Nu 246 (Exam 2)-Sickle Cell Anemia

Front 1

CRISIS CAUSED BY ANEMIA

Back 1

MAY REQUIRE BLOOD TRANSFUSION
-PREVENTATIVE MEASURE DRUGS:
**HYDROXYUREA (HYDREA)-LOWERS HOSPITALIZATION BY 50 PERCENT
**ERYTHROPOIETIN

Front 2

CRISIS CAUSED BY DEHYDRATION

Back 2

100CC/KG/DAY OF FLUIDS-CHILD
3-5 LITERS/DAY-ADULT

Front 3

CRISIS CAUSED BY INFECTION

Back 3

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)

Front 4

SICKLE CELLL CRISIS CAN BE PRECIPITATED BY:

Back 4

1.INFECTION
2.DEHYDRATION
3.CHILLING COLD
4.HYPOXIA(EMOTIONAL DISTRESS,HIGH ALTITUDES)
5.ANEMIA

Front 5

GOALS

Back 5

1.PREVENT SICKLING PHENOMENON
2.TREAT CRISIS/PAIN
3.PROMOTE ADEQUATE O2 AND HYDRATION
4.AIDE FAMILY IN ADJUSTING

Front 6

NSG DIAGNOSIS FOR SICKLE CELL

Back 6

1.PAIN
2.ACTIVITY INTOLERANCE
3.KNOWLEDGE DEFICIT
4.HIGH RISK FOR INFECTION

Front 7

SICKLE CELL PREP AND SICKLED EX

Back 7

**NOT RELIABLE**
NYS MANDATORY NEWBORN SCREENING

Front 8

HEMOGLOBIN ELECTROPHORESIS

Back 8

CAN DISTINGUISH BETWEEN TRAIT AND DISEASE

Front 9

ASSESSMENT

Back 9

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

Front 10

SICKLE CELL CRISIS

Back 10

"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"

Front 11

SICKLE CELLS MORE FRAGILE

Back 11

LIFE SPAN 30 DAYS
-INCREASES DESTRUCTION SUCH AS ANEMIA

Front 12

SICKLE CELLS RIGID

Back 12

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)

Front 13

SICKLE CELLS CARRY AS MUCH O2 AS

Back 13

AS A NORMAL RBC

Front 14

WHEN SICKLE CELLS CHANGE SHAPE, MEDICAL PROBLEMS OCCUR:

Back 14

1.SICKLE CELLS RIGID
2.SICKLE CELLS MORE FRAGILE

Front 15

CHANGE INTO SICKLE CELL SHAPE IS PRECIPITATED BY:

Back 15

1.UNDER LOW O2
2.LOW PH-ACIDOSIS
3.INCREASED BLOOD VISCOSITY-MAY ERUPT FROM DEHYDRATION

Front 16

SICKLE CELL DISEASE

Back 16

Hgb S

Front 17

NORMAL=

Back 17

Hgb A

Front 18

2 TRAITS=

Back 18

25 PERCENT CHANCE OF CHILD GETTING DISEASE

Front 19

TRAIT=

Back 19

CARRIER=ASYPMTOMATIC

Front 20

SICKLE CELL ANEMIA

Back 20

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

Front 21

TREATMENT FOR SICKLE CELL CRISIS

Back 21

-BED REST
-HYRDRATION (W/ELECTROLYTE REPLACEMENT)
-ANALGESICS
-ANTIBIOTICS
-BLD REPLACEMENT
-O2
1.AVOID OVERPROTECTING
2.INCREASE SELF WORTH
3.BALANCED PHYSICAL ACITIVITY

Front 22

BONE MARROW TRANSPLANT HAS A __ SUCCESS RATE

Back 22

80-90 PERCENT

Front 23

RISK TO BABY

Back 23

FETAL DEATH BEFORE AND AFTER SICKLE CELL CRISIS
-DEATH DECREASED BY GOOD FETAL CARE AS YRS GO ON

Front 24

BLOOD REPLACEMENT/ERYTHROCYTOPHORESIS

Back 24

ONLY IF BLOOD < 11
CHECK FOR IRON OVERLOAD

Front 25

O2 IS ONLY GIVEN FOR SICKLE CELL CRISIS IF

Back 25

ONLY IF PT HAS HYPOXEMIA

Front 26

OTHER NSG MEASURES

Back 26

1.NUTRITION
2.CBC Q 6 HRS
3.ROUTINE EYE EXAM
4.COMPREHENSIVE SERVICES
5.GENETIC COUNSELING

Front 27

PREGNANCY AND MARROW TRANSPLANT

Back 27

HIGH RISK
-CHRONI VILLI SAMPLING
-INCREASED RISK OF INFECTION

Front 28

WHEN IS GENE THERAPY DONE

Back 28

BEFORE CHILD BORN