Sickle Cell Anemia
The priority problem with sickle cell crises is?
a. pain
b. reduced life span of RBCs
c. it is a genetic disorder
d. perfusion and oxygenation of tissues
What is perfusion and oxygenation of tissues.
All are problems/characteristics of SCD, but perfusion/oxygenation of tissues is the priority to prevent cell damage/death.
The nurse has taught a client with SCD how to prevent crises. The nurse knows the client needs further education after which statement?
a. I will drink at least 3-4L of fluids each day
b. I will avoid alcohol and caffeine
c. I will avoid extremely cold and hot environments
d. When I fly, I don't have to worry if the cab is pressurized or not.
d - When I fly, I don't have to worry if the cab is pressurized or not.
Client's with SCD should be taught to:
1. drink at least 3-4L of fluids each day
2. avoid alcohol and caffeine to prevent dehydration
3. avoid extremely cold and hot environments
*Wear socks and gloves on cool days
4. Contact their HCP at first s/s infection
5. Get flu shot (and pneumonia) as recommended
6. Avoid unpressurized airplane cabins
7. Avoid places of high altitude - Ex: Denver
8. Ensure all HCPs knows you have SCD
9. Consider genetic counseling
10. Avoid strenuous activity (low impact 3 x's each week when not in crisis should be recommended)
Vitamin B12 deficiency anemia leads to macrocytic (large cell) anemia. Causes of Vitamin B12 deficiency include which of the following?
a. Vegan Diet
b.Diets high in dairy productsc. chronic constipation
d. Poor iron intake
A - vegan diet
Causes of B12 deficiency include:
vegan diet
lack of dairy products in diet
small bowel resection
chronic diarrhea
diverticula
tapeworm
overgrowth of bacteria in intestines
pernicious anemia - when the body lacks "intrinsic factor" to absorb the B12 from the intestines
A client with Vitamin B12 deficiency has been educated on foods to increase dietary intake. The nurse knows further education is required after the client states they will increase the intake of which food?
a. fish
b. dairy
c. nuts
d. alcohol
Correct - alcohol; this will decrease B12 levels
Foods to increase B12 include:
animal proteins
fish
eggsnuts
dairy products
dried beans
citrus fruits
green leafy veggies
Patients with polycythemia vera are at increased risk of which of the following?
a. iron deficiency
b. fluid volume overload
c. clotting
d. infection
answer - c clotting
Polycythemia vera is a cancer where there are increased RBCs.
RBC > 6
Hgb > 18 in men; >16.5 in women
Hct > 55%
Increased WBC and platelets are also typically present (opposite of aplastic anemia/pancytopenia)
Large amounts amounts of RBCs causes hemoconcentration which leads to increased risk of clotting. (monitor for DVT, PE, MI, stroke, peripheral occlusion, etc)What race/ethnicity is at greatest risk of developing SCD?
a. Caucasian
b. Native American
c. African American
d. Asian American
What is African American.
Pregnancy in women with SCD can be life threatening. What type of contraception would be contraindicated for this client?
a. combination hormone therapy
b. condoms
c. cervical cap or diaphragm
d. progesterone only hormone therapy
a. combination hormone therapy
This therapy increases clotting risk - which greatly increases the pt's risk of developing crisis; If this is taken, the pt should not smoke (as smoking increases clotting risk even further)
Causes of folic acid deficiency anemia include which of the following?
a. malabsorption
b. diet high in dark, green, leafy vegetables
c. Chronic constipation
d. lack of alcohol intake
A - malabsorption
Folic acid deficiency is most commonly caused by poor nutrition. Specifically, poor intake of:dark green leafy veggies, liver, yeast, citrus fruits, and dried beans and nuts
The second most common cause is malabsoprtion (Ex: Chron's disease)
Anticonvulsants and oral contraceptives may be a factor as well
A client has pernicious anemia. The nurse knows which of the following should be completed to increase their B12 levels?
a. Increase foods such as diary products and animal meats
b. Take an iron supplement
c. Administer an IM or subcutaneous injection of B12
d. Increase dark green leafy veggies
Correct answer - injection of B12
Remember, pernicious anemia results b/c the GI tract can't absorb the B12 correctly. So, increasing dietary amount isn't helpful.
