It had been several years since I last saw my pulmonary/sleep medicine doctor. Part of the reason was that he was inconveniently far away -- an hour's drive. Fortunately, I learned that our local clinic, just 20 minutes away, was now affiliated with the group. Not only that, the local clinic had also established its own sleep lab where my new sleep study could take place. I made an appointment with one of the new doctors.

 

The new doctor was excellent and was impressed that I have been using noninvasive nighttime ventilation since 1988. 

 

My new pulmonologist ordered a sleep study and suggested I try an ASV (adaptive support ventilation) machine during the study. I've always used an S/T machine but was willing to give ASV a try.

 

I had the one-night sleep study in June, and the technician tried a wide variety of ASV settings during the night. The ASV settings did not seem to work very well. I slept very poorly except for the last 90 minutes of the study. I assumed the right combination of settings had been found.

 

By early July, I had my new vent, a ResMed AirCurve 10 ASV. The first night I used it, I knew it was not going to work for me. It was horrible. I was starved for air and gave up after only 20 minutes. Fortunately, I still had my old Respironics Bi-PAP S/T. I talked with my RT the next day, and the two of us concluded that after 27 years on S/T machines, I should continue using that type of vent. One good feature of these new ResMed machines is that they are connected to the internet, so the RT could remotely monitor and retrieve data and see exactly what was going on.

 

The RT contacted the doctor, discussed the data and eventually convinced him that an S/T vent was best for me. I really appreciate how she persevered with the doctor on my behalf, because he is a big proponent of ASV. A couple of weeks later, I exchanged the ASV machine for a ResMed AirCurve 10 ST. It has been excellent. I can go online and view data about my device, including the amount of mask leakage, hours of usage, etc. I kept my old one, and it is a relief to know I have a backup if the new machine fails.

 

My current diagnosis is obstructive sleep apnea, sleep-related hypoventilation and hypoxemia. My AirCurve device is a capped rental. I am using the same mask and circuit that I used with my previous bi-level machines, with the exception that I now have heated tubing and a humidifier, neither of which I had ever used before receiving the AirCurve. I do not find that the humidifier makes much difference in my comfort, but perhaps I will appreciate it more when dry winter weather sets in.

 

The RT worked with the doctor and sleep lab to get the necessary documentation for Medicare coverage. From the time of my first appointment with my new pulmonologist until I received the S/T vent was about 10 weeks.

 

I was dreading the whole task of getting approved for a new machine, but a great doctor and RT cooperated to make it happen without much drama. A good RT can make all the difference. It's worth shopping around for an RT who really listens to you.

 

Jeff’s History; Honoring Dr. Celli

I contracted polio in 1955, when I was 4 years old. Initially, I was completely paralyzed and could not breathe on my own. I spent 3 months in an iron lung, and then transitioned to a chest cuirass until my respiratory system recovered to the point where I could breathe unassisted.

 

Altogether, I was hospitalized for nine months. Even while in the iron lung, I received physical therapy in an attempt to regain movement and muscle tone. I continued physical therapy for ten more years, and I eventually regained reasonable strength in my arms and shoulders, allowing me to walk with arm crutches and leg braces (KAFO -- knee-ankle-foot orthosis). I developed severe scoliosis, and as a result wore a back brace until my teenage years.

 

I went to college at the University of Illinois, and then worked in the broadcasting and publishing industries. While living in New Hampshire in the mid-1980s, I began waking up with horrible headaches and falling asleep at my office in the middle of the day. My primary care physician ordered lab work and found an elevated red blood cell count. He recognized this as my body's response to insufficient ventilation -- its attempt to compensate by making more red blood cells. He referred me to a sleep medicine doctor.

 

CPAP had by then become a useful treatment for some types of nocturnal breathing problems, so my first sleep medicine doctor ordered two separate sleep studies for me to try CPAP. It did not help, and meanwhile my symptoms grew steadily worse. What the doctor (and I) did not know was that some of my problems were an indication of post-polio syndrome (PPS). The medical community was just recognizing that PPS manifests in some polio survivors as a progressive weakening of the muscles affected by the original onset of the polio. In my case, it was my breathing.

 

I learned that my nighttime breathing grew very shallow until my body was desperate for air. At that point I would become nearly awake, gasp for air, then fall back into an uneasy sleep until the next apnea episode, often only a few minutes later. This cycle repeated all night long, resulting in a very poor night's sleep, plus a high level of CO2 in my blood, which caused the headaches.

 

Since CPAP did not help me, my sleep medicine doctor said he had nothing to offer except a tracheostomy and a ventilator. To his credit, he said he knew of a doctor in Boston -- 90 minutes away -- who was having some success with noninvasive ventilation (NIV). So, in 1988, I was referred to Dr. Bartolome Celli at Boston University.

 

Dr. Celli determined that NIV might help me. I checked into Boston University Hospital for a three-night trial of NIV. On the first afternoon, I was set up with a LP-6 volume ventilator and a nasal mask for a 15-minute test just to see if I could tolerate the therapy. Not only did I tolerate it, but I fell into the deepest sleep I had enjoyed in three years. It was fantastic!

 

Over the next two days, I trialed various pressures and breathing rates until a good combination of settings was found. Each night's sleep was better than the last. I went home with the LP-6 and an off-the-shelf nasal mask.

 

The LP-6 ventilator was a huge machine, weighing in at about 40 pounds, but I used it for 3 years. As soon as bi-level machines became available in the early 1990s, Dr. Celli switched me to a Respironics Bi-PAP S/T vent that weighed about 8 pounds -- a major improvement in convenience. I need the S/T model so that the Timed feature will provide a breath when my own body fails to do so, which happens constantly while I am asleep. I've used a variety of ever-smaller S/T ventilators until I now have a ResMed AirCurve 10 ST that weighs a little over 2.5 pounds.

 

Dr. Celli recognized that the standard nasal masks of the day were not designed for long-term use. My face developed sores after only one night with the standard mask. The answer came from a member of Dr. Celli's team, a reconstructive dental surgeon, who made a custom mask for me from silicone and fiberglass, molded to fit my face. The mask fit perfectly, without pressure points or leaks.

 

I ended up using it for almost 20 years. That's how perfect and well-made it was. The custom mask finally fell to pieces, and although mask technology has advanced a great deal, to this day I have never found an off-the-shelf mask that fits and works as well as that custom mask.

 

For the past few years, I have used a Respironics Wisp nasal mask. The Wisp is the best of a rather poor assortment of masks I have tried. I am not impressed with any of them. They simply do not measure up to my original custom mask.

 

I saw Dr. Celli regularly for 16 years. I certainly would not have the quality of life I enjoy today were it not for his superb care and pioneering work. He is a kind and caring person and a strong advocate for noninvasive ventilation.