen Ovale (FO)
• Bypasses the lungs and blood shunted to LA
• ? Blood returned ? Superior Vena Cava (SVC)
• SVC ? RA ? RV ? PA? DA ?Aorta ? Lower Body Legs
• Ductus Arteriosus (DA)
• Bypasses the lungs (?? Pressure)
• Only small portion of blood goes to pulmonary
  system
• Blood gets re-oxygenated via placenta
• Ductus Venosis
• Bypasses the liver and shunts blood to
  IVC

5
Fetal to Neonatal Circulation

• At birth lungs expand
• ? O2 causes pulmonary vasodilation
• ? Pulmonary pressure (resistance)
• ? Systemic pressure (resistance)
• LA pressure gt RA pressure
• Foramen Ovale closes within 1st hour of life
• ?O2 ?Prostaglandins (from Placenta)
• Ductus Arteriosus closes within 10-24hours.
• Permanent closure by 3-4 weeks.
• PDA Patent Ductus Arteriosus
• In some cases can stay open for 3 months

6
Neonatal Circulation

• Blood flows from higher to lower pressure
• Systemic Pressure gt Pulmonary Pressure
• L side gt R side
• Blood flows from L ? R side

7
Acyanotic Defects

• L ?R side shunt of oxygenated blood
• ? Pulmonary blood flow
• Pulmonary congestion
• Heart is ineffective pump
• Children prone to CHF
• Prophylactic administration of antibiotics
  needed

8
Patent Ductus Arteriosus (PDA)

• Opening between the pulmonary artery (PA) and
  Aorta
• Oxygenated blood shunted from Aorta ??PA
• ?? Systemic resistance
• Blood shunted to LA ? LV ? PA
• ?? Pulmonary Congestion
• ?? Back up to LA LV
• LV Hypertrophy

9
PDA Clinical signs

• Soft - harsh systolic newborn murmur
• Machinery type systolic and diastolic murmur in
  older children
• ? RR moist Breath sounds
• Bounding pulses
• ? HR
• Widened pulse pressure
• Large difference between the systolic
• and diastolic pressure

10
Therapy

• Indomethacin (indocin)
• Prostaglandin inhibitor promotes vasoconstriction
  and closure of PDA
• 3 Dose maximum q 12 hours
• Ligation of Ductus Arteriosus
• Close connection to prevent return of oxygenated
  blood to lungs
• No open heart surgery

11
Atrial Septal Defect (ASD)

• Abnormal opening between the RA LA
• Blood flows from ??press LA to ??press RA
• ?? blood volume to right side of heart
• Leads to RA and RV hypertrophy
• ?? blood volume to lungs
• Pulmonary Congestion
• DOE/ CHF symptoms
• Crescendo/decrescendo systolic
• ejection murmur

12
ASD Therapy

• ASO (Amplatzer Septal Occluder)
• via cardiac cath
• Medications for CHF
• Open heart surgery and bypass, performed before
  school age
• Dacron patch
• Low mortality rate

13
Ventricular Septal Defect (VSD)

• Abnormal opening between RV and LV
• ??O2 blood from LV to RV
• ?? blood to RV RV hypertrophy
• ?? pulmonary flow
• ?? systemic flow
• Spontaneous closure in 20-60 within first year
  of life.

14
VSD Clinical signs

• CHF ??CO, ?HR, ?RR, scalp sweating,
• ? weight gain, irritability
• Pulmonary edema
• DOE, fatigue, ?? PO intake
• ??Aorta Blood Flow
• ?? femoral and brachial pulses
• ?? BP x 4
• Harsh holosystolic murmur with thrill
• Therapy same as ASD

15
Pulmonic Stenosis (PS)

• Narrowing of the pulmonary valve
• ?? PA pressure/resistance
• ?? Pulmonary Blood Flow
• Blood backs up into RV
• RV Hypertrophy

