What is the best next step in managing a 17-year-old girl with homozygous sickle cell disease (SCD) presenting with chest tightness and dry cough, worsened at night, and a history of acute chest syndrome and vasoocclusive episodes, currently taking hydroxyurea (hydroxycarbamide), folic acid, and a multivitamin?

From the Guidelines

The best next step in management for this 17-year-old girl with sickle cell disease presenting with chest tightness and dry cough for 4 weeks is spirometry (option G). Spirometry should be performed first because the patient's symptoms of nighttime cough and chest tightness suggest possible asthma or reactive airway disease, which is common in patients with sickle cell disease 1. Spirometry is a non-invasive test that can evaluate for airflow obstruction and assess lung function, providing valuable diagnostic information before proceeding to more invasive or radiation-exposing tests. The normal chest x-ray already rules out acute chest syndrome recurrence or obvious pulmonary pathology, making spirometry an appropriate next step. If spirometry reveals obstructive patterns, a trial of bronchodilators or inhaled corticosteroids might be warranted. Other options like CT scans, bronchoscopy, or echocardiogram would be more appropriate if spirometry results are abnormal or if symptoms persist despite initial management. The nocturnal pattern of symptoms strongly suggests reactive airway disease, making pulmonary function testing the most logical first diagnostic step.

According to the American Heart Association and American Thoracic Society guidelines, pulmonary hypertension is a significant concern in patients with sickle cell disease, and echocardiography can be used to estimate pulmonary artery systolic pressure 1. However, in this case, the patient's symptoms and normal chest x-ray suggest that reactive airway disease is a more likely diagnosis, and spirometry is a more appropriate initial test.

It's also important to note that the patient is already taking hydroxyurea, which is recommended for patients with sickle cell disease who have an increased risk for mortality 1. However, this does not change the fact that spirometry is the best next step in management for this patient's current symptoms.

In addition, the 2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR guideline for the evaluation and diagnosis of chest pain recommends prompt evaluation for patients with sickle cell disease who are experiencing chest pain, but it also notes that older adolescents and adults with sickle cell disease who present with chest pain and shortness of breath should be evaluated for AMI or myocardial ischemia 1. However, in this case, the patient's symptoms and normal chest x-ray suggest that reactive airway disease is a more likely diagnosis, and spirometry is a more appropriate initial test.

Overall, spirometry is the best next step in management for this patient, and it should be performed before considering other diagnostic tests.

From the FDA Drug Label

The FDA drug label does not answer the question.

From the Research

Patient Presentation

The patient is a 17-year-old girl with homozygous sickle cell disease, presenting with chest tightness and dry cough for 4 weeks, worsening at night. She has a history of acute chest syndrome and vasoocclusive episodes.

Relevant Studies

• The use of echocardiography in assessing pulmonary hypertension is supported by several studies 2, 3, 4, 5.
• Echocardiography can estimate pulmonary artery pressure and resistance, right atrial pressure, and derive indirect information about right heart structure and function 2.
• A study published in 2021 highlights the importance of echocardiography in the diagnostic algorithm of pulmonary arterial hypertension, allowing for the assessment of the right heart's structure and function, estimation of pressure in the right ventricle, right atrium, and pulmonary trunk, and exclusion of other causes of elevated pulmonary bed pressure 4.

Best Next Step in Management

Given the patient's symptoms and medical history, the best next step in management would be to perform an echocardiogram to assess for pulmonary hypertension, as supported by the studies 2, 3, 4, 5.

• Key points to consider in the echocardiogram include:
  • Estimation of pulmonary artery pressure and resistance
  • Assessment of right atrial pressure
  • Evaluation of right heart structure and function
  • Exclusion of other causes of elevated pulmonary bed pressure
• The echocardiogram can help identify potential causes of pulmonary hypertension, such as congenital heart disease or left ventricular diastolic dysfunction 2.
• The study published in 2023 provides guidance on differentiating acute pulmonary embolism and chronic pulmonary hypertension using echocardiography, which may be relevant in this patient's case 6.