Increasing iron will not increase B12 levels.
A client with polycythemia vera has been taught ways to promote health and prolong life expectancy. (life expectancy is just 2 years if not treated/maintained.) Which would indicate the client has understood the teaching? Select all that apply.
a. Limit fluid consumption to 1 L each day
b. Avoid tight/restrictive clothing (especially around the extremities and waste)
c. Wear gloves outdoors when temp < 50F
d. Avoid anticoagulants and antiplatelets
e. Elevate your feet when sitting
f. Stop activity and seek medical care if chest pain develops
g. Use an electric razor and soft bristled tooth brush while on anticoagulants
h. Stop smoking
i. It is ok to miss some of your phlebotomy appointments for apheresis since it is several times per week
B, C, E, F, G, H
The client should consume at least 3L of fluid each day to prevent concentration and subsequent clotting.
Anticoagulants and/or antiplatelets maybe prescribed to prevent clotting of thick blood
Apheresis will filter the blood outside the body - removing RBCs but putting the plasma back into the pt (increases life expectancy approx 10-15 years)
Potential causes of a sickle cell crisis include which of the following? (Select all that apply)
a. Poor fluid intake
b. low altitudes
c. excessive intake of caffeinated beverages
d. Extreme, prolonged exercise
e. hot climate/temperature
f. Trauma
A, C, D, E, F
Anything that can result in dehydration and/or hypoxia can cause sickle cell crisis. Trauma is also a causative factor.
What lab findings would a nurse expect to find for a client with anemia? Select all that apply.
a. WBC 14.2
b. RBC 7.2
c. Hct 29%
d. Hgb 8.5
e. platelets 500,000
C, D
With anemia, you can expect a low RBC, Hgb, & Hct
Normal values:
RBC 4.7-6.1
Hgb 12-16
Hct 36-50%
Also... Remember, anemia is the clinical indicator of another problem. It isn't the actual problem...
Which of the following lab values would you expect for a client with pancytopenia?
a. RBC 5.2
b. WBC 14.4
c. platelet 80,000
d. INR 0.4
Answer - C. low platelets
Pancytopenia includes low red (anemia), white (leukopenia), and platelet (thrombocytopenia) cells in the blood.
While aplastic anemia may occur alone, it typically ocurrs with the others pancytopenia.Typical causes include:
long term exposure to toxins, drugs (see Table 39-3), radiation, or infection (usually viral)
How is sever folic acid deficiency anemia treated?
a. Increase in green, leafy veggies
b. Decrease in nut products
c. Folic acid injection
d. increase in alcohol intake
Answer - folic acid injection
Severe folic acid deficiency can be treated with an injection of folic acid (IM, subcut, or IV)
Mild to moderate folic acid deficiency can be treated with increased intake through diet (green leafy veggies, nuts, citrus fruits, and dried beans) and/or supplement (ex: prenatal vitamin or just folic acid alone)
Excessive Alcohol intake should be avoided as it leads to malabsorption
A nurse is caring for client with hemochromatosis in a longterm care faciltiy that has an order for phlebotomy to remove 500mL of blood twice a week until ferritin levels reach normal levels. What is the most appropriate action by the nurse at this time?
a. Clarify the order with the HCP.
b. Continue to monitor the client.
Correct answer - B. continue to monitor the client
Removing 500mL of blood twice weekly will help to remove excess amounts of iron. Once normal levels are reached, removal may only take place once every 2-4 months.
This prevents iron from building up in the tissues and organs - which would eventually lead to organ failure.