16
Clinical Signs Therapy

• Depends on size of stenosis
• Pale, lethargic, slow feeder
• Systolic ejection murmur
• EKG and CXR show RV Hypertrophy
• Therapy
• Pulmonary Valvotomy
• Angioplasty
• Enlarges ? pulmonic valve opening

17
Aortic Valvular Stenosis

• Narrowing of aortic valve
• ?? Resistance to blood flow from LV
• causing LV Hypertrophy
• ?? back-up of blood in pulmonary system
• ?? Pulmonary congestion
• ?? blood via aorta
• ??Systemic perfusion ?? CO

18
Clinical

• Faint peripheral pulses RT ?? CO
• ?? pulse pressure
• Chest pain RT myocardial ischemia
• Systolic ejection murmur
• Therapy
• Commissurotomy
• Enlarge aortic valve opening via angioplasty.
• Additional surgery may be needed
• later.

19
Coarctation of the Aorta

• Narrowing of the aorta right after arch
• ??Pressure proximal to narrowing
• ?? BP upper body, arms head
• Bounding pulses warm, ruddy skin
• JVD
• ??Pressure distal to narrowing
• ?? BP lower body legs
• Weak pulses cool, pale skin
• Difference of 20mm for systolic BP

20
Clinical signs

• ?? BP in arms ?? BP legs
• Weak or Absent femoral pulses
• Headache, blurred vision and nose bleeds
• ??risk for stroke
• Older kids leg pain on exertion RT ? blood
• Therapy
• Prostaglandin E keep PDA open
• Surgery
• Resect coarcted portion and
• reanastomosis

21
Cyanotic defects

• Unoxygenated blood enters systemic system
• Right to Left shunt (R? L)
• Blood is shunted from venous to arterial
• ?? CHF and hypoxic episodes
• Now classified as
• ?? Pulmonary blood flow or
• Mixed blood flow defects

22
Transposition of Great Vessels (TGA)

• Two separate circulations!
• Aorta arises from RV
• Unoxygenated blood enters aorta ?Systemic
• Pulmonary artery (PA) arises from LV
• Oxygenated blood enters PA ? recycled lungs ?
  Pulmonary veins ? LA
• No Oxygenated blood in systemic
• circulation!

23
TGA CLINICAL SIGNS

• Depends on type and size of associated defects
• Severely cyanotic at birth
• minimal communication between 2 systems
• Large septal defects or PDA
• Less cyanotic but may have CHF symptoms
• ?? HR, ?? RR and cardiomegaly
• Fatigue when feeding
• ?? Intake
• ??Output Edema

24
THERAPY

• Prostaglandin E1 (Prostin VR or Alprostadil)
• Vasodilator
• Relaxes smooth muscle of ductus arteriosus
• Keeps PDA open
• Provides mixing of oxygenated and deoxygenated
  blood to systemic circulation.
• Rashkind procedure
• Cardiac cath to create ASD
• Maintains mixing of blood
• Arterial switch procedure usually performed
  
• in first few weeks of life

25
Tetralogy of Fallot

• Involves four cardiac defects
• VSD
• Blood shunted RV? LV
• Pulmonary Stenosis
• ?? blood to PA
• Overriding Aorta
• Sits over VSD
• RV Hypertrophy
• ? pressure from stenosis

26
Clinical signs of Tetralogy

• First cry hypoxic and cyanotic
• ?? Activity ?? Hypoxia and ?? Cyanosis
• Pulse oximeter in low 70s
• ?? Pulmonary stenosis ?? Cyanosis
• ?? HR, ?? RR
• Tire easily cant finish feedings ?? Intake
• Chronic O2 deficit ? Polycythemia
• ?? RBCs to supply 02 to body
• ?? Risk of CVA or embolism with dehydration

27
Clinical Manifestations

• Tet Spells
• ?? Activity or ?? Crying ?? blood flow to brain
• ?? hypoxia, cyanosis and fainting
• Squatting
• compensatory action
• Knee chest position
• ??femoral blood flow ??blood flow upper body
• Clubbed fingers
• Mental retardation

