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Life Lessons From An Ultra Rare, Potentially Fatal Disease

Sue Levy and her family on vacation in Buenos Aires fall 2013. (Courtesy)

Sue Levy and her family on vacation in Buenos Aires fall 2013. (Courtesy)

By Dr. Annie Brewster
Guest contributor

What if you were suddenly diagnosed with a potentially fatal disease just when your life, work and marriage were on track and your plans to start a family were underway?

That’s what happened to Sue R. Levy.

In 2008, at age 37, she was diagnosed with Pulmonary Lymphangioleiomyomatosis, otherwise known as LAM, a rare, chronic, progressive lung disease in which the lungs fill up with cysts. The result is gradual destruction of the normal lung architecture, compromised breathing and, in many cases, an eventual lung transplant — a procedure with major risks. The LAM Foundation reports 10-year survival, following a lung transplant, at 47 percent.

Fueled by estrogen, LAM primarily affects women in their childbearing years. With only 1,300 documented cases in North America, LAM is poorly understood; currently, there are a few experimental medications in use, but no proven treatments exist.

Prior to the diagnosis, Sue, who lives in Brookline, Mass., had a successful career as a marketing executive, she was happily married, and she and her husband had decided to have kids. Though they struggled with infertility, undergoing six unsuccessful rounds of IVF, Sue still felt that this would work out eventually.

“My whole life I thought the way the world worked is that if you were a good person and you worked hard you could avoid bad things,” she said. LAM changed everything.

Suddenly, Sue was forced to redefine herself as someone with a chronic disease and squarely face her own mortality. In addition, she had to let go of some of her dreams, notably, her desire to get pregnant, as the high levels of estrogen associated with carrying a child would accelerate her lung destruction.

Initially, she was angry. But the disease helped her focus on what she really cares about: she went to school to study nutrition and became a natural foods chef. In 2011, inspired by her own healthier lifestyle changes, she quit her marketing job and started Savory Living-Healthy Eating, a nutrition and health company that provides online healthy eating and cooking classes.

In addition, Sue and her husband now have two young daughters, conceived using egg donors and a gestational carrier.

Listen to Sue’s story here:

Interview highlights:

From ‘Healthy’ To ‘Terrifying’

If you had asked me before my LAM diagnosis I would have told you that I was a healthy person, that I am living a healthy life. There isn’t a disease in my family. This is something I don’t have to worry about and I’m doing great. What was so interesting is that the signs couldn’t have been clearer that I wasn’t. I was heavier, the energy wasn’t great. I had a lot of digestive problems and I faced infertility. But I just thought that was the way life was. I had had a lot of pain and it felt almost like this boa constrictor was around my midsection squeezing my rib cage.

So I went to the doctor and he said ‘You know I’m worried that maybe you have a blood clot in your lung, I want to go get a CT scan.’ And they did the scan and on our way back to our house I got a call from the doctor and he said ‘You need to come in right away,’ and I said ‘Oh, is it a blood clot in my lung?’ And he said no. And I said ‘Oh great!’ And he said ‘No, you need to come in right away.’

We got into the office and he actually said to me because he knew we were trying to conceive, and he said, ‘Life as you know it is about to change considerably. Continue reading

Q&A: 5 Steps To Take Charge Of Your Health

Dr. Gary R. Epler, author of "You're The Boss"

Dr. Gary R. Epler is a lung consultant at Brigham & Women’s Hospital and the Dana-Farber Cancer Institute. He has also just published his first book for the lay public, titled “You’re The Boss: Manage Your Disease,” and subtitled “Five steps to take charge of your health.” In it, he shares lessons that initially derived from his work with patients who have a rare lung disease he discovered in 1985 — but that apply to a far broader swath of the medical public. Just about all of us, in fact.

The book brims with instructive tales of over-aggressive patients and doctors, but I found its overarching tone to be an encouraging, “Don’t worry, you can do it!” Here, lightly paraphrased, he describes the five steps and beyond.

