Do facilities managing sickle cell crisis need to have hematology (hematology) oncology services?

Facilities Managing Sickle Cell Crisis Should Have Access to Hematology Expertise

Facilities managing sickle cell crisis should ideally be designated sickle cell centers with hematology expertise available, though not necessarily hematology-oncology services specifically. 1

Requirements for Facilities Managing Sickle Cell Patients

Essential Components

• Facilities should have a nominated lead hematologist for adults or pediatrician/pediatric hematologist for children with sickle cell disease 1
• Access to automated or manual exchange transfusion services either on-site or through pre-arranged access is necessary 1
• Specialist hematology advice must be available, even if patients are admitted to hospitals without dedicated sickle cell expertise 1
• A multidisciplinary team approach is required with clear communication pathways between hematology, anesthesia, blood transfusion laboratory, and surgical teams 1

Organizational Structure

• National Quality Standards recommend that patients with sickle cell disease be managed within a clinical network with annual specialist review 1
• The lead clinician in the Department of Anesthesia should ensure local departmental guidance exists for perioperative care of sickle cell patients, developed collaboratively with the hematology team 1
• Surgical teams must clearly communicate the sickle cell diagnosis to all relevant teams throughout the patient pathway 1

Transfusion Services and Blood Management

• Blood transfusion laboratory must be notified about sickle cell patients with adequate time to prepare 1
• For difficult-to-transfuse patients, multidisciplinary team discussions should occur to develop detailed transfusion plans 1
• Blood should be available on-site on the day of surgery for all but the most minor procedures, even if transfusion is not planned 1
• Facilities should have capabilities to manage transfusion complications, including alloimmunization and hyperhaemolysis 1, 2

Perioperative Considerations

• Preoperative assessment should be conducted by senior anesthetic staff to evaluate need for:
  • Pre-operative transfusion 1
  • ICU bed requirements 1
  • Suitability for day surgery 1
• Patients should be scheduled early on operating lists 1
• Administrative cancellations must be avoided for patients who have received pre-operative transfusions 1

Management of Complications

• Facilities must be equipped to manage common sickle cell complications including:
  • Vaso-occlusive crisis requiring prompt analgesia 3, 4
  • Acute chest syndrome, which is a common post-surgical complication 1
  • Stroke, which requires immediate intervention 1, 4
  • Infections requiring aggressive antibiotic therapy 1, 3

Pediatric-Specific Considerations

• Children with sickle cell disease may require specialized pediatric facilities, particularly those:
  • Under 5 years old 1
  • With history of severe obstructive sleep apnea 1
  • With high transcranial Doppler flows who are at risk of stroke 1, 4

Common Pitfalls to Avoid

• Failure to communicate sickle cell status across the care team 1
• Inadequate preparation of blood products for patients with alloantibodies 1, 2
• Last-minute cancellations after pre-operative transfusions 1
• Lack of specialist hematology input for transfusion planning 1
• Delayed recognition of serious complications like acute chest syndrome or hyperhemolysis syndrome 1, 2

While comprehensive pediatric sickle cell centers may have specific requirements 5, the most critical element for any facility managing sickle cell crisis is access to hematology expertise and appropriate transfusion services, rather than specifically requiring hematology-oncology services 1.