Pts who can't tolerate the blood loss (ex: anemic too) can do chelation therapy. The drug is injected or ingested and binds to the iron and is exreted in the urine and stool. (Can cause severe drop of neutrophils and damage DNA, so why not used as first line treatment)
A client with sickle cell crisis may experience clumping of cells in arteries, especially those along joints. What S/S would the nurse note if this happened?
a. improvement of pain in these areas
b. distal pulses 2+
c. cool/pale skin distal to site
d. cap refill < 3 seconds distal to site
Cool/pale skin distal to site - Clumping of the sickle cells leads to decreased perfusion distal to the blockage site. S/S include cool/pale skin, cap refill > 3 seconds, distal pulses 1+ or less, etc. Pain would increase if the cells were clumping.
Keep in mind, depending on where the blockage occurs, different s/s may result. (Ex: to brain - stroke; heart - MI; lung - PE; kidney - decreased UO and AKI;
Note... Acute Chest Syndrome can occur (when multiple arteries of lungs blocked off - like multiple PEs) - one of leading causes of death in SDCIron deficiency anemia is caused by which of the following?
a. autosomal recessive disorder
b. x linked genetic disorder
c. Diet lacking fortified foods, dark green veggies, and red meat
d. exposure to radiation
Iron deficiency anemia results from blood loss, poor GI absorption of iron, and inadequate diet (lacking iron fortified foods, dark green veggies, and red meats).
Low iron levels prevents production of RBCs or creates small RBCs (microcytic).
autosomal recessive disorder creates SCD
x linked causes enzyme G6PD deficiency anemia
Hemolytic anemia occurs by which of the following?
a. lack of iron
b.excess amounts of B12
c. autoimmune reaction
d. lack of oxygen
Correct - C
hemolytic anemia occurs when RBCs are destroyed by the body's own antibodies (IgG or IgM reaction)
Aplastic anemia is typically treated with blood transfusions (dependent on cause) until the bone marrow begins working again. What type of fluid should be run with the PRBCs during infusion to prevent clumping/clotting of the blood?
a. D5W
a. LR
c. D51/2NS
d. NS
D - Normal saline (NS) (0.9% NaCl)
Other solutions may cause clotting/clumping or destruction of the blood product.
Other treatments for aplastic anemia (dependent on cause include:
immunosuppresants (teaching for infection prevention)
Hematopoietic stem cell transplant with donor cells (if others unsuccessful)
***Hydration is important during hemolysis to prevent excess debris and Hgb from causing an AKI. Mannitol can help keep blood dilute and the kidneys flushing it out.
Transfusion of PRBCs may be required.
A client with severe anemia requires a blood transfusion. The nurse will complete which of the following during the administration process. Select all that apply.
a. verify a patient IV (at least 20 guage) is available
b. Check vital signs before, at the beginning of administration, periodically during administration, and after administration of the blood
c. Verify the patient, blood compatibility/numbers, and blood expiration date with an LPN
d. Utilize filtered blood tubing Y'd in with normal saline to administer the blood
e. Remain with the patient for the first 5 minutes of the infusion
f. Infuse the blood at the prescribed rate while ensure it is completed within 4 hours of being removed from the blood bank
g. Monitor for S/S fluid volume overload
h. Adminster diphenhydramine but continue the infusion if the patient develops difficulty breathing and hives
A, B, D, F, G
A double check of the patient, blood compatibilty, and expiration date should take place with two RNs according to national standards.
The RN should remain with the client for the first 15 minutes of the infusion
If the patient displays any signs of hemolytic transfusion reaction (S/S back pain apprehension, chest pain, tachypnea, hypotension, fever, DIC, etc d/t antibody formation destruction of infused cells and systemic inflammation), the transfusion should be immediately stopped. Call rapid reponses if appropraite to floor. Apply O2. A flush or infusion of NS should be started to keep the line open. Benadryl maybe given. Monitor for S/S shock and treat as indicated.
Allergic reaction can also occur (IgE reaction). (S/S hives/urticaria, bronchospasm, etc). Pt's with Hx can have washed PRBCs (removal of WBCs, plasma, and immunoglobulin A).
