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Sickle Cell: 10 Things Every EP Should Know about SCD

SCD is marked by recurrent acute events (pain, stroke, acute chest syndrome), many of which result in an ED visit. While evidence to guide the emergency care of SCD patients is limited, there are several important points we can glean from the available literature. As one of very few emergency physicians with an interest in SCD, I’ve compiled a list of ten things I think every emergency practitioner should know.

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  Pearls for Treating Patients with Sickle Cell Disease

As in all of emergency medicine, physicians need to think of the worst diagnoses first. Therefore, it is imperative not to assume that a patient with SCD who is in pain is suffering from a sickle cell crisis. If the pain is different from previous pain crises, broaden your differential diagnosis to include not only all the painful conditions that should be considered in all emergency patients but also sickle cell–specific conditions such as acute chest syndrome.

 For people in sickle cell crisis, specialized infusion centers offer far better care than the ER, study finds

People with sickle cell disease who were experiencing acute pain crises received far better care at specialized infusion centers than emergency departments, with faster access to pain medication and lower rates of hospital admissions, researchers reported Monday.

 Pain Management in Vaso-occlusive Sickle Cell Crisis

Sickle cell disease (SCD) is the most common genetic blood disorder in the United States, affecting an estimated 100,000 persons.1 Vaso-occlusive crisis (VOC) is the hallmark of this disease and the most common cause of hospitalization

 Sickle Cell in the ED: An Update

Sickle Cell Disease (SCD) is a complex medical entity, associated with significant morbidity and mortality. Patients with SCD suffer from acute and chronic pain, along with a seemingly endless list of additional complications (from hemolysis and sequestration, to aplastic crises and acute chest syndrome).

 What Is It Like to Visit the ER With a Pain Crisis?

“It sucks. You never know if you will have a good experience or if you have to fight to get the care you need.” – Survey respondent living with SCD.

Articles of Interest

Acute Chest Syndrome

Acute Chest Syndrome is one of the most common complications of Sickle Cell Disease (SCD).

ED Rounds – Sickle Cell Anemia

Normal vital signs do not exclude sickle – acute painful crisis.

Emergency Management of Sickle Cell Disease

Reserve supplemental oxygen for patients who are hypoxic and fluid boluses for patients who are hypovolemic. Use hypotonic fluids at maintanence for uncomplicated pain crisis.

Ketamine administration for acute painful sickle cell crisis: A randomized controlled trial

Early use of ketamine in adults with VOC resulted in a meaningful reduction in pain scores over a 2-h period and reduced the cumulative morphine dose in the ED with no significant drug-related side effects in the ketamine-treated group.

Laboratory Evaluation of Sickle Cell Disease in the ED

Unfortunately, there is no laboratory test that can definitively determine if a patient is having a pain crisis. The best approach is a good history and physical exam, and trusting patient reported pain.

POTD- Sickle Cell

Think about these dangerous causes and don't just dismiss as "drug seeker"

The Sickle Cell Patient – ED Management of Acute Complications

ACS is a leading cause of morbidity and mortality among patients with SCD.9 Patients may present with fever, cough, chest pain, hemoptysis, or dyspnea.

Three Things You Should Know about Sickle Cell Disease

All patients with SCD who are critically ill or deteriorating despite supportive therapy should be considered for exchange transfusion. It is of little consequence whether the patient has acute chest syndrome or a different life-threatening event.

Resources

LFTL

Always consider the presence of all types of sickle cell crisis, regardless of the dominant presentation.

Emergency Department Sickle Cell Care Coalition

In recognition of the need to improve the care offered to patients with sickle cell disease in the ED, the American College of Emergency Physicians in collaboration with multiple public, private and professional partners created the Emergency Department Sickle Cell Care Coalition.

StatPearls

The main clinical feature of sickle cell disease is the ''acute painful crisis,’’ which often requires hospitalization. The term "sickle cell crisis" is used to describe several acute conditions such as the vaso-occlusive crisis (acute painful crisis), aplastic crisis, splenic sequestration crisis, hyperhemolytic crisis, hepatic crisis, dactylitis, and acute chest syndrome.