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Flashcards in 53. Sickle Cell Disease Deck (24):
1

A patient using hydroxyurea complains that her stools are "loose and watery." Choose the correct statements. (Select ALLthat apply.)

A. Hydroxyurea can cause loose stools, or diarrhea.
B. Hydroxyurea can cause constipation.
C. Hydroxyurea has no effect on stools in most patients.
D. Patients on hydroxyurea usually require loperamide, taken daily, up to 8 tablets/day.
E. Hydroxyurea causes the stool to appear lighter, or clay-colored.

A, B. This is one drug that really can cause constipation in some patients, and diarrhea or "loose stools" in others; counsel on the possibility of either occurring.

2

Select the correct counseling sequence for a patient receiving Exjade:

A. Open the capsule. Sprinkle into a small amount (spoonful) of applesauce or fruit juice. Swallow without chewing.
B. If the capsule cannot be swallowed whole, sprinkle into a small amount (4 ounces) of water and drink. Follow with another glass of water in the same cup to get any drug that has stuck to the cup.
C. Exjade tablets are taken whole, or ground and mixed with a small amount of applesauce or pudding.
D. Drop into apple juice, orange juice, or water. Stir until the mixture is even. Drink all of it, including washing down any that is stuck to the cup.
E. Exjade comes only as a suspension that is mixed with fruit juice or water; do not mix with dairy products.
 

D. Exjade counseling has 3 primary steps: Drop-Stir-Drink. It is unusual in the sense that it comes as tablets which must (must!) be dissolved in in liquid.

3

Children with sickle cell disease have been cured with the following treatment modality:

A. Spleen and pancreas transplant
B. Remicade infusions
C. Cyclophosphamide and possibly other anti-inflammatory/chemotherapeutic agents
D. Bone marrow transplant
E. Kidney transplant

D. The chemotherapy required before bone marrow transplant is generally too toxic for adults with SCD.

4

Latanya is a nine-year old African American who is hospitalized with acute sickle cell crisis. She complains of pain in the chest, lower back and legs. She has been hospitalized previously with acute pain crises. Known allergies include severe rash to amoxicillin. Her pain is rated as “20”  on a scale of 1-10.  At the last hospitalization six months ago she received adequate pain treatment with intravenous morphine at a dose of 5 mg every four hours. Calculate the daily dose of morphine that will likely be required to provide adequate pain control for this child.

A. 5 mg
B. 20 mg
C. 24 mg
D. 30 mg
E. 34 mg

D.

5

Folic acid supplementation is generally required with the following conditions: (Select ALL that apply.)

A. Alcoholics at risk for Wernicke's encephalopathy
B. Sickle cell disease
C. Females of child-bearing age who do not regularly consume folic acid fortified foods (or in any with questionable intake)
D. Scurvy
E. Macrocytic anemia caused by vitamin B9 deficiency

B, C, E. Vitamin B9 is folic acid. A deficiency of folic acid is one of the causes of macrocytic (large-cell) anemia. Thiamine (vitamin B1) causes Wernicke's; alcoholics require vitamin B1 supplementation to avoid this condition. Scurvy is due to a vitamin C deficiency.

6

Chief Complaint:  “ My daughter is having a sickle cell crisis"
History of Present Illness:  TG is a 16 y/o black female who presents to the Emergency Department with her mother.  She is in severe pain and the history is obtained from her mother.  TG was diagnosed with sickle cell disease at age 12 and initially did well with only 1-2 crises per year that were relatively easy to manage at home.  The past 12 months have been more difficult with 5 hospital admissions for severe pain crises and more time off from high school. All of TG’s childhood immunizations are up to date and TG has had no pregnancies or surgeries.
Allergies:  sulfa
Past Medical History: Sickle cell disease since age 12 (last crisis 2 months ago - admitted for 48 hours) 
Medications: Norco 5/325 1-2 tabs Q4-6H PRN, OTC folic acid (unknown strength) 
                
