Sickle Cell Disease Flashcards
All of the following are safety concerns related to the use of hydroxyurea EXCEPT:
a. Neutropenia
b. Skin contact (hazardous drug)
c. Use of live vaccines
d. Iron overload
e. Thrombocytopenia
d
- Hydroxyurea has a BBW for myelosuppression, which increases the risk of infection and bleeding.
- Pts taking hydroxyurea should avoid live vaccines and need to have a CBC with differential checked every 2-4 weeks initially to monitor the ANC. It’s a hazardous drug.
JP is a newborn female infant diagnosed with sickle cell disease during routine screening at birth. Which of the following complications is JP at risk for in the future?
a. Vaso-occlusive crises
b. Infections
c. Alzheimer’s disease
d. Anemia
e. Functional asplenia
a, b, d, e
Vaso-occlusive crises (VOC) are the most common complication of sickle cell disease. Functional asplenia puts the patient at risk for infections caused by encapsulated organisms.
CC: My daughter is having a sickle cell crisis.
HPI: TG is a 19 y/o female who presents to the ED on 11/14 with her mom. She’s in severe pain and has had no appetite because the pain makes her nauseous. She has been able to swallow her pain pill. Her mom states that TG has had 5 hospitalizations in the past 12 months for severe pain crises and is having trouble finishing high school. All of TG’s childhood vaccines are UTD and she has had no pregnancies or surgeries.
PMH: Sickle cell disease since 18 months old (last crisis 2 months ago - admitted for 72 hours)
Meds: Oxycontin 20 mg Q12H, Norco 5/325 1-2 tabs Q4-6H PRN (used 2 tabs Q4H in last 48 hours), OTC folic acid (unknown strength)
11/14 PE and Vitals:
BP: 120/75 HR: 102 RR: 13 Temp: 98.4F Pain: 10/10
General: Thin young female in apparent distress, crying
CV: Tachycardia, normal rhythm
GI: Mild-moderate abdominal pain
Musculoskeletal: Severe hip and back pain
Abnormal Labs 11/14:
BUN = 21 (7-20)
Glucose = 88 (100-125)
Hgb = 9.2 (12-16)
Hct = 29.8 (36-46)
Plan:
- 11/14L IV pain management: Morphine 5 mg q3h PRN. IV fluids: NS infused at 150 mL/hr x 24 hours, then decrease to 100 mL/hr. Order RBC indices, B12 and folate.
- 11/15: Pain improved (now reports 6 out of 10). D/c IV fluids. Continue IV morphine.
- 11/16: Pain improved (now reports 2 out of 10). Wean IV morphine and transition to PO morphine PRN, pharmacy to calculate dose (used 30 mg of IV morphine in last 24 hours).
- 11/17: Plan to discharge today. Start hydroxyurea, consult pharmacy for dosing recommendation. Transition to home pain regimen.
Prior to discharge, what counseling should TG receive about pregnancy and contraception?
a. Taking hydroxyurea decreases her chance of becoming pregnant.
b. She doesn’t have to worry about contraception until she’s older.
c. If she’s sexually active, she doesn’t need to use contraception as long as her partner does.
d. Her risk of miscarriages and premature births if she gets pregnant is the same as females without sickle cell disease.
e. She should use contraception during hydroxyurea tx and for 6 months after discontinuation.
e
CC: My daughter is having a sickle cell crisis.
HPI: TG is a 19 y/o female who presents to the ED on 11/14 with her mom. She’s in severe pain and has had no appetite because the pain makes her nauseous. She has been able to swallow her pain pill. Her mom states that TG has had 5 hospitalizations in the past 12 months for severe pain crises and is having trouble finishing high school. All of TG’s childhood vaccines are UTD and she has had no pregnancies or surgeries.
