Sickle Cell Information Center Guidelines

Edited by James Eckman, M.D. and Allan Platt, PA-C

Anesthesia Management Pre and Post Operative Care  

A recent cooperative study of preoperative transfusion demonstrates that sickle cell patients should have simple transfusions to raise the patient's hemoglobin to 10 gm/dL before surgery. These simple transfusions are safer and as effective in preventing postoperative complications as exchange or aggressive transfusions to decrease the hemoglobin S level below 30%. Postoperative complications such as Acute Chest Syndrome, fever, and alloimmunization with delayed transfusion reactions are common, still occuring in up to 30% of patients.

Alloimmunization can be minimized by giving antigen matched blood (matched for K, C, E, S, Fy, and Jk antigens). All patients should receive incentive spirometry, adequate hydration,
oxygenation, and reduce body exposure to he cold temperatures of the operating room.

Past Medical History. A careful history is taken to determine the presence of recent or recurrent respiratory infections, cardiac problems, hepatic or renal disease. Specific questions include recent cough, fever, chest pain, wheezing, dyspnea on exertion, paroxysmal nocturnal dyspnea, orthopnea, heart murmurs, ankle edema, palpitations, right upper quadrant pain, change in jaundice, stool or urine color, hematuria, nocturia, or dysuria.

Past Medical History. Transfusion reactions and alloimmunization should be highlighted. A careful history of past complications is determined including pneumonias, acute chest syndrome, asthma, hepatitis, kidney infections, frequency of pain episodes, seizures, strokes, subarachnoid hemorrhage, and other significant complications. Use of alcohol and smoking history should be quantitated. History of past anesthesia and surgeries is detailed. All present and recent medications and allergies are defined.

- Vital Signs. Record Temperature, blood pressure, pulse and respiratory rate.

- HEENT. Determine obstructions to intubation, enlarged tonsils.

- Chest. Examine for prolonged expiratory phase, wheezing, rales, egophony. Determine exercise tolerance by walking.

- Cardiac. Estimate central venous pressure from neck veins. Heart size. Define characteristics of A2, P2, and all heart murmurs. Are S3, S4, or edema present?

- Abdominal. Hepatosplenomegaly.

- Extremities. Pulses.

- Neurologic. Focal neurologic findings.

- Minimal Lab. CBC with platelet and reticulocyte count, SMA 18, urinalysis, chest x-ray.

- Additional Lab. The preoperative evaluation is based on the patient’s medical problems and nature of the surgery. Patients with history of chest syndrome, pneumonias, smoking, or respiratory symptoms and findings should have blood gases or oxygen saturation by pulse oximetry. Pulmonary function testing may be indicated. Patients undergoing ophthalmological, neurologic, or bypass surgery should have PT, PTT, and bleeding time. Older patients, those with murmurs, or cardiac history should have an ECG. Significant murmurs should be characterized by echocardiography. Patients on a transfusion program should have quantitation of Hb A and Hb S.

- General Management. The principles of managing all patients include adequate hydration, good oxygenation, avoidance of hypothermia, acidosis, and local vascular stasis, and careful monitoring. Preoperative intravenous hydration is frequently overlooked in patients that are kept NPO. Early ambulation and incentive spirometry are used postoperatively.

- Preoperative Transfusion. The need for and value of preoperative transfusion is one of the more controversial areas in managing patients with sickle cell disease. The benefits cited are the past history of frequent complications, increased tissue oxygenation, reduced blood viscosity, and a "margin of safety". The most consistent benefit may result from prevention of the need to transfuse after surgery because of bone marrow suppression. The disadvantages include induction of hyperviscosity, significant alloimmunization, delayed transfusion reactions, exposure to infectious disease, cost, and provision of a false sense of security. Most agree that transfusion is not indicated for minor procedures under local anesthesia unless the patient is severely anemic or has other complications. Most would transfuse patients undergoing major surgical procedures, however, the definition of which procedures may vary significantly. Cardiothoracic operations, neurosurgical procedures, ophthalmological operation even under local anesthesia, and prolonged operations under general anesthesia are considered indications by most clinicians. Some feel all operations under general anesthesia require preoperative transfusion.

A recent cooperative study of preoperative transfusion demonstrates that sickle cell patients should have simple transfusions to raise the patient's hemoglobin to 10 gm/dL before surgery. These simple transfusions are safer and as effective in preventing postoperative complications as are exchange or aggressive transfusions to decrease the hemoglobin S level below 30%. Postoperative complications such as chest syndrome, fever, and alloimmunization with delayed transfusion reactions are common. Alloimmunization can be minimized by giving antigen matched blood ( matched for K, C, E, S, Fy, and Jk antigens). All patients should receive incentive spirometry, given adequate hydration and oxygenation.

