Living With Chronic Pain 'In The Kingdom Of The Sick'

Transcript

TERRY GROSS, HOST:

This is FRESH AIR. I'm Terry Gross. Chronic illnesses, including autoimmune diseases, are not only challenging to live with, they're often difficult to diagnose. People with chronic pain and fatigue often face skepticism when they do get a diagnosis. Recent research shows there may even be a gender gap in how men and women experience pain.

These are some of the issues that my guest Laurie Edwards writes about. Her new book is called "In the Kingdom of the Sick." Edwards has a chronic respiratory disease so rare, she's met only one other person with it, and that was with the help of the Internet. Edwards wasn't accurately diagnosed until she was about 23, about 10 years ago. Her previous book, "Life Disrupted," is about living with chronic illness in your 20s and 30s. She teaches health and science writing at Northeastern University.

Laurie Edwards, welcome to FRESH AIR. Since you're writing from the perspective of living with chronic illness, let's just start with what you have.

LAURIE EDWARDS: I am a rare disease patient. So I have, primarily, a disease called primary ciliary dyskinesia, which is a mouthful, so most people just refer to it as PCD. And it is somewhat similar to cystic fibrosis, and in my case, it means that the cilia - which are the tiny structures that line the respiratory tract - don't work, or are not there. And so there's really no way to sort of flush out bacteria and clear secretions.

So it just means that I'm susceptible to infections a lot more frequently than the average person, and when I get sick, I get much sicker, and it lasts much longer.

GROSS: And you've been in and out of the hospital all your life.

EDWARDS: I have, yes. So I've been sick since birth, actually, but I was not correctly diagnosed until I was 23, which is unfortunately somewhat typical for rare disease patients.

GROSS: What did it mean to you to actually have a diagnosis, an accurate one?

EDWARDS: It was life-changing, in so many ways. First of, emotionally, it was a sense of validation. I had been told all my life that I was an atypical asthma patient. And when my symptoms didn't respond to medications the way doctors expected them to, it sort of became this issue of, well, maybe I was doing something wrong.

Was I not taking my medicine correctly? Was I simply too stressed or anxious, and was that causing my respiratory exacerbations? I mean, I was in the intensive care unit having those questions thrown at me when I was in college. So it was extremely validating, certainly. But more than that, it just changed my quality of life dramatically, because I knew what was wrong.

I could have more effective treatment and more effective prevention. So it - that means I'm in the hospital less. It means I'm spending less time at doctors' offices. I'm able to be a more productive member of society because I have the information I need to make the best choices for my health.

GROSS: And I'm sure you meet a lot of doctors who have never heard of the rare condition that you have.

EDWARDS: Yes, all the time, all the time, which is understandable, in the sense that if you just don't see it very often and the only time you heard of it was maybe a passing sentence in medical school, it is understandable. But, you know, I've often been in the emergency room, or even admitted as an inpatient, and I have to spell it and define it, and people just don't know what it is or what to do with me, because they just don't see patients like me very often.

GROSS: Now, you have a couple of other chronic illnesses, too.

EDWARDS: Yes, I do. So have I bronchiectasis, which is another respiratory condition. It's a secondary condition, as a result of damage to my lungs from PCD. But I also have things that are a lot more common. So I have thyroid disease, and I take a medication for that every day, and I have celiac disease, which certainly is a lot more known or recognized now than it was even 10 years ago, when I was diagnosed.

GROSS: And I just want to quote something that you write in your book. You write: On the other hand, I've done so much of what healthy people do. I've graduated high school and college, studied abroad for a year, pursued a graduate degree and got married, spent four long years trying to have a child and made it through a medically intensive, complicated pregnancy to deliver a healthy little girl.

You also work fulltime teaching at Northeastern University, and you do freelance work. So you have a very full life, in spite of the illnesses that you have. So just want people to know that.

EDWARDS: Thank you. Yes. Yes. And it is constantly a juggling act. Certainly, it is for everyone, you know, sick or not. But when you do have chronic illness and it is unpredictable, and you're sort of tasked with maintaining all of the things that you were, anyway, but also these flares and these symptoms that are sometimes really quite pronounced, and other times sort of fade away.

