Sickle Cell

Question 1

What is a genetic disorder that’s most common in African American’s where people inherit an abnormal/defective hgb molecule known as HB S trait in their RBC’s from BOTH parents?

Answer 1

Sickle Cell Disease

Question 2

What if people only inherit the hgb S trait from ONE parent?

Answer 2

Have sickle cell trait

Question 3

What is a normal hemoglobin?

Answer 3

HB A

Question 4

The shape of a normal HB A is circular, what’s the shape of HB S?

Answer 4

Sickled shape

Question 5

What is a chronic hemolytic anemia and is the most common type of sickle cell disease?

Answer 5

Sickle Cell Anemia

Question 6

In sickle cell disease the person will produce normal ____, but when _____ attaches to the RBC the cell _____ during a time of _____.

Answer 6

RBC’s, Hgb, sickles, stress

Question 7

How long is the lifespan of a normal RBC? What about a sickled RBC?

Answer 7

120days; 20days

Question 8

RBC’s get destroyed faster than normal RBC’s if they are sickled, so when they break apart bilirubin is sent out which can’t get rid of fast enough so can cause what?

Answer 8

Jaundice

Question 9

How many grams of hgb per mL of blood does a normal RBC have? A sickled RBC?

Answer 9

12-14g; 6-9g

Question 10

What can be done to put more normal RBC’s in the body of someone w/ sickle cell disease?

Answer 10

Blood transfusion

Question 11

The membrane of a normal RBC is smooth and round, what’s the shape of HB S that cause it to get stuck and occludes the blood vessel?

Answer 11

Hard and rigid

Question 12

What causes the RBC to sickle?

Answer 12

Episodes of hypoxemia causing HB S to deoxygenate

Question 13

Dehydration, fever, physical/emotional stress, increase/decrease in environmental temps, high altitudes, poorly pressurized airplanes, and smoking are all what?

Answer 13

Causes of hypoxemia

Question 14

If a pt. wasn’t getting good O2 causing their RBC’s to sickle can their RBC’s revert back to normal shape after they start getting good O2?

Answer 14

Yes

Question 15

What is painful periods resulting from ischemia where RBC’s get stuck everywhere and are not reverted?

Answer 15

Sickle cell crisis (Vaso-occlusive crisis)

Question 16

What can occur from a sickle cell crisis?

Answer 16

Thrombosis or infarction

Question 17

Dehydration, cold, acidosis, localized hypoxemia, and severe ischemia in limb are all what?

Answer 17

Precipitations of sickle cell crisis

Question 18

How does oxygenation relate to sickle cell disease?

Answer 18

Decrease RBC’s decreases amnt of O2, bc RBC sickles and blocks blood vessel

Question 19

How does perfusion relate to sickle cell disease?

Answer 19

Blocks perfusion to major organs/body bc shape of RBC blocks in blood vessels

Question 20

How does pain relate to sickle cell disease?

Answer 20

The ischemia from lack of O2 cause excruciating pain when the RBC’s get stuck

Question 21

How does infection relate to sickle cell disease?

Answer 21

Inadequate tissue perfusion causes tissues to break down and necrosis can occur

Question 22

RBC are most responsible for what in the body?

Answer 22

Carrying O2 to all part of the body

Question 23

Pallor, fatigue, jaundice and irritability are all what?

Answer 23

S/S of hemolytic anemia from sickling of RBC’s

Question 24

What is tissue death (necrosis) caused by a local lack of O2 from an obstruction of the tissue’s blood supply like hypoxemia?

Answer 24

Infarction

Question 25

How long does sickle cell crisis usually occur?

Answer 25

4-6days

Question 26

Priapism (painful, prolonged erection), abd pain, skin ulcers, possible swelling, gallstones, enlarged spleen/liver are all what?

Answer 26

S/S of sickle cell disease

Question 27

What is a symptom complex that includes fever, chest pain, increase in WBC's and pulmonary infiltration may develop?

Answer 27

Acute chest syndrome

Question 28

Pneumonia, pulmonary infarction/embolism and even death are all what?

Answer 28

Complications of acute chest syndrome

Question 29

Children affected w/ sickle cell disease are often asymptomatic until when?

Answer 29

4-6mon

Question 30

A child w/ sickle cell disease may experience?

Answer 30

Chest tightness, SOB, stroke, splenic infarction and/or hematuria

Question 31

What is a medical crisis, especially in children?

Answer 31

Acute chest syndome

Question 32

What is the typical treatment for sickle cell disease?

Answer 32

O2, hydration and analgesics like morphine

Question 33

What treatment decreases the production of abnormal blood cells and leads to a lesser amnt of pain, increases fetal hgb production and red cell mean corpuscular volume?

Answer 33

Hydroxyurea (Cytotoxic med)

Question 34

What are some adverse effects of hydroxyurea?

Answer 34

Bone marrow suppression, HA, N/V, and dizziness

Question 35

If a child is in b/t the ages of 2mon-5yrs what does their treatment include?

Answer 35

Daily admin of prophylactic penicillin

Question 36

What's a complication of multiple transfusions?

Answer 36

Alloimmunization

Question 37

What is it called when the child's immune system reacts against antigens on the donated tissues like blood and stem cells?

Answer 37

Alloimmunization

Question 38

Blood transfusions can prevent what?

Answer 38

Ischemia and strokes

Question 39

Impaired gas exchange/physical mobility, risk for ineffective breathing pattern/decreased cardiac tissue perfusion/imbalanced fluid volume/ineffective cerebral tissue perfusion/infection, acute pain, caregiver role strain, interrupted fam process and delayed growth and development are all what?

Answer 39

Diagnosis

Question 40

Nursing care for what includes: pulse ox, vitals, checking/auscultating respirations, admin O2, low stimuli room, check H/H and iron

Answer 40

Oxygenation

Question 41

Nursing care for what includes: being up-to-date on vaccines, hygiene, antibiotic treatment if needed, assessing skin integrity, turning q2h, wound care, C/S, contact precautions, hand washing and maintaining fluids/nutrition?

Answer 41

Infection

Question 42

Nursing care for what includes: admin analgesics (morphine/toradol), monitor during bc of risk of increase RR so could go into crisis, have narcan available, admin O2, imagery, pain scale q4h, turn q2h, assess vitals?

Answer 42

Pain

Question 43

Nursing care for what includes: assess for edema, admin blood transfusion, check all especially pedal pulses, monitor labs for kidney/liver function, reposition q2h, PCB's, IVF's and physical assessment- pallor/cap refill/color/temp?

Answer 43

Perfusion