Episode #211 Safeguarding Pediatric Patients with Sickle Cell Disease During Anesthesia: Expert Insights and Strategies

July 17, 2024

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Welcome to the next installment of the Anesthesia Patient Safety podcast hosted by Alli Bechtel.  This podcast will be an exciting journey towards improved anesthesia patient safety.

Our featured article today is from the June 2024 APSF Newsletter. It is “Preoperative Transfusion and Sickle Cell Disease in the Paediatric Patient” by Rahul Baijal, Priti Dalal, and Megha Kanjia.

Thank you so much to Megha Karkara Kanjia for contributing audio clips for the show today.

Patients with sickle cell disease are at risk for sickle cell crisis during surgery and anaesthesia if homeostasis is not maintained during the perioperative period. Triggers include the following:

  • Hypothermia
  • Hypoxemia
  • Hypovolemia
  • Infection
  • Acidosis
  • Pain
  • Stress

Paediatric patients with sickle cell disease are at increased risk for postoperative complications including acute chest syndrome, stroke, and 30-day mortality. The good news is that there are interventions that can help prevent sickle cell crisis including intravenous hydration, thermoregulation, and adequate oxygenation.

Here are the citations for our literature review today.

  1. Howard J, Malfroy M, Llewelyn C, et al. The transfusion alternatives preoperatively in sickle cell disease (TAPS) study: a randomized, controlled, multicenter clinical trial. Lancet. 2013; 381:930–938. PMID: 23352054.
  2. Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med. 1995;333:206–213. PMID: 7791837.
  3. Salvi PS, Solomon DG, Cowles RA. Preoperative transfusion and surgical outcomes for children with sickle cell disease. J Am Coll Surg. 2022;235:530–538. PMID: 35972175.

APSF is responding to the call to action to eliminate workplace violence.

We have created video-triggered workshop materials and tools designed to increase awareness, take perspective and stimulate conversation on responses within your workplace. The tools are ideal for integration into staff meetings, grand rounds, in-service training, and existing workplace violence curricula.

Please continue to return to this website as we add resources and use-case examples. Together we can eliminate workplace violence.

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© 2024, The Anesthesia Patient Safety Foundation

Hello and welcome back to the Anaesthesia Patient Safety Podcast. My name is Alli Bechtel, and I am your host. Thank you for joining us for another show. Our topic today is helping to keep paediatric patients with sickle cell disease safe during anaesthesia care. Patients with sickle cell disease have an increased risk of postoperative acute chest syndrome, infections, vaso-occlusive pain crises, and 30-day mortality. In addition, we must keep in mind that paediatric patients with sickle cell disease have a different perioperative risk profile from adults with sickle cell disease due to the cumulative effect of sickled red blood cells leading to end-organ dysfunction. For these patients, the preoperative transfusion plan should be patient-specific with considerations for the sickle cell genotype, baseline haemoglobin, disease severity, risk classification of the surgery, and history of prior surgical complications.

Before we dive into the episode today, we’d like to recognize Merck, a major corporate supporter of APSF. Merck has generously provided unrestricted support to further our vision that “no one shall be harmed by anesthesia care”. Thank you, Merck – we wouldn’t be able to do all that we do without you!”

Our featured article today is from the June 2024 APSF Newsletter. It is “Preoperative Transfusion and Sickle Cell Disease in the Paediatric Patient” by Rahul Baijal, Priti Dalal, and Megha Kanjia. To follow along with us, head over to APSF.org and click on the Newsletter heading. First one down is the current issue and then scroll down until you get to our featured article today. I will include the link in the show notes as well. We are so excited to have two of the authors here to discuss their article. Here is one of the authors now to help kick off the show.

[Kanjia] “I am Megha Karkara Kanjia, a Paediatric Anesthesiologist and Director of Quality Improvement for the Department of Paediatric Anesthesiology at Texas Children’s Hospital in Houston, Texas. We can all agree that across the world our systems are getting more volatile and uncertain while our patients are increasing in complexity.

In a perioperative world where we are always striving to do more with less, we use this as an opportunity to streamline our process for the sickle cell disease patient. Rahul Bajal and I started a local newsletter at our own hospital for the perioperative team with a goal of discussing and clarifying confusing perioperative guidelines that make for challenging issues in the perioperative period.

Sickle cell disease patients, especially in paediatrics, pose special challenges. We have long struggled with the optimization of these patients regarding preoperative hydration and transfusion, but we have tried our best to gather evidence-based outcomes and streamline processes through guidelines and best practices when possible.

