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28 Cards in this Set

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CRISIS CAUSED BY ANEMIA
MAY REQUIRE BLOOD TRANSFUSION
-PREVENTATIVE MEASURE DRUGS:
**HYDROXYUREA (HYDREA)-LOWERS HOSPITALIZATION BY 50 PERCENT
**ERYTHROPOIETIN
CRISIS CAUSED BY DEHYDRATION
100CC/KG/DAY OF FLUIDS-CHILD
3-5 LITERS/DAY-ADULT
CRISIS CAUSED BY INFECTION
1.PNEUMOCOCCUS INFECTION (PNEUMONIA)
2.OSTEOMYLITIS(INFECTION OF BONE)
3.CALL MD EVEN FOR LOW GRADE FEVER
4.KEEP PT AWAY FROM SICK PPL
5.CHILD-GETS PROPHYLACTIC PENICILLIN
FROM 4MOS-4/5 YRS OLD (GETS VACCINATION FROM PNEUMOCCOCAL)
SICKLE CELLL CRISIS CAN BE PRECIPITATED BY:
1.INFECTION
2.DEHYDRATION
3.CHILLING COLD
4.HYPOXIA(EMOTIONAL DISTRESS,HIGH ALTITUDES)
5.ANEMIA
GOALS
1.PREVENT SICKLING PHENOMENON
2.TREAT CRISIS/PAIN
3.PROMOTE ADEQUATE O2 AND HYDRATION
4.AIDE FAMILY IN ADJUSTING
NSG DIAGNOSIS FOR SICKLE CELL
1.PAIN
2.ACTIVITY INTOLERANCE
3.KNOWLEDGE DEFICIT
4.HIGH RISK FOR INFECTION
SICKLE CELL PREP AND SICKLED EX
**NOT RELIABLE**
NYS MANDATORY NEWBORN SCREENING
HEMOGLOBIN ELECTROPHORESIS
CAN DISTINGUISH BETWEEN TRAIT AND DISEASE
ASSESSMENT
AFTER 4 MOS-FETAL HgB PROTECTS
HISTORY-FAMILY HISTORY
1.HAND/FOOT SYNDROME**SEEN IN BABY**
2.JOINT SWELLING 3.PAIN 4.PALLOR/JAUNDICE 5.FATIGUE (Hgb<11)
5.FEVER(CAN BE CAUSE OR RESULT)
6.LOW NORMAL GROWTH-HYPERACTIVE BONE MARROW,SHORT TRUNK,SWINDLE LEGS
7.CHECK SPLEEN
SICKLE CELL CRISIS
"ATTACKS" PRECIPITATED BY ANY CONDITION THAT CAUSES HYPOXIA +/OR INCREASED VISCOSITY OF THE BLOOD +/OR ACIDOSIS
-EPISODES USUALLY SELF LIMITING(RECOVERS ON OWN)
-DAMAGE UNREPAIRABLE
-LASTS DAYS-WKS
-BETWEEN CRISIS FCN-"NORMALLY"
SICKLE CELLS MORE FRAGILE
LIFE SPAN 30 DAYS
-INCREASES DESTRUCTION SUCH AS ANEMIA
SICKLE CELLS RIGID
UNABLE TO FLOW EASILY IN SMALL BLOOD VESSELS (MICROCIRCULATION)
-LEADS TO:LOG JAMS,STOPS BLD FLOW
BLOCKAGE-->ISCHEMIA(MORE SICKLING)->INFARCTION->PAIN->SWELLING->FEVER(INCREASES O2 DEMAND BY 50 PERCENT)->SICKLE CELL CRISIS(ALL ORGAN AFFECTED)
SICKLE CELLS CARRY AS MUCH O2 AS
AS A NORMAL RBC
WHEN SICKLE CELLS CHANGE SHAPE, MEDICAL PROBLEMS OCCUR:
1.SICKLE CELLS RIGID
2.SICKLE CELLS MORE FRAGILE
CHANGE INTO SICKLE CELL SHAPE IS PRECIPITATED BY:
1.UNDER LOW O2
2.LOW PH-ACIDOSIS
3.INCREASED BLOOD VISCOSITY-MAY ERUPT FROM DEHYDRATION
SICKLE CELL DISEASE
Hgb S
NORMAL=
Hgb A
2 TRAITS=
25 PERCENT CHANCE OF CHILD GETTING DISEASE
TRAIT=
CARRIER=ASYPMTOMATIC
SICKLE CELL ANEMIA
TYPE OF HEMOLYTIC ANEMIA
-RBC'S HAVE SHORTENED LIFE SPAN
-INHERITED D/O OF ABNORMAL Hgb
-1/12 AFRO AMER. HAVE SICKLE CELL TRAIT
-1/500 AFRO AMER. ACTUALLY HAVE THE DISEASE
TREATMENT FOR SICKLE CELL CRISIS
-BED REST
-HYRDRATION (W/ELECTROLYTE REPLACEMENT)
-ANALGESICS
-ANTIBIOTICS
-BLD REPLACEMENT
-O2
1.AVOID OVERPROTECTING
2.INCREASE SELF WORTH
3.BALANCED PHYSICAL ACITIVITY
BONE MARROW TRANSPLANT HAS A __ SUCCESS RATE
80-90 PERCENT
RISK TO BABY
FETAL DEATH BEFORE AND AFTER SICKLE CELL CRISIS
-DEATH DECREASED BY GOOD FETAL CARE AS YRS GO ON
BLOOD REPLACEMENT/ERYTHROCYTOPHORESIS
ONLY IF BLOOD < 11
CHECK FOR IRON OVERLOAD
O2 IS ONLY GIVEN FOR SICKLE CELL CRISIS IF
ONLY IF PT HAS HYPOXEMIA
OTHER NSG MEASURES
1.NUTRITION
2.CBC Q 6 HRS
3.ROUTINE EYE EXAM
4.COMPREHENSIVE SERVICES
5.GENETIC COUNSELING
PREGNANCY AND MARROW TRANSPLANT
HIGH RISK
-CHRONI VILLI SAMPLING
-INCREASED RISK OF INFECTION
WHEN IS GENE THERAPY DONE
BEFORE CHILD BORN