Sickle Cell Worksheet

Question 1

Hemolytic Anemia~

Answer 1

destruction of blood cells#

Question 2

Hemoglobin S~

Answer 2

abnormal hemoglobin
caused by mutation in beta-globin gene

causes RBC to distort into a rigid sickle shape#

Question 3

Autosomal recessive~

Answer 3

non-sex linked gene that requires two copies to express phenotype#

Question 4

Sickle Cell Disease~

Answer 4

several diseases that result from abnormal hemoglobin genes#

Question 5

Sickle Cell Trait~

Answer 5

When a person inherits an abnormal hemoglobin gene and a regular one resulting in some percentage of their hemoglobin affected but generally doesn’t cause observable disease#

Question 6

Reticulocyte~

Answer 6

New RBC that hasn’t matured yet
makes up 0.5% to 2.5%
High count indicates loss of RBCs
Low count indicates vitamin B or marrow deficiencies#

Question 7

Systolic Murmur~

Answer 7

Woosh between s1 and S2
indicates:
HTN
Defects
valve disorders

Can happen in SCD due to obstructed blood flow#

Question 8

What can lead to decreased O2 and SC crisis~

Answer 8

Viral illness
Bacterial illness
Exercise
High altitude
Smoking
Drugs#

Question 9

Bone Marrow Transplant procedure~

Answer 9

1. autologous or allogenic
2. chemo or radiation to kill bad cells
3. donor stem cells collected via needle or peripheral blood
4. infuse healthy cells - travel to bone marrow and replicate - grows healthy cells
5. Hospital stay for monitoring#

Question 10

Pathophysiology of SCD~

Answer 10

Abnormal hemoglobin is triggered
Sickling causes sticking together
Occlusion leads to clots
decreased O2
Jaundice from hemolysis
and pain#

Question 11

Pathophysiology of Autosomal recessive SCD~

Answer 11

If only one gene for Hemoglobin S is passed on, a child will have SCTrait

If two people with SCT conceive, 25% will have no HgbS, 50% will have one HgbS (and therefore SCT), and 25% will have SCDisease#

Question 12

Why fluid status matters~

Answer 12

occlusive illness is exacerbated by dehydration and more viscous blood.
Adequate fluids are essential to keep blood flowing and increasing perfusion#

Question 13

Cardiac assessments~

Answer 13

Rhythms
dysrhythmias
pressure fluctuation (occlusion) HTN, HOTN, deficits between areas
pulses#

Question 14

Why jaundice~

Answer 14

HgbS is short lived, and crisis increases hemolysis

the bilirubin builds up faster than liver can filter#

Question 15

Face bones are~

Answer 15

Englarged from perpetual creation of more RBCs to replace those lost from SCD#

Question 16

Priority nursing interventions~

Answer 16

Monitor labs
Monitor vitals
Meds
Pain assessments
Neuro assessments
I&O
Fluid balance (PO/IV)
O2 PRN
Vascular assessments#

Question 17

Client Education topics~

Answer 17

Diet: decrease foods that contribute to poor health outcomes
avoid vitamin deficiencies
Avoid drugs, smoking, booze
Avoid temperature extremes
Avoid getting sick (crowds and sick people)
Vaccinations up to date
Sleeeeeeep
Hydrate
Regular exams#

Question 18

Normal Hgb~

Answer 18

12-17.5#

Question 19

Normal Hct~

Answer 19

36-54#

Question 20

Normal RBC~

Answer 20

4.2-6.1 million#

Question 21

Normal MCV~

Answer 21

80-100#

Question 22

Normal RDW~

Answer 22

11.5%-14.5%#

Question 23

Normal Retic Count~

Answer 23

0.5%-2.5%#

Question 24

Normal WBC~

Answer 24

4k-11k#

Question 25

Normal Bilirubin~

Answer 25

1-12 mg/dL#

Question 26

Normal Iron~

Answer 26

60-150#

Question 27

Normal BUN~

Answer 27

6-24#

Question 28

Normal Creatinine~

Answer 28

0.6-1.3#

Question 29

Hydroxyurea~

Answer 29

Inhibit DNA synthesis, causing bad RBCs to die Stimulates Y-globin genes, producing Fetal hemoglobin (HbF) SE Bleeding Pain (back or pee) Sore throat SOB Hallucinations Seizures Extremity swelling#

Question 30

Opioids~

Answer 30

Activate analgesic pathways SE Decr RR Fatigue Dependence Constip#

Question 31

Ketamine~

Answer 31

NMDA receptor agonist dissociative anesthetic SE hallucinations SOB BP changes Liver dmg N/V/chest pain Loss of reality#

Question 32

Ferrous Sulfate~

Answer 32

Bind globin chains form hemoglobin SE constip N/V/N Dark stool Stained teeth GI discomfort#

Question 33

Oxygen~

Answer 33

Increase cellular availability of O2 SE HA Dizzy Epistaxis/dry Fatigue SOB#

Question 33

Epogen~

Answer 33

Promote erythroid progenitors in bone (RBCs) SE N/D HA Rash Arthralgia HTN Clots#

Question 33

Folic Acid~

Answer 33

Folate helps with protein synthesis and erythropoiesis SE Bad taste Irritability N CFN Anorexia#

Question 33

Pentoxifylline~

Answer 33

Inhibit enzymes and cytokines = reduced blood viscosity; increased microcirculatory perfusion incr blood flow and reduce pain SE N/V/D discomfort Dizzy chest pain erythema face/neck#

Question 34

Corticosteroids~

Answer 34

Inhibit inflammatory pathway Immunosuppressive SE GI upset Hyperglycemia Weight gain Osteoporosis Adrenal suppression Sodium/fluid retention#

Question 34

Broad Spectrum Antibiotics (Cephalosporins)~

Answer 34

Inhibit cell wall synthesis in bacteria SE Diarrhea HOTN Fever Rash GI pain Bleeding Sore throat Superinfection