From the Guidelines
Individuals with Sickle Cell Trait (SCT) can experience complications that resemble sickle cell crisis pain, but this is rare and typically occurs under extreme circumstances. According to a 2018 study published in the Annual Review of Medicine 1, SCT can cause death during training and competition in athletes, with known precipitating factors including high temperatures, high altitudes, and deconditioning. The study notes that death due to SCT has a distinctive prodrome, including cramping, dyspnea, and muscle pain and weakness. Key points to consider include:
- SCT is present in 8% of black Americans and can be a cause of death during intense physical activity
- Precipitating factors for SCT complications include high temperatures, high altitudes, and deconditioning
- Recommendations for individuals with SCT include preventive measures such as hydration, rest, and awareness of acute medical strategies
- Individuals with SCT should be cautious when engaging in physical activity at high temperatures or altitudes, and should seek medical attention promptly if they experience unexplained pain or other symptoms. Some important considerations for individuals with SCT include:
- Staying well-hydrated to prevent dehydration
- Avoiding extreme physical exertion without proper conditioning
- Taking precautions at high altitudes to prevent complications
- Seeking medical attention promptly if symptoms occur, as this could indicate either an unusual complication of SCT or another medical condition requiring treatment.
From the Research
Sickle Cell Trait and Sickle Cell Crisis Pain
- Individuals with Sickle Cell Trait (SCT) are generally considered to be asymptomatic or have mild symptoms, but they can still experience some complications, including sickle cell crisis pain, although it is less common and severe compared to those with Sickle Cell Disease (SCD) 2, 3.
- Sickle cell crisis pain is typically caused by vaso-occlusions in the vascular bed of the bone marrow, leading to necrosis, edema, and increased pressure 2.
- The management of sickle cell crisis pain often requires analgesic therapy, which can include oral non-opioid analgesics, oral codeine, or IV morphine, depending on the severity of the pain 2, 4.
- Studies have shown that patients with SCD can experience severe acute pain and chronic pain, and that opioids have emerged as the standard of care for severe acute pain in SCD 3, 5.
- However, there is limited information available on the specific experience of individuals with SCT, and more research is needed to understand the relationship between SCT and sickle cell crisis pain.
Pain Management in Sickle Cell Disease
- Pain management is a critical aspect of caring for individuals with SCD, and it often requires a multidisciplinary approach, including the use of analgesics, hydroxyurea, and other therapies 3, 6.
- Hydroxyurea has been shown to reduce the frequency of vaso-occlusive crises and improve outcomes in patients with SCD, but its use in individuals with SCT is not well established 3, 6.
- Other therapies, such as L-glutamine, crizanlizumab, and voxelotor, have been approved for the treatment of SCD, but their use in individuals with SCT is not well established 3.
- Further research is needed to determine the best approaches to pain management in individuals with SCT and to develop effective therapies for this population.