by Frank Deford
Of course, then springs won’t mean nearly so much to you because you’ll just take them for granted, like everybody else. Oh well, you can’t have everything.
Alex, I guess I’ll be somewhere in my fifties when I give you this. Either that or I’ll be gone and your mother will find this and give it to you for me. Either way, I want you to know that that will be my happiest moment ever.
I look at you now, and I see those big round blue eyes of yours staring back at me, wondering, asking, hurting, and I think you know more than you let on. Already you possess things I’ve never had, because you have had to struggle to earn life. And soon you’ll have a spring too.
Well, thanks for everything. You’ve already given me so much, and you must get well to help me grow better, with you. I want so much to get to know you. Now Chaucer and I will go to sleep. God bless you always, Alex.
With love,
Your father
I put that in an envelope, addressed it to Alex, and put a stamp on it. Eight cents. Then, across the stamp, in the manner of a postmark, I wrote the year, 1972, in orange grease pencil, and, the next day, I put the envelope in the safe deposit box, where it has stayed until now.
At the time, I firmly believed that Alex would be cured and would get to read the letter. I believed that with all my heart.
Chapter 5
The next few years were nearly beautiful. What was different and difficult about Alex because of her disease was papered over by our dreams. She did not know yet about dying, and did not have to give any time or thought to that. Why, we were almost like any family, Alex almost like any child. Apparently, we were nearly as perfect as those families Alex began to meet on television—“The Brady Bunch,” “The Partridge Family,” “I Love Lucy” reruns, “Happy Days”—where nobody ever gets sick. People in television families get pregnant and beat up, they get mixed up and insulted, they’re not invited to the prom. But they never get sick. It is against the law for anyone in a television family to get sick.
It was easy to be deluded, too, because Alex did appear so healthy for a while. She was so pretty, and she was all but free of pain. The pneumonia and the ear problems had all been cleared up, and the medicines and physical maintenance techniques kept her protected.
Oh, I wish you could have seen her then. She was christened in a long white gown of lace that had been in my mother’s family for generations. Her blue eyes had already turned to brown, which fit her better, matched her hair. She grew even more beautiful in the next couple years, taking on an almost eerie resemblance to the photographs of her mother as a baby.
She grew up exceptionally feminine, but not in the old-fashioned way; she was a feminine feminist. No one ever liked silly frilly things as much as Alex did. The more gossamer, the better. She couldn’t abide pants—too masculine—even when it was bitter cold. Alex fibbed, like any child, but the only time I ever recall contrived, premeditated, real A-1 lies came in nursery school when she advised Carol that the teacher wanted her to wear dresses to school, and then turned around and told the teacher that her mother would not allow her to wear pants to school—no matter how cold it was.
Alex was heavy into cosmetics when she was hardly out of diapers, and you could usually hear her coming, rattling like Marley’s ghost with all the cheap jewelry that clanked about her. Alex would have killed for jewelry. We would bury her with a lot of her favorite jewelry.
Earrings were her favorites. One day, when she was about five or six, Alex and Carol went out shopping to a store with a going-out-of-business sale. Alex’s eyes were drawn to a pair of the ugliest earrings you ever did see. It was not enough that they were hideous. They were huge, hanging down to the poor wearer’s shoulder, and they glimmered, drawing further attention. Not only that, but they didn’t come cheap. Even 50 percent off, going-out-of-business, they were still commanding $12.50, plus tax. “Please, Mother, please,” Alex pleaded.
Carol patiently showed Alex that the earrings were simply too big. On Alex, they almost came to the waist. But she would not be denied. Hadn’t Carol already made the mistake of saying that she adored them too? “You’ve got to get them for yourself, Mother.”
“But they’re too expensive,” Carol said.
“Oh, don’t worry, Mother. I’ll talk to Daddy. I will. It’ll be all right.”
Carol was running out of excuses. “The trouble is, Alex,” she said, “that these earrings are just so pretty, so magnificent, that I would never have the right occasion to wear them.”
