Sickle Cell Disease & Pain Discrimination

Pain is one of the top five reasons people seek immediate medical care in the Emergency Department. The assessment of pain includes the following: time of onset, location, duration, temporal pattern, character and quality of the pain, aggravating factors, alleviating factors, associated symptoms and impact on sleep or mood.

For patients with Sickle Cell Disease, the consistent answers to these questions alone may be off-putting and frustrating to Healthcare Providers who through a complete lack of understanding of the underlying Pathophysiology of the etiology of the pain.

Vaso-occlusive pain crisis occurs when unforeseen factors cause sickled red blood cells to become trapped within the patient’s veins. This entrapment in the veins is caused by deoxygenated red blood cells mostly in the postcapillary veins. The presence of hypoxia causes the already sickled cells to become more “sticky” and the cells further adhere to the postcapillary vein walls.

This blockage creates pain and swelling of the blood vessel. Because these processes are not visible to the naked eye, the corresponding pain assessment by Sickle Cell Anemia patients is frequently vague, mostly with no focal location, is often described as “all over” no distinct onset, temporal pattern, character, aggravating factors, alleviating factors and always has a substantial impact on sleep and mood.

This Clinical “mismatch” of how pain is both assessed and treated, causes Healthcare Professionals to create doubt and mistrust of the patient. This is universal, regardless of Sex and Ethnicity. However, in certain settings- the Sex of the patient, the type of complaint and the race of the patient will heighten the mistrust from the provider. In Sickle Cell Anemia patients, the frequency of Emergency Department visits with the Chief Complaint of pain is viewed as more legitimate if the patient exhausts the outlined Emergency Department management protocol and agrees to be hospitalized to obtain pain relief.

The goal standard of treatment for Sickle Cell Crisis is not narcotics, but vigorous hydration that is augmented by narcotics for immediate, but temporary relief.

Patients with chronic diseases that require frequent hospitalizations obviously undergo a significant amount of phlebotomy procedures and installation of Intravenous Catheters. This now creates a type of iatrogenic cause of the patient’s trepidation, fear, and disdain when presenting to the Emergency Department. These patients undoubtedly, have poor veins due to scarring and require multiple attempts to obtain blood and insert an Intravenous Catheter.

Often, the patient has to undergo, a slightly more aggressive placement in the External Jugular Vein of the neck to obtain intravenous access. The patient’s frustration with their condition is often read as aggression by the Medical Staff, which then unknowingly creates Avoidance behavior.

Unlike an acute musculoskeletal injury, the pain of Sickle Cell Disease is not alleviated by the administration of a single Intramuscular injection. The duration of treatment of a patient within the Emergency Department further frustrates the Staff which creates its own prejudice, as the Staff attempts to “determine” when pain relief should occur, based on the dosages and time intervals medications have been administered to Sickle Cell Anemia patients.

The combination of misperceived notions about the ubiquitous reaction to pain medications coupled with a disease process the produces pain complaints without physical deformities continues to widen the bridge of Provider-patient prejudice. Unfortunately, despite the availability of Sickle Cell Disease pain treatment guidelines, individuals with Sickle Cell Disease report undertreatment of pain and poor interpersonal treatment in Healthcare settings.

Reference:

Haywood Jr. C., et al. Perceived discrimination in health care is associated with a greater burden of pain in sickle cell disease. Journal of Pain Syndrome Management. November 2014. Volume 5. Issue 48. 934-43.
Mathur, Vani A., et al. Discrimination in Health-Care Setting is Associated with Enhanced Laboratory Pain Sensitivity in Sickle Cell Disease. The Clinical Journal of Pain. December 2016. Volume 32. Issue 12. 1076-1085.