Sickle Cell Flashcards

1
Q

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?

A

Sickle Cell Disease

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2
Q

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

A

Have sickle cell trait

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3
Q

What is a normal hemoglobin?

A

HB A

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4
Q

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

A

Sickled shape

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5
Q

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

A

Sickle Cell Anemia

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6
Q

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

A

RBC’s, Hgb, sickles, stress

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7
Q

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

A

120days; 20days

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8
Q

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?

A

Jaundice

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9
Q

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

A

12-14g; 6-9g

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10
Q

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

A

Blood transfusion

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11
Q

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?

A

Hard and rigid

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12
Q

What causes the RBC to sickle?

A

Episodes of hypoxemia causing HB S to deoxygenate

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13
Q

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

A

Causes of hypoxemia

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14
Q

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?

A

Yes

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15
Q

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

A

Sickle cell crisis (Vaso-occlusive crisis)

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16
Q

What can occur from a sickle cell crisis?

A

Thrombosis or infarction

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17
Q

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

A

Precipitations of sickle cell crisis

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18
Q

How does oxygenation relate to sickle cell disease?

A

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

19
Q

How does perfusion relate to sickle cell disease?

A

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

20
Q

How does pain relate to sickle cell disease?

A

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

21
Q

How does infection relate to sickle cell disease?

A

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

22
Q

RBC are most responsible for what in the body?

A

Carrying O2 to all part of the body

23
Q

Pallor, fatigue, jaundice and irritability are all what?

A

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

24
Q

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

A

Infarction

25
Q

How long does sickle cell crisis usually occur?

A

4-6days

26
Q

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

A

S/S of sickle cell disease

27
Q

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

A

Acute chest syndrome

28
Q

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

A

Complications of acute chest syndrome

29
Q

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

A

4-6mon

30
Q

A child w/ sickle cell disease may experience?

A

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

31
Q

What is a medical crisis, especially in children?

A

Acute chest syndome

32
Q

What is the typical treatment for sickle cell disease?

A

O2, hydration and analgesics like morphine

33
Q

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?

A

Hydroxyurea (Cytotoxic med)

34
Q

What are some adverse effects of hydroxyurea?

A

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

35
Q

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

A

Daily admin of prophylactic penicillin

36
Q

What’s a complication of multiple transfusions?

A

Alloimmunization

37
Q

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

A

Alloimmunization

38
Q

Blood transfusions can prevent what?

A

Ischemia and strokes

39
Q

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?

A

Diagnosis

40
Q

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

A

Oxygenation

41
Q

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?

A

Infection

42
Q

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?

A

Pain

43
Q

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?

A

Perfusion