“Oh, yes you will,” Alex said.
“When?” Carol asked.
“When they find the cure for cystic fibrosis and we have that big family party and get all dressed up—you can wear them then.”
Carol bit the tears back and said of course and bought the earrings. They’re still in a drawer.
Makeup was as much an obsession as jewelry for Alex. As she grew older and got more sophisticated with her drawings, for example, every face had to have two big red circles on the cheeks—rouge. My favorite drawing of hers said:
Happy Valentin Day God Love Alex
With it there were renderings of the obligatory heart, rainbow, and lollipop flower and the word God, with an arrow pointing to a large, smiling face. And this face had two red dots for rouge on the cheeks. Only Alex would put God in makeup.
But although I never heard Alex directly suggest that God was a woman, I wouldn’t be surprised if she had believed it. Alex instinctively believed in women, and believed they got a raw deal. From an early age, and without any prompting, she questioned why there were no women this or no women that. “Where are the girls?” she would say. “Why aren’t there any ladies?” On “Star Trek,” she strictly concerned herself with Lieutenant Uhura, and on “Captain Kangaroo” with Debbie (good choice: I had a crush on Debbie too). Instead of the male superheroes, Alex inclined to the likes of Wonder Woman, the Bionic Woman, and Isis. Unlike a lot of little girls, Alex never had much interest in horses—not until the great filly Ruffian came along and Alex started hearing that she could beat the colts, the boy horses. Alex wasn’t the least bit impressed that I was off to cover the Olympics in 1976 until Nadia Comaneci suddenly started making perfect scores and stealing all the thunder from male athletes.
I want to guard against reading significance into everything about Alex, but I do believe that her almost instinctive feminism came out of some greater sense she had to detect the unfairness of life. Wouldn’t this have been great? Next to love itself, equality and beauty were the things that interested her the most.
Chapter 6
One of the reasons I find it difficult sometimes to comprehend quite all that Alex meant to other people—especially to those who only met her nearer the end—is that, much as I loved her, proud as I was of her, I always knew her as a little person, a child, a very fallible human being. Alex could be petulant and cross, and, especially early on, when she was relatively at her healthiest, she was inclined to take things too seriously, too literally, and show little humor. The Halloween of ’74, the day after her third birthday, she dressed up as bossy Lucy, from “Peanuts,” and we all laughed at Alex because we thought the casting was so apt. She didn’t get the joke.
That was the time in her life when Alex looked and felt her best. But even then we never had the luxury of being able to close our eyes and pretend. When a child has cystic fibrosis, she never simply lives. She must be kept alive. It’s a lot of work, too. Even now, long after Alex has gone, I wake up some mornings with a vague feeling that something is incomplete in my life, that there is some obligation I’ve forgotten.
Even now it seems strange for me and for the family just to get up, brush our teeth, put on our clothes, eat breakfast, and go on about the business of another day of living. Because when Alex was with us, even when she was doing pretty well, we had to go through lengthy, set routines every day just to help her live. And after you’ve done that for a while, after helping to keep someone living has become part of
the rhythm of your existence, then merely getting up again in the morning and accepting life—no questions, no effort—can be very flat, even disorienting.
Some diseases, even some fatal ones, don’t put too many demands on the family of the patient. But cystic fibrosis is a tyrant that rules everyone in the family. There is no avoiding it; it is at once clinging and creepy, smothering and penetrating; maddening for sure; often it can destroy the whole family, even as it takes the one child’s life.
Cystic fibrosis utterly defies a cure. It is a genetic disease, carried almost exclusively by Caucasians, but with little fluctuation in incidence anywhere in the white world. Apparently it’s been with us from antiquity. Cystic fibrosis patients give off an excess of salt; this is reflected in a German folk saying: “The baby who is kissed at birth and tastes salty will have a short life.”
