Living Long, Thanks to Iron Lungs, Cuirasses, and BiPAPs

It's 1949 and I'm eight years old, about to have my first experience with a life-saving respirator/ventilator: waking up encased in an iron lung in a gloomy polio ward at Los Angeles County Hospital amid other wheezing machines — startled, scared, and screaming, my arms turning useless, my familiar family-son school-boy life suddenly gone, and … well, you know what I mean.

IRON LUNGS TO ASSURE SURVIVAL

I would lie vacantly in that iron lung for the next eight (nine?) months before being moved by ambulance to a rustic airy ward at Rancho Los Amigos in Downey, California, to begin rehab and reentry. I don't recall how many months I spent there (four? six?), but my breathing and walking slowly recovered enough to leave the iron lung behind, sleep on a bed without mechanical respiration, walk around Rancho's playful grounds, visit with family, and begin thinking about going home. Which I did, after physical therapy stints for a few months at Kabat-Kaiser Hospital in Santa Monica and then Casa Colina in Chino.

Polio patients receiving treatment at Rancho Los Amigos, Downey, California, 1952. Courtesy WHO/March of Dimes.

Only a few good and many bad memories remain from those two/three years in that series of otherwise admirable institutions. Especially awful were the daily curare injections and hot-pack treatments that preceded arm and leg exercises at Kabat-Kaiser. For an hour or two, that barbaric curare depressed my breathing, which was already down to a third of normal lung capacity, barely sustained by polio-weakened chest muscles. I complained, but it took weeks before the staff ceased the curare in my case. Treatment there (not to mention its occasional TV coverage) was designed for polios with weak lower bodies, not ones like me with atrophied upper bodies.

Almost as bad a memory came a year or two later, now back home in good old Claremont (thank God!). A pre-teen growth spurt meant my spine began to curve, my torso tilting and twisting. So, a renowned orthopedist in Pasadena wrapped my torso in a plaster cast as a corrective. The problem was, as the cast dried and tightened, it compressed my ability to inhale as the hours went by, resulting in a hasty after-hours drive back to Pasadena for the solution, which consisted of cutting a large rectangular opening in the front of the cast, allowing my chest to pop out and my breathing to re-stabilize. The first time this happened was quite scary. It happened the next several times too, whenever the cast needed changing — a tiring back-and-forth routine (my poor parents!) for a year or two that became less scary but was always distressing. I still wonder whether the doctor could have come up with a better solution.

For the next twenty-five years — all across my Webb prep school, Pomona college, and Stanford grad-school years, and then into my early RAND work years — I never worried about my limited breathing capacity. It remained at about one-third normal, adequate for everyday life. It even kept me going at higher altitudes, such as during camping and hiking trips in California's High Sierras and multiple trips and stays in Mexico City.

Breathing while sleeping in Mexico City did eventually become problematic, so I scrutinized my normal daytime breathing pattern — hard-exhale, soft-inhale, especially when walking — and figured out how to reverse it after I lay down for a night's sleep. I developed an exaggerated gasping exercise to sharpen my inhale and soften my exhale. Decades later, I learned that a nosy school-mate in the adjacent bedroom — we were sharing an apartment while doing doctoral-thesis research from 1967-1969 — presumed I was indulging in something else. In fact, my breathing-reversal exercise worked well enough for me to sleep okay night after night thereafter.

RETURNING BRIEFLY TO AN IRON LUNG

Thus, until 1978, I never gave a thought to maybe needing mechanical ventilation again someday. But then, kablam, I suffered two pulmonary collapses, a few months (weeks?) apart. Post-polio syndrome (PPS) had started taking hold, though neither I nor anyone around me knew it at first.

I knew I was having inexplicable spells of heavy-headed drowsiness and wondered what to do. Get some advice? At the time, I was about five years into working as a researcher on US–Latin American and other national-security issues at RAND in Santa Monica (curiously, just across the street from where Kabat-Kaiser had been); and I had chosen Kaiser-Permanente for my medical plan. But I had never visited a Kaiser office, nor signed up for a doctor, much less a medical exam — I just felt it was an option I had in reserve for an emergency. I recall mentioning my drowsy-headedness to a fellow researcher on a terrorism project — he was a psychiatrist, maybe he'd know something medical about my symptoms — but he figured low-sugar might be the cause and suggested orange juice. Respiratory insufficiency never came up.

