Wednesday, January 4, 2017

Respiratory Issue with SBMA

SMA News Today published an article on respiratory issues with patients with Kennedy's Disease. A portion of the article as well as a link to the full article and actual study results are shown below. Most of us are aware of this issue; especially the implications of pneumonia and KD.

SBMA Patients Prone to Develop Respiratory Problems Due to Reduced Muscle Strength, Study Says

Patients with spinal and bulbar muscular atrophy (SBMA) may be at risk of developing respiratory complications due to a decline in the levels of genes responsible for muscle strength, according to a new study. The authors assert that monitoring the breathing decline in these patients may help provide better clinical management of the disease.

The study, Decreased Peak Expiratory Flow Associated With Muscle Fiber-Type Switching In Spinal And Bulbar Muscular Atrophy,” was published in the journal PLoS One.

At more advanced stages of the disease, SBMA patients may develop dysphagia (difficulties in swallowing) and dyspnea (shortness of breath), as well as pneumonia and respiratory failure. Thus, the management of swallowing and respiratory function is indispensable for the long-term care of these patients.

However, respiratory difficulties associated with SBMA are not well characterized, which limits the knowledge of how these processes occur in patients and how they might be managed.

The objective of the study was to evaluate respiratory function of SBMA patients and identify the mechanisms underlying this condition, in comparison to patients with amyotrophic lateral sclerosis (ALS), another motor neuron disease in which dyspnea has been observed and well-studied.

The study included 40 SBMA and 25 ALS male patients, as well as 15 healthy individuals, in whom researchers evaluated respiratory function (by assessing the peak expiratory flow, %PEF, a measure of breathing capacity, and the forced vital capacity, FVC), ....

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