The patient with hemoglobin SS or SC with hemoglobin levels of 10 g/dl requires special consideration. Hyperviscosity is a clear contraindication to raising the hemoglobin level above 11 gm/dL until the percentage of Hb S is below 30%. Therefore, exchange transfusion or repeated simple transfusion over several weeks is required if the patient is undergoing surgery where the clinician feels it is necessary to provide cells containing hemoglobin A.

 Patients and family must be informed about the risk and benefits of surgery and transfusion using the most recent data available.

 There is increased risk for sickle cell patients undergoing surgery and informed consent is mandatory.

 Care should be taken during surgery to maintain adequate body temperature to prevent sickling.

 Pulse Ox should be closely monitored to prevent hypoxia.

 Patients require intravenous hydration while NPO for surgery.

 Monitor intake and output to avoid excessive hydration leading to respiratory complications postop

 Encourage ambulation, postoperative coughing, deep breathing and the frequent use of incentive spirometers.

Whenever possible, undertake surgery on an elective basis, so that the patient is in the best possible physiologic condition. Transfusion is not likely indicated for procedures under local anesthesia, with the exception of intraocular surgery. No matter what the approach to transfusion, careful preoperative, intraoperative, and postoperative care are mandatory. Over-transfusion to high hemoglobin levels is dangerous unless the percentage of Hb S is very low. Incentive Spirometry should be given to all patients to prevent chest syndrome.

Antibiotic prophylaxis for subacute bacterial endocarditis should be considered, especially for patients with valvular insufficiency due to distortion of the heart valve by severe dilated cardiomegaly.

Patients and parents must be informed and educated about the risks and benefits of surgery and transfusion using the most recent data available. Because there is increased risk for sickle patients undergoing surgery, true informed consent is mandatory.
 
 References

Vichinsky EP, Haberkern CM, Neumayr L, et al: A comparison of conservative and aggressive transfusion regiments in the perioperative management of sickle cell disease N Engl J Med 222:206-213, 1995.

Riddington C, Williamson L Preoperative blood transfusions for sickle cell disease (Cochrane Review).Cochrane Database Syst Rev 2001;3:CD003149 http://www.update-software.com/abstracts/ab003149.htm

Haberkern CM, Neumayr L, Earles AN, Robertson S, et al. Cholecystectomy in sickle cell anemia patients:Report of 364 patients from the preoperative transfusion study. Blood 1995:86:142a

Vichinsky EP, Earles A, Johnson RA, et al: Alloimmunization in sickle cell anemia and transfusion of racially unmatched blood. N Engl J Med 322:1617-1621, 1990.

Holzman L, Finn H, Lichtman HC, et al. Anesthesia in patients with sickle cell disease: A review of 112 cases. Anesth. Analges. 48:566-572, 1969.

Goldman L, Cardera DL, Nussmbaum, et al. Multifactorial index of cardiac risk in noncardiac surgical procedures. New Engl. J. Med. 297:845-850, 1977.

Janik J, Seeler RA. Perioperative management of children with sickle hemoglobinopathy. J. Pediatr. Surg. 15:117-120, 1980.

Wilson ME, Williams NB, Baskett PJF, et al. Assesment off fitness for surgical procedures and the variability of anaethetists’ judgements. Br. Med.

Fullerton MW, Philippart AI, Sarnaik S, Lusher JM. Preoperative exchange transfusion in sickle cell anemia. J. Pediatr. Surg. 16:297- 300, 1981.

Metras D, Coulibaly AO, Ouattara K, et al. Open-heart surgery in sickle-cell haemoglobinopathies: report of 15 cases. Thorax 37:486-491, 1982.

Forrester K. Anesthetic implications in sickle cell anemia. J. Amer. Assoc. Nurse Anesth. 54:319-324, 1986.

Gibson JR. Anesthesi for the sickle cell diseases and other hemoglobinoapthies. Semin. Anesth. 6:27-35, 1987.

Turnbull JM, Buck C. The value of preoperative screening investigations in otherwise healthy individuals. Arch. Intern. Med. 147:1101-1105, 1987.

Bischoff RJ, Williamson A, Dalali MJ, Rice JC. Assessment of the use of transfusion therapy perioperatively in patients with sickle cell hemoglobinopathies. Ann. Surg. 207:434-438, 1988.

Esseltine DW, Baxter MRN, Bevan JC. Sickle cell states and the anesthetist. Can. J. Anaesth.35:386-403, 1988.

Ware R, Filson HC, Schultz WH, Kinney TR. Elective cholecystectomy in children with sickle hemoglobinopathies: Successful outcome using preoperative transfusion regimen. Ann. Surg. 208:17-22, 1988.

Macpherson DS, Snoe R, Lofgren RP. Preoperative screening: Value of previous tests. Ann. Intern. Med. 113:969-973, 1990. 

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Copyright © 1997 Sickle Cell Information Center
Last modified: December 05, 2001