The first collapse downed me hours after returning from a trip to Mexico City, where my breathing had become problematic. Fortunately, my parents met me at LAX airport (had I phoned ahead?), just as paramedics began giving me oxygen. So, my parents took me to their home in Claremont, and kept an eye on the increasingly worrying signs. When I began talking strange and nodding off, they rushed me to the ER at nearby Pomona Valley Hospital, where I passed out and stopped breathing. I awoke in an ambulance, fitted to a mask and a pneumo bag, with my father, a musician, proving far superior at pacing my breaths than had the ambulance technician.

Destination: Kaiser-Permanente in Fontana, where I was soon intubated, on a hospital ventilator. I don't recall how many days. But no longer at Mexico City's altitude, I soon recovered enough to return first to my parent's home, and next to my own in Manhattan Beach. I suppose I went back to work after a week. It's all a hazy memory, presumably because, unbeknown to me and those around me, my O2 and CO2 blood-gas levels were surely out of whack, for I still kept wanting to nod off, especially when driving my car.

As I recall, I returned to Kaiser Fontana for a follow-up exam that was supposed to include an arterial blood-gas test. This might have revealed quite a lot. But, after various unsuccessful tries to insert a needle into an elusive artery, the staff demurred and sent me on my way — I seemed functional enough — with advice to find a Kaiser doctor near my home or workplace for further analysis.

Meanwhile, my brother was already calling around to find a pulmonologist who might know about polio and its effects. And he found one, Dr. Edward Oppenheimer – a wonderful physician who became crucial to my longevity – who, to our amazed delight, happened to be at Kaiser Los Angeles.

Dr. Edward "Tony" Oppenheimer, Japan, 2005.

My first meeting with him was easy-going, a general familiarization. He agreed to take me on as a patient. But, as I recall, that first appointment did not involve any tests, nor did it generate any urgency. Because I was good at appearing to be okay? Because when I walked and talked, increasing my respiration, my blood gases would re-normalize? I suppose we were going to schedule a follow-up exam before long. In any case, the key problem was yet to be identified and diagnosed: my body was losing its natural ability to breathe and sleep at the same time.

Early one morning a short time later, I awoke totally disoriented and out of breath, nearly unable to breathe at all. I don't recall how or who decided what next, but someone came over (my mother from 30 miles away?) and drove me to Kaiser's ER in Los Angeles. I remember being laid under an oxygen tent, which hardly helped, and then coughing up some hot thin acidic fluid that signaled infection. I figured intubation might be next — and was it ever.

For the next several weeks, I lay intubated in the ICU (excellent attentive staff) while Dr. O attended to my case steadfastly, first to fight the infection, then to seek a solution to my respiratory problems. He knew about polio and now began learning about PPS from colleagues at Rancho. But none of us knew what to do, apart from maybe giving me a trach, the option I dreaded.

Then movement in a new direction came into view: I gather I mentioned to my mother (well, scribbled on a writing pad, since I couldn't talk) that it felt like I might need an iron lung again. She, I learned years later, insistently passed my remark to Dr. Oppenheimer. And he, to everyone's amazement, quickly located and ordered one shipped into Kaiser's ICU.

It worked! Once the intubation was ended, god-awful phlegm suctioned out, and the relic iron lung started up, my recovery progressed rapidly in the ICU. I even got to walk around, take showers, and play chess with a jolly nurse.

But going home with an iron lung to sleep at night didn't seem practical. So, the good doctor had me ambulanced to Rancho for further assessment. A tracheostomy was still being contemplated; I'd become especially anxious about this whenever I'd see Rancho's famed Dr. Jacqueline Perry eyeing me or my medical records. But another option was also available at Rancho: fitting me with a small lightweight plastic cuirass (chest shell) attached via hose to a negative-pressure machine that functioned much like an iron lung.

CUIRASSES TO KEEP ME GOING

Cuirasses — I’d not known about these specialty items before — came in pre-molded shapes and sizes but could be heat-bent and trimmed to tailor-fit a non-standard chest, within limits. Fortunately, after working on a few edges, Rancho's staff modified one to fit my chest very well. This enabled me to transition from the iron lung to the cuirass almost immediately.

