A story about cystic fibrosis and genetics has been making rounds on the internet fairly recently. Many people and many publications are talking about it. A student was removed from school because of his “genes.” The student (child A) with “genetic markers of cystic fibrosis” was removed from school and sent away merely because of a test he received as an infant. The truth is slightly more complicated.

The Current Background

Cystic fibrosis is a recessive-genetic disease. As with many forms of medical testing, the test for a child with cystic fibrosis is a two-step process. An inexpensive and inaccurate screening test is given to see if there’s a chance that you have a disease. After the inaccurate test comes back positive, this justifies the more laborious and accurate diagnostic test to give the real diagnosis. Genetic tests can be done, but sometimes the results are downright ambiguous. The accepted definitive diagnostic test to see if you actually have cystic fibrosis, no matter what the genes are, is a sweat test.

Child A at one point received the screening test for cystic fibrosis, which showed “genetic markers” for cystic fibrosis. The further diagnostic test showed he did not (and does not) actually have the disease of cystic fibrosis. It seems this screening-test information was included in the child’s medical history at school. Seeing this result, and without permission from the parents, a teacher told the mother of another two students (Children B) at the school who did have cystic fibrosis of this news. Being unclear about its meaning, the teacher communicated that child A had cystic fibrosis when he actually did not. This news made the parents of children B first remove their children from school, and then seek to have child A removed from school. Their request was granted, but soon voluntarily reversed. A lawsuit was filed. The case was dismissed. The decision was appealed. The plot thickens.

Now, as the appeal will soon be decided, there is lots of talk about these nebulous “genetic markers,” which brought about the situation. Wired magazine points to the insufficiency of laws like the Genetic Information Non-Discrimination Act, which only covers insurance and employment, and does not cover other situations like school attendance. The Justice Department has also declared an interest in the case for two reasons. First, the DOJ believes that the mere belief that someone has a disability is enough to be covered by the ADA, and it is not necessary that the belief of the discriminatory actor be correct. Second, the DOJ believes that when the judge decided the issue of the reasonableness of the school’s action, dismissing the complaint in the process, it was improper. Instead, that issue should have been left to the jury, not the judge.

But despite all this legal interest and public attention, as an individual who has lived with cystic fibrosis for 29 years, this case doesn’t really interest me at all.

The Real Issue

There is a huge elephant in the room that everyone is missing.

The case is an interesting legal and public interest story because a person without cystic fibrosis was removed from school because people thought he had cystic fibrosis due to his “genetic markers.” He didn’t actually have cystic fibrosis…

…but what if he did?

Has anyone though about why the parents of Children B were so scared that they first pulled their own children out of school and then asked the school to pull the other child out of school? Are they crazy? Has anyone thought about why the school obliged the request? Is the school crazy, too? You can find this information in one sentence in most stories and in the case’s briefs: “kids with the inherited lung disease can’t be near each other because they’re vulnerable to contagious infections” or “As a general matter, there is a risk of infection between people who have cystic fibrosis because a person with the disease can be a carrier of bacteria that is easily transmitted and harmful to others with cystic fibrosis.” But in practice, what does that really mean?

Well, that’s the real issue. It means it is often totally okay to “discriminate,” according to the ADA, against those with cystic fibrosis.

The Real Danger

People with cystic fibrosis are no danger to you. They are quite simply not contagious, no matter how much that cough concerns you. The real danger of contagion, however, is between two people with cystic fibrosis.

In normal people, thin watery mucus lines the lungs and protects them from the dust of the air. But with cystic fibrosis, a genetic defect inhibits the regulation of salt in respiratory tissue and makes this mucus thick and sticky. Rather than cleaning the lungs, the mucus clogs the lungs. And what do lungs do to clean themselves of clogs? They make more mucus, of course. To break this cycle, coughing can physically remove this mucus junk from the lungs, but this daily battle of getting thick sticky mucus out of the small airways of the lungs is the daily grind of living with cystic fibrosis.

The thick mucus of cystic fibrosis is also a petri dish for bacteria. It is both food and shelter for whatever one-celled organism is lucky enough to find its way inside a CF lung and reproduce billions upon billions of times. Once a strain of bacteria lands in that petri dish and “colonizes” the lungs, it doesn’t go away very easily.

