Originally published at Pediatric EM Morsels on July 26, 2013. Reposted with permission.

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We have discussed previously (in Splenic Sequestration – Be a Spleen Detective!) how being vigilant for “badness” is paramount when caring for patients with sickle cell disease. Often, the dreaded complications that we are taught to look for don’t present to us in the Emergency Department, but develop and evolve during the hospitalization that began with the patient seeing us in the ED. Our skill can help the child in distress, but our vigilance can detect the evolving Acute Chest Syndrome and perhaps even prevent it.

Acute Chest Syndrome

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

of Sickle Cell Disease (SCD). Pulmonary complications (Acute Chest Syndrome and Pulmonary Hypertension) are the most common causes of death in patients with SCD. Some estimate that Acute Chest Syndrome accounts for 25% of all SCD- related deaths.

in patients with SCD. Acute Chest Syndrome is a new pulmonary infiltrate, often accompanied by fever,chest pain, cough, wheezing, or tachypnea.

Causes of Acute Chest Syndrome

Pulmonary Infection Most common agents are atypical bacteria and viruses. Streptococcus pneumoniae is also a prevalent culprit Incidence of Acute Chest Syndrome has actually declined since conjugated 7-valent pneumococcal vaccine. Unclear of whether there is a causal relationship… but a lower incidence is still a good thing! Fat Embolization from Bone Marrow Vaso-occlusive crisis involving multiple bones, especially pelvis and femur, leads to infarction and edema of the marrow. The marrow then undergoes necrosis, leading to its contents (like fat) being released into the bloodstream. Vaso-occlusion of the pulmonary vasculature by Sickled RBCs

These, and other factors, all play a role in the downward spiral of Acute Chest Syndrome

As the lung becomes involved, V/Q mistmatch develops leading to hypoxemia, which then leads to more sickling of RBCs, accelerating the process.

Pain also leads to chest wall splinting, hypoventilation, and atelectasis, further increasing the V/Q mismatch.

Reactive-airway disease, which is present in up to 53% of children < 10yrs of age, can further accelerate this process.

of children < 10yrs of age, can further accelerate this process. Over-sedation from narcotics can also lead to hypoventilation and V/Q mistmatch.

Clinical Appearance of Acute Chest Syndrome

Unfortunately, Acute Chest Syndrome may present with severe respiratory distress. In the patient who has increased work of breathing, but doesn’t yet require intubation, consider noninvasive ventilation early to help improve V/Q mismatch.



More often, Acute Chest Syndrome develops within the first 1-3 days of a Vaso-occlusive crisis. Almost 50% of patients diagnosed with Acute Chest Syndrome develop respiratory complication during hospitalization for another diagnosis, such as pain crisis.

of a Vaso-occlusive crisis.

Fever is the most common presentation of Acute Chest Syndrome. Acute Chest Syndrome needs to be considered when evaluating a patient with SCD and fever.

Cough is the second most common presenting symptom. Acute Chest Syndrome often presents with cough and fever Even without initial sickling, a pneumonia leads to hypoxemia which can lead to a secondary Acute Chest Syndrome. Pneumonia in a patient with Sickle Cell Disease and Acute Chest Syndrome are often clinically managed similarly… so consider them the same!

Chest Pain may be present, but chest pain is less commonly seen in children than in adults with Acute Chest Syndrome.

in children than in adults with Acute Chest Syndrome. Physical Exam findings are less reliable – 35% of children with Acute Chest Syndrome have initially normal exams.

Risk Factors for Acute Chest Syndrome

{from Chang, 2013}

History of Acute Chest Syndrome (Adjusted OR = 15.9)

ANC >/= 9×10^9/L (Adjusted OR = 2.4)

URI symptoms (Adjusted OR = 2.1)

Noncompliance with Penicillin (Adjusted OR = 3.2)

Hemoglobin < 8.6 g/dL (Adjusted OR = 1.6)

Hypoxemia < 93% on RA (Adjusted OR = 1.9)

Help treat Acute Chest Syndrome before it occurs!

Incentive Spirometry – help keep those small alveoli open!

PCA Pump delivered narcotics – to help avoid oversedation and hypoventilation!

Albuterol and bronchodilators!

Treat Acute Chest Syndrome aggressively once it is present!

Frequent reassessments is key, as the patient may decline rapidly.

Be prepared to manage the airway!

Provide and encourage good pulmonary toilet (bronchodilators, positive expiratory pressure, incentive spirometry)

Antibiotics (include azithromycin to cover atypical bacteria)

RBC transfusion Provide this in concert with the hematologist. Based on the patient’s hemoglobin level a straight transfusion or exchange transfusion may be required. More mild versions of Acute Chest Syndrome may not require initial transfusion, but this needs to be a part of the discussion with the hematologist.



Chang TP, Kriengsoontorkij W, Chan LS, Wang VJ. Clinical Factors and Incidence of Acute Chest Syndrome or Pneumonia Among Children with Sickle Cell Disease Presenting with Fever: A 17 year Review. Pediatr Emer Care 2013; 29: 781-786.

Crabtree EA, Mariscalco MM, Hesselgrave J, et al. Improving Care for Children with Sickle Cell Disease / Acute Chest Syndrome. Pediatrics 2011; 127: e480

Gladwin MT, Virchinsky E. Pulmonary Complications of Sickle Cell Disease. NEJM 2008; 359: 2254-65.