From the Guidelines
The next best course of action for a patient with sickle cell disease presenting with severe acute abdominal pain is to initiate prompt pain management with intravenous opioids (such as morphine 0.1 mg/kg or hydromorphone 0.015 mg/kg) while simultaneously beginning a thorough evaluation to identify the cause. This approach is supported by the most recent guidelines, including those from the Association of Anaesthetists 1, which emphasize the importance of prompt and effective pain management in patients with sickle cell disease.
The patient should also receive intravenous fluids at 1.5 times maintenance rate to address potential dehydration, which can worsen sickling, and supplemental oxygen should be provided if oxygen saturation is below 95% 1. Laboratory tests, including complete blood count, reticulocyte count, comprehensive metabolic panel, and blood cultures, should be ordered to assess for vaso-occlusive crisis, acute chest syndrome, splenic sequestration, or other complications. Imaging studies, such as abdominal ultrasound or CT scan, may be necessary to rule out gallstones or other abdominal pathologies common in sickle cell patients.
Key considerations in the management of these patients include:
- Prompt pain management with intravenous opioids
- Aggressive hydration to prevent dehydration
- Supplemental oxygen as needed
- Laboratory tests to assess for complications
- Imaging studies to rule out other abdominal pathologies
It is also important to note that patients with sickle cell disease are at increased risk of peri-operative complications, both sickle-related and non-sickle-related, and that careful planning, assessment of comorbidities, and multidisciplinary team working can help mitigate these risks 1. The American Society of Hematology guidelines for sickle cell disease also provide recommendations for transfusion support, including the use of immunosuppressive therapy in certain situations 1. However, the initial management of acute abdominal pain should focus on prompt pain control, hydration, and evaluation to identify the underlying cause.
From the Research
Management of Acute Abdominal Pain in Sickle Cell Disease
- The patient's acute abdominal pain rated 10/10 requires immediate attention and treatment with strong opioid analgesic drugs, combined with additional supportive care measures 2.
- Guidelines for managing acute pain crisis in sickle cell disease have been established, but reports indicate that these guidelines are often not followed, leading to suboptimal care in emergency departments and acute medical wards 2.
- The use of opioids, such as morphine, is common in managing severe pain in sickle cell disease, but it is essential to consider the potential organ-specific pathological effects of opioids and develop strategies to prevent these effects without compromising analgesia 3.
Opioid Use in Emergency Departments
- A study in Saudi Arabia found that emergency physicians face challenges in managing sickle cell anemia, including department overcrowding and the opioid epidemic, which restricts the use of Schedule-II opioid prescriptions 4.
- Another study in Saudi Arabia found that morphine is the most commonly used agent for acute pain management of sickle cell anemia in emergency departments, often accompanied by oral analgesics such as NSAIDs and acetaminophen 5.
- Patterns of opioid use in sickle cell disease vary widely, with some patients taking only short-acting opioids, while others take a combination of short-acting and long-acting opioids, and the dose of opioids correlates with the number of admissions due to vaso-occlusive crisis 6.
Potential Interventions
- Implementing a comprehensive opioid administration tracking system and standardizing protocols for opioid use in emergency departments may help improve patient outcomes 4.
- Developing individual care plans agreed between patients and treating specialists, and providing innovative means of delivering initial opioids to reduce time to first analgesia, may also improve care 2.
- Further clinical randomized-controlled trials are needed to determine the effectiveness of different agents, such as tinzaparin, isoxsuprine, and pethidine, in managing acute pain crisis in sickle cell disease 5.