PMH: Sickle cell disease since 18 months old (last crisis 2 months ago - admitted for 72 hours)
Meds: Oxycontin 20 mg Q12H, Norco 5/325 1-2 tabs Q4-6H PRN (used 2 tabs Q4H in last 48 hours), OTC folic acid (unknown strength)
11/14 PE and Vitals:
BP: 120/75 HR: 102 RR: 13 Temp: 98.4F Pain: 10/10
General: Thin young female in apparent distress, crying
CV: Tachycardia, normal rhythm
GI: Mild-moderate abdominal pain
Musculoskeletal: Severe hip and back pain
Abnormal Labs 11/14:
BUN = 21 (7-20)
Glucose = 88 (100-125)
Hgb = 9.2 (12-16)
Hct = 29.8 (36-46)
Plan:
- 11/14L IV pain management: Morphine 5 mg q3h PRN. IV fluids: NS infused at 150 mL/hr x 24 hours, then decrease to 100 mL/hr. Order RBC indices, B12 and folate.
- 11/15: Pain improved (now reports 6 out of 10). D/c IV fluids. Continue IV morphine.
- 11/16: Pain improved (now reports 2 out of 10). Wean IV morphine and transition to PO morphine PRN, pharmacy to calculate dose (used 30 mg of IV morphine in last 24 hours).
- 11/17: Plan to discharge today. Start hydroxyurea, consult pharmacy for dosing recommendation. Transition to home pain regimen.
Which of the following PO regimens provides an equivalent daily amount of morphine as the IV daily amount documented on 11/16 (use an IV:PO conversion ratio of 1:3)?
a. Morphine 10 mg PO Q6H
b. Morphine 15 mg PO Q6H
c. Morphine 15 mg PO Q4H
d. Morphine 20 mg PO Q4H
e. Morphine 30 mg PO Q6H
c
CC: My daughter is having a sickle cell crisis.
HPI: TG is a 19 y/o female who presents to the ED on 11/14 with her mom. She’s in severe pain and has had no appetite because the pain makes her nauseous. She has been able to swallow her pain pill. Her mom states that TG has had 5 hospitalizations in the past 12 months for severe pain crises and is having trouble finishing high school. All of TG’s childhood vaccines are UTD and she has had no pregnancies or surgeries.
PMH: Sickle cell disease since 18 months old (last crisis 2 months ago - admitted for 72 hours)
Meds: Oxycontin 20 mg Q12H, Norco 5/325 1-2 tabs Q4-6H PRN (used 2 tabs Q4H in last 48 hours), OTC folic acid (unknown strength)
11/14 PE and Vitals:
BP: 120/75 HR: 102 RR: 13 Temp: 98.4F Pain: 10/10
General: Thin young female in apparent distress, crying
CV: Tachycardia, normal rhythm
GI: Mild-moderate abdominal pain
Musculoskeletal: Severe hip and back pain
Abnormal Labs 11/14:
BUN = 21 (7-20)
Glucose = 88 (100-125)
Hgb = 9.2 (12-16)
Hct = 29.8 (36-46)
Plan:
- 11/14L IV pain management: Morphine 5 mg q3h PRN. IV fluids: NS infused at 150 mL/hr x 24 hours, then decrease to 100 mL/hr. Order RBC indices, B12 and folate.
- 11/15: Pain improved (now reports 6 out of 10). D/c IV fluids. Continue IV morphine.
- 11/16: Pain improved (now reports 2 out of 10). Wean IV morphine and transition to PO morphine PRN, pharmacy to calculate dose (used 30 mg of IV morphine in last 24 hours).
- 11/17: Plan to discharge today. Start hydroxyurea, consult pharmacy for dosing recommendation. Transition to home pain regimen.
What criteria does TG meet to begin hydroxyurea tx?
a. Diagnosis of SCD before age 15
b. Hemoglobin < 10 g/dL
c. >/= 3 moderate-severe crises in 1 year
d. Duration of SCD >/= years
e. Inability to manage crises with PO opioids
c
CC: My daughter is having a sickle cell crisis.
HPI: TG is a 19 y/o female who presents to the ED on 11/14 with her mom. She’s in severe pain and has had no appetite because the pain makes her nauseous. She has been able to swallow her pain pill. Her mom states that TG has had 5 hospitalizations in the past 12 months for severe pain crises and is having trouble finishing high school. All of TG’s childhood vaccines are UTD and she has had no pregnancies or surgeries.
