Fast Five Quiz: Sickle Cell Disease Presentation and Diagnosis

Sophie M. Lanzkron, MD, MHS


September 24, 2021

An expert panel report from the National Heart, Lung, and Blood Institute (NHLBI) recommends annual screening for albuminuria, proteinuria, urine albumin to creatinine ratio, and serum creatinine in all patients with SCD after 10 years of age. The American Society of Hematology (ASH) has also developed a series of guidelines for screening for complications in patients with SCD.

According to the ASH guidelines, routine screening echocardiograms to identify pulmonary hypertension are not recommended for asymptomatic patients with SCD. However, considering the risk for cardiopulmonary disease in individuals with SCD, routinely taking a targeted history for signs and symptoms that might indicate a need for further evaluation, including consideration for a diagnostic echocardiogram, is advisable.

Routine screening PFT is not recommended for asymptomatic patients with SCD. Some indications for diagnostic PFT include wheezing or increased cough at rest, with exertion, or during episodes of acute respiratory infection; dyspnea at rest or with exertion that is either unexplained or increased from baseline; unexplained chest pain at rest or with exertion, or chest pain that is increased from baseline; unexplained or increased exercise limitation from baseline; and history of recurrent acute chest syndrome or pulmonary embolism.

Formal polysomnography is not recommended for asymptomatic patients with SCD. When symptoms justify further study, appropriate, validated tools (eg, Epworth Sleepiness Scale or Pittsburgh Sleep Quality Index) should be used to further identify patients who should be considered for formal sleep testing.

Learn more about diagnostic procedures in SCD.

For additional information, refer to a summary of the ASH clinical guidelines.


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