Sickle Cell Disease (SCD)


Sickle cell disease (SCD) is an inherited blood disorder characterized by the presence of abnormally shaped red blood cells that do not bend or move inside blood vessels as easily as normal red blood cells. In addition, sickled red blood cells have a shortened life span, which leads to the development of anemia, and they adhere to other cells and block blood vessels, disrupting normal blood flow and causing severe pain.1,2 Several complications besides severe anemia and painful crises are associated with SCD. These include acute chest syndrome; eye, cardiac, and joint problems; gallstones; and infections.2 Patients with SCD may need to undergo surgical procedures for complications of their disease, such as cholelithiasis, or for surgical indications that are also common in the general population.3,4 Surgical procedures frequently performed in patients with SCD include cholecystectomy, pressure equalization tube placement, splenectomy, cardiopulmonary bypass, and transurethral resection.4

Preoperative Management

According to various reports, perioperative mortality rates and risks for vaso-occlusive crisis, acute chest syndrome, heart failure, and infections may be increased among patients with SCD undergoing surgical procedures.5,6 Mortality and morbidity may result from associated anemia and the potential blockage of blood vessels, with resultant hypoxia and organ damage.3 Because SCD is a multiorgan disease, patients should undergo a comprehensive preoperative assessment before any surgery.

During the preoperative assessment, it must be determined whether the patient is medically fit to undergo the surgical procedure, and an evaluation of the risks for possible complications of the surgery is required. A plan for the management of perioperative complications, if they develop, should be prepared.4 Preparations before a surgical procedure should include an evaluation of the patient’s cardiac and pulmonary functional status. Electrocardiography, echocardiography, chest radiography, and pulse oximetry may be useful for assessing pre-existing cardiac and pulmonary conditions.7

Other relevant clinical information, such as the patient’s sickle cell genotype, frequency of painful crises, typical number of days of hospitalizataion during crises, blood cell counts, and medical history regarding blood transfusions, should be obtained.4

Triggers of acute SCD crisis, such as stress, anxiety, infection, and dehydration, must be avoided perioperatively. Patient education and counseling are crucial during the time preceding the surgery to reassure the patient and prevent potential triggers of a crisis.4

Decreasing the risks of the surgical procedure requires that the medical team be aware of the patient’s SCD diagnosis. Correction of anemia with simple or exchange transfusion and postoperative respiratory care can be helpful during this time.3

Blood Transfusions

Blood transfusions may be performed either to decrease the hemoglobin S (HbS) level to below 30% or to correct anemia. Several risks are associated with this procedure, including infection, alloimmunization, increased blood viscosity, iron overload, and delayed hemolytic transfusion reaction.7

A few trials have focused on preoperative transfusion; however, the reports are limited because of the small number of participants in the studies and the challenges involved in their follow-up. A study of pediatric surgical patients suggested that routine preoperative blood transfusion may not be needed.8 For adults undergoing low- to medium-risk surgical procedures, the rate of complications such as acute chest syndrome and vaso-occlusion may be reduced with preoperative transfusions.9

Anesthesia

Anesthesia planning is important to prevent vaso-occlusive events. Epidural anesthesia is thought to decrease the possibility of vaso-occlusion during the perioperative period; however, the risk for complications such as hypotension and bradycardia, which can cause vaso-occlusion, should also be considered.7 The use of general anesthesia may result in inadequate analgesia after surgery; however, it has the advantage of reducing the anxiety that patients typically experience when they awaken and that can trigger a painful crisis. Another benefit of general anesthesia is the use of controlled ventilation, which improves oxygenation and reduces the risk for a vaso-occlusive event.7

Pain Management After Surgery

Postoperative pain must be distinguished from the pain of an SCD crisis. The administration of opioids remains the approach most commonly used to alleviate pain in patients with SCD. General measures for preventing vaso-occlusive crises must also be taken, which include monitoring the patient’s hydration, sedation, antibiotic prophylaxis, analgesia, oxygenation, and temperature. The placement of ice on the surgical site must be avoided.7

References

1. Sickle cell disease. National Organization for Rare Disorders (NORD). Accessed November 28, 2021.

2. Sickle cell disease. National Heart, Lung, and Blood Institute. Updated September 1, 2020. Accessed November 28, 2021.

3. The management of sickle cell disease. National Heart, Lung, and Blood Institute. Division of Blood Diseases and Resources. NIH Publication No. 02-2117. Revised June 2002. Accessed November 28, 2021.

4. Adjepong KO, Otegbeye F, Adjepong YA. Perioperative management of sickle cell disease. Mediterr J Hematol Infect Dis. 2018;10(1):e2018032. doi:10.4084/MJHID.2018.032

5. Holzmann L, Finn H, Lichtman HC, Harmel MH. Anesthesia in patients with sickle cell disease: a review of 112 cases. Anesth Analg. 1969;48(4):566-572 

6. Firth PG, Head CA. Sickle cell disease and anesthesia. Anesthesiology. 2004;101(3):766-785. doi:10.1097/00000542-200409000-00027

7. Khurmi N, Gorlin A, Misra L. Perioperative considerations for patients with sickle cell disease: a narrative review. Can J Anaesth. 2017;64(8):860-869. doi:10.1007/s12630-017-0883-3

8. Griffin TC, Buchanan GR. Elective surgery in children with sickle cell disease without preoperative blood transfusion. J Pediatr Surg. 1993;28(5):681-685. doi:10.1016/0022-3468(93)90031-f

9. 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(9870):930-938. doi:10.1016/S0140-6736(12)61726-7

Reviewed by Debjyoti Talukdar, MD, on 11/26/2021.

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