PMH: Sickle cell disease since 18 months old (last crisis 2 months ago - admitted for 72 hours)
Meds: Oxycontin 20 mg Q12H, Norco 5/325 1-2 tabs Q4-6H PRN (used 2 tabs Q4H in last 48 hours), OTC folic acid (unknown strength)
11/14 PE and Vitals:
Height: 5’8” Weight: 119 pounds
BP: 120/75 HR: 102 RR: 13 Temp: 98.4F Pain: 10/10
General: Thin young female in apparent distress, crying
CV: Tachycardia, normal rhythm
GI: Mild-moderate abdominal pain
Musculoskeletal: Severe hip and back pain
Abnormal Labs 11/14:
BUN = 21 (7-20)
Glucose = 88 (100-125)
Hgb = 9.2 (12-16)
Hct = 29.8 (36-46)
Plan:
- 11/14L IV pain management: Morphine 5 mg q3h PRN. IV fluids: NS infused at 150 mL/hr x 24 hours, then decrease to 100 mL/hr. Order RBC indices, B12 and folate.
- 11/15: Pain improved (now reports 6 out of 10). D/c IV fluids. Continue IV morphine.
- 11/16: Pain improved (now reports 2 out of 10). Wean IV morphine and transition to PO morphine PRN, pharmacy to calculate dose (used 30 mg of IV morphine in last 24 hours).
- 11/17: Plan to discharge today. Start hydroxyurea, consult pharmacy for dosing recommendation. Transition to home pain regimen.
The hydroxyurea starting dose is 15 mg/kg/day for patients with normal renal function. If hydroxyurea is available in 200, 300, and 400 mg caps, how should the discharge prescription be written?
a. Hydroxyurea 400 mg #120, Take 4 caps PO daily
b. Hydroxyurea 200 mg #60, Take 2 caps PO daily
c. Hydroxyurea 300 mg #75, Take 2 1/2 capsules PO daily
d. Hydroxyurea 300 mg #90, Take 3 caps PO daily
e. Hydroxyurea 400 mg #90, Take 3 caps PO daily
d
TG’s CrCl is normal. She’s 119 lbs (54.09 kg) x 15 mg/kg/day = 811.36 mg/day. Using the available dosage forms and rounding up to the nearest capsule, the dose would be three 300 mg capsules daily.
Which of the following is an appropriate tx for vaso-occlusive crisis in sickle cell disease?
a. Morphine
b. Aspirin
c. Levofloxacin
d. Loperamide
e. Enoxaparin
a
Vaso-occlusive crises are episodes of acute, severe pain from sickled blood cells blocking blood flow, which leads to decreased oxygen and ischemia in the tissues. IV opioids, including PCA, are needed for the severe pain associated with VOCs.
Which of the following best describes the MOA of hydroxyurea when used as part of the tx plan in sickle cell disease?
a. Blocks painful impulses from traveling to the CNS
b. Stimulates production of erythropoietin
c. Stimulates production of fetal hemoglobin
d. Stimulates production of endogenous endorphins, which limit pain sensations
e. Helps neutralizes urine, which preserves renal function
c
Fetal hemoglobin (HgbF) blocking the sickling action of RBCs, which is why infants with SCD are asymptomatic until the first 2-3 months of life when HgbF is replaced with HgbS (sickle hemoglobin).
Which of the following encapsulated organisms are vaccinated against in patients with SCD?
a. Streptococcus pneumoniae
b. Haemophilus influenzae
c. Neisseria meningitidis
d. Pseudomonas aeruginosa
e. Mycoplasma pneumoniae
a, b, c
Functional asplenia (a complication of SCD) increases the risk of infection with encapsulated organisms.
Children with SCD have been cured with the following treatment modality:
a. Spleen and pancreas transplant
b. Infliximab infusions
c. Cyclosphosphamide
d. Bone marrow transplant
e. Kidney transplant
d