Because sickle cell disease affects more than one out of 365 African American patients in the U. S., this is a common comorbidity with significant perioperative outcomes and deserves appropriate attention to ensure that we’re providing the best care to all of our patients. Sickle cell disease process has the potential to cause significant perioperative morbidity and complications, as well as decreasing efficiency and utilization of our operating rooms.

Therefore, optimization of these patients is crucial to best care for our patients. The details of the process can be found on our website at www. sicklecell. com. Best practice for preoperative transfusion can be ambiguous, so we took this opportunity to further dissect these recommendations from various sources.”

[Bechtel] Thank you so much to Kanjia for helping to introduce this topic. And now it’s time to get into the article. The authors highlight the current focus on delivery of safe and high-quality perioperative care with appropriate optimization during the preoperative period. Preoperative optimization is especially important for paediatric patients with sickle cell disease. Before we can discuss preoperative optimization though, let’s take a step back and do a review of sickle cell disease.

Sickle Cell Disease occurs when there is a substitution of valine for glutamic acid on the beta chain of haemoglobin. Normal haemoglobin is Haemoglobin A. Patients with sickle cell disease have Haemoglobin S. This occurs in about 1 out of 365 African American births. In the United States, there are about 70,000-100,000 people with sickle cell disease with about 2.6% of individuals of Mediterranean, Asian, and African origin affected. Patients may be homozygous, Heterozygous, or have an associated thalassemia. Carriers of sickle cell are heterozygous with about 40% of their haemoglobin as haemoglobin S and usually do not have clinical disease manifestations. Patients who are homozygous have 70-98% of their haemoglobin in the Haemoglobin S form. Red blood cells usually have a 120-day lifespan while sickled RBCs have a much shorter lifespan of about 10-20 days. Following triggers of hypothermia, hypoxemia, dehydration, infection, acidosis, pain, surgery, and physiologic stress, the red blood cells undergo polymerization leading to RBC deformity and sickling. This results in haemolysis and vaso-occlusion and a sickle crisis leading to pain crisis, acute chest syndrome, chronic organ damage, and musculoskeletal complications.

Now that we have done our review of sickle cell disease, let’s return to the perioperative space. Patients with sickle cell disease are at risk for sickle cell crisis during surgery and anaesthesia if homeostasis is not maintained during the perioperative period. Remember, the triggers include hypothermia, hypoxemia, hypovolemia, infection, acidosis, pain, and stress which may occur during surgery and anaesthesia care. Paediatric patients with sickle cell disease are at increased risk for postoperative complications including acute chest syndrome with an incidence of about 3%, stroke 0.2%, and 30-day mortality at 0.2%. The good news is that there are interventions that can help prevent sickle cell crisis including intravenous hydration, thermoregulation, and adequate oxygenation.

What about a preoperative blood transfusion? This requires weighing the risks and benefits in order to transfusion when necessary while avoiding any unnecessary transfusions. The most common paediatric procedures are low to moderate risk compared to higher risk procedures in adults. These procedures may include ear tubes, laparoscopic cholecystectomy or appendectomy, tonsillectomy and adenoidectomy, laparoscopic splenectomy, and umbilical hernia repair. Risks from blood transfusions may include alloimmunization, volume overload, and immunosuppression. Let’s look at the risk of alloimmunization which has an incidence in sickle cell disease from 7-58% and depends on the patient’s age, number of previous transfusions, and use of red cell phenotypic matching. Be on the lookout for any paediatric patients with a history of multiple alloantibodies, delayed haemolytic transfusion reaction, and/or haemolysis since they have an increased risk of complications following transfusion and should only receive a blood transfusion after careful consideration.

The authors highlight that there is controversy over appropriate preoperative transfusion strategies for patients with sickle cell disease. Haematologists and anaesthesia professionals must work together to optimize sickle cell patients and consider preoperative blood transfusion to decrease the percentage of sickled red blood cells with the goal to decrease the risk for perioperative complications.