One out of every twenty whites carries the defective gene, as I do, as Carol does, as perhaps 10 million other Americans do—a population about the size of Illinois or Ohio. Indeed, the CF gene is so prevalent that one of my colleagues at the Foundation, Dr. Charles Lobeck, even speculates that at some point in human development the defect served a necessary—even beneficial—purpose. But carriers, the tens of millions of us in the world, are unmarked in any way, and no carrier has any way of knowing that he is a carrier until he marries another carrier who has no way of knowing that she is a carrier until they have a baby who is diagnosed with CF. Research into carrier identification does appear to be well along the path to success, and it is reasonable to expect that an effective and fairly inexpensive test may be ready within the next few years, but as yet one does not exist. If Carol and I had been lucky, if our second-born had not been cursed with cystic fibrosis, then we probably never would have had another child, and our bad genes would merely have been passed on, blissfully unknown to us.
The laws of genetics are pretty unyielding, of course. One out of every four children born to two carriers will have the disease. One out of four will be free and clear. The other two will be carriers. But it’s like flipping coins. There is no reason for the unlucky ones to pop up in the order that they do. Surely, many undiagnosed CF carriers have numerous children, none afflicted. Similarly, others have a sick child right away, their first-born. And some of these parents, by ignorance or design, go on having babies. Many families have two CF kids, a few even three or more.
Carol and I never even considered trying to have another child. If you’re carriers, you can get better odds, of 5—1, playing Russian roulette than the 3—1 cystic fibrosis offers you at life. But I must say this: Had Alex died at Children’s, as she was supposed to, Carol and I would never have known what hell cystic fibrosis truly is, and maybe, in that ignorance, we would have decided to take the 3—1 and gamble. I think that is possible, because although you may understand, intellectually, that cystic fibrosis is painful, fatal, incurable, depressing, expensive, and altogether horrible, until you have lived with it you can never comprehend how impossible it is. For CF families the divorce rate is several times the national average, and the incidences of separation, alcoholism, desertion, wife beating, and so on are all comparably high. And the physically healthy siblings, no less than the parents, are often crippled in many emotional ways.
I was lucky, you could say; I only had my child die.
Even in the best of times, when Alex was younger, she required almost as much daily attention as she did later, on the verge of death. The care, the demands, are constant; all the worse that there is a Sisyphean quality to them. In those first years, too, on top of everything else, Alex had to sleep every night in a mist tent. She despised that, as well you can imagine. Mercifully, after a while the expert consensus shifted, and the doctors decided that such tents were of dubious merit. However much they may help, marginally, in the strict medical sense, they probably do more damage psychologically. So at least then Alex was permitted to sleep like any child, without the stigma of a tent.
Not too long ago I was crawling around up in our attic, searching for some old chairs. There are no lights in the attic, and suddenly, behind some worthless, nostalgic debris, loomed an apparatus I did not immediately recognize, a ghostly superstructure reminiscent of some rotting ship’s hulk grounded on a sandbar. I peered closer in the dark, and finally recognized that it was the frame for Alex’s mist tent. I had shoved it up there, out of my sight, when it had been declared unnecessary.
Softly, then, alone, I began to cry, for I could see Alex there in her crib, as a baby, as any baby, curled up with a doll and her pink “blankie,” looking so cheerful, so chubby, even (it seemed) … so healthy. Chris would usually be the one to get her, then, to flip off her mist machine, pull back the plastic covering, clouded and dewy by now, snatch her up, and bring her into our bed, where we could all four snuggle together, putting off the morning, and the day’s first treatments.
Cystic fibrosis is, notwithstanding its name, a disease primarily of the lungs. It has nothing to do with cysts. It was not identified as a distinct clinical entity until the midthirties, and not until some years later was the full pathology comprehended. Inexplicably, the disease attacks not only the lungs but other disparate parts of the body: the pancreas, the major digestive organs; and, in males, the testes. So it undermines breathing, eating, reproduction—all of life itself.
The common agent in all cases is mucus. The cystic fibrosis victim’s body manufactures too much mucus, or the mucus is too thick, or both. So baffling is the disease that nobody knows for sure which basic factor is the issue. Whatever, the mucus obstructs the airflow in the lungs and clogs the pancreas and the testes. Adding to the perplexity is the fact that no two patients have the same history, except in the sense that CF is always progressive, always terminal.