Physical Exam / Vitals: 
Height: 5’8”  Weight: 119 pounds    
BP: 115/75 mmHg  HR: 84 BPM  RR: 13 BPM Temp: 98.4°F  Pain: 10/10 
General: Thin young female in apparent distress, crying 
Lungs: CTA 
CV: RRR 
GI: Mild abdominal pain, mild 
Musculoskeletal: Hip and back pain

Labs:
Na (mEq/L) = 140 (135 – 145)              

WBC (cells/mm3) = 5.2 (4 – 11 x 10^3)       
K (mEq/L) = 4.8 (3.5 – 5)                      

Hgb (g/dL) = 9.8 (13.5 – 18 male, 12 – 16 female)
Cl (mEq/L) = 102 (95 – 103)                

Hct (%) = 26.8 (38 – 50 male, 36 – 46 female)
HCO3 (mEq/L) = 24 (24 – 30)              

Plt (cells/mm3) = 215 (150 – 450 x 10^3)
BUN (mg/dL) = 10 (7 – 20)                  

AST (IU/L) = 15 (10 – 40)
SCr (mg/dL) = 0.8 (0.6 – 1.3)              

ALT (IU/L) = 18 (10 – 40)
Glucose (mg/dL) = 88 (100 – 125)      

Albumin (g/dL) = 4.6 (3.5 – 5)
Ca (mg/dL) = 9.8 (8.5 – 10.5)                
Mg (mEq/L) = 1.7 (1.3 – 2.1)                 
PO4 (mg/dL) = 4.1 (2.3 – 4.7)                
                                                               
Plan:  IV pain management.  Order RBC indices, B12 and folate.  
Question: 
Prior to discharge the case manager speaks to TG about pregnancy and contraception.  Which of the following points should be emphasized?

A. Oral contraceptive pills with higher estrogen content are preferred for women with SCD.
B. TG should not worry about contraception until she is older.
C. Pregnancies in women with SCD are the same as those of other women.
D. Higher risk of miscarriage and premature birth are associated with pregnancies in SCD.
E. Children born to women with SCD have increased incidence of diabetes.

D.

7

What is the primary cause of iron overload in patients with sickle cell disease?

A. Increased gut absorption
B. Chronic transfusions of healthy red blood cells
C. Chronic use of erythropoietin
D. Chronic use of hydroxyurea
E. Chronic use of beta lactam antibiotics

B. Iron overload can damage the liver, heart and other organs.

8

Which of the following statements concerning deferoxamine is correct?

A. The brand name is Exjade.
B. It is the primary antidote for iron overdose.
C. It is administered as oral tablets.
D. It improves hearing.
E. It has low risk of hypersensitivity reactions.

B.

9

Which of the following are benefits with using deferasirox versus the older deferoxamine therapy for iron chelation? (SelectALL that apply.)

A. Deferasirox is an oral medication and deferoxamine is an injection.
B. Deferasirox is easier to tolerate than deferoxamine.
C. Deferasirox can be used with severe renal impairment while deferoxamine cannot.
D. Deferasirox has less interactions with other medications in the gut (GI tract) than deferoxamine.
E. Deferasirox is less time consuming to administer.

A, B, E. Only deferasirox has gut interactions (with magnesium and calcium) since deferoxamine is an injection. Both are not used with severe renal impairment.

10

Which of the following goal hemoglobin levels is correct for treatment of anemia associated with sickle cell disease?

A. No higher than 8 g/dL
B. No higher than 9 g/dL
C. No higher than 10 g/dL
D. No higher than 11 g/dL
E. No higher than 12 g/dL

C.

