• People with PPS were likely to have good lungs but weak muscles. Even people with muscles so weak that they have no measurable ability to breathe have successfully used noninvasive ventilator support for up to 63 years.
   

• People with PPS should not ever need a tracheostomy. Rather, noninvasive support may be used.
   

• Conventional sleep management with oxygen and low span BiPAP (inspiratory pressure - expiratory pressure less than 10 cm H2O) instead of noninvasive support must inevitably result in respiratory failure and intubation for patients with progressive muscle weakness.
   

• Noninvasive mechanical support can provide full respiratory muscle rest along with ventilator support.
   

• People with muscle weakness as a result of polio should not use oxygen (unless there is another lung problem). Supplemental oxygen reduces be need to breath which causes carbon dioxide (CO2) to builds up. The high CO2 leads to major problems. Continuous CO2 monitoring is needed if oxygen is given to a PPS patient!
   

• The normal pulmonary function tests are designed to evaluate lungs and airways rather than muscle function. Poor muscle function is detected by CO2 measurement, spirometry (air capacity of the lungs) in both a seated and a prone position, cough peak flows and oximetry.
   

• A weak cough peak flow can lead to pneumonia because the cough flow is inadequate to clear airway mucus. A mechanical “Cough Assist” can help clear airways.
   

My goal for the appointment with Dr. Bach was to get better settings for the ventilator. Since I often can feel mucus in my airways that is difficult for me to clear by coughing, I was also interested in finding out about the Cough Assist.
 

I first contacted PHI to get contact information on Dr. Bach and then sent him an e-mail describing my problems. He replied with the suggestion that I make an appointment. He is located at Rutgers University Hospital, Newark, NJ, about 500 miles from where I live in West Virginia.
 

I had given my local pulmonologist some of Dr. Bach’s articles. When I decided to visit Dr. Bach, she was very helpful and referred me to him and sent along my records. We made an appointment when the weather was expected to be good and stayed at a hotel recommended by the hospital. The hotel even gave a “hospital discount” when I told them I was coming for a doctor’s appointment. The hotel was near Newark Airport and had an international clientele. It was fun to see the different styles of clothing. We were able to have dinner with some of my family who lived in the area the night before the appointment.
 

The appointment lasted about an hour. We discussed my condition. I had filled out the

“My Polio Life” form developed by Polio Survivors Network in the United Kingdom which allowed me to document the effect of polio over time. I think it helped speed the conversation. My pulmonologist’s office had emailed my records ahead of time, but they somehow got deleted or misplaced by Dr. Bach’s office. Fortunately, I had checked with his secretary just before we left and brought along a hard copy. ALWAYS TRAVEL WITH A HARD COPY! 
 

“In 1977, Childrens Hospital Los Angeles (CHLA) took a bold step forward and sent its first patient, an infant with Congenital Central Hypoventilation Syndrome (CCHS), home, where he was on a mechanical ventilator and cared for by his family. CCHS is a rare, lifelong condition where a person cannot breathe on his own due to a lack of nerve signal from the brain to the lungs to breathe. There are approximately 1,000 cases in the world and there is no cure.

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“Since then, CHLA has established itself as a leader in the field by having one of the largest pediatric diaphragm pacer programs in the world and one of the largest home mechanical ventilation programs in the world.... CHLA patient Jim Harrison is one of the patients who has benefitted from the program. Harrison, now a 29-year-old San Diego-based chartered financial analyst, was diagnosed at infancy with CCHS. At 18 months he had surgery for a diaphragm pacemaker, which receives electrical impulses from a breathing unit stored in a small backpack he wears during the day to his diaphragm to help him take 22 breaths per minute. At night, Jim takes the backpack off and connects to a ventilator via his trachea tube to help him breathe as he sleeps. The routine, which he has followed since he was a toddler, hasn’t deterred his desire or his ability to do whatever he wants.” 

June 20-23, 2018 at The Chase Park Plaza, St. Louis, Missouri, USA. Registration is open.