321 - Exam 3 P. 7-13 (Sickle Cell, Anemia)

Front 1

Blood Transfusion
-Whole blood (erythrocytes and plasma) rarely given, why?

Back 1

b/c this adds to volume and they often don’t need this.

Front 2

Blood Transfusion
-IV size?

-Fluid given with?

-What kind of tubing?

Back 2

•IV: size (18-20 g needle, you want a larger needle because the erythrocytes are big and if they have to go through a small IV, you will elongate the erythrocyte and break it)



-NS


•use IV tubing specifically for blood. it usually has a filter in it. Use Y-blood tubing (Hang NS on one side and blood on the other)

Front 3

Blood Transfusion

-What do you make sure to do before giving this

Back 3

Double check blood – have to send down a slip to the blood bank

Front 4

Blood Transfusion
-when should you give this blood?
(time frame)

Back 4

Give ASAP b/c blood at room temperature breaks down
-Give within 20-30 minutes of receiving the blood

Front 5

Blood Transfusion
-Stay with the patient for how long? Why?

Back 5

Stay with patient during first 15 minutes or 50 ml b/c pt can go into an anaphylactic reaction first

Front 6

Blood Transfusion

-Infusion at ____ ml/min during first 15 minutes

Back 6

2mL/min

Front 7

Blood Transfusion

How long should you infuse the blood over?

Back 7

Infuse over 2 – 4 hours

Front 8

Blood Transfusion

When would you use the blood warmer?

Back 8

for rapid infusion (for sickle cell disease)

Front 9

Transfusion Reaction – what is the first thing you do?

Back 9

stop the transfusion, flush with NS in NEW tubing,

Front 10

Blood Transfusion
-what do you do with the unit of blood once it has infused?

Back 10

send back to blood bank ALONG WITH the tubing

Front 11

Blood Transfusion
-People can get a cocktail to prevent reactions, what 2 drugs are usually given?

Back 11

(Benadryl and Tylenol)

Front 12

Blood Transfusion Reaction
-who is at the highest risk?

Back 12

-people who receive multiple reactions are the highest at risk

Front 13

Blood Transfusion Reaction

Acute hemolytic
-what is it caused by?

Back 13

-caused by blood type or Rh incompatibility .

-When blood containing antigens different from the pts own antigens are infused, antigen antibody complexes are formed in his or her blood. These complexes destroy the transfused cells and start inflammatory responses.

Front 14

Blood Transfusion Reaction


Acute hemolytic
-onset:

Back 14

within minutes to 24 hours of infusion

Front 15

Blood Transfusion Reaction


Acute hemolytic
-clinical manifestations?

Back 15

fever or chills
flank pain
reddish or brown urine (because of rhabdomylosis-muscle breaking down, it is not blood)

Front 16

Blood Transfusion Reaction


Acute hemolytic
-clinical manifestations within the cardiovascular system?

Back 16

tachycardia & hypotension leading to shock, cardiac arrest, and death

Front 17

Blood Transfusion Reaction


Acute hemolytic is a life threatening complement mediated immune response causing: (4)

Back 17

simultaneous intravascular coagulation and hemolysis

DIC

renal faillure

Shock

Front 18

Blood Transfusion Reaction


Acute hemolytic
-Nursing interventions:

Back 18

-stop transfusion immediately
-hydrate with NS as ordered

Front 19

Blood Transfusion Reaction


Acute hemolytic
-Nursing interventions: what are we checking and supporting?

Back 19

-check VS frequently
-support airway, BP, HR, and UO

Front 20

Blood Transfusion Reaction


Acute hemolytic
-Nursing interventions:
Be aware that the patient usually requires ______ management

Back 20

ICU

Front 21

Blood Transfusion Reaction

What is the most common type of reaction?

Back 21

Febrile nonhemolytic reaction

Front 22

Blood Transfusion Reaction

Febrile nonhemolytic reaction
-caused by?

Back 22

-recipient antibodies react with donor WBC antigens
OR
-from cytokine accumulating during blood storage

Front 23

Blood Transfusion Reaction

Febrile nonhemolytic reaction
-Clinical manifestations:
HR?
BP?
RR?

Back 23

tachycardia
hypotension
tachypnea

Front 24

Blood Transfusion Reaction

Febrile nonhemolytic reaction
-Does it cause hemolysis

Back 24

hemolysis = breakdown of RBCs

NO

Front 25

Blood Transfusion Reaction

Febrile nonhemolytic reaction
-Clinical manifestation
What happens to their temperature?

