HWN Suggests

Feeling Sick(le) in the Chest

Patients may present with classic symptoms that include: Chest pain, dyspnea, fever, or cough. Clinicians should also consider ACS if a patient complains of back, shoulder, or abdominal pain, particularly in young children who may have difficulties localising their pain. Signs include tachypnea, tachycardia, and hypoxia. Sometimes these signs may be present, even while the patient does not appear overtly ill. About half of patients diagnosed with ACS are actually admitted with a different diagnosis such as a vaso-occlusive pain crisis before developing ACS. It is not uncommon for a patient to be admitted for a pain crisis, then develop ACS on day two or three of hospitalization. Other common…

read full article

Featured

 Sickle Cell in the ED: An Update

Acute chest syndrome (AChS) is an intimidating entity for many ED clinicians. It is the second most common reason for admission and the leading cause of mortality in SCD patients, with case fatality rates ranging from 4 – 25%. The majority of information regarding etiology, signs, symptoms, and outcomes is taken from two robust cohort groups: MACSS and CSSCD.13,14 The etiology of adult AChS is predominantly infectious, but often multifactorial, including fat embolism, and pulmonary thrombosis/embolism... there is a large predominance of atypical organisms, and given that the majority of these patients are functionally asplenic, they are also at significant risk for infection with encapsulated bacteria.

Articles of Interest

SGEM#166: Which febrile child with sickle cell disease should get a CXR?

Children with SCD presenting to the ED with fever and shortness of breath, tachypnea, cough, rales, or chest pain should receive a CXR due to high ACS rates. A higher WBC count or history of ACS in a child without one of those symptoms may suggest the need for a CXR. Prospective validation of these criteria is needed.

Acute Chest Syndrome

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

Acute Chest Syndrome

Acute Chest Syndrome is one of the most common complications of Sickle Cell Disease (SCD). Acute Chest Syndrome is a new pulmonary infiltrate, often accompanied by fever,chest pain, cough, wheezing, or tachypnea.

Acute Chest Syndrome (Sickle Cell Disease)

There are three major causes to consider: infection, fat embolization from bone marrow, and vaso-occlusion of the pulmonary vasculature by sickled RBCs. Can acute chest syndrome be prevented? Almost 50% of patients develop acute chest syndrome during a hospitalization for another illness, such as an acute pain crisis

Best Case Ever 38 Sickle Cell Acute Chest Syndrome

Sickle Cell Acute Chest Syndrome remains the leading cause of death in patients suffering from Sickle Cell Disease.

EM@3AM – Acute Chest Syndrome

Acute chest syndrome is the leading cause of death in sickle cell patients in the U.S. (12% mortality). Infection is most common cause of ACS ( C. Pneumoniae and RSV). A normal pulmonary exam is most frequently associated with ACS. ACS rapidly progresses to ARDS = admit to ICU level care

How I treat acute chest syndrome in children with sickle cell disease

Acute chest syndrome is (ACS) the second most common cause of hospitalization and challenges infection1 as the leading cause of sickle cell-related mortality in children. Still, management is largely determined by the experience of individual practitioners, and there are no conclusive randomized controlled clinical trials to guide therapy.

Sickle Cell Acute Chest Syndrome

Acute chest syndrome occurs in the majority of people with sickle cell anemia at some point during their lives. The overall approach is similar to that for any critically ill patient with pain. However, increased attention is required in this context, because it will be harder to strike the right balance. A pain-dose ketamine infusion may be extremely useful as an adjunctive analgesic agent which will not suppress respiration. This is especially useful in patients who may be less responsive to opioids.

Sickle Cell Disease: The Acute Chest Syndrome

The role of transfusion support is not clearly defined, although there are sporadic case reports of rapid reversal of chest x-ray findings and symptoms immediately post transfusion. It seems clear that there are at least two broad indications for transfusion.

Visual Dx Answer: a) Acute Chest Syndrome

Management requires hospitalization for hydration, oxygenation, intravenous antibiotics, pain control, and blood transfusions and, in some cases, exchange transfusion.

Resources

ALiEM

Acute Chest Syndrome occurs in both children and adults regardless of sickle cell disease phenotype. Treatment focuses on minimizing vaso-occlusion in pulmonary vasculature: Oxygen, IV fluids, antibiotics, blood transfusion Although pulmonary infection is less common in adults, cover with antibiotics since cannot distinguish from embolic cause in initial phase.

Life in the Fastlane

correct hypoxia. ensure adequate hydration, transfuse of symptomatic anemia targeting Hb >50 (avoid over-transfusion e.g. Hb100, due to risks of hyperviscosity or rebound if splenic sequestration).

StatPearls

The presentation of acute chest syndrome differs between pediatric and adult patients. In pediatric patients, who are more prone to infectious causes, symptoms such as wheezing, coughing, increased work of breathing, and fevers are commonly observed. On the other hand, adult patients often present with chest pain, pain in the extremities, dyspnea, or may exhibit signs of vaso-occlusive crises in other parts of the body (eg, priapism).