How does pain affect people with sickle cell disease?

Pain is the most common symptom that people who have sickle cell disease experience. Pain is also the leading cause of emergency room visits and hospitalizations. There are various types of pain that people experience including acute, episodic pain that comes and goes related to vaso-occlusive pain episodes and more chronic pain that is more continuously present. Sickled cells can block the blood flow to various parts of the body which contributes to pain. Over time, the way a person feels and responds to pain can also change, which leads to more chronic pain.

The burden of pain varies between individuals and can have a significant effect on the quality of life of people with sickle cell disease. Pain can start in early childhood, leading to missed days of school and inability to participate in normal activities and can continue to progress into adulthood. Up to 30% of adults with sickle cell disease can have chronic pain (1). While the use of hydroxyurea and more focus on adequate pain management have been important steps to addressing pain, this continues to be a major complication of sickle cell disease.

Curative therapy does not guarantee that pain symptoms will go away. In fact, there are some studies that show that pain continues to be a major burden of disease, in particular within the first one to two years after curative therapy. This is complicated by the fact that curative therapy in itself can lead to pain in a way that is not directly related to sickle cell disease.

We hope to understand more about how people experience pain before and after curative therapy. The COALESCE study will use surveys so people can report their pain before and after transplant in order gather more data on how pain changes after transplant.

References

1. Lee S, Vania DK, Bhor M, Revicki D, Abogunrin S, Sarri G. Patient-Reported Outcomes and Economic Burden of Adults with Sickle Cell Disease in the United States: A Systematic Review. Int J Gen Med. 2020;13:361-377. https://doi.org/10.2147/ijgm.S257340