I have Cystic Fibrosis. There, I’ve pointed to the large, white elephant in the middle of the room that is me. It’s a funny thing to talk about. In some ways, I feel it’s a small part of who I am, but in other ways, it’s the most defining aspect of my life. It’s all I’ve ever known, and has shaped me in too many ways to count; yet I fight to give it as little influence as possible. Truth is, I wish all of this was not the case, but none of us are able to decide what baggage we’re handed right after they check the arrival box our birth certificate, or some strained metaphor to that effect.
First off, I should give a definition to those of you who don’t know what Cystic Fibrosis is. To state it simply, CF is a huge pain in the ass. Oh, I can give you the diagnostic explanation, if you want:
Cystic fibrosis is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract. It is one of the most common chronic lung diseases in children and young adults, and may result in early death.
There. But believe me, that medical descriptive diagnosis does little to really help you understand what CF is. My explanation is better, more succinct, and more accurate, actually. The only qualitative addition I would make is how much of huge a pain in the ass it is, and how that amount changes throughout a lifespan.
Let me start at beginning. I must state this first part of my personal history as being information relayed to me by my parents, of course, since I was either too young to understand any of it or was not a participant in this reality at the time. Don’t worry, that will make sense later.
I arrived on this good old planet (specifically New York City) in 1959, the second of three children born to my Irish-American parents. They were both immigrants to this country, who met here a few years after their arrival. I had an older sister, Maureen, who was 2 years my senior. Later, my folks had a second daughter, Helene, born 5 years after me.
Right from the beginning, my older sister seemed to get colds a lot more often than one might expect, at least it seemed that way to my folks. My mom and dad were often back and forth to the doctor, who gave them various diagnostic explanations: respiratory infection, bronchitis, lung inflammation, allergies, strep throat, whooping cough; the list went on and on.
When I was born, it started all over again. Now my parents were toting the two of us on the constant trek back and forth to good old Dr. Knownothing (whose name we do not speak). The good doctor was your classic, good old hometown local doc, which in this case meant he didn’t have a clue as to what the hell was going on. I remember him vaguely as a fat man with big hands and a painfully strong grip, handy for dispensing shots that required one not to move. For some strange reason, he had a huge satellite dish sized sun lamp in his examination room. I remember that thing to this day and it always terrified me.
It’s not that he was a stupid man, quite the contrary. His main problem, it would turn out, was not that uncommon back in the good-old days: he had graduated from medical school sometime in the 40s, and had not cracked open a book since. As you can imagine, that could be a big problem, but my folks had no idea, and besides, that was an age when most never questioned your doctor.
My folks spent money hand over fist at his office and the local pharmacy, and we just got sicker and sicker. I often wonder how they put up with the constant pressure and fear of their children hurting and nothing they did seemed to help.
My sister Maureen was worse off than I was. She was thin as a rail, and no matter how much my mom fed her, she would lose more and more weight.
One day Dr. Knownothing’s brilliant solution was to feed her a constant diet of milkshakes, as that was sure to pack on the pounds! My mom dutifully complied, and my sister got a severe case of diarrhea. As fast as mom poured the shakes in, they came out the other end. It seemed as though she had little ability to absorb the food that was given to her. The doctor was puzzled, saying he would start looking into digestive disorders. My parents were at their wits’ end.
But bless them both; they still tried to make our family life as normal as possible, which obviously was not an easy task. Summer was coming on, I had just turned 2 and my sister was now 4. It was common back then for a vacation to pack up the car with the family and head out to the country. In our case, the place to go was the Emerald Isle of the Catskills, an area in Greene County.
The town we headed for was East Durham, which still to this day wears its Irish roots on its sleeve, hat, tee shirt, and socks. You get then idea. You couldn’t swing a shillelagh and not hit a guy or gal from the old sod, as they say. My folks felt very much at home, and surely could use a few days off from the constant pressure of doctor visits. Besides, we were sick no matter where we were. So we packed up the old Plymouth and off we went.
After we arrived upstate and got ourselves a housekeeping cottage (the most popular way to vacation in that area back then) my parents looked around for ways to keep us kids occupied. Back in the early 60s, when the Catskills was hopping with families from the city, there were several tourist attractions designed to pull those city slicker dollars in. One of the biggest was a spot called Carson City & Indian Village, a re-creation of a real shoot-em-up Wild West town, complete with more cowboys and Indians than you could shake a six-shooter at. Just the place to take two kids for a day of riding horses or the stage coach, staged gun battles and saloon fights, and face-painted Indians; just about all the tacky Old West schlock a kid could handle.
Of course, there were balloons and pennants and all sorts of souvenirs, but I wanted and got a straw cowboy hat, which I wore with the seriousness demanded of my appointed position as a deputy sheriff. A tall mustached guy in a big, Stetson hat and a shiny silver star on his leather vest deputized me. We still have a faded family photo of my parents, Maureen and myself posing in rootin’ tootin’ buckboard coach. It’s what you don’t see in that photo that later made all the difference.
