Mark was a child with a lot of friends, and in spite of his disease he led an active life, but it was a life punctuated by visits to the hospital. In contrast to some CF kids, who by the age of 13 may have been in as many as 30 times, Mark, says his mother, “wasn’t in really a lot. Maybe seven times. There was one period where he went for almost two years without a hospital stay.”
Usually such hospitalizations are for what is referred to in CF jargon as a “tune-up.” Tune-ups become necessary when a cold stirs up the bacteria in a CF patient’s lungs; they usually last 10 to 14 days, and their main treatments are the administration of intravenous drugs and intensive chest physical therapy that involves physically beating on the patient’s chest. This can be done with either a mechanical vibrator or by hand in a clapping manner which evokes the sound of a horse’s clippity-clop; a few minutes spent observing this procedure can yield a keen perspective on the limitations of modern medicine. The purpose of this activity is to loosen and dislodge the thick, sticky mucus (termed “junk”) which a person with CF can cough up in mind-boggling quantities after a treatment. One adult commented that he coughed up eight ounces a day when he felt well and twice that when he had an infection.
CF patients are advised to do treatments at home as well as in the hospital; they can be done by a child’s parents, or, when old enough to use the special vibrators, children can do their own. The Longfellows came to view the treatments as a part of life, a habit “like brushing your teeth every morning. It took an hour every time we did them and we routinely did them twice a day.” But while some patients have no doubt about the value of treatments (one proclaiming, for instance, that “if I stopped doing my treatments today I would probably be in the hospital in two weeks, maybe not even that long”), others avoid them fanatically because they symbolize the unremitting grip CF maintains upon those it affects: two, three, sometimes up to four hours a day spent clearing one’s lungs in a ceaseless effort simply to stay alive. One mother said, “It’s just like pulling hen’s teeth” to get her 11-year-old son to take a treatment. “He hates it. He thinks if he works on himself he doesn’t need that pounding. In the morning he goes in and he coughs and he coughs and he gets most of that stuff up himself. He’ll cough for maybe five or ten minutes.”
It is one of CF’s more unnecessary tragedies that the type of callous (and often ignorant) welcome to the medical world which Judy Longfellow received is far from uncommon. And though families have had the amazing luck of having a child correctly diagnosed the very first time he got sick, the stories of wrong diagnoses, missed diagnoses, and refused diagnoses are legion among the CF community. One man told of having “pneumonia, or some problem, as an infant”; “repeated illnesses” all through his youth; “what was diagnosed as viral pneumonia three times” in his early teens; “mononucleosis during college”; “and other problems off and on.” One physician was “convinced” he had tuberculosis and “kept running skin tests. He couldn’t understand why they kept turning out negative.” He spoke of being given 40 different diagnoses — with any one as good as another — and of being treated for “allergies” until his mid-20s.
It was the ironic ill fortune of this man, now 35, to be too healthy and longlived to be considered a candidate for cystic fibrosis. Nor is this sort of episode confined to the backwoods of medicine: as recently as two years ago, and based upon the erroneous results of a sweat test conducted by placing a plastic baggie over one hand, a pulmonary specialist at one of San Diego’s most respected medical institutions persisted in treating for tuberculosis an adult patient who had been clearly diagnosed elsewhere as having CF — presumably because the patient was too old and too well to be “cystic.”
Just as common as the litany of false diagnoses and incorrect treatments are the attendant tales of mental turmoil compounded by physicians who failed either to recognize the medical subtleties and variability of CF or to comprehend and appreciate the emotional difficulties generated by the disease, or both. When Ellen Derner was finally diagnosed, she was understandably relieved, as she put it, to “find out what it was, to learn that the symptoms were all related and that it had a name.” But her doctors, she says, couldn’t understand that. “One of them stood in my room and yelled at me that this was something that was going to kill me and that I had better start taking it seriously. They couldn’t understand what a relief it was to have it all explained, to have it all make sense.”
The medical mistakes are understandable given that virtually nothing in CF is orderly — diabetes, liver disease, and sterility can develop in addition to the respiratory and digestive disorders; and it can be found among blacks and Hispanics, though it is disproportionately a disease of whites. But what is harder to understand is that the predominant attitude within the medical community seems based on an image of CF drawn largely from the ’50s. The general medical community maintains a picture of CF similar to that put forth recently by the prestigious New England Journal of Medicine. In an editorial, they pronounced CF “a grim sentence, usually prolonged through an unhappy adolescence to a sad, inevitable end.” This attitude, unfortunately, is often reflected in the medical care dispensed by physicians who hold it, and it is this situation which contributes to the unrestrained joy most CF patients and families feel when they finally do find a physician familiar with their disease.