Interview Contents:

• Introduction
• About the Book
• The Imperative to Discriminate
• The Good News: The Future of Therapy
• Resources for NHL Patients

Introduction

Lymphomas in general are cancers of the immune system. However, so much remains unknown about NHLs that these cancers are more readily classified by what they are not.

NHLs exhibit vast diversity in how and where the disease manifests in the body and the nature of its progression, as well as its responses to treatments. Thus, a diagnosis of NHL frequently inducts a person into an existence fraught with uncertainty and a frustrating lack of cohesive information. Beyond the difficulties of simply getting an accurate diagnosis lies a sometimes nebulous journey with no clear-cut course of action.

"NHLs are a group of cancers that are sometimes poorly understood, and the diagnoses can often be confusing," acknowledges Patient Centered Guides editor Linda Lamb. "I wasn't even sure initially whether this is really a homogenous group of people, but Lorraine convinced me that this was a subject that needed to be written about. Much of the overall uncertainty has to do with the fact that even the slow-moving form of this disease can switch unpredictably to a worse form. It's not at all like the much more determined stages of breast cancer, for example. Many people with NHL diagnoses live in the watch-and-wait scenario for years, and the information available can be very confusing."

NHL now rates statistically as the sixth most common type of cancer in the U.S., increasing at an annual rate of 3.3 percent. Should the increase proceed unchecked, the number of people diagnosed by the year 2021 will double. "If this trend continues," the author reports, "NHL soon will rival colon, lung, breast and prostate cancers as a leading cause of illness and death in the U.S."

Annealed by her own family's cancer experiences, and fortified by a background in biology, Johnston has managed to create her book in a way that guides NHL patients and their loved ones through a wealth of practical information, resources, and patient experiences. She also emphasizes the good news on the horizon: the bright future of current genetic research and exciting new biological treatments undergoing clinical trials. Little of this emerging information filters readily or accurately to the general public due to its complex nature.

About the Book

Houston:

Tell me how the book came about.

Johnston:

Eight years ago, my husband was diagnosed with lymphoma. For a couple of years we foundered away on our own with many sleepless nights. About two years after what appeared to be successful treatment, I stumbled onto an Internet support group for cancer survivors, and it just lit a flame. I've been part of these groups for five or six years now. Somewhere in the course of that time, Nancy Keene (author of Childhood Leukemia, Your Child in the Hospital, and Working With Your Doctor) noticed my e-mails, and said, "I think you'd do a good job writing a book about lymphoma." She encouraged me to contact O'Reilly's Patient Centered Guides editor, Linda Lamb.

Linda and I talked about which lymphomas were not getting addressed by books currently on the market. For those who may not be aware, lymphomas tend to fall into two categories: Hodgkin's lymphomas and non-Hodgkin's lymphomas. About 8,000 people are diagnosed annually with Hodgkin's disease in the U.S., while statistically a great many more people are diagnosed with non-Hodgkin's lymphomas. Estimates range from 45,000 annually to more than 60,000. In fact, the rate of increase in NHLs is frightening. If it were to continue at its current pace, in the next 100 years NHLs will eclipse the more common cancers like breast and colon cancer. However, it's not very likely that the increase will continue.

Houston:

Why do you say that?

Johnston:

Typically, sharp increases tend to abate. They don't usually sustain a large rate of increase over long periods of time. Nonetheless, this trend is still a very serious threat, particularly since no lifestyle factors seem to account yet for NHL. We think that we can decrease the incidence of colorectal cancer with diet, for example -- calcium and vitamin D appear to be key -- and we know we can decrease the incidence of lung cancer by not smoking. But that doesn't appear to be the case for the non-Hodgkin's lymphomas.

Houston:

Why do you think there's such a lack of information in many of the cancer-related books on the market?

Johnston:

I perceive that we have a burgeoning awareness among the population about medical issues. Books like The Andromeda Strain and television programs like "ER" have made people more aware of and hungrier for genuine, sound medical information. I think writers and publishers are catching on to people's need for this kind of information. Often older books don't contain much detailed medical information because the prevailing wisdom at one time was that people would be turned off by medical information. Now, in fact, the reverse is happening.

Houston:

To that end, what do you hope to achieve with the publication of your NHL book?

Johnston:

I hope that NHL patients, family members, and caretakers find enough in this book to go forward and learn even more. Treatments are evolving so quickly that patients who end up with a second-rate doctor at a second-rate institution may not be getting the best care. If this book encourages them to get another opinion at a university medical school or a cancer center designated by the National Cancer Institute, it might make the difference between whether they live or die. So this book is a springboard for them. The intent of the book is to show them that they can learn even more from well-grounded sources such as the National Cancer Institute's data bases and various medical journals.