11

Chief Complaint:  “ My daughter is having a sickle cell crisis"
History of Present Illness:  TG is a 16 y/o black female who presents to the Emergency Department with her mother.  She is in severe pain and the history is obtained from her mother.  TG was diagnosed with sickle cell disease at age 12 and initially did well with only 1-2 crises per year that were relatively easy to manage at home.  The past 12 months have been more difficult with 5 hospital admissions for severe pain crises and more time off from high school. All of TG’s childhood immunizations are up to date and TG has had no pregnancies or surgeries.
Allergies:  sulfa
Past Medical History: Sickle cell disease since age 12 (last crisis 2 months ago - admitted for 48 hours) 
Medications: Norco 5/325 1-2 tabs Q4-6H PRN, OTC folic acid (unknown strength) 
                
Physical Exam / Vitals: 
Height: 5’8”  Weight: 119 pounds    
BP: 115/75 mmHg  HR: 84 BPM  RR: 13 BPM Temp: 98.4°F  Pain: 10/10 
General: Thin young female in apparent distress, crying 
Lungs: CTA 
CV: RRR 
GI: Mild abdominal pain, mild 
Musculoskeletal: Hip and back pain

Labs:
Na (mEq/L) = 140 (135 – 145)              

WBC (cells/mm3) = 5.2 (4 – 11 x 10^3)       
K (mEq/L) = 4.8 (3.5 – 5)                      

Hgb (g/dL) = 9.8 (13.5 – 18 male, 12 – 16 female)
Cl (mEq/L) = 102 (95 – 103)                

Hct (%) = 26.8 (38 – 50 male, 36 – 46 female)
HCO3 (mEq/L) = 24 (24 – 30)              

Plt (cells/mm3) = 215 (150 – 450 x 10^3)
BUN (mg/dL) = 10 (7 – 20)                  

AST (IU/L) = 15 (10 – 40)
SCr (mg/dL) = 0.8 (0.6 – 1.3)              

ALT (IU/L) = 18 (10 – 40)
Glucose (mg/dL) = 88 (100 – 125)      

Albumin (g/dL) = 4.6 (3.5 – 5)
Ca (mg/dL) = 9.8 (8.5 – 10.5)                
Mg (mEq/L) = 1.7 (1.3 – 2.1)                 
PO4 (mg/dL) = 4.1 (2.3 – 4.7)                
                                                               
Plan:  IV pain management.  Order RBC indices, B12 and folate.  
Question: 
The following order is written:  “Transfuse 2 units of packed red blood cells and recheck hemoglobin after the second unit.  If hemoglobin is < 11 g/dL after second unit, transfuse an additional unit”. Which of the following statements is correct about this order and transfusion in SCD?    

A. The order should be processed as written.
B. The order should be modified to state “If hemoglobin is < 12 g/dL, after second unit, transfuse an additional unit”.
C. The goal of transfusion in SCD is to normalize the hemoglobin to the laboratory reference range.
D. Transfusions are not used for SCD patients with acute episodes of ACS and stroke.
E. Blood transfusion is not recommended in uncomplicated vaso-occlusive crisis.

E. TG is having an uncomplicated pain crisis. Her hemoglobin is very close to the goal hemoglobin for patients with SCD after a transfusion. So transfusing her would expose her to more risk than benefit.

12

Which group of products cannot be taken concurrently with Exjade? 

A. Maalox, milk, and Oscal
B. Milk, orange juice, and yogurt
C. Oscal, milk, and applesauce
D. Gaviscon, yogurt, and hot beverages
E. Fruit juice, yogurt, and coffee

A. Exjade cannot be taken with calcium, magnesium, or aluminum containing products. It will chelate with the cations and needs to be available to chelate with iron.

13

A pharmacist has a prescription for deferasirox. What is the correct brand name for this iron chelation agent?

A. Exjade
B. Extrent
C. Exacto
D. Desferal
E. LoFerrous

A. The generic name of deferasirox is Exjade.

14

Which of the following statements concerning sickle cell disease are correct? (Select ALL that apply.)

