Perioperative considerations sickle cell disease

Surgical planning

Any acute sickle cell related complication such as acute pain episode or acute chest syndrome is a contraindication to proceed with elective surgery.
Screen for comorbidities that may increase surgical/anesthesia risk (pulmonary disease, asthma, pulmonary hypertension, kidney disease).
For patients with a history of cardiopulmonary disease or suggestive symptoms, use echocardiography to screen for pulmonary hypertension and chronic lung disease.
Determine whether RBC alloantibodies are present and inform the transfusion medicine service.
Multidisciplinary communication between the surgeon, anesthesiologist, and hematologist/sickle cell specialist should occur to evaluate potential risks and their prevention.
Establish a perioperative pain management plan with the patient, which may involve a pain management service for interventions such as nerve blocks or ketamine.

Preoperative

Ensure adequate hydration.
- Oral clear liquids until 2 hours before surgery for individuals who are able and surgeries that allow.
- Intravenous fluids (typically 5% dextrose with 0.45% NaCl) for those who have NPO status for longer periods.
Obtain CBC, reticulocyte count, type and crossmatch, and chemistry panel with creatinine.
Ensure an active type and cross before any transfusion, and, if possible, ensure a RBC phenotype is on file with the blood bank or transfusion medicine service.
Patients with Hb SS or Hb S/beta⁰ thalassemia who are undergoing any procedure with general anesthesia or moderate to deep sedation lasting ≥1 hour should have a simple transfusion to increase the hemoglobin to 10 g/dL.^*

Intraoperative

Avoid prolonged used of torniquets if possible.
Consider regional anesthesia if possible.
While under anesthesia, pay attention to:
- Fluid balance and hydration to maintain euvolemia.
- Oxygenation (hemoglobin oxygen saturation).
- Acid-base status (pH).
- Thermoregulation.
If preoperative transfusion was indicated but not administered, strong consideration should be given to administering the transfusion in the OR.^*

Postoperative

Use adequate pain management to prevent splinting and atelectasis, with care to prevent oversedation. Refer to UpToDate for concerns and recommendations about use of NSAIDs.
Monitor closely for signs and symptoms of acute chest syndrome.
If transfusion was indicated but not administered preoperatively or in the OR, strong consideration should be given to administering the transfusion in the recovery room to reduce the risk of acute chest syndrome and other serious adverse events.^*
Monitor pulse oximetry for at least 12 to 24 hours postoperatively; provide oxygen to maintain saturation ≥95% in most individuals.
Use incentive spirometry, 10 maximal breaths every 2 hours while awake, to reduce the risk of acute chest syndrome.
Obtain CBC with reticulocyte count and creatinine every morning.
Consider occult blood loss if there is postoperative decrease in blood pressure or increase in pulse rate, respiratory rate, or increased oxygen requirements to keep the hemoglobin oxygen saturation >92%.
Provide VTE prophylaxis (pharmacologic, mechanical, or both) per institutional standards as soon as acceptable by the surgeon.
Avoid early discharge. We recommend inpatient observation until the patient is ambulating well and pain is well-controlled without significant sedation.
Provide a post-discharge phone call or telemetry visit within 24 to 48 hours after discharge and a clinic visit within 1 week of discharge.

Most deaths in patients with SCD occur during hospitalization, and acute events often occur in patients who at admission were thought to be relatively stable. Unexpected events that may develop after admission include acute chest syndrome, pulmonary embolism, fat embolism syndrome, stroke, acute kidney injury, cardiac events, severe vaso-occlusive pain, and thrombosis. Knowledge of these risk factors and comorbid chronic disease may improve early detection and treatment. Racial bias and health-related stigmas can lead to maladaptive or distrustful patient behavior that can impact hospital outcomes. Knowledge of existing risk factors for each patient may require modification of routine orders to minimize complications during hospitalization. Refer to UpToDate for details of simple transfusions and anesthesia considerations.

CBC: complete blood count; Hb: hemoglobin; NaCl: sodium chloride; NPO: nothing by mouth; NSAID: nonsteroidal antiinflammatory drug; OR: operating room; RBC: red blood cell; SCD: sickle cell disease; TAPS: Transfusion Alternatives Preoperatively in Sickle cell disease; VTE: venous thromboembolism.

* The purpose of simple preoperative transfusion is to reduce the risk of serious adverse events including acute chest syndrome associated with moderate and high risk surgeries requiring general anesthesia, as documented in the TAPS trial^[1]. It is not based on the amount of surgical blood loss.

The benefit of preoperative simple transfusion was seen for moderate-risk and high-risk surgeries in the TAPS trial. However, the benefit has not been evaluated for all procedures.
For patients with Hb SCD or sickle cell-beta⁺ thalassemia, the decision to transfuse preoperatively must be made on a case-by-case basis depending on disease severity and surgical procedure. Since these patients often have a higher baseline hemoglobin, exchange transfusions may be necessary.
On a case-by-case basis, exchange transfusion may also be indicated to reduce Hb S to <30% for patients with severe disease, significant medical comorbidities, or those undergoing high-risk surgeries such as neurosurgery or cardiac surgery.

Reference:

Howard J, Malfroy M, Llewelyn C, et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: A randomised, controlled, multicentre clinical trial. Lancet 2013; 381:930.

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Perioperative considerations sickle cell disease