I was then returned to Kaiser's ICU for a brief period – just to be sure – where Dr. Oppenheimer and I were viewed as something phenomenal for a while – first because of the obsolete iron lung and now the newfangled cuirass, neither of which had ever been seen there before. I was released to my parents' care within the week. Not long afterwards, I returned to my own home and went back to work on an accommodative part-time schedule (whew, thanks to RAND).

That cuirass system, provided by Kaiser (or Rancho?) through LifeCare kept me going for the next fifteen years. It was easy to put on for napping and sleeping at home and was so effective that my 02 and CO2 levels went back to normal. Plus, with the cuirass packed in one box and the machine and hose in another, I could travel ably again around the US, Canada and Mexico. I even returned to Mexico City numerous times (where airport customs agents usually isolated my boxes until I could find them and explain).

However, in the early 1990s my torso's shape began to shift, such that by 1994 my cuirass ceased to fit well, resulting in increasingly poor ventilation and awful sleep problems for months. Making matters worse, I was unable to locate a replacement in my size or have a custom cuirass competently molded by an outside vendor. Thus began a distressing unwanted ordeal to switch to mask ventilation and a BiPAP machine in December 1994 — an effort Dr. Oppenheimer had actually begun suggesting months earlier.

TRANSITIONING TO A BIPAP

Shifting from a bulky technology that sucked my chest upward to a smaller lighter one that pushed air through my nose may sound like a no-brainer, but it really stressed my brain and my body. Back then, 1994, BiPAPs and nasal masks were still a new technology for home ventilation. The machines were basic and the masks ill-fitting and awkward. Moreover, few doctors and therapists were skilled at providing patients with a BiPAP on the best personal settings and a mask that fit without leaking or hurting.

I had a terrible time; the shift became a major ordeal. Because the cuirass had become so dysfunctional, I rushed to transition, trying to force the issue during the Christmas/New Year's break while taking time off from work. But after a couple weeks of uncertain ventilation, mounting sleep deprivation, and erratic progress despite efforts by all parties to give me good advice and set the machine up, including in Kaiser's pulmonary lab, I retreated back to a poorly-modified cuirass for several months.

Then I renewed my efforts to make the shift, this time using a gradual approach in which I tried to start a night's sleep on the mask, then switch to the cuirass after a few hours for the rest of the night. I also added afternoon catnaps to gain experience adapting to the mask. Eventually, over about a six-week period, I increased the time I could spend on mask ventilation.

Most importantly, I began experimenting with the BiPAP's settings on my own, seeking refinements through trial-and-error spread over months. Back then, fortunately for me, my BiPAP had five openly-accessible knobs for adjusting the settings.

Thus I found I needed a higher pressure when asleep than when awake; so I raised the inhalation pressure (IPAP) from its initial 10 (i.e., 10 cm. H2O) to 12 (higher years later). Meanwhile, after trying various breaths-per-minute (BPM) rates, I kept returning to 20-21 BPM, which is what I had used with the cuirass (later I would prefer 19 BPM).

Another knob let me choose the T (machine-timed), S (spontaneous patient-triggered), or S/T (combination) setting. My inhale was too weak to trigger the S, so the choice was between T and S/T. At the time my body preferred T, maybe because I was accustomed to it from my years on the cuirass, but also because on S/T, my every swallow or move triggered an untimely and unsettling blast of air into the mask.

Neither the directions that came with the BiPAP nor the therapists told me about the %IPAP knob. Yet, it plays a crucial role in the T and S/T modes by determining the length of time air is pushed into my lungs during the IPAP phase. It took me a while to discover this, and also to find the best setting for me – 40-45%.

Gradually, as I got used to the blasted mask and refined the BiPAP settings, I started falling asleep for brief periods with less delay, both at night and for afternoon naps. Another threshold was being crossed — but it took weeks, and I was still not staying asleep for long.

