(http://emergencymedicinecases.com/wp-content/uploads/2015/08/sickle-cell-acute-chest-syndrome-300x225.jpg)A recent needs assessment completed in Toronto found that Emergency providers are undereducated when it comes to the Emergency Management of Sickle Cell Disease. This became brutally apparent to me personally, while I was researching this topic. It turns out that we’re not so great at managing these patients. Why does this matter?

These are high risk patients.

In fact, Sickle Cell patients are at increased risk for a whole slew of life threatening problems. One of the many reasons they are vulnerable is because people with Sickle Cell disease are functionally asplenic, so they’re more likely to suffer from serious bacterial infections like meningitis, osteomyelitis and septic arthritis. For a variety of reasons they’re also more likely than the general population to suffer from cholycystitis, priapism, leg ulcers, avascular necrosis of the hip, stroke, acute coronary syndromes, pulmonary embolism, acute renal failure, retinopathy, and even sudden exertional death. And often the presentations of some of these conditions are less typical than usual.

Those of you who have been practicing long enough, know that patients with Sickle Cell Disease can sometimes present a challenge when it comes to pain management, as it’s often difficult to discern whether they’re malingering or not. It turns out that we’ve probably been under-treating Sickle Cell pain crisis pain and over-diagnosing patients as malingerers.

Then there are the sometimes elusive Sickle Cell specific catastrophes that we need to be able to pick up in the ED to prevent morbidity, like Aplastic Crisis for example, where prompt recognition and swift treatment are paramount. A benign looking trivial traumatic eye injury can lead to vision threatening hyphema in Sickle Cell patients and can be easy to miss.

In this episode, with the help of Dr. Richard Ward, Toronto hematologist and Sickle Cell expert, and Dr. John Foote, the Residency Program Director for the CCFP(EM) program at the University of Toronto, we’ll deliver the key concepts, pearls and pitfalls in recognizing some important sickle cell emergencies, managing pain crises, the best fluid management, appropriate use of supplemental oxygen therapy, rational use of transfusions and more...

 

Written Summary and blog post prepared by Dr. Keerat Grewal, edited by Dr. Anton Helman, August 2015

Sickle Cell Pain Crisis
Drug seeking behaviour in emergency management of sickle cell disease

The majority of Sickle Cell Disease patients suffer real pain, but may not look uncomfortable because they have learned to adapt to a lifetime of chronic pain. In the ED, they may appear calm, preoccupied with their handheld device or casually chatting. The typical appearance of a non-Sickle Cell patient in pain may be absent.

The rates of true opioid addiction in Sickle Cell patients are low (< 5% of patients), and the literature suggests that emergency physicians under-treat pain in sickle cell patients.

Our experts recommend that unless there is clear evidence the patient does not have Sickle Cell Disease, take the patient’s complaint seriously and use analgesics aggressively.

 
Sickle cell pain crisis is a diagnosis of exclusion

* Sickle Cell Disease has been described in all races. Do not assume that a patient does not have sickle cell disease just because they have a light skin colour.

* Ask patients whether this is their usual pain or not; if the pain is different from previous pain crises, broaden your differential diagnosis to include not only all the painful conditions that we consider in all emergency patients but also the sickle cell-specific conditions such as acute chest syndrome.

* The diagnosis of a pain crisis is a clinical diagnosis - no laboratory test will reliably determine whether the patient is suffering from a pain crisis or not.