Let’s take a look at the literature. Guidelines from the American Society of Haematology in 2020 suggest transfusion to a haemoglobin level of 9 or 10g/dL in all patients with sickle cell disease undergoing surgery with general anaesthesia that lasts for more than one hour, but with the available evidence there is still controversy about the preoperative transfusion strategy. Here is a study on paediatric patients with sickle cell disease undergoing surgery. The Transfusion Alternatives Preoperatively in Sickle Cell Disease trial included adults and children in a randomized controlled trial evaluating the incidence of perioperative complications in patients who received a preoperative transfusion or underwent an exchange transfusion depending on their starting haemoglobin level. In this TAPS study, there was a lower incidence of perioperative complications in patients who received a preoperative blood transfusion compared to patients who did not receive a transfusion. Patients randomized to the transfusion arm either received a simple transfusion to increase the haemoglobin to 10g/dL for patients with a starting haemoglobin less than 9g/dL or patients received a partial exchange transfusion to decrease sickle cell haemoglobin percentage to less than 60% in patients with a starting haemoglobin level greater than 9g/dL. Patients who received a preoperative blood transfusion had a significantly lower risk of postoperative acute chest syndrome and life-threatening complications. There was no difference in the incidence of postoperative pain crisis, hospital length of stay or readmission rates. Limitations of study included the small size with N of 67 and heterogeneous participants with 40 children and 27 adults so it does not fully answer our question for preoperative transfusion strategy.

Let’s look at another study, “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” published in the New England Journal of Medicine in 1995. This is a randomized multicentre study in adult and paediatric patients with sickle cell disease who received a simple transfusion to increase the haemoglobin level to 10g/dL compared to an exchange transfusion to decrease the haemoglobin S level to less than 30%. Surgical procedures ranged from cholecystectomy to head and neck surgery to orthopaedic surgery. Results revealed that transfusion-related complications occurred more frequently at 14% of patients in the exchange transfusion arm compared with 7% in the simple transfusion arm. Postoperative acute chest syndrome was found to be 10% in both groups. The authors concluded that a simple transfusion and exchange transfusion were effective in helping to prevent perioperative complications.

We are fast-forwarding to a more recent study published in 2022 in the Journal of the American College of Surgeons by Salvi and colleagues, “Preoperative Transfusion and Surgical Outcomes for Children with Sickle Cell Disease.” This study looked at data from the American College of Surgeons National Surgical Quality Improvement Program Paediatric Database related to sickle cell disease and blood transfusions. This was a retrospective cohort study of 357 paediatric patients with sickle cell disease undergoing low to moderate risk surgery such as laparoscopic cholecystectomy, splenectomy, or appendectomy. The results reveal no difference in the following outcomes between patients who were transfused preoperatively and those who did not receive a transfusion:

  • 30-day readmission rates
  • Surgical site infections
  • Wound Dehiscence
  • Pneumonia
  • Unplanned reintubation
  • Venous thromboembolism
  • Urinary tract infection
  • Postoperative transfusion
  • Cardiac arrest
  • Stroke
  • Sepsis
  • And death.

There was also no difference in the 30-day rate of surgical complications in both groups. The benefit of using this large database is being able to look closer at the data and further evaluation comparing preoperative haematocrit greater than 27.3% or less than 27.3% revealed no difference in postoperative sickle cell crisis in patients who received a transfusion compared to those who did not. In addition, preoperative transfusion was no associated with a reduced rate of postoperative transfusions.

We still have more to talk about so you will need to join us next week. The authors highlight that routine preoperative blood transfusion in children with sickle cell disease is not recommended.

If you have any questions or comments from today’s show, please email us at [email protected]. Please keep in mind that the information in this show is provided for informational purposes only and does not constitute medical or legal advice. We hope that you will visit APSF.org for detailed information and check out the show notes for links to all the topics we discussed today.

For more great resources related to anesthesia patient safety, we hope that you will head over to APSF.org and click on the Patient Safety Resources heading. One important resource is the video on Workplace Violence Prevention. Are you interested in stopping the toxic effects of healthcare workplace violence? Did you know that anesthesiology faculty had the largest proportions of reported harassment experiences in the past 12 months in 2022 in men 21.3% and women 52.6%. Plus, 71.6% of perioperative survey respondents of anesthesiologists, surgeons, CRNAs, CAAs, PACU nurses, and OR nurses report experiencing non-physical violence. The APSF is responding to the call to action to eliminate workplace violence. This resource includes video-triggered workshop materials and tools to increase your awareness, take perspective, and stimulate conversation on responses within your workplace. You can use these tools during staff meetings, grand rounds, in-service training, and existing workplace violence curricula. This is an important step towards eliminating workplace violence and an excellent resource that you can use at your institution.

Until next time, stay vigilant so that no one shall be harmed by anesthesia care.

© 2024, The Anesthesia Patient Safety Foundation