Two more adjustments settled matters. My BiPAP was set to an exhalation pressure (EPAP) of 4, a standard choice I'd not questioned. One night I dropped it to 3 and immediately had my first good night's sleep. (I would later drop it to 0.) Another improvement that helped my lungs do what they wanted to do – now that I understood better how to breathe in tune with the machine's sensitive technology – was to shift from the T to the S/T mode.

These experiences showed me that a change of just one point or step on any setting may make a major difference, for better or worse. This suggests that patients who have difficulty adapting might be well-advised to try experimenting with the settings – and expect that the settings that work best at first may not remain optimal as the process of adaptation advances.

Most doctors, therapists and vendors prefer to determine (and lock) the settings. Yet, in my experience, patients may have to take an active role in figuring matters out, even if this means trial-and-error experimentation on one's own. Dr. Oppenheimer agreed. Thus as 1995 came to a close, I was fully on the BiPAP system — no more iron lungs or chest cuirasses for me.

BIPAPS TO ENABLE LIVING LONG AND WELL

My life has depended on using BiPAPs ever since – at first Respironics’ original BiPAP S/T, a decade or so later the sleek System One S/T with a humidifier, next the AVAPS model, and now the sophisticated Trilogy 100. Initially I had just the one machine by my bed, for napping and sleeping. Today I have an assortment of five machines deployed around my home for all-day use as I roam around – next to my bed, TV sofa, toilet/shower, dining room table, and front-room desk. Just in case, I have four other machines deployed as well – in a travel bag for sitting outside and/or taking car rides, on the front room floor in case I go to ground, plus upstairs and in the garage in case I need to go there.

Why so many? My condition has gone from requiring only the one bedside machine after 1994, to needing another one or two handy for daytime relief elsewhere at home after a car crash in 2012, to needing progressively more on hand for round-the-clock ventilation after a bad fall in 2017. Normal aging with post-polio syndrome (PPS) might have caused my torso to shift, crumple, and gain belly weight anyway; but the crash and the fall accelerated and worsened these structural shifts — all of which further diminished my breathing ability and required raising the IPAP to 19-21 while increasing the tidal volume (Vt) as well.

Also, while my legs have always been pretty strong, I was alarmed to find I could not get back up on my feet, and thus reach my BiPAP, after going to ground twice while attending to my ailing kitty-cat pal last year. Both times turned into brief but exhausting breathless struggles as I crawled on my knees to find ways to re-erect myself. Just in case I go to ground again, I now keep my TV-sofa and desktop BiPAPs running all day long, their hoses dangling over the edge. At other spots, I keep the travel bag BiPAP and a backup Trilogy on the floor, ready to fire up, just in case.

There is lots more I could add about living with ventilators: my thankfulness for my genetic code, for my parents, brother, and sister, for my friends and neighbors, for my education and career, for not only Dr. Oppenheimer but also his hand-picked successor at Kaiser, Dr. Luis Moreta-Sainz, for rapid responses by respiratory therapists Marie Barton and Digna Berry at Kaiser's vendor Apria, for Diana Guth and her Home Respiratory Care outfit in West Los Angeles where I acquired my best nasal masks and used BiPAPs, for the Rancho Los Amigos Post-Polio Group and its spinoffs, etc. They have all helped keep me going (as have improvements in home delivery services).

I could also note personal dreads that stem from my dependence on ventilators, e.g., a power outage that exceeds my home battery-backup capacities; or isolation in a Kaiser ward where I am unable to attract immediate attention if my body or equipment goes wrong. All too much to add here.

CODA

But I do have a final observation: polio creates losses. Yet in looking back, I find that after surviving the initial shock in ‘49 and returning home by ‘51, my thoughts and my emotions have rarely dwelled on loss. Instead, they have found comfort and even taken pride and pleasure in coming up with liberating adaptations along the way. My first such sense of liberation, once home at age 10, came from figuring out techniques for putting my shirt on all by myself, without parental help.

My decades of living with ventilators — first the cuirass, then the BiPAPs — figure strongly in this as well. Far from viewing them as lamentable hindrances, I admire them as personal force-multipliers, impressive enablers of what matters most to me: continuing to read and write clear-headedly about theoretical and strategic ideas that matter to me. I hear other long-lived polio survivors, most of whom are not on vents, share this kind of positive outlook in their own ways. Thus, onward we go.