Sometimes it's simply a matter of knowing what to ask your doctor, and so my book addresses the doctor/patient relationship as well. My husband's doctor, for example, never mentioned clinical trials to us. Perhaps he felt it wasn't appropriate because my husband had achieved a successful remission. When I brought up the issue of bone marrow transplantation if Larry were to relapse, his response was simply that of course my husband would be sent to a nearby well-known hospital. But I know from my own research that this particular hospital is, in my opinion, not on the forefront of bone marrow treatments for lymphoma, so I thought to myself, "No, he won't."

Houston:

Fortunately, you were aware of that. What about someone who isn't aware or doesn't know what questions to ask?

Johnston:

Of course, my book is not going to be the final answer, but most likely it will enable one to build a framework from which a tremendous amount of curiosity may arise. Hopefully, readers will begin asking questions and looking at resources. If the material published about their particular type of lymphoma is coming from one or two cancer centers in a certain part of the country, they're going to know those are the physicians they should be seeing. I think people are a lot smarter than doctors sometimes give them credit for.

At some point it might be necessary to change doctors. This can be hard for some people, but it shouldn't be. There's the time-honored tradition of getting a second opinion. People should not feel guilty, or feel as if they are jumping ship by getting a second opinion, particularly if that second opinion causes them to switch doctors. The Hippocratic Oath compels doctors to admit -- or at least it should compel doctors to admit -- the need to seek out a second opinion for their patients when they don't have the answer. That being the case, patients should feel free to get a second opinion on their own if they think it's warranted. Two oncologists who reviewed this book, for instance, stated emphatically that lymphoma biopsy material must be reviewed by a pathologist who is experienced in analyzing lymphomas in order to avoid incorrect diagnoses and wrong treatment choices. Patients need to know that their biopsy material should be reviewed by specialists at NCI or at an NCI-designated cancer center.

Houston:

Who do you hope reads this book?

Johnston:

The patient, if he or she is up to it. Reading any material at all about cancer might be so frightening they'll only read it in pieces. Certainly, the people who are taking care of patients would benefit: family members and loved ones. Also, medical professionals. Oncology nurses in particular have expressed a lot of interest in the book.

The Imperative to Discriminate

Houston:

Why was it so important to establish early on in the book the difference between NHL and other types of cancers that affect the lymphatic system?

Johnston:

Because once other cancers that are neither lymphomas nor leukemias enter the lymphatic system, the prognosis may be more grim for complex biological reasons. But patients with lymphomas and leukemias are not necessarily "at the brink." If a person's cancer is the type that alters lymphatic tissue or the lymph nodes, as lymphomas do, that's the cancer cells' "home" to start with, and not an invasion from elsewhere. Nonetheless, it's very frightening for patients to hear someone say, "My sister-in-law had breast cancer and ten lymph nodes were involved. She was classified as Stage 4 and only lived for two years."

If that same patient with a lymphoma diagnosis hears that some of their own lymph nodes are filled with cancer, it's terrifying. However, it isn't necessarily the case that a lymphoma invading your lymph nodes is going to give you a Stage 4 disease that cannot be cured. There are people with Stage 4 NHLs who certainly can be cured.

Houston:

What do you mean when you say there are "complex biological reasons" that differentiate the prognosis for NHL versus other non-lymphoma type cancers?

Johnston:

Cancers tend to target various organs based on the characteristics of the organ where they originate. If you are unfortunate enough to have a colorectal cancer, for instance, that invades first lymphatic tissue or the liver, and then eventually the spine, lungs, and brain, that's a very grave spread of disease. But a lymphoma is a cancer of white blood cells that happen to be sitting in a lymph gland. Because that's where it started, this is not necessarily a grave sign of spread. That's its home. Likewise, although leukemias generally arise in bone marrow, some of these can lodge in a lymph gland as well.

Houston:

So, if the immediate prognosis for some NHLs tends to be less severe, does your book give any kind of summary or generalities about NHL survival and recurrence rates?

Johnston:

In general -- and it's very important to note explicitly that this could change tomorrow because research is so good in this country -- the intermediate and high-grade non-Hodgkin's lymphomas often respond very well to chemotherapy and radiation therapy, and can truly be cured in some cases, especially when caught in early stages. Low-grade disease -- meaning those cases in which the cancer cells divide more slowly and spread more slowly -- doesn't respond as well, however those patients may live for a long time with disease under control. Frequently there can be a remission of one, two years, or more. Then, if the patient relapses, treatment can be repeated to get another remission, perhaps of multiple years. This might go on for 12 years -- and a lot of research can be done in 12 years.

Therefore, the generalization about prognoses is that there is this dividing line between low-grade disease and intermediate and high-grade diseases. But it's not necessarily something patients should perceive as a bad thing. They might instead choose to look upon low-grade disease as 10 to 12 years of opportunity during which advancing medical research might help them.