28
Therapy

• Prostaglandin E1
• Maintain PDA
• ?? Pulmonary perfusion
• Surgery
• Patch the VSD
• Open stenotic pulmonary valve
• Heart Transplant with severe defects

29
Hypoplastic Left Heart (HLHS)

• Fatal anomaly
• Non-functioning LV
• Severe Aortic Stenosis
• ASD
• Aortic valve and Mitral valve Atresia
• PDA
• Clinical Signs
• ?? systemic output
• ?? B/P
• ?? Perfusion
• Faint, weak pulses (lt1)

30
Treatment

• ExtraCorporal Membrane Oxygenation (ECMO)
• ?? Risks Costs (250,000/day)
• ?? Availability _at_ Regional centers
• Heart Transplant
• ?? Donor hearts
• 3 Stage surgery if child can tolerate it.
• DNR Letting Go
• Bereavement

31
Tricuspid Atresia (TA)

• Three major defects
• No tricuspid valve
• ASD VSD
• RV Hypoplasia
• Lungs receive blood via
• PDA
• small VSD
• bronchial vessels
• As long DA remains open the child
• receives adequate O2.

32
Clinical Signs

• Cyanosis
• ?? HR ?? RR
• Dyspnea with activity
• Systolic murmur
• Squatting
• Polycythemia
• Clubbed fingers

33
Therapy

• Prostagladin E
• Maintain PDA for 2 weeks then need surgery.
• Surgery-
• Anastomosis to allow blood flow to lungs.
• Three stages
• Blalock-Taussing _at_ 2 weeks of age
• shunt btwn PA and Aorta
• Glenn _at_ 6 month to a year
• shunt from SVC to PA to lungs
• 3) Fontan _at_ 2-3 years
• shunt from IVC to PA to lungs
• No more mixing of blood

34
Truncus Arteriosus

• One common artery arises from LV RV.
• Overrides a large VSD
• No separate PA or Aorta
• Unoxygenated blood enters systemic circulation
• ?? blood volume flows to lungs
• ?? pulmonary blood flow
• ?? pulmonary edema
• ?? CHF

35
Treatments

• CHF and fluid overload
• Lasix (1 mg/kg/dose)
• Diurectic ?? edema ?? Na, ?? K
• Digoxin (Digitalization Dosing)
• Cardiac glycoside ?? edema
• Surgery
• VSD
• R side graft

36
Nursing Interventions

• v Maternal History
• Rubella, DM, ETOH or Cardiac disease
• Congenital heart disease
• Chronic maternal illness
• Perinatal infections (TORCH)
• ertain meds maybe linked
• Substance Abuse
• ETOH may be associated with FAS and Tetralogy of
  Fallot

37
Physical Exam

• Thoracic Exam
• Cardiac Sounds
• v Location of PMI (5th LICS MCL)
• v Rate
• v Rhythm
• v Murmurs
• location, intensity and where in cardiac
  respiratory cycles
• v visible pulsation on thorax
• v JVD
• Breath Sounds
• v Rate, rhythm
• v Dyspnea and Grunting (keep alveoli open)
• v Adventitious sounds
• Moist- Pulmonary congestion or CHF

38
COLOR

• v Mucous membranes
• Lips, conjunctiva and nail beds.
• v Cyanosis
• _at_ rest or with activity
• v clubbed fingers
• Flushed cheeks Polycythemia
• KEEP INFANTS HYDRATED!
• WHY?

39
Pulses

• Palpate bilaterally
• Compare upper and lower extremities
• Absent or ?femoral pulses in Coarctation
• v Rate/Rhythm/strength (0-4)
• v BP all four extremities
• Widened pulse pressure in PDA
• ?? BP upper extremities in Coarctation.

40
Nutritional Status

• v Intake
• Rest periods needed?
• Time needed to complete feedings
• ?? intake, tiring due to ?? available O2
• v HT, WT and HC
• v Activity level-tires easily?
• Developmental tasks achieved?

