From the Guidelines
Sickle cell crises are acute complications of sickle cell disease that occur when abnormally shaped red blood cells block blood vessels, causing pain and organ damage, and can be categorized into vaso-occlusive crisis, acute chest syndrome, splenic sequestration, and aplastic crisis. The most common type is the vaso-occlusive crisis, characterized by severe pain in the chest, abdomen, joints, or bones that may last for days to weeks 1. Other types include acute chest syndrome (lung involvement with fever, chest pain, and breathing difficulties), splenic sequestration (blood trapped in the spleen causing rapid enlargement), and aplastic crisis (temporary cessation of red blood cell production) 1. Management includes:
- Prompt pain control with NSAIDs and opioids (morphine 0.1-0.15 mg/kg IV every 3-4 hours or hydromorphone 0.015-0.02 mg/kg)
- Hydration with IV fluids at 1.5 times maintenance rate
- Oxygen therapy if oxygen saturation is below 95%
- Sometimes blood transfusions for severe cases, with the benefit of red cell transfusion for acute chest syndrome described in case series and observational studies 1 Prevention involves staying hydrated, avoiding extreme temperatures, managing stress, and taking medications like hydroxyurea (15-35 mg/kg/day) which increases fetal hemoglobin production and reduces crisis frequency 1. These crises occur because the abnormal hemoglobin S molecules in red blood cells form rigid polymers when deoxygenated, causing cells to become sickle-shaped and sticky, leading to blood vessel blockage and tissue damage from oxygen deprivation. Key points to consider in management include:
- Early recognition and treatment of acute chest syndrome, as it is one of the leading causes of death in patients with sickle cell disease 1
- The use of red cell transfusion, with simple or exchange transfusion, depending on the severity of the case 1
- The importance of multidisciplinary care, including specialist haematology clinics, to manage complications and improve survival 1
From the Research
Types of Sickle Cell Crises
- Vaso-occlusive crisis: the most common type of crisis, characterized by the occlusion of blood vessels due to sickled red blood cells 2
- Haemolytic crisis: a type of crisis characterized by the rapid destruction of red blood cells 2
- Sequestration crisis: a type of crisis characterized by the trapping of red blood cells in the spleen or liver 2
- Infarctive crisis: a type of crisis characterized by the death of tissue due to lack of blood supply 2
- Aplastic crisis: a type of crisis characterized by the failure of the bone marrow to produce new red blood cells 2
- Bone pain crisis: a type of crisis characterized by pain in the bones due to infarction or inflammation 2
Characteristics of Sickle Cell Crises
- Acute painful crisis: the hallmark of sickle cell disease, characterized by sudden and severe pain episodes 3
- Chronic pain syndromes: associated with avascular necrosis and leg ulcers 3
- Neuropathic pain: not well studied in patients with sickle cell disease, but has been modeled in the transgenic sickle mouse 3
- Frequency of vaso-occlusive crises: varies widely, ranging from a mean or median of 0 VOCs/year to 18.2 VOCs/year 4
Management of Sickle Cell Crises
- Treatment of acute painful crisis: typically involves hydration, anti-inflammatory drugs, aggressive analgesia, and possibly vasodilators 3
- Management of vaso-occlusive crisis: may involve individual care plans, innovative means of delivering initial opioids, and availability of a specialist unit for expert care 5
- Importance of early intervention: aborting the acute painful episode at the prodromal phase could potentially prevent or minimize tissue damage 3