We had a nice, restful vacation, but sadly soon Maureen and I were up coughing every night. Since our parents were not sure if the damp air of the countryside was causing us more harm than good, they decided to head back to the city.
It wasn’t long after we got home from vacation that my mother noticed I was sleeping more than normal. At first she just chalked it up to me being tuckered out from the excitement of the trip. But it continued, and seem to be getting more pronounced. I was walking by that time, but now seemed to be having more trouble navigating than I had been just weeks before. Worried, my mom called Dr. Knownothing, who like many doctors back then, still made house calls. He came the next day and had my mom take me out of the crib to take a little walk. I stumbled and rocked like a drunken sailor, and finally fell over on my butt. I’ll give the doctor credit for this one, anyway. He took me to the hospital immediately.
By the time I got to the hospital, I seemed to be having trouble keeping conscious. I was rushed to the emergency room, but little by little, I was slipping away. By later on the next day, I had lapsed into a coma.
Tests were done; (x-rays and the like), basically all you could do back in those days. It wasn’t long before they had a diagnosis: I had Encephalitis, which is a swelling of the brain brought on by a virus. It’s much more common in countries where people live near a lot of livestock; whose waste attracts biting insects, such as a mosquito or horsefly, then the virus is transmitted by their bite.
Remember that buckboard coach photo of my family? What you didn’t see in that snapshot from Carson City was the Volkswagen sized horseflies that were buzzing everywhere in that good old-fashioned fake Wild West Town. Lots of livestock for authenticity, making lots of crap that then brought lots of horseflies. Buy the time we left we were all quite bitten up. I guess one that bit me was infected with the virus. Just a lucky little guy, that was me all right.
Encephalitis was no joke. In its mild form it can make you feel woozy for a week or so, in a severe form it can cause coma, or death. Progressing to coma at the age of two was considered just about as bad as it could get, before, well, you get the idea. My folks were given the prognosis; there was a good chance that I would never wake up.
But my parents being who they were: strict Irish Catholics, the first thing they did was start praying. There is a saint named Jude, who is considered the patron saint of “Hopeless Cases”, and I guess I fit the bill. My mom and dad (and a bunch of their friends and relatives) started storming the heavens to get St. Jude’s attention. My mom was at my bedside everyday, banging on St. Jude’s door.
The doctor came by everyday, took my temperature, blood pressure, checked my pupils for some sign of brain activity. He would just look at my folks and shake his head. No change, day after day. But week one passed, and I was still here. Then two weeks passed, then three; Dr. Knownothing would sigh and leave the room, and mom and dad hunkered down and kept at it. Suddenly, 23 days into the coma, I woke up, much to the surprise of Dr. Knownothing and the staff of Parsons Hospital. My parents’ prayers were answered, and they gave thanks.
All better? No, not really. Now the doctor had more good news. It was likely that because I had been in a coma with brain swelling for that length of time, I would probably be partially or fully paralyzed, and sure enough, I was. I had no movement from the neck down. So I was sent home, nothing more could be done. But did my folks buy that? No way, it was back to St. Jude again. As far as they were concerned, he was doing a hell of a better job for me than all the doctors were.
About once a week, Dr. Knownothing would stop by for a house call and check on me, which basically entailed more taking of my vital signs and shaking his head.
After I was home for about a month or so, the doctor stopped by for his regular visit. My mom left him alone to look me over while she got dinner together. He knelt down next to me as I lay motionless, took his doctor bag and placed it on the bed, and then got back up to look in his pocket for a pen. No pen. Maybe it was in his coat pocket, which he had thrown on my sister bed, behind him. Finding one in the coat, he turned back around, and reached for his bag. It was no longer on my bed.
He looked around, and found it on the floor on the other side of the bed. Scratching his head, he picked it up, knelt back down and placed it back on the bed, on top my leg, which was under the covers. I promptly kicked the bag back on the floor, which caused the doc to fall back on his butt in shock. He pushed himself up and called for my mom. She came running in, and he repeated the bag trick, which I promptly complied with by kicking it once again to the floor. “Okay, this is out of my hands now,” he stated, “I have no idea what’s going on here, but I’ve got little or nothing to do with it.” My mom just smiled, and winked up to heaven. Her and St. Jude, they were in charge now. Maybe they always were.
Dr. Knownothing was not done yet, however. He had more good news. Mom was now warned that because of the Encephalitis and my period in a coma, and although I was now recovering movement, his last diagnosis was that I might have brain damage. Those who know me to this day might have varying opinions on that matter I guess, but medically, I seemed to have dodged that bullet too. No medical reason was ever found for my full recovery. Dr. Knownothing just closed the file.
And how do I feel about all this? To this day I wear a St. Jude medal around my neck, given to me on my 40th birthday, 38 or so years after I was written off. Scoff if you will, but St. Jude and I, we got this thing going on. I believe in him, because I’ve got to figure he believed in me.