A. Painful vaso-occlusive crises occur when sickle-shaped cells get stuck in narrow vessels, preventing blood flow to the subsequent tissue.
B. The common organism causing infections in infants children with sickle cell disease is P. aeruginosa.
C. Infections are common in infants, children and young adults with sickle cell disease.
D. Sickle cell disease is caused by a genetic defect of a single base substitution on the chromosome that codes for the beta chain that forms part of hemoglobin.
E. Hemoglobin carries oxygen in red blood cells.

A, C, D, E. 

15

What is the primary cause of sickle cell anemia (anemia associated with sickle cell disease)?

A. Shorter life span of the red blood cells
B. Lack of erythropoietin
C. Lack of iron
D. Renal disease
E. Over-use of transfusions

A.

16

A female pharmacist is attempting pregnancy. She has a child with sickle cell disease. She is careful to use the following precaution:

A. She uses gloves when handling the hydroxyurea tablets, and washes her hands afterwards; hydroxyurea is pregnancy category A.
B. She uses gloves when handling the hydroxyurea tablets, and washes her hands afterwards; hydroxyurea is pregnancy category D.
C. Hydroxyurea poses no risk to an unborn child; after all, it is given to children.
D. Hydroxyurea is beneficial to the unborn child because it stimulates production of fetal hemoglobin.
E. Hydroxyurea is not supposed to be given to children with sickle cell disease; she should contact the prescriber.

B. If any of the contents are spilled, clean up with a damp cloth and seal in a plastic bag.

17

Select the correct statement concerning the dosing for folic acid in sickle cell disease:

A. The dose of folic acid in children with chronic hemolytic anemia is 1 mg/day, up to 1 year of age, with higher requirements as the child grows older.
B. Children with sickle cell disease do not generally require folic acid supplementation.
C. Folic acid supplementation must be given by injection.
D. The dose of folic acid in children with chronic hemolytic anemia is 0.1 mg/day, up to 1 year of age, with higher requirements as the child grows older.
E. The dose of folic acid in children with chronic hemolytic anemia is 0.1 mg/day, up to 1 year of age, with lower requirements as the child grows older.

D. Folic acid is well absorbed. Vitamin B12 is often given by injection if the absorption is impaired due to a lack of intrinsic factor; this occurs with pernicious anemia.

18

When prophylaxis for pneumococcal infection is necessary in a young child with SCD, the preferred antibiotic is:

A. Penicillin
B. Ciprofloxacin
C. Tetracycline
D. Cefuroxime
E. Rifaximin

A. Erythromycin is used in patients with penicillin allergy.

19

Chief Complaint:  “ My daughter is having a sickle cell crisis"
History of Present Illness:  TG is a 16 y/o black female who presents to the Emergency Department with her mother.  She is in severe pain and the history is obtained from her mother.  TG was diagnosed with sickle cell disease at age 12 and initially did well with only 1-2 crises per year that were relatively easy to manage at home.  The past 12 months have been more difficult with 5 hospital admissions for severe pain crises and more time off from high school. All of TG’s childhood immunizations are up to date and TG has had no pregnancies or surgeries.
Allergies:  sulfa
Past Medical History: Sickle cell disease since age 12 (last crisis 2 months ago - admitted for 48 hours) 
Medications: Norco 5/325 1-2 tabs Q4-6H PRN, OTC folic acid (unknown strength) 
                
Physical Exam / Vitals: 
Height: 5’8”  Weight: 119 pounds    
BP: 115/75 mmHg  HR: 84 BPM  RR: 13 BPM Temp: 98.4°F  Pain: 10/10 
General: Thin young female in apparent distress, crying 
Lungs: CTA 
CV: RRR 
GI: Mild abdominal pain, mild 
Musculoskeletal: Hip and back pain

Labs:
Na (mEq/L) = 140 (135 – 145)              

WBC (cells/mm3) = 5.2 (4 – 11 x 10^3)       
K (mEq/L) = 4.8 (3.5 – 5)                      

