Fast Five Quiz: Sickle Cell Disease Presentation and Diagnosis

Sophie M. Lanzkron, MD, MHS


August 17, 2023

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 guidelines for screening for complications in patients with SCD. The guidelines state that routine screening ECGs 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 ECG, is advisable.

Routine screening pulmonary function testing (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.

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

Learn more about diagnostic procedures in SCD.


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