UBP 4.1 (Long Form): Obstetrics - Sickle Cell Disease Flashcards

Secondary Subject -- Amniotic Fluid Embolism / Local Anesthetic Toxicity / Magnesium: Imbalance - Use in Treatment / Perioperative Pain Management / Postpartum Hemorrhage / Uterine Atony / Preeclampsia and Eclampsia / Pregnancy Testing, Pre-operative / Nitrous Oxide and Retinal Detachment Surgery / Substance Abuse – Withdrawal and Post-op Pain Management / Citrate Toxicity / TRALI / Neuraxial Anesthesia in the Coagulopathic Patient

1
Q

Intra-operative Management:

Would you choose regional or general anesthesia for this case?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

Given the potential for difficult airway management due to her obesity, pregnancy, preeclampsia (airway edema), and Mallampati 3 airway exam,

I would prefer to utilize regional anesthesia for this case.

However, I recognize that – a platelet level of 88,000, in conjunction with the thrombocytopathia associated with preeclampsia, places her at increased risk for epidural or spinal hematoma.

Moreover, I understand that these patients are often intravascularly contracted, and that the sympathectomy associated with neuraxial anesthesia could result in significant hypotension.

Therefore, prior to providing neuraxial anesthesia, I would –

  • evaluate the patient for signs of coagulopathy (bruising, mucosal bleeding, bleeding at IV sites),
  • make sure that there was not an unacceptable downward trend in her platelet level, and
  • judiciously provide fluids for volume replacement (overaggressive fluid administration risks pulmonary and cerebral edema in the setting of severe preeclampsia).

In order to further reduce the risk for significant hypotension, I would slowly induce epidural anesthesia (rather than spinal anesthesia), giving the patient sufficient time to compensate for the loss in sympathetic tone occurring below the level of blockade.

Postoperatively, I would ensure adequate platelet levels and allow for complete resolution of motor and sensory function prior to removing the neuraxial catheter (to allow for better detection of the change in motor or sensory function associated with epidural or spinal hematoma formation).

Following the removal of the catheter, I would order hourly neuro-checks for 12 hours, to more quickly identify the signs and symptoms of epidural or spinal hematoma –

  • (i.e. severe backache, bowel or bladder dysfunction, radiculopathy, tenderness over the spinous or paraspinous area, unexplained fever).

Finally, while there is a theoretical concern that compensatory vasoconstriction above the level of blockade may increase the risk of vaso-occlusive crisis, this has not been supported by evidence.

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2
Q

Intra-operative Management:

The surgeon requests a spinal rather than an epidural regional anesthetic.

What would you say?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

I would explain to him that epidural anesthesia has traditionally been considered the more appropriate technique in the setting of severe preeclampsia due to –

an ability to slowly titrate both local anesthetic and fluids in order to avoid a precipitous drop in systemic vascular resistance and/or fluid overload

(preeclamptic patients are more susceptible to pulmonary and cerebral edema with aggressive fluid administration).

However, if there were a good reasont to utilize spinal anesthesia, I would consider placing a combined spinal-epidural (CSE) for this case.

I would prefer a CSE to a single-shot spinal technique because her obesity and the scarring from three previous cesarean sections may prolong surgery beyond the duration of analgesia provided by the inital intrathecal dose, requiring additional medicine via the epidural catheter.

Having the option to maintain adequate neuraxial blockade using the epidural catheter is of particular important in this patient with a potentially difficult airway.

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3
Q

Intra-operative Management:

You are unable to place a spinal anesthetic and decide to employ epidural anesthesia for the case.

What will you do to reduce the risk of sickling during this cesarean section?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

In order to reduce the risk of sickling, I would:

  1. avoid hypotension and venostasis by ensuring adequate fluid administration to compensate for the sympathectomy induced by regional anesthesia;
  2. ensure adequate oxygenation with supplemental oxygen and pulse-oximetry, recognizing that deoxygenated hemoglobin S is more susceptible to polymerization;
  3. ensure adequate oxygen-carrying capacity by transfusing PRBCs as necessary;
  4. maintain normothermia, recognizing the need to avoid the increased oxygen consumption associated with hyperthermia and the vasoconstriction with subsequent blood stasis associated with hypothermia; and
  5. ensure adequate ventilation to avoid acidosis, which predisposes to hemoglobin deoxygenation.
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4
Q

Intra-operative Management:

Shortly after achieving adequate levels with the epidural, the case begins and the baby is quickly delivered.

A few minutes after delivery, the mother complains of difficulty breathing and then begins seizing.