The luckiest patients are those born without lung involvement. Others have such mild cases that they go undetected for years; quite possibly there are even some CF patients who never know they have the disease, but die relatively young of some misunderstood pulmonary involvement. At the other end of the spectrum, some infants are essentially born dead, their tiny bodies so ravaged by the disease that they cannot even begin to draw breath.
As events proved, Alex was toward the worse end of the spectrum. While she died at eight, half of the children now born in the United States with cystic fibrosis who are diagnosed and treated live to the age of eighteen. Be grateful for small favors. Back in the midfifties, when the Cystic Fibrosis Foundation was started, a child with CF could not even expect to live to kindergarten. Regrettably, early steady advances stopped just about the time Alex was born. Until the early seventies almost every passing year saw another year of life expectancy added for a CF kid, but these advances were somewhat illusory. They were largely prophylactic, stemming almost entirely from better maintenance and more powerful antibiotics. The longer life span in no way indicated an approaching cure, nor even a control (as, for example, insulin keeps diabetes under control). In a sense, it isn’t accurate to say that we kept Alex alive—we merely postponed her dying.
Alex’s day would start with an inhalation treatment that took several minutes. This was a powerful decongestant mist that she drew in from an inhaler to loosen the mucus that had settled in her lungs. Then, for a half hour or more, we would give her postural drainage treatment to accomplish the same ends physically. It is quite primitive, really, but all we had, the most effective weapon against the disease. Alex had to endure eleven different positions, each corresponding to a section of the lung, and Carol or I would pound away at her, thumping her chest, her back, her sides, palms cupped to better “catch” the mucus. Then, after each position, we would press hard about the lungs with our fingers, rolling them as we pushed on her in ways that were often more uncomfortable than the pounding.
Some positions Alex could do sitting up, others lying flat on our laps. But a full four of the eleven she had to endure nearly upside down, the blood rushing to her head, as I banged away on her little chest, pounding her,
rattling her, trying somehow to shake loose that vile mucus that was trying to take her life away. One of her first full sentences was, “No, not the down ones now, Daddy.”
Psychologists have found that almost any child with a chronic disease assumes that the illness is a punishment. Soon, the treatment itself blurs with the disease and becomes more punishment. Sick children have highly ambivalent feelings about their doctors, on the one hand hating them for the pain and suffering they inflict, on the other admiring them, wanting to grow up and be doctors. Wendy Braun and Aimee Spengler, Alex’s best friends, told me after Alex died that whenever the three of them played doctors and nurses, Alex participated with enthusiasm, but when she played the doctor, it was always cancer she was seeking to cure. She could not bring herself to be a cystic fibrosis doctor. As much as she adored and trusted her specialist, Tom Dolan, she must have associated too much pain with him ever to want to be him.
In cystic fibrosis a child must transfer this attitude toward the parents, as well, for we were intimately and daily involved in the medical process. Imagine, if you will, that every day of your child’s life you forced medicines upon her, although they never seemed to do any good; you required her to participate in uncomfortable regimens, which you supervised; and then, for thirty minutes or more, twice a day, you turned her upside down and pounded on her. And this never seemed to help either. I have been told that parents let their self-conscious resentment of the illness surface during the treatments, and I must face the fact that this was sometimes surely true of me too. In some moments I must have thought that I was also being punished.
And say what you will, explain to me intellectually all you want about how much the postural drainage helped Alex—still, when every day I had to thump my little girl, pound away on her body, sometimes when she was pleading with me, crying out in pain to stop, something came over me, changed me. I guess, over eight years, I did therapy two thousand times, and Carol many more, probably three thousand, having to manage both times each day when I was traveling. I never understood how she managed. But still, me: Two thousand times I had to beat my sick child, make her hurt and cry and plead—“No, not the down ones, Daddy”—and in the end, for what?