Back 25

they will have a fever
-increase in temperature of at least 1 degree celsius above baseline

Front 26

Blood Transfusion Reaction

Febrile nonhemolytic reaction
-5 other clinical manifestations

Back 26

-Chills
-Flushing
-Nausea
-Headache
-Vague discomfort

Front 27

Blood Transfusion Reaction

Febrile nonhemolytic reaction
-Nursing intervention:

Back 27

-stop transfusion immediately
-help rule out other causes of fever
-manage symptoms as appropriate

Front 28

Blood Transfusion Reaction

Febrile nonhemolytic reaction
-How do you prevent this from happening?

Back 28

premedicate with antipyretic and antihistamine
-give leukocyte-reduce or irradiated products
-benadryl and tylenol prior to gettign the blood

Front 29

Blood Transfusion Reaction

Febrile nonhemolytic reaction
-Prevention: what would you screen for?

Back 29

HLA antibodies in pts with repeated febrile reactions

Front 30

Blood Transfusion Reaction

Febrile nonhemolytic reaction
-Onset:

Back 30

within minutes to hours of transfusion

Front 31

Blood Transfusion Reaction

Mild allergic reaction
-why does this occur:

Back 31

when transfused allergens in plasma activate recipient's IgE antibodies

Front 32

Blood Transfusion Reaction

Mild allergic reaction
-marked by release of:

Back 32

histamine by recipients mast cells and basophiles

Front 33

Blood Transfusion Reaction

Mild allergic reaction
-Onset:

Back 33

immediately or within 24 hrs after transfusion

Front 34

Blood Transfusion Reaction

Mild allergic reaction
-Clinical manifestations (4)

Back 34

Uticaria (hives - red elevated patches) with or without itching

edema
bronchospasms
flushing

Front 35

Blood Transfusion Reaction

Mild allergic reaction
-Nursing interevention:

Back 35

stop transfusion immediately
-give antihistamine, steroids, or both
-if ordered, resume transfusion when symptoms subside

Front 36

Blood Transfusion Reaction

Mild allergic reaction
-Prevention:

Back 36

premedicate with antihistamine (benadryl)

Front 37

Blood Transfusion Reaction
-what can result if a blood product is infused too quickly?

Back 37

Circulatory overload. What are CM??

hypertension, bounding pulse, distended jugular veins, dyspnea, restlessness, confusion

Front 38

Blood Transfusion Reaction


Anaphylactic reaction:
-onset:

Back 38

immediately or within 24 hours after transfusion

Front 39

Blood Transfusion Reaction


Anaphylactic reaction:
-what causes this to occur:

Back 39

in pts with IgA deficiency who have anti-IgA antibodies, causing a severe to life threatening immune response

Front 40

Blood Transfusion Reaction


Anaphylactic reaction: (4)
-cardiovascular clinical manifestations

Back 40

hypotension
no fever
flushing
chest tightness

Front 41

Blood Transfusion Reaction


Anaphylactic reaction: (4)
-respiratory CM

Back 41

stridor
bronchospasm
dyspnea
decreased oxygen saturation

Front 42

Blood Transfusion Reaction


Anaphylactic reaction:
-GI clinical manifestations

Back 42

cramps

Front 43

Blood Transfusion Reaction

Anaphylactic reaction:
-Nursing interventions

Back 43

-stop transfusion immediately
-call a code
-obtain VS often
-support airway, BP, and HR

Front 44

Blood Transfusion Reaction

Anaphylactic reaction:
-Nursing interventions
If ordered, give what 2 drugs

Back 44

steroids and epinephrine

Front 45

Blood Transfusion Reaction

Anaphylactic reaction:
-Prevention:

Back 45

ensure special preparation of blood products, such as washed cells and products from IgA deficient donors

Front 46

Blood Transfusion Reaction

Anaphylactic reaction:
-What do you do with the IV?

Back 46

-keep vein open with NS

Front 47

Transfusion Reaction - Action
-After you stop the transfusion, what do you do with the tubing?

Back 47

•Change tubing and run IVF – NaCl

Front 48

Transfusion Reaction - Action

-Notify which 2 pepole?
-Recheck what?
-Monitor what 2 things?