What do you think is going on?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

The timing of her dyspnea and seizure, occurring shortly after delivery of the baby, is consistent with amniotic fluid embolism.

However, given her preeclampsia and the recent administration of large volumes of local anesthetic, I would also be considering eclampsia and local anesthetic toxicity, respectively

(the dyspnea in the case of preeclampsia could be from pulmonary edema;

the dyspnea in the case of local anesthetic toxicity could be from pulmonary edema or high neuraxial blockade).

Another less likely consideration would be sickle cell crisis leading to acute chest syndrome and a stroke-induced seizure.

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5
Q

Intra-operative Management:

What will you do?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

I would immediately –

  1. apply cricoid pressure,
  2. supply 100% oxygen,
  3. call for difficult airway equipment,
  4. administer a small dose of midazolam,
  5. position the patient in reverse-trendelenburg
    • (to facilitate intubation and reduce the risk of passive regurgitation),
  6. perform careful laryngoscopy, and secure the airway with an endotracheal tube.
  7. I would then – evaluate the patient’s ECG, blood pressure, and oxygen saturation;
  8. verify left uterine displacement;
  9. ensure adequate ventilation
    • (hypercarbia lowers the seizure threshold and hypoxia and hypercarbia increase the risk of sickling); and
  10. support maternal circulation with fluids and vasopressors as necessary
    • (myocardial depression and vasodilation are associated with both amniotic fluid embolism or local anesthetic toxicity).
  11. Finally, I would – ensure adequate intravenous access,
  12. order a magnesium level
    • (inadequate levels may have led to an eclamptic seizure),
  13. type and cross the patient
    • (in the case of amniotic fluid embolism, blood products are often required secondary to coagulopathy),
  14. place an intra-arterial catheter
    • (to aid in blood pressure management and blood draws), and
  15. continue to monitor her for –
    • coagulopathy,
    • arrhythmias
      • (local anesthetic toxicity, amniotic fluid embolism, or increased intracranial pressure associated with eclampsia could result in arrhythmias),
    • hypoxia, and/or signs of shock
      • (with amniotic fluid embolism, transient pulmonary vasospasm can lead to right heart failure –> hypotension –> V/Q mismatch).
  16. If she became hemodynamically unstable, I would – place a pulmonary artery catheter, obtain rapid transfusion equipment, and administer blood products, fluids, and vasopressors as required.
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6
Q

Intra-operative Management:

What are the signs and symptoms of amniotic fluid embolism?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

The signs and symptoms of amniotic fluid embolism occur in two phases.

  • The first or early phase consists of –
    • pulmonary hypertension (secondary to pulmonary vasospasm),
    • hypotension (secondary to right heart failure),
    • hypoxia (secondary to ventilation-perfusion mismatching),
    • seizure, and
    • cardiac arrest.
  • For those who survive the first phase, the second phase consists of –
    • left ventricular failure,
    • pulmonary edema, and
    • coagulopathy (the latter is possibly secondary to circulating trophoblast).
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7
Q

Intra-operative Management:

If you were concerned that this was amniotic fluid embolism, would you pull the epidural catheter at the end of the case?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

Recognizing that the placement and removal of an epidural catheter are the two times when the patient with a coagulopathy is at the highest risk for bleeding in the epidural space leading to hematoma formation,

I would NOT pull the catheter immediately following the case.

Given the potential for rapidly changing coagulation status during the initial disease process (despite the administration of appropriate blood products), and

recognizing that an indwelling catheter presents minimal risk even when the patient is coagulopathic (the times of highest risk are when placing and removing the catheter),

I would prefer to leave the catheter in situ until her coagulation status normalized.

While leaving the catheter in place presents some risk of infection, I believe this risk is minimal in the first several days.

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8
Q

Intra-operative Management:

Let’s assume this was just a seizure secondary to eclampsia. She is intubated, the baby is delivered, and the surgeon tells you that the uterus is “boggy”. The resident wants to reduce the volatile agent and supplement with nitrous oxide. What do you think of her plan?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

While supplementation with nitrous oxide may be desirable to allow for a reduction in volatile agent concentration and, therefore, reduce the contribution of the volatile agent to uterine relaxation,

it may not be advisable in this case due to the patient’s sickle cell disease and recent retinal detachment surgery.

  1. First, the administration of 100% oxygen helps to reduce the risk of significant sickling by reducing the amount of deoxygenated hemoglobin S.
  2. Second, the ophthalmologist who performed her retinal detachment surgery may have injected sulfur hexafluoride or perfluoropropane into the vitreous in order to tamponade the retina, holding it in place until healing occurs.

