What do you do with children who weren't supposed to grow up, but did?
That cute, little, pathetic, sad-eyed poster child wins your sympathy, perhaps your financial contribution. The organization promoted in the poster provides whatever services the poor (doomed) child needs. And of course the doctors are working constantly, in research and in clinics, to find a way to help.
In some case, scientific advances have been so successful that the afflicted child becomes an afflicted adult.
No longer cute, no longer on a parent's health insurance, unable to qualify, in many cases, for his or her own. Services for children have been outgrown. Services for adults aren't yet there . . .
This is the case now with cystic fibrosis (CF).
There is no cure for cystic fibrosis. It is a genetic illness that used to spell doom for its victims within a few months, or at best a few years.
Now, with greater (although still woefully incomplete) understanding of what it is and how it can be controlled, victims of cystic fibrosis can grow up, get married and, in some cases, have their own children.
They aren't making it much beyond the 30s, though--not yet, anyway, and the older they get, the harder it is . . .
Larry Morrison doesn't talk about it much, but he's 33, and he knows the odds are beginning to go against him. He is one of about 15,000 CF victims in this country who are over 21. Another 15,000 are younger, with better and better chances of reaching adulthood. It's been 30 years since Morrison's cystic fibrosis was diagnosed. It's only been about 40 years since the disease was recognized as such.
Morrison has been lucky in the sense that breakthroughs in the treatment of CF seemed to come along just as he needed them. So at first, when it appeared that he might not make it past age 3 or so, the life expectancy lengthened to adolescence and then to the 20s, even the 30s . . . Today the average survival rate is age 14, but any child born today with CF has a 50 percent chance of living to be 21.
Cystic fibrosis is an enzyme deficiency disease of the pancreas.
It can masquerade as a lot of other seemingly unrelated ailments, from pneumonia to celiac disease to asthma, allergies to all sorts of digestive difficulties, even to just being "sickly." In the light of new breakthroughs in allergy research, scientists are now looking at biochemical similarities in CF patients and those who have various allergies.
But one of the things that "bugs" Larry Morrison is that the illness, even today, is so often misdiagnosed that both its incidence and prognosis cannot be accurately gauged and many of its victims are never given those treatments that are available.
Part of the problem is the way the illness varies from victim to victim, sometimes with one symptom, sometimes with a dozen, sometimes mild, sometimes so severe even today's treatments cannot help, even for a short while.
A positive diagnosis of cystic fibrosis must be made at a center that specializes in this so-far inevitably terminal illness.
But one good clue is this: If a baby or child tastes salty to another person when he or she has been inactive -- after a nap or a bath, for example, CF should be suspected, even when no other symptoms exist. The illness causes its victims to lose inordinate amounts of sodium and chloride in their sweat. (In fact, the CF informational slogan exhorts, "kiss your baby . . . ")
Once the illness has been diagnosed, treatment begins with diet: The CF patient requires up to 100 percent more calories than a normal person, administration of enzymes from animals and, possibly most importantly, a physical-therapy routine known as postural drainage.
The enzyme deficiency causes a buildup of thick bodily secretions which eventually will take their toll on pulmonary and cardiac functions, among other things. The postural drainage involves a minimum of twice-daily sessions of pounding, thumping, chest-clapping, back-clapping and especially designed vibration procedures to help the patient clear a respiratory tract constantly attacked by its body's own thick mucous.
It is not hard for a parent to do this with a small child, but for adult patients like Morrison, it gets harder and harder.
Coughing persistently and almost constantly is a hallmark of the CF patient, essential to his or her wellbeing, yet often embarrassing, and, in adolescence, recalls Morrison, downright humiliating.
The trouble has been, still is, as one CF therapist at Georgetown University Hospital puts it, "The health-care system hasn't really addressed itself to looking at what the needs of these young people are," although in about 21 states, legislation has extended "crippled children" benefits to adult CF patients.
But for example, says Morrison, "There was no career counseling when I was growing up."
Morrison had relatively mild symptoms and became a science teacher. He met his wife when he was a teaching assistant at the University of Maryland (in parasitology) and she was one of his students.
"One of the problems a lot of CF people have," he says, "is that they don't project the fact that they're going to get old. If," he adds, in obvious reference to his own 33 years, "you see what I mean.
"I wish I'd been counseled earlier about going into a career that I could last longer in when I became more disabled. Or a hobby I could take and change into a career."
Younger CF adults are now encouraged to go into fields which permit them to work at home--in advanced computer programming, for example--or at least during unscheduled hours. They are also receiving psycho-social counseling about sex, marriage and parenthood. Most CF males are sterile, but although pregnancies may be difficult, CF females can become mothers. Counseling, however, includes information on the odds for parenting a CF baby, or a carrier. (One in four chances for the first and 50-50 for the second when both parents are carriers.)
Larry Morrison is no longer able to work in his own field. His condition has deteriorated. He spent 18 days in the hospital this summer and now, with his wife's help, spends 4 or 5 hours a day in the essential physical therapy. He spends his time writing poetry, taking photographs and serving as an information resource for the Cystic Fibrosis Foundation.
This is another thing that disturbs him. The (CF) foundation points to people like him as hopeful examples.
"Yet," he says, "even if Joe Smith is president of this and does this and that right now, two or five or six years and he won't be able to. It gets progressively worse and eventually wears you down to the point you just can't handle it. It kind of bothers me they (CF foundation spokesmen) aren't being totally truthful about the whole thing."
Another ironic annoyance for Morrison and other CF adults is, curiously, that they don't always look sick. In fact, they mostly look pretty good. Yet, he notes, "If I walk 10 steps, I'm out of breath." And such things as special handicapped auto plates are challenged regularly. It is a source, he says, of unending frustration.
("I'm not totally unreasonable," he will say, using the parking "hassle," perhaps, as a more reasonable target to rail at than fate, "but at the same time it is embarrassing and humiliating. There aren't many things I can get with this disease. It's bad enough I have to go through life with this stigma on me . . . at least I can get something like parking.")
Piled on this, of course, are the growing expenses of the illness -- about $6,000 a year minimum -- and the difficuly in obtaining health insurance or even jobs. Morrison was able to maintain his insurance from his teaching days at "astronomical premiums, but worth it." Others are not so fortunate.
A major concern centers around possible political and Moral Majority challenges to genetic testing, screening and research, really, says Morrison, the only potential hope, although there are as yet no ways to screen for CF carriers or to test through amniocentesis. There is also the possibility that current research will be successful in finding a way to either provide the missing enzyme or inhibit the secretions.
"It may be too late for me," sighs Morrison, with more regret than bitterness, "but there are lots of little kids -- for them it could be the life-saver . . . "
For more information, write:
Cystic Fibrosis, Washington area chapter: Metro-Plex Building, Suite 110, 8401 Corporate Dr., Landover, Md. 20785.
National Cystic Fibrosis Foundation, 6000 Executive Blvd. Rockville, Md. 20852.