Dear Andrea,
I got a BiPAP today, and I’m scared and nervous about it. What tips do you have to make this easier? —Nervous NIV Newbie, USA

Dear Nervous NIV Newbie,
I won’t pretend to have all the answers, but I know starting noninvasive ventilation can be scary and full of unknowns. First, know there will be an adjustment period. The most important thing for you to do is to be patient, determined, and know that the results of using NIV are all worth the challenges you may have adjusting to it. 

Good communication between NIV user and respiratory care provider is a must! The best situation is one in which a respiratory care company staffs a Registered Respiratory Therapist (RRT) who keeps in close contact with the NIV newbie, calling after the first night or two of use, being available to come to the home to adjust settings and to answer a variety of questions at your convenience, and to offer additional interfaces (masks and nasal pillows) for you to try. 

A huge factor in comfort is the mask or nasal pillows. There are dozens of options, and you should try several. Masks fit and adjust differently sitting up versus lying down, so try them both ways. In the Breathe with MD Support Group, we keep a folder of “mask selfies” that help to showcase masks group members like and why. 

Another area of challenge can be device settings. Sometimes they need to be adjusted for comfort or even completely changed from the original prescription. A good RRT will be patient and identify options for adjustments and changes, communicating with you and your ordering physician along the way.

Identifying the right level of moisture and heat needed on your device’s onboard or stand alone humidifier can bring challenges too. You may need to experiment to identify which humidification setting is best for you so that nasal passages don’t dry out or get too much moisture. Allowing your nasal passages to get too dry can trigger over production of mucus, something you want to avoid. Conversely, the tubing can become “water-logged,” if the humidity is set too high. 

Some find that starting to wear assisted ventilation while watching TV, reading, or playing video games helps them to adjust. They gradually increase their usage time until eventually they are wearing it for an entire night of sleep. Insurance will require you use the ventilation so many hours each day or they won’t pay your rental and other associated fees. Check with your supplier to identify your compliance requirements. (Most follow a four-hour per night requirement, and many also allow an adjustment period with less usage.)

Lastly, reach out to others who use NIV to ask for their advice. No one understands like your peers in the NMD community! That’s why your friends in the Breathe with MD Support Group at
https://m.facebook.com/groups/breathewithmdsupportgroup are a great resource. Know that we will help you through it; you CAN do this! 

Wishing you the best sleep breathing,
Andrea

One of our followers asked, “Does respiratory muscle weakness worsen over time?”

Just like our other body muscles weaken over time with progressive muscle wasting, our breathing muscles (those between the ribs and the diaphragm), lose strength over time. As they weaken, we take shallower breaths, filling our lungs with less and less air (Oxygen), eventually having areas of our lungs that Oxygen does not reach. Small areas of lung can collapse (called atelectasis).

The issue in neuromuscular disease (NMD) is with the muscles that move air into and out of the lungs, a process called ventilation. So that means the primary issue is not the lungs themselves. Weak respiratory muscles can cause under ventilation and weak or ineffective cough, and that can lead to secondary lung issues like atelectasis and pneumonias that can damage our lungs.

Because we don’t exert ourselves doing strenuous tasks, we don’t get short of breath or have symptoms to clue us in that something is wrong with our breathing. Instead, the symptoms like morning headaches, daytime drowsiness, fatigue, and anxiety when lying down can be subtle and explained away as something else.

Overtime, left untreated, respiratory muscle weakness can become emergent, particularly if you develop a respiratory infection. For example, you may have reduced forced vital capacity (the maximum amount of air you can exhale after taking your deepest breath) on pulmonary function testing. It may not be so reduced that you need ventilation assistance (via a bi-level device or ventilator), but when you become ill with a respiratory infection, your forced vital capacity that was borderline abnormal is now significantly decreased, and you need help with ventilation and mechanically assisted cough (i.e. Respironics CoughAssist). You may struggle to the point you must go to the hospital. 

This is where it can quickly become scary, and you may not be in control of the situation. You may not have previously read up on the potential breathing problems for those with NMD and the options for care, and in the hospital, you could be forced to make uninformed decisions.

That’s why with Breathe with MD, Inc. we stress that you should be as knowledgeable as possible about breathing muscle weakness, in advance of any significant problems developing. That allows you to have a plan in place and proactively address your respiratory care. With a little knowledge, you are equipped to advocate for the care that’s right for you!

If you believe your respiratory muscles are weakening, schedule a visit with a pulmonologist who specializes in the breathing issues of those with NMD. They are most often located in university healthcare systems. You may benefit from ventilation assistance, mechanically assisted cough, or if you’re already using these breathing muscle aids, your settings may need periodic adjustments.

Wishing you all the best,
Andrea