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