41
Respiratory Infections

• ??Risk
• Pulmonary vascular congestion
• Bacterial invasion and growth
• RT stasis of secretions (prophylaxis meds)
• Therapy
• Meds
• Bronchodilators
• Steroids
• PD C
• O2

42
Compensatory Mechanisms

• Cardiomegaly
• ? pumping action of heart ? SV
• ? use of cardiac muscle ? O2 availability
• ? size hypertrophy
• Tachycardia gt160 in infant
• ? rate ? CO
• ? O2 to tissues and vital organs
• Polycythemia
• ? production of RBCs
• ? availability of O2 to tissues
• ? viscosity of blood
• ?? flow, sluggish
• ?? decreased peripheral circulation
• High risk for CVA
• Tachypnea gt 60 in infants
• ?? RR ?? O2

43
Compensatory

• Posturing
• ?? O2 to vital organs by ?? workload of heart
• Less area for blood to flow ?? venous return
• TET Spells
• Infants
• May be flaccid with extremities extended
• Knee chest position (infant seat)
• Preschool
• Squatting position
• occludes femoral vein ?? venous return
• ?? workload on heart
• ??O2 sat ?? blood to vital organs

44
Congestive Heart Failure CHF

• Childrens CHF due to congenital heart defects
• CHF ?? Contractility of heart ?? CO
• ?? blood volume for systemic circulation
• ?? pulmonary congestion
• ?? O2 and ?? nutrition.
• Unable to meet metabolic demands

45
Interventions

• Parent teaching
• Review defect and s/s when to call MD
• Meds - dose, schedule, SE
• Prophylactic antibiotics
• Immunizations
• Nutrition - ? cal formula, ? Fe, ? K, ? Protein,
  ? fat, ? Na
• Activity- allow for rest periods for fatigue
• ? Cardiac demands
• Position, thermoregulation
• Cardiac Cripple
• Parents overprotect and child manipulates
• Set limits discipline WNL
• Emotional support (access to NP/RN 24
  hours)
• Encourage support groups (specific to
  defect)

46
Medications

• Digoxin
• Action -cardiac glycoside
• ? Contactility of heart ? efficacy ? CO
• Slows down SA node ? HR
• Digitalization
• Loading Dose 30-40 mcg/kg/dose (½, ¼, ¼)
• Maintenance dose 4-5 mcg/kg/day q 12
• Nursing interventions
• v Apical pulse for one full minute before giving
  med. Hold med if
• Infant lt100 Toddler lt90
• Preschool lt70 School age lt60
• Document Apical HR next to dose on MAR

47
Nursing Interventions

• v I and O and v K level
• ?? K ?? Dig toxicity
• v Serum Digoxin level (0.5-2ng/dl)
• Digoxin Toxicity (gt3ng/dl)
• vomiting (earliest sign), nausea (?? Po intake)
• lethargy and bradycardia
• Administer with 2 RNs
• Review order v HR parameters
• v Dosage and calculation
• v Actual dose in syringe a administering
• Document on MAR HR Initials _at_ dose

48
Diuretics

• Action- eliminates excess H2O and Na ? fluid
  loss
• ?edema and ?work for heart and lungs
• Furosimide-Lasix (Strong acting) 1mk/kg/dose
• Blocks reabsorption of Na H2O _at_ loop of Henle
• ??? loss of Na, K and H2O
• Thiazides-Diuril 10-20 mg/kg/dose
• Blocks reabsorption of Na H2O K distal tubules
• ??? loss of Na, K and H2O
• Aldactone (Aldosterone Inhibitor)- (K Sparing)
• Blocks action of Aldosterone
• hormone that retains Na and H2O
• Promotes H2O and Na loss Retains K

49
Nursing Interventions

• v Weight
• Same time, scale and amount of clothing None!
• v I and O
• weigh all diapers
• v skin tugor on sternum (tenting dehydrated)
• v Serum electrolytes
• K, Na, BUN and Creatine
• Administer K supplements
• KCL, Slo-K, K-Lor, K-Dur
• K level affects Digoxin efficacy!