So, you know, it is even more of a juggling act, but I think one thing that growing up with chronic illness and sort of never knowing what it is to be healthy has shown me is how to prioritize. You know, you have limited energy sometimes. You have more time that you need to spend on managing your health on a daily basis, a monthly basis.

So you begin to sort of make the choices about what is the most important, and what things are going to get your best time and energy.

GROSS: Is your respiratory problem what would be defined as an orphan illness? And I'm going to ask you to explain what an orphan illness is.

EDWARDS: Sure. Yes, absolutely. So, rare diseases, or orphan diseases are those that affect fewer than 200,000 patients. And that's in the United States. That's how we define it here. And there are upwards of 7,000 classified rare diseases that affect somewhere around 30 million Americans. So there a lot of us, but we are spread out into these very small disease populations.

GROSS: And what are some of the problems if you have, like, an orphan illness, in terms or prioritizing research to find either cures or just treatments that will help with symptoms of the condition?

EDWARDS: There are many challenges. First of all, until very recently, it was not financially appealing for pharmaceutical companies to invest in treatments for rare disease populations, because the populations are so small. Simply, you're not going to make enough money.

The Orphan Drug Act, which was first passed in 1983, has certainly made a big difference in that respect. And much more recently, in the past couple of years, we've seen a lot of investment in biotech firms - and also in larger pharmaceutical companies - into researching and developing better, more effective treatments for rare diseases. But that is absolutely a challenge.

Sort of on a larger, social scale, you know, funding is so important for research, and it's scarce, and there are a lot of diseases trying to get a piece of the pie, as it were. So the smaller your population is and the smaller you voice is, the harder it is to get that hard-earned research funding. So that the challenge that we see.

You know, my rare disease group, Primary Ciliary Dyskinesia Foundation, for example, has wristbands for awareness month. But, you know, you can't compare that what we see with, say, breast cancer awareness and sort of the month of pink.

And, you know, there are reasons for that, and the numbers make sense, but still, we miss out on some of that awareness-raising, certainly.

GROSS: A lot of people in the chronic illness category have fatigue syndromes or pain syndromes that are kind of controversial and some doctors say don't exist and some doctors say do, but it's very difficult to have a definitive diagnosis right now. So that includes chronic fatigue and immune dysfunction syndrome, and it would include fibromyalgia. What else would you put in that category?

EDWARDS: Those are the two big ones, I think, also pelvic pain disorders, sort of those quote-unquote "women's diseases" are poorly understood. Irritable bowel syndrome is another one, migraine disease - sort of these more nebulous pain conditions where we don't always know the exact mechanism that causes it, can't really test for it, but, you know, patients are certainly suffering and experiencing symptoms.

GROSS: So, in your research and in your contact with people who have, you know, various chronic illnesses, do you accept that those conditions really do exist as chronic illnesses, fibromyalgia, chronic fatigue?

EDWARDS: I do, absolutely. I believe that they exist. I believe that we are getting closer, in some cases, to finding out more in terms of the mechanisms that cause them. In think fibromyalgia is a great example of that. We do have some interesting research on functional MRI scans that show differences in the way fibromyalgia patients actually experience and feel pain.

So I think any time you can get that type of research that can help us figure out why does this happen, and then we'll figure how we treat it, is encouraging. But, I mean, the uphill battle is steep in terms of greater societal recognition, and I think that there is this underlying idea among many that, well, everyone gets tired. So why can't you just push through it? Or everyone has aches and pains. So why don't you just stop malingering and complaining and hyper-focusing on your symptoms? So it's absolutely a challenge.

GROSS: Would you describe what fibromyalgia is, or is believed to be?

EDWARDS: Sure. So fibromyalgia is primarily a pain disorder, where it's diffuse pain throughout the body. There are certainly certain trigger points in the body where it is more common. Fibromyalgia also includes a lot of debilitating symptoms like profound fatigue, brain fog, sort of cognitive challenges. And what's really hard about it is that, you know, there - it's a diagnosis of exclusion.

So you can't just get a blood test, and you can't just go get a scan and suddenly have an answer, and I think that ambiguity can cause a lot of problems.

GROSS: If you're just joining us, my guest is Laurie Edwards. She's the author of the new book "In the Kingdom of the Sick: A Social History of Chronic Illness in America." Laurie, let's take a short break here, and then we'll talk some more. This is FRESH AIR.

