What are the management strategies for a patient with Sickle Cell Disease (SCD) in critical care?

From the Guidelines

Management of sickle cell patients in critical care requires a comprehensive approach focusing on preventing and treating vaso-occlusive crises while addressing complications, as outlined in the guidelines from the Association of Anaesthetists 1. Immediate interventions should include:

• Aggressive pain management with opioids such as morphine (0.1-0.15 mg/kg IV every 2-4 hours) or hydromorphone (0.015-0.02 mg/kg IV every 3-4 hours), with patient-controlled analgesia often being optimal.
• Hydration is crucial, with IV fluids at 1.5 times maintenance rate using isotonic solutions like 0.9% normal saline.
• Oxygen supplementation should be provided to maintain saturation above 95%, even if the patient appears well-oxygenated, as hypoxemia can trigger sickling. Some key considerations in the management of sickle cell patients include:
• The importance of meticulous peri-operative care and transfusion to reduce complications, while avoiding unnecessary transfusion to minimize the risk of allo-immunisation 1.
• The need for a low threshold for admitting patients to the high dependency or intensive care unit after surgery, depending on the surgical risk and patient comorbidities 1.
• The use of cell salvage for peri-operative blood conservation, which may be effective in adults with sickle cell disease who have had a red cell exchange pre-operatively 1.
• The management of difficult to transfuse patients, which requires careful multidisciplinary team discussion and a detailed transfusion plan 1.
• The prevention and management of complications such as acute chest syndrome, stroke, and hyperhemolysis, which can be life-threatening if not recognized and treated promptly 1. Overall, the management of sickle cell patients in critical care requires a multidisciplinary approach, with careful consideration of the patient's individual needs and comorbidities, as well as the potential risks and benefits of different interventions.

From the Research

Management of Sickle Cell Patients in Critical Care

• The management of sickle cell patients in critical care involves the treatment of pain, which is a hallmark of sickle cell disease (SCD) 2.
• Opioids are commonly used for treating severe pain in SCD, but their use can be associated with liabilities and organ-specific pathological effects 2.
• Early and aggressive pain management is a priority in the emergency department, and emergency providers must also diagnose other emergent diagnoses in patients with SCD and differentiate them from vaso-occlusive crisis 3.

Fluid Management

• The choice of intravenous fluid (IVF) is important in the management of SCD, as some IVF formulations may induce localized microvascular obstruction due to alterations of sickle red blood cell biomechanical properties 4.
• Lactated Ringer (LR) solution may be preferred over normal saline (NS) for fluid resuscitation in patients with SCD and vaso-occlusive episodes, as it has been shown to have a small but significant improvement in hospital-free days and secondary outcomes 5.

Pain Management

• Intranasal fentanyl may be a viable alternative to intravenous morphine for the treatment of severe painful sickle cell crises in children, with a study protocol aiming to establish its non-inferiority 6.
• The primary endpoint of this study is the severity of pain scored at 10 minutes from administration of the study medications, with secondary endpoints including pain severity measured at various time points and the incidence of adverse events 6.