50
Prostaglandin E1 (Prostin VR)

• Vasodilator (0.1 ug/kg/min)
• Relaxes vascular smooth muscle
• Keeps open Ductus Arteriosus (DA).
• ? Pressure in L heart ? pressure in Aorta
• Blood shunted from Aorta ? PDA ? PA
• ? Blood to lungs ? perfusion ? oxygenation
• ? O2 to systemic circulation
• Maintains mixing of oxygenated and
• deoxygenated blood in cyanotic defects.

51
Prostin VR Adverse SE

• Apnea- must intubate
• Cutaneous generalized flushing
• ?? BP ?? HR
• ?? Seizures
• ?? I O
• Hemorrhage and thrombocytopenia
• ?CBC ? PLTS ? PT/PTT

52
Indomethacin Na (Indocin)

• NSAID (0.1 -0.3 mg/kg/dose q12h
• Max 3 doses)
• Action
• Inhibits prostaglandin synthesis
• Promotes PDA closure
• Assess presence of murmur
• () murmer, give med
• (-) closed, hold med

53
Indocin Adverse SE

• ?? Renal ?? GI blood flow
• ? I O ? UA ? BUN/creatine
• ? Bowel sounds
• Guiac stools for necrotic bowel
• (NEC) Necrotizing enterocolitis
• ?? Platelet function
• ? CBC ? PLTS ? PT ? PTT

54
Kawasaki Disease

• Most common acquired heart disease in children lt8
  years of age
• Acute febrile multi-system disorder
• Autoimmune
• Skin, mucous membranes, lymph nodes
• Vasculitis ? cardiac complications
• ?? incidence near fresh H2O
• Late winter/early spring

55
Clinical signs

• Fever gt5 days
• Febrile seizures
• Cervical lymphadenopathy gt1.5 cm
• Bilateral non-exudative conjunctivitis
• Strawberry tongue
• Dry, red, cracked lips

56
Clinical signs

• Truncal rash
• Erythema edema of palms and soles
• Shedding skin
• Desquamation from fingers
• ? WBC ? ESR ?Plts
• Cardiac sequella
• Pericarditis
• Myocarditis
• Arrhythmias
• Coronary Artery Aneurysm
• If untreated 15-25 develop MI

57
Nursing Interventions

• IV Immune Globulin (IVIG)
• ?? the incidence of coronary aneurysm lt3 Single
  dose IVPB over 24 hours
• ??Dose ASA (100 mg/kg/day)
• v thrombocytosis
• Bed rest
• ? O2 Demands
• Petroleum jelly to lips
• v CHF ?HR ?RR dyspnea crackles
• Strict I O
• Tepid sponge bath
  
• Complete and spontaneous recovery in 3-4 weeks!

58
Subacute Bacterial Endocarditis (SBE)

• Infection of valves and inner lining of heart
• High risk patients congenital heart disease
• Bacteremia
• Strep Viridians- most common 70,
• Staph Aureus 20, Candida Albicans 10
• Enters blood stream via teeth, gums, tonsils,
  UTI.
• Slow insideous onset
• Attaches to congenital anomalies or prosthetic
  valve sites
• Vegetations
• Bacteria, fibrin and plt thrombi grow on
  endocardium
• Invade Aortic and Mitral valves
• ?? turbulent blood flow and break off as
  embolism
• spleen, kidney, CNS, lung and skin.

59
SBE Clinical Signs

• Fever- low grade, intermittent or unexplained
• Anorexia- malaise.
• feel like getting the flu
• Murmur
• New or change in previous murmur
• Cardiomegaly
• Splenomegaly
• Osler nodes-
• Red, painful nodules at finger tips
• Janeways spots
• Painless, hemorrhagic areas on palms and soles
• Splinter hemorrhages
• thin black lines under nails
• Petechiae on oral mucous membranes
• HA, ??motor coordination CVA!!