Q: I really enjoyed your book, especially its use of Socratic dialogues to make for totally painless teaching. But the general impression I came away with is that as patients or potential patients, our main goal needs to be to keep ourselves out of the clutches of the medical system as much as possible. As a doctor who works in that system, is that indeed your message?

A: It is my message. I was thinking about the most important thing I could say today, and it is that people need to have a positive approach to their disease. They need to say, ‘I can manage this disease.’ Not, ‘Why did it happen to me?’ and look for blame, but ‘I can manage this disease,’ and as a result, people will learn how to manage their diseases with the least amount of ending up in hospitals. They’re dangerous places. They’re fantastic places if you need them but they’re really quite dangerous if you don’t. And learning about your disease, learning these five steps, will help you keep that to a minimum. Sometimes it may be needed, of course, but it will be kept to a minimum.

Q: So what are your five steps??

Step One: Learn all you can.
The first step is to learn everything you can about what you’re facing, about your disease. Talk to the doctor, talk to the nurses, but also go on the Internet. There’s amazing information on the Internet about diseases that affect one in a million, and not only that, but you can talk to people with the same issues and problems, and learn things. People worry that there’s some really bad information on the Internet, and that is correct, but trust yourself. You will find what you need to manage your health.

Step Two: Understand the diagnostic process.
This means that doctors are going to be be asking you questions and examining you, and then they’re going to be telling you about tests. Be sure to ask your questions: What is this test going to show? Is it going to help me in my situation? Is there only a 1% chance it will answer the question? What are the risks? Some of these procedures are quite hazardous, and if the benefit is worth it, they’re okay, but if there’s no benefit, all risk, it’s just not worth the test.

A story about a friend of mine: He was having some morning vomiting in January. He went to the doctor, who took some X-rays and found nothing. They did blood tests — normal. They did an endocoscopy test, looking into the stomach — normal. The vomiting continued, but everything else was fine. The doctor said he wanted to do a colonoscopy. Why? My friend asked. The doctor said, ‘We need to be thorough.” My friend said, ‘That’s not a very good reason,’ but they went ahead and it was normal. Then the doctor said, ‘We need to open up your belly and look inside.’ My friend said, ‘Why would you do that?’ The doctor said, ‘To be thorough.’ My friend said, ‘Look. We had an abdominal CT scan, there were no masses, no cancer.’ His weight was fine, his appetite was good, everything was perfect, he was just vomiting in the morning. And he said, ‘I don’t think so. This procedure is a fishing trip. There’s risk associated with it.’

By the way, he had felt like he wasn’t getting enough sleep in January, so he had gone to a health food store and picked up a natural sleep aid, melatonin. He’d been taking two tablets every day. He went to the Internet and the first sentence he saw was ‘May cause gastric distress and vomiting in some people.’ He stopped taking it, the vomiting went away, and that was the end of it. But look at the diagnostic process he went through! That’s why you need to know about the diagnostic process.

Step Three: Know the treatment options. Continue reading

New Cigarette Warning Labels: Death, Disease, Burning Skin

Some of the new warning labels the FDA is considering for cigarettes

If these graphics don’t give smokers pause, nothing will.

The New York Times reports that the U.S. Food and Drug Administration is proposing a series of graphic new warning labels be placed on cigarette packs, with bold messages, like “Smoking Can Kill you,” and “Cigarettes Cause Cancer,” and horrifying pictures depicting the many ravages of smoking.

Designed to cover half of a pack’s surface area, the new labels are intended to spur smokers to quit by providing graphic reminders of tobacco’s dangers. The labels are required under a law passed last year that gave the Food and Drug Administration the power to regulate tobacco products for the first time.

The proposed labels include pictures of a man smoking from a tracheostomy tube inserted into his throat; a diseased lung; and a woman holding a baby in a smoke-filled room. The proposals stayed away from some of the more gruesome labels used in other countries, where pictures of blackened teeth and diseased mouths are common.