This is of concern because the administration of nitrous oxide (a much more soluble gas) could result in expansion of the intra-vitreal bubble, with resultant increased intra-ocular pressure, central retinal artery occlusion, and retinal and/or optic nerve ischemia.

Moreover, the subsequent discontinuation of the nitrous oxide can lead to rapid reabsorption of the intra-vitreal bubble, leading to a precipitous drop in intraocular pressure that could result in another retinal detachment.

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9
Q

Intra-operative Management:

If the opthalmologist utilized intra-vitreal air for retinal tamponade, would you be ok with nitrous oxide administration?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

Since intra-vitreal air would likely be reabsorbed within 5 days, I would be less worried about nitrous oxide expansion of an intra-ocular bubble.

However, given my ongoing desire to maintain a high oxygen saturation to reduce the risk of hemoglobin S sickling, I would still avoid nitrous oxide as a means to reduce the volatile agent concentration.

Rather, I would enable the reduction or discontinuation of volatile agents by administering narcotics and propofol as tolerated by the patient’s hemodynamic status.

Clinical Notes:

  • Nitrous oxide should be discontinued 15 minutes prior to the injection of intra-vitreal gas for retinal detachment surgery.
  • Nitrous should be avoided for a variable amount of time depending on the type of gas injected:
    • Air – avoid nitrous oxide for 5 days
    • Sulfur Hexafluoride – avoid nitrous oxide for 10 days
    • Perfluoropropane – avoid nitrous oxide for at least 30 days
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10
Q

Intra-operative Management:

Bleeding continues and you institute the massive blood transfusion protocol. You have given 5 units of PRBCs and 4 units of FFP. The patient’s blood pressure drops to 78/44 mmHg and is not responsive to additional doses of phenylephrine. What do you think is going on?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

The most likely causes of her nonresponsive hypotension are inadequate blood or fluid replacement and/or hypocalcemia secondary to massive blood transfusion (the citrate preservative in blood products can chelate calcium during massive blood transfusion).

However, I would also be considering other potential causative or contributive factors, such as:

  1. myocardial depression from acidosis or anesthesia;
  2. magnesium toxicity (vasodilation and myocardial depression);
  3. right heart failure due to the pulmonary hypertension associated with sickle cell lung disease;
  4. transfusion reaction; and
  5. pulmonary thromboembolism.
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11
Q

Intra-operative Management:

You give 2 grams of calcium gluconate and her blood pressure returns to low normal.

Why do you think the administration of calcium helped?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

Her response to calcium suggests that citrate-induced hypocalcemia or magnesium toxicity was at least partly responsible for her hypotension.

However, the timing of her hypotension with the transfusion of blood products would make it more likely that citrate intoxication, rather than magnesium toxicity, was the problem.

With the transfusion of large volumes of stored blood, especially FFP, the citrate preservative can lead to significant chelation of ionized calcium with subsequent myocardial depression.

Other signs of citrate intoxication would include increased central venous pressure, narrow pulse pressure, prolonged QT interval, flattened T waves, widened QRS complexes, and increased intraventricular end-diastolic pressure.

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12
Q

Post-operative Management:

How will you manage her pain post-operatively?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

Despite her concerns about possible opioid addiction, my goals in managing this patient with sickle cell disease, who is chronically using opioids to control pain, would be –

to avoid opioid withdrawal and provide adequate pain control.

While her concerns about addiction may be valid, this does not change the need to provide adequate pain control in the perioperative period due to the risk that poorly controlled pain could exacerbate the complications associated with sickle cell disease and/or lead to additional chronic pain related to the surgery itself

(there is increased risk of developing chronic post-surgical pain, independent of the source of her pre-operative pain, when post-operative pain is not controlled).

Therefore, I would manage her pain as follows:

  1. reassure the patient pre-operatively that her potential opioid dependence will not deter me from providing adequate pain control post-operatively;
  2. explain to the nurses in recovery that she will likely require significantly more pain medicine than their typical patient (one of the main risk factors for inadequate pain control in opioid dependent patients is the biases of healthcare personnel);
  3. supply intravenous or oral opioids equivalent in strength to her normal daily dose;
  4. provide epidural morphine, recognizing that higher than normal amounts may be required to achieve the desired amount of analgesia; and
  5. make 2-3 times the normal amount of anticipated opioids available to her for breakthrough pain.
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13
Q

Post-operative Management:

The nurse calls you immediately post-op to report that the patient is completely comfortable with just the epidural.

She asks if it is ok to discontinue all scheduled intravenous/oral narcotics until the patient complains of pain.