60
Diagnosis

• CBC with differential
• BC identifies the agent
• ?? ESR
• CXR cardiomegaly
• ? RBC anemia
• EKG prolonged PR interval
• Echocardiogram
• Vegetations

61
Therapy

• Bed rest
• High dose (Meningitic) Antibiotics
• PCN, Gentamycin, Ampicillin
• IV therapy 4-6 weeks
• ? med SEs- ?hearing ?renal status
• Serial BC
• Counsel parents regarding antibiotic
• prophylaxis a p invasive
• procedures

62
Rheumatic Fever

• Autoimmune response to
• Group A ß Hemolytic Strep
• Caused by untreated/partially treated
• group A strep pharyngitis
• Symptoms appear 2-6 weeks after infection
• Diffuse inflammatory collagen disease
• connective tissue, joints
• subcutaneous tissue
• Brain, heart and blood vessels

63
Diagnosis Jones Criteria

• Carditis
• Cardiomegaly, murmur RT Mitral regurgitation
• valvulitis (Endocardium ? Pericardium), ? HR
• Ashkoff bodies
• Hemorrhagic lesions in heart
• Polyarthritis
• Reversible and migratory
• knee ? shoulder ? elbow
• Subcutaneous nodules
• 1 cm non-tender swelling over bony prominences.
• Erythema marginatum
• Red macular wavy rash with clear center
• Chorea St. Vitus dance
• Involuntary movements of extremities and face
• ? c anxiety ? c rest

64
Diagnosis

• ?CPR (C-Reactive Protein)
• ?ESR
• Throat culture
• ? ASO titer gt333
• Anti-streptolysin reflects lysis of RBC
• ? 7 days p onset
• Max. level 4-6 weeks
• BC
• EKG prolonged P-R interval

65
Therapy

• Complete bed rest 2-6 weeks
• Gradual activity
• Medications
• Antibiotics
• PCN Prophylactic RX q Month IM
• Erythromycin for PCN allergy
• ASA -joints
• Prednisone valvular inflammation
• Nutrition
• ? protein ? carbs ? fluids

66
Iron Deficiency Anemia

• Inadequate supply of dietary iron (Fe)
• Infants
• ? risk _at_ 6 months
• Fetal Fe stores are depleted
• ? milk consumption ?? protein/solid intake
• Adolescents
• ? growth spurt
• poor nutrition
• ? blood loss c menses
• No whole milk until after 1 year.

67
Clinical signs

• Tachycardia
• Pallor
• Infants chubby and white
• Hypoxia
• Muscle weakness
• Fatigue ?Alertness
• Irritability
• HA Dizziness
• Koilonychia
• Spoon shaped fingernails
• Glossitis
• ?Hgb lt10 Hct lt30
• ? Ferritin lt7 ?Serum Fe lt30
• ?TIBC (Total Iron Binding Capacity)
  gt350

68
Therapy

• Fe supplement (2-3 mg/kg/day)
• Give between meals with acidic fluids.
• Takes at least 4 months to replace loss
• SE Stains teeth, black tarry stools
• Dextran (parental iron)
• Z-track deep IM - buttocks only
• Nutrition
• Green leafy vegetables, whole wheat,
• beans, shellfish, egg yolk, Organ meats,

69
Hemoglobinopathies

• Sickle Cell Disease
• Defective HgB chain (HgS)
• RBCs are sickle shaped
• Unable to carry O2
• RBCs have a shorter life span 16-30 days

70
Sickle cell

• Autosomal recessive
• AA WNL
• AS trait (carrier)
• SS Disease
• Both parents have trait
• 25 AA normal
• 25 SS disease
• 50 AS (carriers)
• Only 35-45 HgB is sickled
• Majority Asymptomatic
• ? Risk in African Americans 15-40

