Acute Chest Syndrome in Sickle Cell

Adapted from Colton Hood's August 17th 2016 Grand Rounds lecture.

One of the most common and severe complications of sickle cell disease, clinically may resemble a pneumonia, and can develop suddenly, and is more common in patients with asthma or those with prior acute chest events. ​Typically presents with cough, shortness of breath, and rales accompanied by a new infiltrate on chest X-ray. 

Mortality of acute chest syndrome is 9% in adults, and can lead to pulmonary hypertension, right heart failure, and risk of sudden death. 

Source: http://www.ssajm.org/text.asp?2014/1/3/111/138930

Evaluate patients with sickle cell disease with acute onset of lower respiratory tract disease with or without fever for acute chest syndrome, including chest x-ray and pulse oximetry to measure oxygen saturation (NHLBI Consensus-Panel Expertise)

Typically presents with sudden onset pain and;

• cough
• shortness of breath
• rales
• children typically present with fever but adults are often afebrile

Signs of severe acute chest syndrome event include:

• multilobe disease
• inability to maintain oxygen saturation > 95% with supplemental oxygen
• increased difficulty breathing
• pleural effusions
• no distinctive laboratory features, but rapid decrease in hemoglobin below baseline value often occurs

Common causes of acute chest syndrome include:

• infection (viral, bacterial, chlamydia, or Mycoplasma)
• bone marrow fat embolism
• intrapulmonary aggregates of sickled cells
• atelectasis
• pulmonary edema
• may cause rapid respiratory failure or multisystem organ failure

Treatment of Acute Chest Syndrome

Evaluate your sickle cell patients with labs, chest imaging, pain control and FEV. Be sure you are getting a chest X-ray and pulse-ox on any sickle cell patient complaining of chest pain. Incentive spirometry is strongly recommended and is associated with decreased rates of pulmonary complications.

• Bronchodilators
• Pain Management: See our Sickle Cell Pain Control Post
• Incentive Spirometry
• Antibiotics
• Transfusion: Exchange transfusion in patients with persistent hypoxia <90% , or a simple transfusion in patients with Hgb <1.0 from baseline.
• Steroids: Be aware, increased risk of bounce-back, however may be utilized in patients with wheezing or asthma co-morbidity. 

Pain Control: See our Sickle Cell Pain Control Post

• Opioids are first line therapy with rapid reassessment within 20 minutes with titration to pain control: Morphine 0.1-0.15mk/kg max initial dose 10mg or Hydromorphone 0.02-0.05mg/kg, max initial dose 1.5mg
• Ketamine: dosage 0.3mg/kg given as a push or over 10 minutes
• Adjuncts: Hydration (risk of developing acute chest due to excess volume), Oxygen (for sats <93%), Ketorolac, 

Be aware and prevent the following:

• Over-hydrating
• Narcosis
• Splinting 
• Poor pain management