Of course, the general sickness problem had not gone away, just put on hold until the coma/dying thing had been resolved. Now that I was no longer at death’s door (as Dr. Know-nothing used to refer to it), my parents tried to concentrate on narrowing down what the hell was going on with my sister and myself. If this were going on today, specialists would have been consulted; multiple tests would have been done. But this was 1962. Such things did not exist then, or if they did, not for folks of my parents’ income.
The trips to Dr. Knownothing continued month after month, year after year. We would get better, and then worse, and something else would be tried. Antibiotics? Check. Giving Vitamin B12 shots? Check. Giving Gamma Globulin shots? Check. Sun Lamp treatments? Check.
And so it went, and we continued to take two steps forward, and then two back. Then something rather remarkable happened, and it was because of the radio.
My mom was home with both of us kids all day, and by this time she had become pregnant with my soon to be sister, Helene. Consequently, it was often that she would need to hear another adult voice, just to keep her from going nuts. Her answer was the same as most moms’ was back then; she would have the radio on the talk station most of the day in the kitchen. Most of the time it was just background noise, a pleasant but uninteresting parade of one guest after another pushing this or advertising that. Sometimes she would pause from cooking dinner or cleaning and sit and just listen for a while, but most times, the radio and it’s hosts would just buzz pleasantly as she dealt with us or the housework.
One summer afternoon she was starting dinner, and the radio host had just introduced a doctor from a center at Columbia Presbyterian Hospital in upper Manhattan. Mom was listening with only half an ear, picking up a word or phrase here or there. After a bit, some words like coughing, or a phrase such as “lack of nutritional absorption” caught her full attention. The doctor was Carol Denning, the head of a newly opened clinic in Columbia Presbyterian for Cystic Fibrosis. The disease had been identified as a particular syndrome in the 1930s and 40s, but was still not widely known.
My mom, bless her, went over, turned the radio up, and pulled up a chair at the kitchen table and grabbed a pencil with paper. She’s told me that she sat transfixed, taking notes, and it wasn’t long until she was convinced that Dr. Denning was describing my sister and myself to a T. At the end of the interview, Dr. Denning gave a phone number for the clinic. My mom went right to the phone. When a nurse answered, my mom said, “I just heard Dr. Denning on the radio and I think she was describing both of my children.” She was told to come up to the center the next day.
When my father came home that night, my mom told him about the radio show and Dr. Denning. They immediately called Dr. Knownothing. He had heard of this thing called Cystic Fibrosis, but did not know a lot about it. He recommended my parents take us to the clinic, because they would surely know more. Gee, thanks, my mother thought; no kidding. The next day my father took off from work and we all headed for Columbia Presbyterian.
The standard test to diagnosis Cystic Fibrosis back then was a sweat test. The way it works is a chemical, that causes sweating, is put on a small area on an arm or leg. An electrode is then put over that spot. This lets a doctor apply a weak electrical current to the area to cause sweating. I remember it sort of hurt, but having endured countless needles, I could hack it.
The second part of the test consists of cleaning the area and collecting the sweat on a piece of gauze. Thirty minutes later, the collected sweat is sent to a hospital laboratory to measure the chloride in the sweat. CF patients have a lot more chloride in their sweat than a normal person. Too much chloride in the sample, and you’ve got CF. Both Maureen’s and my test came back positive.
Congrats, Mr. and Mrs. Cotter, both of your kids have Cystic Fibrosis, and lucky you, you’ve got one on the way! Can we go for a trifecta?
So here’s the good news. My folks, after years of frustrating, endless trips to the doctor, finally had a correct diagnosis. But then there was the bad news. In 1964, most CF children didn’t make it to double digits. My parents made one last trip to Dr. Knownothing. His final, knowledgeable advice to my parents was to take us home and enjoy us, because they would not have us for long. My folks looked at each other, then at him, turned and walked out his door. They never returned again.
Years later, my mom and I ran into Dr. Knownothing in front of what was then Booth Memorial Hospital, where we had just visited a sick relative. He was just leaving the hospital, too. We were, to put it mildly, shocked. Dr. Knownothing now looked like he weighed 85 pounds. My mom, as politely as possible, asked him what was wrong. He told us he had skin cancer. Remember that sun lamp in his exam room that terrified me? Well, he used to spend a great deal of his lunch hour baking under it, day after day. He had another home in Florida, and Dr. Knownothing loved a good tan. I’d speak of irony and justice, but truthfully, it all just made me very sad. His error of not keeping up his education had hurt my sister and me, but it was killing him. We wished him all the best, and went on our way.
So now my folks had to deal with a new reality; not just two sick kids, but two kids with an incurable disease that was likely to kill them. What to do? Panic? Bang their fist against the walls? Maybe scream at the top of their lungs and run around in circles? Good plan; but probably not the most productive use of their time. No, instead they squared their shoulders and re-oriented the center of their universe, and headed back to Columbia Presbyterian. It was time to get this party started.
End of Chapter One
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