A colonized strain of bacteria can have a range of effects. On the more common side, it just remixes what antibiotics you take when you get sick. Some of those bacteria are drug resistant, so things are sometimes more complicated than a simple antibiotic. Many times, a new strain of bacteria involves a respiratory infection, which can bring on decreased lung functions, staying home from school, or even going to the hospital to receive heavy IV antibiotics. On the extreme end and with certain rare and very severe bacteria, this could mean death within a few weeks despite aggressive treatment. But absent that rare and scary possibility, the danger is mostly just cumulative damage. People respond to bugs in different ways. My Pseudomonas may not bother me until I get a cold, but maybe the MRSA will be a consistent bother even apart from an exacerbating virus. Maybe the Staph is normally manageable, but what about when my job keeps me from getting enough rest?

What will it do immediately? Nothing. What will it do next? Nobody knows. Will it ever go away? Maybe, but maybe not. Bacteria comes in the air you breathe, from the hospitals and doctors’ offices you visit, and through your environment, too. Different bacteria affect individuals in different ways, and a single strain of bacteria will affect the same individual in different ways at different times. Regardless, the weight adds on. For healthy living, the bugs are to be avoided.

But the unique thing is that the habitat for these bacteria do not exist in people without cystic fibrosis. It only exists in the mucus of individuals with cystic fibrosis. This is why people with CF are not contagious to you. If you have a cold, then you might be contagious to them. But the real danger for CFers is that we are contagious among ourselves.

This can create some cruel ironies. Tough situations can be overcome by joining with others who share your trouble, who can give help, and who will help you soldier through the pain. The same is true for cystic fibrosis. But with cystic fibrosis, no matter what encouragement you hope for, physical contact or proximity is off limits.

From my own memory, the quarantines seem to keep growing, too. When I was young, I remember being told of summer camps for individuals with cystic fibrosis. I never went, but they went away. Hand-washing was, of course, emphatically emphasized. Then masks at doctor’s visits went from available to required. Then a six-foot rule between CFers was the norm. Then gown-and-glove protocol for doctors. Then a no-shared-room order for hospital stays. Recently, the Cystic Fibrosis Foundation created, not long ago, new guides for infection control. Only one individual with cystic fibrosis is allowed at an official cystic fibrosis foundation event. Would you like to attend a conference for people with cystic fibrosis? You’re welcome to come! Unless you have cystic fibrosis, of course. I once had the opportunity to talk to two representatives from the Cystic Fibrosis Foundation on an issue of concern to me about cystic fibrosis. They were helpful, professional, resourceful, and happy to meet with me. But we met at a restaurant that they suggested. I never saw the foundation office. I suspect that was intentional. Can I really blame them? Thank goodness for the internet and webcasting, because otherwise CFers would be almost completely removed from the primary organization that advocates for them and educates them on how to care for themselves. Personally, I am thankful that CF runs in families. At least that is one physical tie that is too strong to be broken by this segregation. But the segregation is real. And it’s not crazy.

I share these facts as background to communicate how real the danger of cross-infection is within cystic fibrosis. This danger is why the Americans with Disabilities Act offers very little if any redress to a child who actually did have cystic fibrosis, even if he underwent the same treatment as Child A, the child of the story who actually didn’t have cystic fibrosis.

The Current Law

Normally, the Americans with Disabilities Act protects individuals with a qualified disability from discrimination. This is what Child A is using to sue his school for being removed. But certain cases have shown, for example Doe v. Judicial Nominating Commission, 906 F. Supp. 1534 (S.D. Fla. 1995) and Guckenberger v. Boston University, 974 F. Supp. 106 (D. Mass. 1997), you can discriminate if it is “necessary.”

“Under federal law, public entities cannot use eligibility criteria that screen out or tend to screen out individuals with disabilities unless they can show that the criteria are necessary.” Guckenberger v. Boston University, 974 F. Supp. at 134.

“[W]hen, as in this case, questions of public safety are involved, the determination of whether an applicant meets ‘essential eligibility requirements’ involves consideration whether the individual with a disability poses a direct threat to the health and safety of others.” Doe v. Judicial Nominating COmmission, 906 F. Supp. at 

 

The ADA has other protections, such as the requirement that any exclusion not be “arbitrary” or “over-broad,” but does this actually help? I can see that someone has cystic fibrosis, but can I see the bacteria in their lungs? I may not have a bug now, but will I have it tomorrow? I may see how much a bug affects me, but can I predict how much it will affect him? Doesn’t the mere fact that I can pose these legitimate questions show that a school’s actions will hardly be arbitrary? Isn’t over-broad a difficult sin to commit when the actual risk is already so broad?

Even the current case of Child A may be advertised as deciding too much. Even in his situation of not having cystic fibrosis, the current appeal does not answer whether or not he wins. Child A is suing because “I was denied my rights by being removed from school.” But, ignoring the advantages for settlement that a win on appeal may bring, a favorable result will not be an answer of “Yes, Child A, you were.” Instead, it merely says “Maybe, Child A. You can pose the question, and when you do pose the question, the jury, not a judge, will decide the answer.”

