Perioperative management of patients with sickle cell disease
Myron Yaster MD
In the very early days of SPA, well before the development of the quality and safety committees and multi-institutional clinical trials groups like the pediatric difficult airway registry, Dr. Sal Goodwin of the University of Florida and the Nemours clinic in Gainesville, Florida, Dr. Tom Mancuso of Boston Children’s Hospital, Dr. Paul Firth of the Mass General, Dr. Chuck Haberkern of the Seattle Children’s Hospital and several others formed one of the very first Special Interest Groups (SIGs) in SPA which was devoted to improving the care of patients with sickle cell disease (SCD) using the best evidence based knowledge available at the time.(1-4) The SIG disbanded when the members felt that consensus had been reached and no further efforts were needed. After reading today’s PAAD I’m wondering of a redo may be a good idea.
Today’s article by Azbell et al.(5) is an excellent review is short and is well worth your time to read in its entirety if you treat these patients even only occasionally. Myron Yaster MD
Review article
Roberta C G Azbell, Sophie M Lanzkron, Payal C Desai. Current Evidence and Rationale to Guide Perioperative Management, Including Transfusion Decisions, in Patients With Sickle Cell Disease. Anesth Analg. 2023 Jun 1;136(6):1107-1114. PMID: 37205803
“When preparing a patient with SCD for surgery, there are many factors to consider. Intraoperatively, surgery may lead to hypotension, acidosis, hypoxia, frequent exposure to low temperatures, vasoconstriction, venous stasis, and increased stress, all of which can promote increased sickling and potentially induce vaso-occlusive episodes (VOEs). Additionally, patients with SCD have higher risks of perioperative infection due to functional asplenia, and increased risk of perioperative thrombosis as a result the underlying hypercoagulable state. Preoperative planning is required to help minimize the risks of perioperative morbidity and mortality.”(5)
Some key points
Always, and I do mean always, plan on consulting the patient’s hematologist before the day of surgery to plan your course of action.
Fluid management. Patients with SCD have decreased renal function and concentrating ability making dehydration and decreased vascular volume a set up for sickling. Avoiding prolonged preoperative fasting and even preadmission to provide IV fluids is common. What fluid to use? I would use/recommend a balanced salt solution like Plamalyte or Normosol. The article recommends the hypotonic solution D5 0.45 NS.
Temperature. Cold temperatures can precipitate a vaso-occlusive crisis. Thermoregulation including warm OR, forced air heat blankets and warmed fluids is necessary.
Perioperative transfusions. Preoperative transfusion is the key to therapy and reduces the perioperative risk of acute chest syndrome and stroke even in low to medium risk surgical procedures like tonsillectomy.(3,6) How much to transfuse? There is no difference between “aggressive transfusion” defined as targeting a hemoglobin of 10 g/dL and HbS <30% via exchange transfusion, or “simple transfusion” defined as targeting a hemoglobin of 10 g/dL irrespective of HbS percentage. Thus, simple transfusion to a post transfusion hemoglobin concentration of 10-11 g/dL is the way most folks treat these patients.(7)
Intraoperative ventilatory management. This was not discussed in the article. Sickling occurs when the patient’s Hemoglobin deoxygenates. Anything that shifts the Oxygen dissociation curve to the RIGHT should be avoided (and hemoglobin SS is already shifted to the RIGHT). Thus, these patients should routinely be treated perioperatively with supplemental oxygen. Further, this is one of those occasions were even I would recommend hyperventilation and permissive hypocapnia in order to shift the curve to the LEFT and thereby minimize desaturation.
Post operative management. There was no discussion in the article of what to do with these patients postoperatively. Should they be admitted to the hospital? Are they candidates for same day surgery? What do you do in your practice? Send your thoughts to Myron who will post in Friday’s Reader Response
References
1. Goodwin SR, Haberkern C, Crawford M, Lerman J, Mancuso T, Yaster M. Sickle cell and anesthesia: do not abandon well-established practices without evidence. Anesthesiology 2005;103:205-7.
2. Firth PG, McMillan KN, Haberkern CM, Yaster M, Bender MA, Goodwin SR. A survey of perioperative management of sickle cell disease in North America. PaediatrAnaesth 2011;21:43-9.
3. Vichinsky EP, Haberkern CM, Neumayr L, Earles AN, Black D, Koshy M, Pegelow C, Abboud M, Ohene-Frempong K, Iyer RV. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group [see comments]. NEnglJMed 1995;333:206-13.
4. Haberkern CM, Neumayr LD, Orringer EP, Earles AN, Robertson SM, Black D, Abboud MR, Koshy M, Idowu O, Vichinsky EP. Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Preoperative Transfusion in Sickle Cell Disease Study Group. Blood 1997;89:1533-42.
5. Azbell RCG, Lanzkron SM, Desai PC. Current Evidence and Rationale to Guide Perioperative Management, Including Transfusion Decisions, in Patients With Sickle Cell Disease. Anesth Analg 2023;136:1107-14.
6. Waldron P, Pegelow C, Neumayr L, Haberkern C, Earles A, Wesman R, Vichinsky E. Tonsillectomy, adenoidectomy, and myringotomy in sickle cell disease: perioperative morbidity. Preoperative Transfusion in Sickle Cell Disease Study Group. JPediatrHematolOncol 1999;21:129-35.
7. Chou ST, Alsawas M, Fasano RM, Field JJ, Hendrickson JE, Howard J, Kameka M, Kwiatkowski JL, Pirenne F, Shi PA, Stowell SR, Thein SL, Westhoff CM, Wong TE, Akl EA. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv 2020;4:327-55.