Back 48

•Notify blood bank and MD
•Recheck identifying tags and numbers
•Monitor VS and UO

Front 49

Transfusion Reaction - Action

-Fill out what?
-Collect what 2 things?

Back 49

• Transfusion reaction report
•Collect blood and urine specimens per policy
•Document

Front 50

Transfusion Reaction - Action
-what do you do with the blood bag and tubing?

Back 50

Save blood bag and tubing and send to blood bank

Front 51

Vitamin C - what is it important for?

-4 examples of foods that it is high in?

Back 51

(conversion of folic acid to its active forms and aids on iron absorption)
–Citrus fruits
–Leafy green vegetables
–Strawberries
–cantaloupe

Front 52

Hemolytic Anemia
-what is it caused by

Back 52

destruction of RBC

Front 53

Hemolytic Anemia
-Destruction of RBCs can occur intrinsically or extrinsically
-what does intrinsic mean?

Back 53

there are defects IN the RBC
--where you have someone who has a deficiency in the body that causes an abnormal erythrocyte (ex: sickle cell) something is fundamentally wrong with the erythrocyte

Front 54

Hemolytic Anemia
-Destruction of RBCs can occur intrinsically or extrinsically
-what does extrinsic mean?

Back 54

somthing outside the RBC is causing the destruction
-more common
-ex: cells trapped in spleen/liver, antibody mediated destruction, toxins, heart valves

Front 55

Sickle Cell Disease
-Etiology?

Back 55

Inherited autosomal recessive disorder
-if 1 parent has the trait (carrier) and the other one is normal, you have a 50% risk of offspring having normal, 50% will have the trait
-if both parents have the trait, 25% will have the disease

Front 56

Sickle Cell Disease
-people that have sickle cell disease need to have genetic counseling, why?

Back 56

to see if they have any history in their family so they can make decisions about having children

Front 57

Sickle Cell Disease
-Is a result of abnormal Hemaglobin. What happens to the hemoglobin?

Back 57

-Hemoglobin A (good kind) is partly or completely replaced by abnormal sickle hemoglobin S.

Front 58

Sickle Cell Disease
-Hemoglobin S is sensitive to what?

Back 58

- changes in the oxygen content of the RBC.

Front 59

Sickle Cell Disease
-Insufficient oxygen causes the Hemoglobin S cell to do what?

Back 59

-become rigid and clumped together, obstructing capillary blood flow.

Front 60

-The CM of sickle cell disease primarily occur as a result of (2)

Back 60

obstruction caused by sickled red blood cells and increased RBC destruction

Front 61

What ethnicity has the highest rate of sickle cell disease?

Back 61

African Americans (1 in 350)

Front 62

Sickle Cell Disease
-the RBC becomes sickled because of the abnormal hemoglobin (S), it doesn’t allow the erythrocyte when there is low amounts of oxygen to keep its concave shape.
-Once the RBC is sickled, what does it do to oxygen/circulation

Back 62

is thin walled and doesn’t carry oxygen very well. it occludes the small vessels and circulation can be impaired

Front 63

Diagnosis of sickle cell disease

-Peripheral blood smear
-Electrophoresis of Hgb identifies and Hgb
-DNA testing

What is the Sicking test?

Back 63

The RBC is exposed to a deoxygenation agent (they remove the oxygen and see what happens to the cells)

Front 64

CM of sickle cell disease
-Bilirubin level?
-What is it caused by?

Back 64

increased or normal bilirubin

(bilirubin is released from damaged and dead
RBCs)

Front 65

CM of sickle cell disease

-Hemoglobin & Hematocrit (increased or decreased)

Back 65

decreased H/H because the life span of the RBC is so much shorter than normal and because many cells are destroyed / sickled cell has a life of 21 days . the spleen sees it as being defective and destroys it

Front 66

CM of sickle cell disease

-Reticulocytes (increased or decreased)

Back 66

increased (over 1.5) reticulocytes
(indicating anemia of long duration – it is high because the bone marrow is releasing immature RBC (reticulocytes) to make up for the low hematocrit and hypoxia

Front 67

CM of sickle cell disease
-what does the X ray show relating to bones and joints?