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GROSS: If you're just joining us, my guest is Laurie Edwards. She's the author of the new book "In the Kingdom of the Sick: A Social History of Chronic Illness in America." And she has several chronic illnesses herself. And her previous book was about chronic illness when you're in your 20s and 30s.

So there's connections - you know, your new book is a social history of chronic illness in America, and you make connections between chronic fatigue and fibromyalgia and conditions that were diagnosed, say, in the 19th century as neuresthenia, you know, a woman's condition, which is what?

EDWARDS: It was basically the throwaway term for nervous exhaustion, which is the idea that it was just sort of this hysterical, emotional state that women worked themselves into.

GROSS: That caused...

EDWARDS: That caused fatigue, and sort of what they would have called malingering, or just general malaise.

GROSS: And so, like, in an old movie when a woman takes to her bed, that would be neuresthenia?

EDWARDS: Yes. Yes.

GROSS: And so what does it tell you, what do you make of it, that there was this condition, say, in the 19th century that was described as neuresthenia, and now we have this condition that's described as fibromyalgia, or another one that's described as chronic fatigue, and they all have similarities, but none of them have had, like, a definitive diagnostic test or anything like that?

EDWARDS: Well, I think it tells us that there is a longstanding connection and perhaps bias about patients who are female. And it's very easy to say, oh, well, she's just hormonal. She's emotional. And I think that was sort of underlying the nervous exhaustion diagnosis, and it's certainly a big part of the attitudes towards patients with these conditions now, who are overwhelmingly female - certainly not exclusively, but are overwhelmingly female. And it's just an iteration of a longstanding attitude.

GROSS: So what does science have to say, if anything, about why it's predominately women who seem to have these pains and fatigue syndromes?

EDWARDS: Right. Well, we certainly have a long ways to go in terms of understanding the mechanisms that underlie pain. However, in 2009, for example, the Journal of Pain published a wonderfully comprehensive review of research into pain and sex-based differences in pain, and found that women do experience a disproportionate amount of pain and pain conditions.

For example, they're two-and-a-half times more likely, I believe, to experience rheumatoid arthritis, or nine times more likely to experience fibromyalgia, four times more likely to experience chronic fatigue syndrome, three times more likely to experience autoimmune disease, which most of them involve pain.

So it's that they are more likely to manifest it, and then when they do, the symptoms are - they're more sensitive to the pain itself. And while we don't know all of the mechanisms yet, it is believed to a confluence of physiological and hormonal differences in the way we both manifest, as well as perceive pain.

GROSS: Does it make you uncomfortable to say that? Because for so many years, like, women have been fighting against the stereotype that they're the weaker sex, that they're more sensitive, that they can't handle things, that they're more vulnerable.

EDWARDS: You know, that's such a great question, and that's one of the more interesting things, personally, that I came across and really began to understand more in writing the book. And, in fact, Paula came in, who wrote a wonderful book "All in My Head," about her experiences with migraines and chronic daily headache and gender and pain more generally, was the first to really introduce this idea - that I know of - that, you know, the women's health movement, for example, made amazing strides in terms of doctor-patient relationship, in terms of women's understandings of their bodies. And there are so many wonderful concrete victories that we could point to, yet they would not touch women in pain.

They wouldn't, because to acknowledge that these biological differences existed took away from the push for equality. So, you know, it is an interesting phenomenon, but I don't think - it doesn't make me nervous to say that, because I think the more information that we have, the better that we can treat and manage and diagnose pain for both genders.

So, you know, sex-based clinical research is one of the most important things that can happen for the 25 percent of all Americans who live with chronic pain.

GROSS: Do you think it's actually true that the women's health movement hasn't touched women's pain syndromes? I mean, is that based on actual evidence?

EDWARDS: I don't think it's that they didn't touch them. It just - it wasn't the same priority as, say, looking at the paternalistic establishment of medicine itself. So, you know, if you look at "Our Bodies Ourselves," which was published right around that time, and, you know, the whole point of that was this sort of change (unintelligible) movement, where women took control of the information they had about their bodies.

