Many of you have reached out to me and asked for advice on how to choose between two different ventilation devices or select between two different device brands.

We are so lucky to live in a time in the U.S. when so many choices exist for complex medical devices, including mechanical ventilation devices!  Many of the machines boast some amazing features that help us breathe our best.    

But when it comes to choosing the right bi-level device (i.e. BiPAP or home use, portable ventilator), it can quickly get complicated. There are many reasons why one would select one ventilation device over another.  Frankly, it seems to be a personal decision, and there is no right or wrong. It only matters that you seek the advice of your respiratory care team, and select the device that best meets your needs today and is likely to meet those needs as your Neuromuscular Disease progresses over time.  

To help with this decision making process and answer basic comparison type questions, the International Ventilator Users Network (IVUN) has done their homework! They maintain a thorough document known as the "Home Ventilator Guide." It's a great resource, and if you are at a crossroad as to which device to select or just new to the mechanical/assisted ventilation arena, I recommend this guide at http://www.ventusers.org/edu/HomeVentGuide.pdf.  

Dear Andrea,
I am new to using ventilation assistance, and I'm wondering how I should wash the re-usable supplies and things that are not frequently replaced by my DME. 

Thanks,
NIV Supply Newbie 

Dear Supply Newbie,
While my supplies cleaning method is not the only way and perhaps not even the best way, it's a method I've used since I began ventilation assistance in late 2013. I'm happy to share it with you but encourage you to ask around and hear what others are doing so that you may find a method that suits you best.

Our members of the Breathe with MD Support Group have a lot of supply cleaning ideas, so be sure to join that group if you have not already.  Then you can search at the top of the group for a keyword like "cleaning," and find all of our past discussions on cleaning supplies.  .

As for my method, once a week I gather all the supplies I used that week that are washable. This includes tubing used for my ventilator's night prescription and my CoughAssist; my mask and mouthpiece parts; and my humidifier water chamber. Unless I have been sick, every two weeks, I gather up my daytime prescription's mouthpiece ventilation tubing. (I only use my mouthpiece ventilation as needed.)  

For times when I have been sick or at least once a month, I will do a vinegar/water soak before the soapy water wash. The vinegar soak is one part white distilled vinegar and two parts water.  I don't like the smell of vinegar, so I prefer to rinse those supplies and then soak them in soapy water. This ensures there is no remaining vinegar smell. Whether I'm doing the vinegar soak or not, I'm careful to rinse everything thoroughly in warm water.  

​When the soak and rinse process is complete, I gather the supplies and hang my tubing to dry in my utility room near the clothes dryer and a floor register that circulates air. I place the short tubes and smaller parts on a thick, folded bath towel on top of my clothes dryer.  The heat from the in-use dyer seems to dry things faster in this area of my home.  You might have the same fast drying results placing your supplies in a room that gets a lot of sunlight.

With limited success, I have tried to dry tubing by folding it and wrapping it in bath towels gathered at the ends and center by large rubber bands. I placed those towel bundles into the clothes dryer on a low heat cycle for 30-45 minutes and tumbled to partially dry. Because I have multiples of each supply item, it's not imperative that my items dry quickly, so this drying option did not seem worth the effort to me. I usually allow 3-4 days for the wetter tubing items to thoroughly dry.      

I hope this gives you some ideas on how best to clean your own supplies.

Wishing you the best,
Andrea

What do you do if there is no NMD-knowledgeable pulmonologist nearby?​

Dear Andrea,
I don’t have a nearby pulmonologist who specializes in patients with Neuromuscular Disease. Will this local person understand my breathing issues once I tell them about my form of Muscular Dystrophy?

Sincerely, 
Need a Pulm

Dear Need a Pulm,
Without seeing and interacting with this pulmonologist, I cannot say if this physician will understand breathing muscle weakness in Neuromuscular Disease (NMD) or be receptive to learning about it from a patient. I personally attempted this, and the pulmonologist was unwilling to read the articles I shared, some of which were from peer reviewed medical journals that advised against what he was prescribing for me. With some physicians, the last thing they want is a patient who knows more about a medical condition than they do. I personally had to ask for a second opinion referral to another physician who specialized in the breathing issues of those with NMD. I honestly felt it meant the difference in my worsening and dying versus living and thriving.

If you choose to see for example a pulmonologist who specializes in asthma or COPD, the burden is on you to know what the appropriate interventions ARE and ARE NOT for evaluating and managing breathing muscle weakness. Pulmonologists with these specialties sometimes want to prescribe unventilated supplemental oxygen and/or CPAP, or simply corticosteroid inhalers. If for example you are experiencing underventilation (hypoventilation) and/or hypercapnia (elevated levels of carbon dioxide in the blood and lungs) at night and have struggles with a weak and/or ineffective cough, none of those interventions will help. 