Back 67

bone and joint deformities and flattening
– the joints can become abnormally shaped and the small vessels are occluded, thus decreasing o2 to joints

-bone marrow is working overtime to produce more erythrocytes and the shape can be deformed

Front 68

CM of sickle cell disease

-WBC (increased or decreased)

Back 68

high WBC because of inflammation caused by tissue hypoxia and ischemia

Front 69

CM of sickle cell disease
-Skin/mucous membranes?

Back 69

Pallor – (examine the lips, tongue, nail beds, conjunctivae, palms, and soles of feet) because of poor oxygenation from decreased perfusion
•Skin grayish cast in black people

Jaundice because of RBC destruction

Front 70

CM of sickle cell disease

-tell me about their energy level?

Back 70

•Fatigue
•Decreased exercise tolerance

Front 71

CM of sickle cell disease

-Why does pain occur?

Back 71

-as a result from tissue injury caused by poor oxygenation from obstructed blood flow

Front 72

CM of sickle cell disease

-Cardiovascular system: (3)

Back 72

-Murmurs or S3 sound
-Tachycardia
-BP is low to average

Front 73

CM of sickle cell disease

-tell me about what happens with the kidneys?

Back 73

the kidneys are less effective at filtration and reabsorption. The urine contains protein and the pt may be unable to concentrate urine. eventually, the kidneys fail, resulting in little or no UO and the buildup of toxic waste products in the blood leads to death.

Front 74

CM of sickle cell disease
• the foundation of the problems is

Back 74

occlusion of the small vessels

Front 75

CM of sickle cell disease

-what can happen in the brain:

Back 75

thrombosis or hemorrhage, causing paralysis, sensory deficits or death

Front 76

CM of sickle cell disease

-what can happen in the lung?

Back 76

acute chest syndrome (chest pain) , pulmonary hypertension, pneumonia

Front 77

CM of sickle cell disease

-what can occur in the eye?

Back 77

hemorrhage, retinal detachment, blindness, retinopathy (new vessels will form as a result of compensation, and that causes an increased in pressure leading to edema and that causes the eye problems)

Front 78

CM of sickle cell disease

-what happens to the heart?

Back 78

congestive heart failure (occlusion of the vessels, ischemia, but the heart also has to pump harder to pump the blood through these occlusions)

Front 79

CM of sickle cell disease

-what happens to the spleen?

Back 79

splenic atrophy and splenomegaly (spleen is working overtime because it sees these erythrocytes and destroys them because they are defective. at some point a large congested spleen will fail.)

Front 80

CM of sickle cell disease

-what happens to the kidney?

what is seen in the urine?

Back 80

renal failure (occlusion of small vessels)


hematuria & proteinuria

Front 81

CM of sickle cell disease

-what happens to the bones and joints?

Back 81

-hand-food syndrome (pain from ischemia),

-osteonecrosis (lack of O2 – destroys tissue in joints)

Front 82

CM of sickle cell disease

What happens to the liver & gallbladder:

Back 82

hepatomegaly
gallstones occurs as an increase in bilirubin (enlarged liver because of organ congestion and fluid can accumulate in the liver)

Front 83

CM of sickle cell disease

what happens to the penis :

Back 83

priaprism (erection that lasts for a long time. the penile vein becomes engorged and the blood can not flow out of the vein) this is a medical emergency, they can insert a needle in the vein and remove the blood.

Front 84

CM of sickle cell disease

Skin:

Back 84

stasis ulcers of hands, ankles, and feet

Front 85

Sickle Cell Disease

Pain Crisis means what:
-when do these crises first occur?

Back 85

ACUTE CM

6 months of age

Front 86

Sickle Cell Disease / Management of Pain Crisis
-Prevent pain crisis from occurring, how?

Back 86

-avoid ischemia/injury,
-avoid cold weather (wear gloves),
-avoid stress,
-avoid anything that can cause vasoconstriction.

Front 87

Sickle Cell Disease / Management of Pain Crisis

-What supplements do you give?

Back 87

Give folic acid supplements because it promotes the production of erythrocytes

Front 88

Sickle Cell Disease / Management of Pain Crisis

Pain:

Back 88

- Oxygen
-fluids (PO or IV)
-analgesic
-rest

Front 89

Sickle Cell Disease / Management of Pain Crisis

Pain: heat or cold?

Back 89

heat

Front 90

Sickle Cell Disease / Management of Pain Crisis

-How does hydration help

Back 90

-hydration is needed because dehydration increases cell sickling and must be avoided

Front 91

Sickle Cell Disease / Management of Pain Crisis

-Why do you give oxygen?