So obstetrics and gynecology was the first frontier, but certainly women with chronic - and all patients with chronic illness - have benefited from a lot of the changes from the women's health movement. We can look at the number of women who are now enrolled in medical school and you are practicing physicians, and that is a huge change, and we can trace it back to that.

We can look at the idea of the patient as empowered, as collaborative and being involved in their decision-making about their own bodies, and we can trace it back to that. So it's not that I think they absolutely ignored it, or this - a ton of research that say no, they wouldn't go near it, but, you know, if the message is equality for all, then highlighting those same differences would have been problematic.

GROSS: Right. Though I just do want to say that OB/GYN was such a priority of women's health, and that's all about the differences between men and women, since men don't have gynecological needs.

EDWARDS: Oh, absolutely. And so maybe I should clarify and say that I'm really talking about pain disorders as maybe separate, because I think it's a really interesting tension with obstetrics and gynecology, because childbirth is the one area where no one really doubts that women have pain, right.

So, you know, it's just chronic pain disorders and chronic pain. It's not that it wasn't important, but it wasn't the same priority or the same focus.

GROSS: And you also write about how scientists are finding that women metabolize certain medications at a different rate than men do, at a different speed than men do.

EDWARDS: Yes, absolutely, and that's really interesting. You know, it wasn't until 1993 that the National Health Revitalization Act first required that researchers include women and minorities in clinical research trials, and that phase three drug trials for drugs to be brought to market had to be analyzed by sex.

So before that, there was no mandate that women even had to be included. So the result of that is, even to this day, the majority of the drugs that we take and that are prescribed to us have been dosed according to the average white American male.

So, for example, some antidepressants metabolize and build up in your blood in different concentrations in men and women. So the dosages, you know, aren't - they don't work the same way. But yet there isn't really any information on a label about that for women. It just says, you know, the only information we usually see is whether or not it's suitable for pregnant women to take.

GROSS: And you write that recently, the FDA announced it was cutting the prescribed dose, you know, lowering the prescribed dose of Ambien for women, because women remain drowsy for a longer time after taking the drug than men do.

EDWARDS: Yes, yes, and really interesting sort of intersection between sex and gender when it comes to prescription drug dosing is - I mean, look at the case of antidepressants, for example. There is a lot of research that shows that women's pain symptoms are dismissed or called psychogenic or emotional, and that's much more likely to happen to women than men.

And at the same time, they're treated less aggressively. So sometimes, for example, men might be treated with painkillers, while women reporting the same symptoms might be getting antidepressants. So if you're giving a woman an antidepressant, and then it's not working in the same way, then it's sort of this vicious cycle, right, where we see all these different tensions between sex and gender.

And I think, you know, in the case of Ambien, which is a sleep drug, you know, we're beginning to see some recognition of that. So I think that that's important, and I think that, again, further sex-based research will really help all genders.

GROSS: So what are some of the possible explanations for why women might metabolize medicine at a different rate than men would, or might, you know, experience pain differently than men would?

EDWARDS: Sure, well, with the drug metabolization, we simply - we have different body compositions. So our organs are not the same size. We have different percentages of body fat. So all of those things can make a difference in the way that our bodies metabolize drugs.

GROSS: Laurie Edwards will be back in the second half of the show. Her new book is called "In the Kingdom of the Sick." I'm Terry Gross, and this is FRESH AIR.

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GROSS: This is FRESH AIR. I'm Terry Gross, back with Laurie Edwards, author of the new book "In the Kingdom of the Sick: A Social History of Chronic Pain in America." She teaches health and science writing at Northeastern University. She has a rare respiratory disease. Her previous book was about living with chronic illness in your 20s and 30s. When we left off, we were talking about research suggesting there's a gender gap in how men and women experience pain and metabolize certain medicines.

You point out that a lot of doctors say to patients with chronic pain it's, you know, stress is causing it, you know, stress is behind it, so you need to learn to relax. But you say that often, it's the pain that's creating the stress. But the whole thing becomes kind of a vicious cycle, maybe.

EDWARDS: Absolutely. And, again, that's something that I can relate to personally, even if the symptoms I'm talking about aren't pain symptoms. So, you know, I would be in the hospital and, you know, I'd be stressed out about missing my internship in college or worried about finals. And I would get all these questions: Are you sure you just can't handle the stress? And I would say over and over, you don't understand. It is this disruption of being in the hospital and missing out on my busy life that causes me stress.

