Chest
Volume 128, Issue 5, November 2005, Pages 3336-3344
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Clinical Investigations: RESPIRATORY SYMPTOMS
Factors Associated With Dyspnea in Adult Patients With Sickle Cell Disease

https://doi.org/10.1378/chest.128.5.3336Get rights and content

Objective: The aim of this prospective study was to determine the cardiorespiratory factors associated with dyspnea in patients with sickle cell SS-hemoglobin disease, with a specific interest in lung vascular involvement.

Measurements: Forty-nine patients (29 women and 20 men; mean [± SD] age: women, 29 ± 6 years; men, 31 ± 11 years) underwent direct evaluations (Borg scale evaluation during a 6-min walk test) and indirect evaluations (modified Medical Research Council [MRC]score) of their dyspnea, pulmonary function tests (PFTs) [spirometry, volumes, diffusing capacity of the lung for carbon monoxide (Dlco), diffusing capacity of the alveolar-capillary membrane, and pulmonary capillary blood volume measurements], echocardiography, and biological evaluation.

Results: Thirty-four patients complained of significant breathlessness (MRC score, > 1). Indirect and direct evaluations of dyspnea were correlated. PFT results depicted a very mild restrictive pattern (mean total pulmonary capacity, 86 ± 11% predicted) and an impairment of Dlco(mean Dlcocorrected for the degree of anemia, 69 ± 13% predicted). The statistical analysis demonstrated that dyspnea and exercise performance were closely linked to indexes of Dlcobut not with any echocardiographic or biological measure including anemia. Nevertheless, only approximately 25% of the variability was explained by these associations. Despite having a similar history of vasoocclusive crisis events, women had more severe anemia, dyspnea, decreases in Dlco(corrected for the degree of anemia), and a higher capillary blood volume (corrected for alveolar volume) than men.

Conclusion: Lung vascular disease contributes to dyspnea and the exercise limitation of patients with sickle cell disease. A sequential assessment of Dlcowould therefore constitute one of the objective functional end points for follow-up studies of these patients.

Section snippets

Patients

Biological evaluation, PFTs, and echocardiography are part of the regular follow-up at the Sickle Cell Disease Center at our institution. Inclusion criteria were as follows: sickle cell SS-hemoglobin disease (documented by high-pressure liquid chromatography), age > 18 years, the absence of the recent (ie, < 3 months) occurrence of a vasoocclusive crisis (VOC), acute chest syndrome (ACS), or blood transfusion, the ability to perform a 6-min walk test (6MWT), the absence of any associated

RESULTS

Fifty consecutive patients who were referred for PFTs were prospectively included during a 10-month period; one patient was excludeda posteriori since she had sickle-hemoglobin C anemia. The characteristics of the 49 patients are described inTable 1. The results of their functional evaluation are described inTable 2.

The MRC scores of the 49 patients are described inFigure 1. Despite a similar previous history of SCD, women, who were more anemic than men, complained of more dyspnea and fatigue

DISCUSSION

This study confirmed that adult patients with SCD during a mild phase of their disease can experience mild-to-severe dyspnea. This functional complaint was illustrated by a rapid breathing frequency. The exact prevalence of dyspnea in SCD patients is, to our knowledge, unknown but could be elevated; for instance, in the study by Miller and Serjeant,117 of 25 patients experienced mild-to-severe dyspnea. Furthermore, Lonsdorfer and colleagues2 noticed empirically that the degree of anemia

ACKNOWLEDGMENTS

This study is dedicated to the memory of Alain Harf.

References (0)

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This study was supported by l'Association Soutiens aux Actions contre les Maladies du Globule rouge.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).

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