71
Pathophysiology

• Vaso-occulusion
• Sickle Shaped cells stack up
• Lodge in small vessels
• ? blood flow
• Tissue Hypoxia
• ?Viscosity of blood ?blood flow
• ? O2 ? metabolic end products
• Tissue Ischemia
• Edema necrosis _at_ site
• Infarction
• Brain, Kidneys Liver

72
Clinical signs

• First sign
• Fetal hemoglobin (HgB) is depleted
• HgS hemoglobin is now dominant
• Low Hgb 5-9 Hct 15-30
• Pallor
• Jaundice
• ? RBCs destroyed RT ?life span
• Frequent URIs
• Generalized weakness
• Hepatosplenomegaly

73
Sickle Cell Crisis Sickling

• Acutely ill RT ?O2 and Dehydration
• ? Stress or ?infection (URI GI GU)
• ? Temp - Dehydration
• ? BMR ? O2 consumption
• leads to tissue hypoxia

74
Clinical Signs

• Abdominal pain-
• Thrombosis to liver and spleen
• Severe bone pain RT sickled joints
• Hematuria and diuresis RT renal ischemia
• Seizures RT Brain thrombosis/CVA
• Acute Chest Syndrome
• Severe chest pain
• SOB, ? HR ? RR
• Pulmonary congestion

75
Therapy

• Bone Marrow Transplant ?Prognosis
• O2 humidified
• Hydration
• ? PO intake 2.5 -3 L/day ? I O
• Pain control
• PCA , MSO4, Fentenyl
• ASA
• No Demerol ( Metabolite ?? seizures)
• Folic acid
• ?RBC
• PRBC Transfusions weekly (Hgb lt10)
• Splenectomy (kids lt5 years)
• Prevents Splenic Sequestration
• Massive entrapment of sickled cells in
  spleen
• HYPOVOLEMIC SHOCK!!!

76
Parent Teaching

• Life long-frequent hospitalizations
• Life Span determined by sickled RBCs
• Genetic Counseling
• Have all children tested
• Monitor fluid losses
• ?diapers ?mucous membranes ?Skin turgor
• ? Infection-Immunize on schedule
• meningococcal, pneumococcal and hep B
• Prophylactic PCN by 2 months of age
• NO day care/malls ?Exposure to other kids
• ? Coping techniques Stress Reduction

?
77
Hemophilia A Classic Hemophilia

• Lacks Factor VIII (AHF)
• AHF Anti hemolytic Factor
• Severe spontaneous bleeding
• Not trauma induced
• Sex linked recessive-X chromosome
• Mom transfers diseases to boys
• Girls are carriers

78
Clinical signs

• 1st indication at circumcision
• Crawling ? bruises on pressure areas
• Hemarthrosis
• Bleeding into joint cavities (synovial space)
• Early sign stiffness, tingling or achy
• Warmth, redness, swelling
• ? ROM function
• Alkylosis of joint
• Spontaneous bleeding
• Epistaxis, loose baby teeth,
• Hematuria
• Spinal Cord Hematoma paralysis
• Intracranial Hemorrhage Death

79
Therapy

• Recombinant Factor VIII (IV)
• Purified, reconstitute a use
• DDAVP (1-deamino-8 D Arginine Vasopressin)
• Synthetic form of vasopressin
• Control bleeding RICE
• Apply pressure x 15 mins (NO Peaking!)
• Splint immobilize area x 24 hours
• Pain meds
• Tylenol
• Corticosteroids
• Opiods
• Exercise
• PT to strengthen joint muscles

80
Patient Teaching

• Genetic testing
• All female members
• Injury/Bleeding prevention
• Soft rugs, soft toothbrush, electric razor
• Review S/S Internal Bleeding
• ? hematuria ? black tarry stools
• Cerebral HA, slurred speech, LOC
• Venipuncture
• Kids gt8 years can self administer
• ? Independence and accountability
• Community Education
• Medical Alert Tag
• Notify all organizations, friends
• Quiet activities, non-contact sports
• National Hemophilia Foundation