It's not the stress that causes me to be here. It's the fact that my symptoms aren't getting better, and I'm sick and I'm in the intensive care unit when I'd rather be working on the campus newspaper that is an enormous source of stress for me. And so I think that that is the cycle.

Of course, you know, stress can cause physical problems, and absolutely, it exacerbates existing conditions. So I'm not saying that there isn't a significant role with stress, but for more patients, it's the other way around, that instead of the stress being the only cause of our symptoms, it's having conditions that haven't been effectively diagnosed and managed, and therefore have a bigger impact on our ability to lead productive lives that is stressful.

GROSS: So when you have any kind of condition that causes pain, the doctor's office is likely to ask you to fill out where you stand on the pain scale and rate your pain one to 10. And you write about this a little bit. How do you deal with that? Do you feel like you can accurately grade your pain? And one to 10 compared to what?

(LAUGHTER)

EDWARDS: And that's exactly the challenge, is that pain is inherently subjective. It - so one person's four could be another person's 10, and vice versa. And I think it's especially tricky when you're someone who has lived with chronic pain for so long and is used to pain, because maybe what registers to you as significant, you know, would be crushing to somebody else if they're not used to it. So I think the inherent subjectivity of pain, as well as the inherent subjectivity of the health care professional who's then interpreting your perception of pain makes it incredibly challenging.

GROSS: Do you try to, like, scale down your grading of pain so doctors don't think you're complaining or something? Or do you try to like grade it up so doctors take very seriously the pain that you're having?

EDWARDS: You know, personally, I tend to go down, because, you know, I'm - as just sort of a longtime patient, anytime I can get myself to not be admitted in the hospital is wonderful. But, you know, what I've heard from interviewing many, many, many patients - particularly younger women with pain - is that, you know, there's this outside perception that women overestimate their pain, and they just complain more than they can't handle it. But actually, lived experiences of patients bears another truth altogether, which is that, you know, they're so hyperaware of this that they are underreporting their pain because they don't want to look like malingerers and they don't want to be thought of as chronic complainers.

GROSS: So one of the things that you do, Laurie, is that you blog, and you blog all about, you know, chronic illnesses and you write about your own experiences. You connect to articles about interesting research. You connect to other people who are writing about their personal experiences with chronic illness. And you write that it's only through the Internet and social media that you've ever met anyone with a primary lung disease that you have, because it's so rare.

EDWARDS: Yes. Absolutely. And I think, you know, we can look at medications, and we can look at improved diagnostics, and we can look at refinement in surgeries as all these wonderful technological breakthroughs, but for the chronic illness community, the Internet and social media are what, you know, I call in the book Health 2.0 - which are these platforms that allow patients to connect to each other - are incredibly life-changing. Absolutely.

So, for me, when I was first diagnosed with PCD several years ago, you know, I didn't know much about it. Everything was new to me in terms of the new treatments I would be getting, and I found a disease group, a Yahoo group. And, you know, there are only 400 Americans who have been appropriately identified with what I have, with PCD. Up to 25,000 people are suspected to have it, but because it's so hard to diagnose, they haven't yet been caught.

So I found this group, and there are people who are talking about what I was thinking about. And, you know, there was this wonderful woman I connected with in Ireland, and she happened to be visiting Boston a couple years ago, and said, hey. You know, we had corresponded over Facebook, and she'd asked me questions. And she said, hey. Let's meet.

And, you know, it was the first time in my life that I sat down next to somebody with what I had and, you know, who didn't sort of give me an evil eye when I coughed a lot and didn't worry that she was going to catch something. And, you know, I doubt that I'll have that experience again. But it was through the Internet that that was ever possible.

And that can be incredibly valuable to patients who, regardless of your diagnosis, you know, if you're feeling well and you're homebound or, you know, you're not as active socially as you used to be, or you feel isolated by your diagnosis, or you feel isolated by your lack of diagnosis - because that's a huge issue, too - then finding people who you can connect to emotionally who understand what you're going through can make a big difference.

