Notes from our webinar with John Bach, MD
by June Kinoshita, FSH Society
The best practices for managing breathing issues were the subject of the June 19, 2018, FSH Society webinar with John Bach, MD. Bach, of the Rutgers University New Jersey Medical School, is a leading authority on respiratory issues that can arise in individuals with neuromuscular conditions, including FSHD.
In the webinar, Bach recommended that every FSHD patient have respiratory function tested regularly, while noting that standard pulmonary function tests are almost useless for people with FSHD because “they do not measure cough flows, carbon dioxide levels, or lung volume recruitment measures.” FSHD patients should have vital capacity measured, but this must be done with the patient lying down, he said. This is “the most important measurement of all to determine if someone needs nocturnal noninvasive ventilatory assistance/support, or NVS,” he explained.
All FSHD patients should have an oximeter available to monitor their blood oxygen when they are ill, Bach said. He noted that these can be bought for less than $20 on Amazon. But if oxygen levels drop, “no one with FSHD should ever be given oxygen at home,” he cautioned. “It turns off the drive to breathe, causes carbon dioxide to go up, and patients often need to be intubated and attached to respirators as a result.” NVS and CoughAssist rather than oxygen supplementation should be used to renormalize oxygen levels, he said.
Sleep apnea is typically caused by the brain skipping a breath or the throat obstructing breathing, but according to Bach, sleep apnea is not the primary problem in FSHD. “In FSHD, the problem is hypoventilation caused by weak respiratory muscles (diaphragm), which results in nighttime waking and feeling unrested in the morning. Typical ‘sleep studies’ are not helpful because they do not measure carbon dioxide levels,” he explained. Instead, patients should have oxygen saturation and carbon dioxide (CO2) levels monitored, he said. CO2 can now be measured transcutaneously, which is noninvasive and painless.
In patients with hypoventilation, “CPAP (continuous positive airway pressure) is worse than useless,” he said. “BiPAP (bilevel positive airway pressure) is helpful if set at high enough inspiratory pressure, but the expiratory pressure is counterproductive.” He recommended a ventilator such as the Trilogy (made by Philips Respironics), which has more than 10 ventilation modes for NVS that include two for BiPAP.
A CoughAssist device, which he also recommends to keep airways clear and reduce the risk of pneumonia and respiratory failure, also needs to be set at high enough pressures, i.e., 40 to 60 cm H2O.
Bach asserted that ventilatory support via tracheostomy tube is never needed for patients with FSH muscular dystrophy and can be avoided by these noninvasive approaches. He noted that after surgery, when the breathing (intubation) tube is removed, an FSHD patient can have weakened breathing, which prompts doctors to put the patient back on the tube. He said that such patients can in fact have the tube removed (extubation) to full noninvasive ventilatory support (NVS)—a process in which his center specializes. Only three or four centers in the United States have this expertise, he noted. They are listed at www.BreatheNVS.com.
These are only a few examples of the wealth of information Bach offered during the webinar. You can watch the one-hour video below:
We refer readers to Bach’s website, www.BreatheNVS.com, for extensive, detailed information as well as a list of centers across the U.S. and internationally that specialize in noninvasive ventilatory support.