In my case, I travel nearly three hours to a university based physician who works with a Neuromuscular Disease clinic. I do know some members of our Breathe with MD Support Group at https://www.facebook.com/groups/BreathewithMDSupportGroup/?ref=share have been successful in self-advocating to get the appropriate care from their local pulmonology practice. To do this successfully, it takes some education on your part, the courage to speak up for yourself and tell the physician what you have learned, and sometimes the ability to find and print materials to share with that physician. Breathe with MD, Inc. can assist you in finding relevant sources of printable information to share with a physician. Just private message us on this page, and a volunteer with our organization will assist you as quickly as possible. 

Best wishes for better breathing and stronger coughing,
Andrea

Are you prepared for a trip to the ER or a hospital stay? A follower asked what she should pack.

Dear Andrea,
I have suspected pneumonia and am going to the emergency room. I feel certain they will admit me. What should I take with me?
Thanks, 
Packing for the Hospital

Dear Packing,
While I am sorry to hear you may have pneumonia, it is a common scenario for those with muscle-wasting diseases. I have fortunately never been in this situation, so I reached out to my peers in the Breathe with MD Support Group at https://m.facebook.com/groups/breathewithmdsupportgroup, and they responded with excellent advice.

If you can contact the specialist you see for Neuromuscular Disease (NMD) related breathing muscle weakness, do so in advance. They may be able to do a “direct admit,” allowing you to bypass the emergency department, avoiding the germs, and reducing your wait. If direct admit is not possible, they may be able to call ahead and tell ER staff what to expect before your arrival.

If the hospital is a long distance from home or you have never been there, you should err on the side of over-packing. Take snacks, bottled water, any toiletries you use such as toothbrush and toothpaste, all medications, and all respiratory equipment (bi-level device or ventilator) with extra supplies. Small hospitals tend to not even know what the Philips Respironics CoughAssist is, so if you have that vital piece of equipment, by all means, take it.

If pre-admit is not possible, bringing your own pulse oximeter may be helpful to monitor your oxygen saturation while you wait. If you have any other medical devices such as a feeding tube, catheter, etc., pack the necessary medical supplies for that too. If you have a surge protector or extension cord, take them along. Many hospital rooms have limited electrical outlets. Don’t forget your wheelchair charger too! 

If you have a blanket, pillow, or stuffed animal that adds to your comfort, take that with pajamas and shoes or slippers. A change of clothes is never a bad idea. A copy of your living will, durable power of attorney, and a medical binder that includes articles about your NMD, recent pulmonary function test (PFT) results, etc. can also be helpful. 

The International Ventilator Users Network (IVUN) has a portfolio of four documents called “Take Charge, Not Chances” that you can save, fill out, print, and bring along. They are designed to assist with advanced preparation of an emergency, communication between patient and medical staff, and to help educate the medical staff. Find them athttp://www.ventusers.org/vume/.

And lastly, it never hurts to take along a well-informed advocate for your care. They can speak on your behalf and help to ensure a smoother hospital visit and/or stay. 

Wishing you a rapid recovery,
Andrea

Dear Andrea,
I was prescribed a CPAP, but I heard Muscular Dystrophy patients are supposed to use a BiPAP. What’s the difference?
Sincerely, 
PAP Confused

Dear PAP Confused,
A CPAP has one continuous positive airway pressure. That means the same pressure blows when you inhale and exhale. BiPAP or a bi-level device is one that has two different positive airway pressures, one higher pressure for inhaling (IPAP) and a second, much more reduced pressure for exhaling against (EPAP). The difference between the two numbers is called SPAN. 

Experts say span should never be low for those of us with neuromuscular diseases (NMDs), so there should be a large difference between the two pressure values. Some respiratory clinicians believe the difference should be 10 or more. Others prefer to set the difference as 50%. And still other experts believe the EPAP value should be set to zero, which requires use of a ventilator and what’s called “active circuit” setup. In all of this respiratory jargon, it’s important to understand that as your respiratory condition changes with your muscle weakness, these PAP (positive airway pressure) values should change too. A clinician who specializes in the breathing issues of those with NMD will understand and be able to guide you to the device and settings best for your level of function. 

Only a bi-level device assists with ventilation, the process of moving air into and out of the lungs. This process can be difficult for those of us with NMDs, as our muscles between the ribs and diaphragm weaken over time. 

Exhaling against a pressure that is strong enough to fill our lungs can be uncomfortable and difficult. That’s why the lowered EPAP of a bi-level device makes it more comfortable for us than using the one pressure of CPAP. When the IPAP and EPAP of a bilevel device are at an appropriate level, breathing muscle rest can be achieved overnight. If you have weakened breathing muscles, CPAP use can be exhausting, preventing breathing muscle rest.

Being prescribed a CPAP as opposed to a bi-level device is a common management error for breathing muscle weakness. You can find more common clinician mistakes compiled by Dr. John R. Bach’s team at https://www.breathenvs.com/most-common-mistakes in the section, “MOST COMMON MANAGEMENT MISTAKES.”

Wishing you breathing muscle rest,
Andrea