GROSS: If you're just joining us, my guest is Laurie Edwards. She's the author of the new book "In the Kingdom of the Sick: A Social History of Chronic Illness in America," and she has several chronic illnesses herself. And her previous book was about chronic illness when you were in your 20s and 30s. And you're in your 30s now, right?

EDWARDS: Yes. I am almost 33.

GROSS: OK. So when you were in your 20s, you met the man who became you husband. And so this leads to a question that I guess anybody with a chronic illness has to ask themselves if they're dating: At what point do you tell the person that you've met and that you're starting to see that you have a serious medical condition? So how did you handle that with the man who became your husband?

EDWARDS: Well, we had a really interesting story in that respect. I mean, I think, generally, the issue of disclosure - whether it's when do you tell your boss you're sick, or romantically, when do you tell the person you're just dating that you're sick - is a huge one, because you don't want to scare people away. You know, people don't often know a lot about what conditions you may have, and, you know, you don't want to scare people.

But I think what was really different about interaction with my husband, John, was that the night that we met I couldn't escape disclosing it, disclosing that I was sick, because I had actually just been released from the hospital a day or two before. It was New Year's Eve, and my roommate and I were throwing a party. And I was wearing a dress, and my arms were covered up and down, both arms in huge ugly bruises from all the IVs I had had.

And even though I had a rap and I was - a shawl, and I was covering my arms, it was still really noticeable. You know, I had - was so frustrated and I was so exhausted and I was so worn out, and I was just trying to make it through the night and be a good hostess that I didn't have the energy to play games about it.

So I just straight out told him. I said, I have a lung disease that's sort of like cystic fibrosis, and I just got out of the hospital, because it was the elephant in the room. I mean, you're looking at me, and I'm absolutely covered in bruises. So I felt like I just wanted to be up front about it, and it ended up being really great for us. It's certainly not how I normally would have operated. You know, I'm not usually one to sort of open with that, but it really ended up working out well.

GROSS: Why did it work out well to get that on the table right away?

EDWARDS: Because he - even though he didn't know a ton about my conditions themselves, he could look into it. He knew right away the unique circumstances of my life. I didn't have to hide things. You know, just a couple months into our dating, I had to have a bronchoscopy just to take some tissue from my lungs. And, you know, I went under anesthesia. And, you know, I was, you know, recovering from that and coming home, and I had all these different tubes and wires that they had to leave to test something else and, you know, it was just very, very present, very there. And he was able to support me and be there for me, and he came home from the hospital with me.

And very quickly into our relationship, he asked if he could learn how to perform chest PT for me. So it allowed him to be a part of things, and it allowed illness to be just something that was a part of our relationship. It wasn't something that defined it, but it also wasn't something that I was hiding and putting up these barriers about. And that really allowed us to approach it as a team, and I think that was a lot better for both of us.

GROSS: So this is probably way too personal to ask - in which case you should tell me, and not answer it. But...

(LAUGHTER)

GROSS: You have a young daughter now, after a difficult pregnancy. Do you worry at all that there'll come a time when she's going to have to take care of you, long before children usually have to take care of their parents?

EDWARDS: You know, that is a wonderful question. Do I worry about it? I mean, sure. And because I live that, you know, I have parents with serious health needs. And so, you know, I'm not always the patient. Sometimes I'm the caregiver, and my siblings, too, absolutely. So I've seen that. You know, I didn't expect to be sort of part of the sandwich generation, as it were, at the age of 30. So I know how I guess stressful and challenging that can be.

You know, I also, on the other hand, though, you know, know that I am managing my health really well, that I have done all I can to prevent disease progression, you know, that I have, you know, a healthy spouse, that we have an extended community of relatives and friends that support us and therefore, support her. So I'm not naive to the challenges that lie ahead. I mean, my father just had a kidney transplant three weeks ago. So I'm certainly not naive. But I have a lot of hope that, you know, that we've set myself up and my family, and therefore, have set her up so that we can all succeed.

GROSS: Well, Laurie Edwards, thank you so much for talking with us. Thank you very much.

EDWARDS: Thank you. It's been an absolute pleasure.

GROSS: Laurie Edwards is the author of the new book "In the Kingdom of the Sick." You can read an excerpt on our website, freshair.npr.org.

Coming up, jazz critic Kevin Whitehead reviews the new box set "Classic Earl Hines Sessions 1928-1945."

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