Hgb (g/dL) = 9.8 (13.5 – 18 male, 12 – 16 female)
Cl (mEq/L) = 102 (95 – 103)                

Hct (%) = 26.8 (38 – 50 male, 36 – 46 female)
HCO3 (mEq/L) = 24 (24 – 30)              

Plt (cells/mm3) = 215 (150 – 450 x 10^3)
BUN (mg/dL) = 10 (7 – 20)                  

AST (IU/L) = 15 (10 – 40)
SCr (mg/dL) = 0.8 (0.6 – 1.3)              

ALT (IU/L) = 18 (10 – 40)
Glucose (mg/dL) = 88 (100 – 125)      

Albumin (g/dL) = 4.6 (3.5 – 5)
Ca (mg/dL) = 9.8 (8.5 – 10.5)                
Mg (mEq/L) = 1.7 (1.3 – 2.1)                 
PO4 (mg/dL) = 4.1 (2.3 – 4.7)                
                                                               
Plan:  IV pain management.  Order RBC indices, B12 and folate.  
Question: 
TG’s physician prescribes hydroxyurea.  What criteria make her a candidate for this medication?

A. Diagnosis of SCD before age 15
B. Hemoglobin < 10 g/dL
C. ≥ 3 moderate-severe crises in 1 year
D. Duration of SCD ≥ 3 years
E. Inability to manage crises with oral opioids

C.

20

Hydroxyurea is difficult for patients to tolerate. Which of the following side effects are common with the use of this drug? (Select ALL that apply.)

A. Bone marrow suppression
B. Increased appetite/weight gain
C. Anorexia, nausea
D. Sedation
E. Hyperpigmentation, scaling skin, rash

A, C, E. Bone marrow suppression is most often leukopenia and neutropenia. Thrombocytopenia and anemia occur, but less commonly. Headache and dizziness are common. The hyperpigmentation is commonly seen as nail and skin darkening. Hydroxyurea is used for other conditions, including cancer and polycythemia vera.

21

Which of the following statements concerning sickle cell disease are correct? (Select ALL that apply.)

A. The cell nucleus in white blood cells is misshapen into a concave, sickle shape.
B. The hemoglobin in red blood cells is misshapen into a concave, sickle shape.
C. Sickle cell disease is an acquired disorder.
D. Sickle cell disease is a genetic disorder.
E. Sickle cell disease affects 1 of every 500 African Americans.

B, D, E. 

22

A pharmacist has received a prescription for hydroxyurea. She is out of the generic and will substitute a brand product (at no additional cost to the patient). Which of the following products contain hydroxyurea?

A. Hydrangea
B. Droxy
C. Hydralazine
D. Hydrea
E. Urea

D. Brand names of hydroxyurea include Hydrea and Droxia.

23

Which of the following best describe the mechanism of action of hydroxyurea when used as part of the treatment plan in sickle cell disease?

A. Hydroxyurea blocks painful impulses from traveling to the CNS.
B. Hydroxyurea stimulates production of erythropoietin.
C. Hydroxyurea stimulates production of fetal hemoglobin.
D. Hydroxyurea stimulates production of endogenous endorphins, which limit pain sensations.
E. Hydroxyurea helps neutralize urine, which preserves renal function.

C. Increasing fetal hemoglobin (HbF) blocks the sickling action of RBCs, which is why infants with SCD are asymptomatic until the level of HbF drops.

24

Which of the following is not consistent with the principles of pain management in sickle cell disease?

A. Healthcare providers should believe the patient's pain.
B. An individualized treatment plan should be used, which can include random drug testing if warranted.
C. Several healthcare providers should write prescriptions for long-term opioids.
D. The patient should be seen by a healthcare provider every 2-3 months
E. The patient should be encouraged to maintain adequate fluid intake and use laxatives as needed.

C. Patients with SCD require trusting relationships with their healthcare providers for optimal management of their disease. These principles are critical.

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