Average volume of 1 unit PRBC is 250-350mL
Can be infused over 2 hours if no cardiac or FVO issues
A client is admitted to the hospital for sickle cell crisis and severe pain. Which of the following interventions would be appropriate for this client? Select all that apply.
a. Administer ibuprofen for pain prn
b. Administer hypotonic solutions as ordered
c. Place cold compresses on the joints
d. Obtain blood cultures if the patient develops a fever or other signs of infection
e. remove restrictive clothing
f. Place the client in high fowler's position
g. Administer oxygen therapy
h. Monitor for jaundicing
i. Administer fresh frozen plamsa
B, D, E, G, H
When severe pain is occurring, opiods like morphine and dilaudid should be given. Also, use a timed/scheduled dose - not just as needed to ensure better pain control.
Hypotonic solutions will help rehydrate the patient and their cells sinc ethey are typically dehydrated during a crisis.
Warm compresses can be used to vasodilate vessles to prevent/relieve clumping of cells/improve blood flow/improve pain. Cold should be avoided as it will cause vasoconstriction.
The patient's spleen and other organs may be damaged over time - making them more susceptible to infection and sepsis. SCC pt's should have a sepsis workup if fever develops. Measures should also be taken to prevent infection - Ex: avoid crowds or sick people; anyone with an upper resp infection should wear a mask around the pt; wash, wash, wash your hands, etc. Monitor for s/s infection - lung sounds, cloudy urine, thrush, etc
Remove restrictive clothing to aid perfusion
Hip, knee, and elbow flexion should be avoided as a means to prevent obstruction of blood flow
Oxygen therapy is needed as hypoxia and dehydration are typically the causes of crisis
Sickled cells have a shorter life span and also can destroy other RBCs. RBC destruction leads to release of bilirubin (increased amts could cause jaundicin) and iron
FFP would lead to further clotting and decrease in perfusion. The nurse may need to administer PRBCs to dilute the sickled cells and aid in perfusion to prevent stroke/MI/etc
S/S of anemia include which of the following? Select all that apply.
a. Pallor of ears, nail bed, conjuctivae, and around mouth
b. strong nails
c. Tachycardia
d. Dyspnea on exertion
e. Low O2 levels
f. increased energy levels
A, C, D, E
S/S include:
Pallor
cool to touch
intolerance of cold
brittle nails
tachycardia at baseline; increased with exertion
orthostatic hypotension
dyspnea on exertion
decreased O2 levels
fatigue
headache
The first line treatment for mild iron deficiency anemia is which of the following?
a. IV infusion of iron
b. increasing iron intake in foods consumed
c. iron supplements
d. liquid oral iron
B - increasing dietary intake of iron will be the first step for iron deficiency anemia. (red meat, organ meat, egg yolk, green leafy veggies, raisins, iron fortified cereals/bread/pasted)
If this is not sufficient, oral supplements will be added. (Can take with food if GI upset occurs).
Liquid iron may also be prescribed (remember to drink through a straw). Educate can cause tarry looking stools.
If deficiency is severe, IV iron can be given. Some brands now carry black box warning due to increased risk of anaphylaxis during administration.
A patient has mild hemolytic anemia. What therapy does the nurse expect to administer to this client?
a. immunosuppressants
b. chemotherapy
c. splenectomy
d. steroids
Answer - steroids may be used for a mild hemolytic therapy
If steroids fail, more aggressive treatments like immunosuppressants, splenectomy, and chemo maybe used.
Plasmapheresis with antibody removal may also be used if above is not successful. (recurrent)
A client with Type A+ blood requires PRBC infusion. Which of the following blood types can the patient receive? Select all that apply.
a. A+
b. B+
c. AB+
d. O+
e. A-
f. B-
g. AB-
h. O-
A, D, E, H
For the letters, each blood type can only have their "letter" plus O.
For - or +, remember... A positive person can tolerate a negative person; but a negative person can't stand to be around a positive person.
If this had been A- (instead of A+), the pt could have only had A- or O-
Remember, O- is a universal donor. So, the ER will have O- available for trauma pts. This way, we don't have to wait for a type and cross before administration.