Houston:

Are you saying that longevity can work in your favor with low-grade disease, even though current treatments aren't as successful, and that with high-grade disease, while treatments tends to be more successful if caught sooner, the downside is the fight against time?

Johnston:

Yes. Sometimes decisions have to be made more quickly. There are a percentage of people who don't survive intermediate or high-grade disease, unfortunately.

Houston:

Now why is this? Has there been research into this low-grade versus high-grade phenomenon?

Johnston:

Part of the reason is that most treatments we have today exploit the fact that cancer cells divide more rapidly than normal cells. Many of the drugs used in chemotherapy, for example, exploit the fact that when a cell is dividing, the chromosomes -- the cell's DNA -- are stripped of their coating. They're unraveled, sort of laying around flat like a string. When DNA is in that state (there are technical names for the different states of DNA, but I'll just refer to this as the "uncoiled" state) drugs and radiation therapy work better on them.

Therefore, for intermediate and high-grade diseases where many more cells are uncoiling and dividing, these treatments usually work well. Cancer cells in low-grade diseases go through that process a lot more slowly, so the target, the DNA, isn't exposed as readily.

Yet we must remember that my previous statement is only true about treatments as we know them today. Many treatments are evolving that don't always depend on DNA being in the uncoiled state.

The Good News: The Future of Therapy

Houston:

That seems like the perfect segue into talking about what's on the horizon for treatment of NHLs. What are some of the recent developments and progress?

Johnston:

In general, the shift now is to begin to use the patient's own body against the disease. Specifically, to use the patient's own cancer against itself. We're trying to fight fire with fire now, whereas before we were fighting fire with poison. Some of the newer treatments -- one was just approved in November 1997 -- use a chemical produced by our white blood cells called an antibody. These are proteins our white blood cells secrete, and these can be produced en masse in the laboratory to attack only tumors. An antibody latches onto the surface of a tumor and changes the behavior of it. There are many, many different kinds of antibodies and they all do different things, but, in a nutshell, those targeted to tumors change a tumor's behavior in a way that makes it more likely to die. This is called biological therapy, or antibody therapy, or monoclonal antibody therapy.

Another very interesting development is a technique that extracts pieces of tumor from the patient's body and grows these cells in the laboratory to formulate a vaccine. This is then re-injected into the patient, causing the immune response to reawaken and recognize the tumor as an enemy. Here's some background information that may help explain this:

One of the puzzling problems about cancer, as you probably know, is that our immune systems tend to ignore tumors. We know that when we have tiny tumors our bodies usually attack and kill them. We always have the phenomenon of DNA misbehaving in our bodies, the way a cancer will misbehave, and normally our body recognizes this and attacks to stop it. But for reasons that no one yet fully understands, one of these tiny cancers will sometimes get a foothold and grow into a tumor that our body will just ignore.

This new direction in treatment, known as an idiotype vaccine, is an attempt to teach the patient's body to attack the tumor once again. It appears that these idiotype vaccines, or tumor-derived vaccines as they're also called, are able to stimulate the growth of a certain kind of white blood cell called a natural killer (NK) cell that really can kill a tumor. No one knows if this is going to be able to kill a very large tumor, or if it will only be effective in killing the outer edges of a tumor rather than the entire tumor. At this point it's too soon to say, yet these vaccines appear to be capable of acting against cancers in new ways.

Houston:

Where do things stand in terms of patients having access to these cutting edge antibody and idiotype vaccine treatments?

Johnston:

These substances and techniques are currently in clinical trials, and this is covered in Chapter 23 of the book, "The Future of Therapy." It goes back to the idea of the book helping someone build a frame of reference. A patient doesn't have to understand a great deal of biology. All they need to know is what to ask their doctor: "I'm interested in a clinical trial. I've heard about this vaccine. Where is this being done? Would I be a candidate for it?"

Houston:

What are you most optimistic about as far as what's coming in the future?

Johnston:

Actually, my perception of our progress is four-fold. We have a nation that's at peace; we have a government that's willing to fund cancer research; we have a collaborative spirit among researchers; and we have benefited enormously from AIDS activists who have demanded attention, research funds, and faster drug approvals for their disease.

Houston:

You mean to say that AIDs research has directly benefited other cancer research? I don't think that's a very well-known fact, do you?

Johnston:

No, I don't. I think there's a tremendous amount of unfortunate prejudice against AIDs activists. There are people who are even outright angry that AIDs is getting so much attention and research funding.

