Vasoocclusive pain episodes are common and usually occur at night with painful crises. Pain relief may be achieved with NSAIDs, CO inhalers, compression garments, and splinting. There should not be any expectation that analgesics are definitive treatment for vasoocclusive pain episodes. These treatments are helpful in reducing the frequency and severity of pain episodes. There is no treatment for the underlying causes of vasoocclusion. Appropriate investigations for evaluation of alternative medical treatments are needed.
There is no direct evidence that sickle cell disease vaso-occlusive pain episodes are responsive to vaso-occlusive agents and the data supporting this treatment are limited. Although there may be benefit, it is unclear whether these observations are clinically relevant, and prospective studies are needed to address these important questions. Vaso-occlusive pain episodes in sickle cell disease may be associated with vaso-occlusive pain syndromes experienced by people suffering chronic pain from vaso-occlusive pain, although such a link remains to be established.
There is a large rate of vaso-occlusive pain crisis in the sickle cell disease in the Unites states, with more than 1 in 3 patients affected. There is significant prevalence of chronic sickle cell disease, even for those who do not have acute pain crises. The prevalence for those without the acute pain crisis is lower than for those with it. The study found that males are more affected than females. There is a weak association between pain crisis and number of hospitalizations. The study suggests a need for more research and understanding of the pathophysiology to make improvements in management for the vaso-occlusive pain crisis.
The present case illustrates the signs of sickle cell pain, which is a common problem in patients. In patients with vaso-occlusive pain, the following are more likely to occur in those with sickle cell disease than in those without sickle cell disease: headache or migraine, abdominal pain or cramp, fever, low blood pressure (usually after exercise), swollen neck or leg veins, painful joints, or a sudden onset of pain with activity. In the United States, the National Center for Education and Economics of Health (NCEH) classifies vaso-occlusive pain episodes into 2 types A and B. Type A pain episodes have no underlying medical cause, and are usually associated with sickle cell disease.
Increased erythropoietin production was suggested to induce an acute vaso-occlusive pain episode in this patient. There was also the possibility of post hoc reperfusion of vascular spasms.
The occurrence of a sickle cell crisis was the same in both groups. Vaso-occlusion pain occurs in sickle cell disease patients but not controls, is reversible with oxygen therapy, and more severe in SCD with a higher haematocrit.
There is very limited evidence to support or refute clinical practice guidelines. There is more need for randomised controlled trials to compare active with placebo treatments for vaso-occlusive pain in sickle cell disease patients.
These data suggest that treatment with inclacumab may lead to reduced numbers of circulating endothelial cells, and thus a more potent state with respect to VOC.
We discuss the possible risks of a sudden onset of pain in sickle cell disease patients; the possibility of a diagnosis of acute stroke in those with prior history of stroke; and suggest that early recognition and prompt intervention are mandatory.
These data suggest that a very low frequency of vaso-occlusive pain episodes is a common event in nsickle cell disease and suggest that it is typically treated by blood transfusion. Incacumab therapy does not affect vaso-occlusive episodes per se but may be effective in certain patient subgroups for which this clinically important complication occurs.
Compared with erythropoietin, which is often used in clinical practice, rINFN-CIII has a favorable safety profile and results in higher hemoglobin levels while maintaining comparable numbers of transfused units. There have been no safety issues of note associated with rINFN-CIII use. The current trial results suggest that INFN-CIII is safe and effective for treating patients with transfusion-independent vaso-occlusive pain crises in SCD.
A quarter of patients randomized to receive either placebo or inclacumab alone required the use of an additional systemic treatment, but the majority of the patients needed an additional systemic therapy to treat an adverse event.