What would you say?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

I would explain to her that patient’s who take opioid chronically are at increased risk for perioperative opioid withdrawal.

Furthermore, the amount of opioids necessary to provide adequate epidural analgesia is not necessarily sufficient to prevent opioid withdrawal.

Therefore, I would continue the scheduled administration of systemic opioids in an amount equivalent to ther pre-admission dose.

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14
Q

Post-operative Management:

A few hours later, the patient is wheezing, dyspneic, and demonstrates a pulmonary infiltrate on chest x-ray.

What do you think may be the cause of these findings?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

Given this patient’s sickle cell disease, preeclampsia/eclampsia, and recent blood transfusion, my differential would include:

  1. acute chest syndrome, which typically presents with fever, tachypnea, cough, hypoxemia, pulmonary infiltrate, and chest pain;
  2. the reduced colloid osmotic pressure and increased vascular permeability associated with preeclampsia/eclampsia;
  3. transfusion related acute lung injury (TRALI), a noncardiogenic pulmonary edema, typically developing within 6 hours of transfusion, and presenting with a pulmonary infiltrate, dyspnea, fever, chills, and hypertension or hypotension;
  4. acute respiratory distress syndrome; and
  5. magnesium toxicity, which may present with respiratory depression and pulmonary edema among other things.
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15
Q

Post-operative Management:

Assuming this were acute chest syndrome (ACS), what would you do?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

Assuming this were acute chest syndrome, I would:

  1. provide supplemental oxygen, bronchodilators, incentive spirometry, and chest physiotherapy;
  2. administer antibiotics to cover atypical and encapsulated organisms;
  3. ensure adequate pain control;
  4. correct any anemia with simple blood transfusion; and,
  5. depending on the severity of the patient’s condition, consider exchange transfusion and/or mechanical ventilation.
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16
Q

Post-operative Management:

She comes back 6 months later for carpal tunnel release. She is refusing a pregnancy test because she has been breast feeding and “can’t be pregnant”.

What will you do?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

While preoperative pregnancy testing is possibly cost effective when considering the cost of abortions, miscarriages, and malpractice lawsuits, the evidence is equivocal when it comes to demonstrating improved outcomes.

Therefore, I would carefully review my institution’s policy, and assuming there was no requirement for mandatory pregnancy testing, I would provide informed consent, have the patient sign a waiver of all legal rights as it pertains to an undetected pregnancy, and proceed with the case.

17
Q

Post-operative Management:

Your institution’s policy does mandate pregnancy testing. What would you do?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

I would explain to her that given the potential for harmful effects on the baby’s development and the increased risk for spontaneous abortion or preterm labor,

the facility requires preoperative pregnancy testing prior to elective surgery.

I would further explain that she has every right to refuse pregnancy testing, but the facility would then be forced to exercise their right to refuse service.

18
Q

Post-operative Management:

The surgeon wishes to utilize a tourniquet for the case. Are you ok with this?

  • (A 32-year-old, 99 kg, G4P3 female requires a repeat cesarean section due to severe preeclampsia. She is at 36 weeks gestation and has a history of three previous cesarean sections. The obstetrician informs you she does not know the patient and that there has been no prenatal care.*
  • PMH: Additional history includes shortness of breath with exertion and frequent hospital admissions due to pain from her sickle cell disease. She has been taking narcotics routinely to control pain and is afraid she may be becoming addicted.*
  • PSH: Her surgical history consists of a splenectomy 18 years ago and retinal detachment surgery under local anesthesia 1 week ago.*
  • Medications: Hydroxyurea and MS-Contin 30 mg BID*
  • Allergies: NKDA*
  • PE: Vital Signs: HR 118; BP = 188/104 mmHg; R = 12; T = 36.8 C*
  • Airway: Mallampati III*
  • Heart: RRR*
  • Lungs: CTAB*
  • Extremities: Edema noted in the lower extremities*
  • CXR: Cardiac enlargement*
  • Lab: Hgb = 7.8 g/dL; BUN = 18 mg/dL; Cr = 1.2 mg/dL; Plt = 88; urine = 3+ protein)*
A

Given the regional venostasis, acidosis, and deoxygenation of hemoglobin that occurs distal to the tourniquet, I would prefer to AVOID tourniquet use in this patient with sickle cell disease.

However, recognizing that tourniquets have been utilized safely for up to two hours in sickle cell patients, I would be OK with this assuming it were necessary for the successful completion of the procedure.

During the case I would attempt to minimize the risk of complication by avoiding factors that would potentially contribute to intravascular sickling, such as hypoxia, acidosis, dehydration, hypothermia, and hyperthermia.