Back 91

-oxygen is given during crises because lack of O2 is the main cause of sickling

Front 92

Sickle Cell Disease / Management of Pain Crisis

If you have to give a transfusion, what must you remember?

-Purpose of transfusions?

Back 92

Transfusions – never administer cold blood

-Transfusion can be helpful to increase HbA levels and dilute HbS levels

Front 93

Sickle Cell Disease / Management of Pain Crisis

Blood transfusion
-what line should we prevent using?

Back 93

- usually central lines are not used because of risk for infection, easy for clots to become occluded,

Front 94

Droxia (hydroxurea) is the drug used for what?

Back 94

Sickle Cell Disease
-this drug stops the production of the abnormal erythrocyte (HbG S)
-the younger you start on this, the better it works

Front 95

Droxia (hydroxurea)
-why is this used?
-How does it work?

Back 95

used to reduce the # of sickling and pain episodes in SCD.
-Hydroxyurea works by stimulating fetal hemoglobin (HbF) production. HbF is present during fetal development, but its production is turned off before birth. Increasing levels of HbF reduces sickling of RBCs in patients with sickle cell disease

Front 96

Droxia (hydroxurea)
-side effects: (2)

-intervention:

Back 96

•increased incidence of leukemia
•it also suppresses bone marrow function

SCD
-regular follow ups & monitor CBCs for drug toxicity is important

Front 97

Droxia (hydroxurea)

-SE specifically for sexually active women
-interevention:

Back 97

SCD
-it also causes birth defects.
- use at least 2 methods of birth control while taking this drug

Front 98

Sickle Cell Disease management:

-what 2 surgeries may they have done?

Back 98

1) BMT (bone marrow transplant) 25% of the people who have a transplant die

2) Spleen can be removed (the spleen removes the erythrocytes)

Front 99

Sickle Cell Disease
-Sickled cells go back to their normal shape when the low oxygen condition is removed and proper tissue perfusion resumes (true or false)

Back 99

True

-administer oxygen and blood transfusions as directed to increase tissue perfusion
--maintain adequate hydration and blood flow through oral and IV fluids

Front 100

Sickle Cell Disease
-What are 4 situations that precipitate sickling which could cause the patient to end up in a sickle cell crisis (acute exacerbation)?

Back 100

1) fever
2) dehydration
3) emotional or physical stress
4) any condition that increases the need for oxygen or alters the transport of oxygen

Front 101

Sickle Cell Disease

-The crisis has a sudden onset, and occur as a result of __________

Back 101

hypoxemia

Front 102

Sickle Cell Disease
-give analgesics how often?

-avoid giving what specific medication for pain? why?

Back 102

-administer analgesics as prescribed (around the clock)

-administration of meperidine (Demoerol) for pain is avoided because of the risk of induced seizures

Front 103

Sickle Cell Disease / Crisis management
-positioning?

Back 103

-keep the extremities extended to promote venous return
-elevate HOB no more than 30 degrees

Front 104

Sickle Cell Disease
-Diet: encourage consumption of what?

Back 104

a high calorie, high protein diet, with folic acid supplementation

Front 105

Sickle Cell Disease Crisis Management


-monitor for signs of complications including: 3

Back 105

1) increased anemia,
2) decreased perfusion,
3) shock (mental status changes, pallor, VS changes)

Front 106

Sickle Cell Disease
-Children are at risk for growth and development problems. What can you teach about preventing susceptibility to infection?

Back 106

-ensure that the child receives the pneumococcal and mengiococccal vaccines and an annual flu vaccine

Front 107

Sickle Cell Disease
-what IV fluid do you use?

Back 107

Hypotonic solution such as: D5W or dextrose 5% in 0.45% sodium chloride are infused at 250mL/hr for 4 hours.

Front 108

Sickle Cell Disease
-The pts cell are in a hypotonic or hypertonic enviornment?

Back 108

-Hypertonic: fluid is being pulled out of the cell (cell is shrinking) as a result of dehydration.

Front 109

Sickle Cell Disease
-Using Hypotonic IV fluid will help bring the patients blood osmolarity back down to the normal range. How?

Back 109

Because fluid will enter a cell and cause it to swell.
The inside of the cell has higher osmotic pressure than the surrounding fluid, so fluid is drawn into the cell.