That is no clear answer to Child A. It is even less of a clear answer to a CFer like me.

The Coming Challenges

The recent history of cystic fibrosis has been a long stream of good news. Cystic fibrosis until recently was considered a childhood disease, not because it only affects individuals in childhood, but because the children did not live to adulthood. That is changing. In the 80s when I was born, the median age of survival was somewhere between 18-21. It is now 35 in the United States. I might not even catch that age before it changes, either. It is as high as 50 years in Canada. When I was born, there was not a single drug that was specifically designed for treating individuals with cystic fibrosis. Now there are more than a dozen specialized drugs both in use and in development, covering multiple aspects of the disease including some with fascinating methods of attacking the underlying causes of cystic fibrosis, and not just the symptoms.

The fact of the matter is, we are living longer, living healthier, and living better than ever before. The danger of infection and lung deterioration might be present, but that does not mean a child with CF born today can’t grow up, finish school, attend college, enter the workforce, start a family, and just live. For myself, that story has always been the expectation. Growing up I played sports, musical instruments, participated in theater, and entered whatever activity I desired. I recently graduated law school. I passed the bar. I am a licensed Virginia Attorney. About the only thing I know I can’t do is smoke cigarettes.

But what about the challenges? All this good news does not erase the danger of a bacterial infection. Individuals with cystic fibrosis are still contagious to other with cystic fibrosis.

What happens when two individuals with cystic fibrosis attend the same school? Is it proper to keep them there and keep them separate? What if they are interested in the same classes or activities and it is impossible to keep them separate? Can you really have two football teams or two cross country teams with two locker rooms? Does one have to choose a new sport? Which one? The one with the best stats and times, or the one with the most to gain from the experience? What happens when two seniors in high school with cystic fibrosis want to take the same AP history course that only has one small section of 6 students, which meets in a small room in the library, and which does not lend itself to separation of students? Does one student have to choose another passion? If so, which one does?

Importantly, no matter what the range of reasonable options may be, who chooses the outcome? Does the principal and the school board make the decision without any input of the parents or students? Can they make this decision despite the parents and students? Do the students’ wishes have equal weight as the parents’ concerns? Does the squeaky wheel get the oil when one set of parents says something but the other set of parents does not? Do privacy rules even allow one parent with a CF child to be told that another student in the school has CF? If those rules do not, then is it a good thing that parents are not allowed to be told that another child in the school has cystic fibrosis and carries certain bacteria?

In a job setting, can an employer discriminate based on cystic fibrosis if he already has an employee with cystic fibrosis? Is an employer allowed to ask about cystic fibrosis in the application process? Should an applicant disclose that information? If an employer is required to make reasonable accommodations to accommodate two individuals with cystic fibrosis, what exactly is reasonable? If the employer must make reasonable accommodations, can the employee be required to take reasonable risks in his interaction with any other employees with cystic fibrosis? What exactly is reasonable?

These are good questions. I will not even pretend to know the answers.

 

The Hopeful Conclusion

Cystic fibrosis is a unique disease with a very specific set of challenges. Currently, we have very general laws with very unspecific solutions. No other ailment presents a situation where the carriers are health risks to other carriers.

But the challenge is not just about laws and rules. Parents and individuals with cystic fibrosis have to make their own rules for themselves. What exactly is healthy living and what is worth sacrificing to achieve it? If you learn that a strain of bacteria which can be deadly for those with cystic fibrosis exists in the soil, do you avoid the earth? If you know that doing regular treatments keeps you healthy, what happens when doing a treatment means being late for work?

The longer individuals live with cystic fibrosis, the more of us will bump into each other at school, at work, and on the street. That is the realm of governments, organizations, and rules. The more we find out how to guard against infection and treat cystic fibrosis symptoms, the more we will have to refuse to do some of those things for the sake of our time and our sanity. That is the job of parents and individuals living with cystic fibrosis.

If your treatment and therapies give you a longer life but keep you from living and enjoying that life with your friends and family, what benefit did you gain from those years? In case you were wondering, that is not a medical question. It touches things much deeper than that. And the political question of rules, discrimination, shared classrooms, and infection control cannot be free from that deeper question, either. The political question IS that deeper question. It is how we put the answer to that question into action.

But the cystic fibrosis community has conquered harder challenges than this. If this issue is too heavy, just watch this awesome music video to feel good about your own life and ours, too. Both the cystic fibrosis community and everyone else needs to see this challenge and recognize it for what it is. We are already equipped, and we should not be worried. I am confident we can overcome this, just like we can overcome everything else.