Ability of pulmonary function decline to predict death in amyotrophic lateral sclerosis patients

Abstract

This study provides tools to calculate the increase in risk of death from a PFT decline. At an individual level, since each test showed some flaws, the use of a combination of PFTs for ALS respiratory monitoring is recommended.

A cross-sectional analysis of clinical evaluation in 35 individuals with mutations of the valosin-containing protein gene

“…The pulmonary function tests results suggested a compromise in the lung function due to myopathic involvement of the diaphragm and other accessory muscles of respiration. Because respiratory muscle compromise is an early sign of VCP disease, low values of FVC, FEV1, MIP and MEP may have prognostic value in reflecting the state of respiratory muscle weakness [32–34]. The strong correlations between pulmonary tests and functional studies indicating respiratory muscle weakness have been reported previously [63,64].…”

Cough peak flow decline rate predicts survival in patients with amyotrophic lateral sclerosis

“…However, CPF generally varies widely across subjects, and is influenced by many factors, such as age, sex, height, and weight. As previously reported, the decline rates of clinical parameters, including pulmonary function tests, are more sensitive and useful in clinical practice than the absolute values, particularly in patients with rapid disease progression . Bach et al .…”

“…Bulbar onset is also predictive of shorter survival . The rates of change in forced vital capacity (FVC), body mass index (BMI), and the revised ALS Functional Rating Scale (ALSFRS‐R) may have much more influence on the disease course of ALS than their values at the time of diagnosis . However, there have been few reports on the relationship between the rate of progression of bulbar dysfunction and survival in ALS .…”

“…The rates of change in forced vital capacity (FVC), body mass index (BMI), and the revised ALS Functional Rating Scale (ALSFRS‐R) may have much more influence on the disease course of ALS than their values at the time of diagnosis . However, there have been few reports on the relationship between the rate of progression of bulbar dysfunction and survival in ALS . CPF has been utilized as a simple measure for evaluating both bulbar and respiratory function, and we aimed to conduct an investigation on the prognostic significance of CPF and its rate of decline in patients with ALS.…”

Caring for the ALS patient today: Are we under‐utilizing our available clinical tools?

“…Attention on the maintenance of proper oral hygiene, aimed to reduce the risk of bacterial aspiration, cannot be over emphasized. Although individual respiratory measures used in clinic have had recognized limitations, a recent study suggests that the use of multiple PFTs might be most useful in predicting survival in patients with ALS …”