The research being done for AIDS survivors is benefiting everyone, but especially those of us dealing with lymphomas and leukemias. Some people simply don't want to hear this. Yet everything the AIDS activists have stimulated in the way of research has directly or indirectly benefited the lymphomas and the leukemias, because AIDS is a disease of white blood cells. We probably can't thank them enough for marching, for raising awareness, and for demanding action.

Resources for NHL Patients

Houston:

Certainly AIDs and breast cancer patients have learned the value of organized advocacy. Is there any sort of similar effort for NHL patients?

Johnston:

There are national groups dedicated to helping find a cure for lymphomas. The Cure for Lymphoma Foundation in New York and the Lymphoma Research Foundation of America in San Francisco are two. The Leukemia Society of America also serves lymphoma patients. These groups are all very good. The LRFA's funding of research for lymphoma is superb. I see them advertising frequently in medical journals, inviting researchers to apply for grants.

These are typically $30,000 grants, and while that's not a lot of money - after all, everyone would like a $3 million renewable grant -- $30,000 is respectable, especially since most of their money goes to research rather than being spent on overhead costs like salaries.

The founder of the LRFA, Ellen Cohen, is a lymphoma survivor. So there is a lot of advocacy being done, and while there's always a lot more that can be done, I don't think NHLs are being neglected.

Houston:

If advocacy represents the broad end of the spectrum, what other resources are there for individual NHL patients?

Johnston:

Join a support group. It's not something I would have thought of doing before my husband's diagnosis. In fact, when I first joined an Internet support group, I just deleted all the messages for the first week because I was too afraid to read them. My opinion is that good emotional support is usually going to come from other human beings experiencing the same or a similar disease. Regardless of any medical information a patient is able to find, the emotional support factors can't be discounted.

Houston:

So you definitely recommend seeking out a support group? Even if someone is not the kind of person inclined to want to do that?

Johnston:

Yes, yes. I certainly wasn't. I thought people would just be complaining to each other, and it would all be too sad to listen to. But it changed me from a crazy lady to a person who could deal with my husband's illness. I would never have thought I would endorse a support group, but here I am! In fact, I feel like the luckiest woman alive to have this terrible illness happen to my husband at the same time the Internet emerged from academia into the public domain and awareness. Even with a degree in biology, I was a basket case until I found other people who could say, "I wake up at three in the morning and lay there and worry about this for hours, too. You're not nuts!"

You have to find the right group for you. For some people, that won't be an Internet group; it will be an in-person group. For others, like me, who are very uncomfortable crying in front of other people, an Internet support group may be the better of the two.

Cancer support groups are really different from other support groups. Often there's a lot of joking, but there's silence when silence is called for. There's not a lot of hashing over the same issues in an unproductive way. Everyone cuts to the chase because, after all, time might be limited.

Houston:

Does your book cover other Internet resources for NHL patients in addition to support groups, such as access to information about NHLs and treatments?

Johnston:

Absolutely, and that's another booming area. In 1993 I was paying about $100 a month to a third-party access provider to search the National Cancer Institute's databases using Telnet, a computer, and a modem. Now it's free. It's on the Web. When you consider that 50% of men and 33% of women will be diagnosed with cancer sometime in their life (according to the American Cancer Society), the fact that this information is free and all you have to do is know how to get it -- that's an extraordinary benefit.

Houston:

And yet, despite the wealth of available information, one overriding theme of your book seems to be that because there are so many different types of NHLs and so many patient variables, there are almost no generalities or benchmarks about the disease's progression and treatment. How can NHL patients deal with that practically as well as emotionally?

Johnston:

If patients have the fortitude and persistence to find out as much as they can about their own sub-type of lymphoma -- and they are indeed able to do this -- then when they hear other patient talking about their disease, one is able to say, "That doesn't apply to me because my sub-type is different from hers or his." I think emotional fortitude will follow from this. One would be less likely to be frightened when they hear that someone else has been given only a month to live because their disease is progressing rapidly. I think that once someone has the facts, the appropriate emotions tend to follow. That can work both ways, though. If the facts they learn are very grim, for instance, emotionally they may be much worse off than if they hadn't known.

Houston:

Can you provide a synopsis of what your book covers in terms of coping with an NHL diagnosis?

Johnston:

The key issues in my mind for NHL patients are:

• Educate yourself.
• Avoid immediately assuming, after listening to other people talk about their cancer, that you will walk the same path, because you probably won't.
• Do your very best to find the best doctor and treatment center available, and be willing to move on if you're not getting good care. Consider traveling for second opinions or for care if necessary.
• Keep your information current. What you learned only two years ago may be getting dusty by now.
• Speak up about pain or any unusual symptoms you're having. Very effective treatments are available for pain - don't suffer needlessly.
• Challenge your insurance company's decisions if they won't pay for your care.
• Live each day, and love yourself. Postpone worry. Trust yourself.