When Cheryl was diagnosed with lung cancer, it came as a complete shock. She was always healthy, ate the right foods, maintained a trim physique, and exercised regularly. Plus, she had never smoked. When she developed a persistent cough six months before I met her, her primary care physician treated her with antibiotics for possible bronchitis. The cough did not improve, so a chest X-ray was performed. This showed an abnormality in the right lung, and a CT scan followed. The findings were startling. The right lung was dotted with multiple little tumors throughout, with a dominant mass where the lung meets the major bronchial tube and enlarged lymph nodes in the middle of the chest. Surrounding the lung was an abnormal collection of fluid called a pleural effusion. Cheryl was told that she probably had lung cancer and that it was in the fourth stage.
The doctors withdrew some of the fluid, and it did contain cancer cells, a type called adenocarcinoma. Cheryl began chemotherapy treatments, and her condition seemed to improve. She put up with the side effects, which were extreme. The paclitaxel and carboplatin that she received caused fatigue, hair loss, and loss of appetite. She became depressed. After three months of treatment, a CT scan showed that the tumors were growing rather than shrinking. She started to feel hopeless.
Cheryl was used to having her ducks in a row. As a grade-school teacher for thirty years, she kept those little darlings in line and well taught. In addition to her healthy lifestyle, she had a loving husband and long, solid marriage. How could she have developed lung cancer? And why wasn't the chemotherapy working? Cheryl's ducks were all over the floor, and her spirit was at its lowest. It was at this point that we met.
My first action was to request that a sample of Cheryl's tumor be sent to a specialized laboratory for molecular testing, specifically for genetic analysis of a gene called EGFR, short for epidermal growth factor receptor. The EGFR gene gives rise to the EGFR protein, which is present on the surface of many of the most common cancers and is one of many molecules that fuel the growth of cancer. Its importance is most evident, however, when it is the main driver of a cancer. This is best determined by analysis of the EGFR gene: if the genetic code contains a mutation in a critical region, then EGFR is the main power source of the cancer, galvanizing its expansion and spread in the body. In this case, blocking the EGFR protein from firing would shut the cancer down.
Cheryl hit pay dirt. Her cancer contained a mutation in exon 18 of the EGFR gene. Intensive and exciting research over the past several years has proven that patients whose lung cancers contain mutations in EGFR can have their cancers successfully treated and their lives significantly prolonged by a pill that prevents the EGFR protein from firing. In the United States, the pill is called erlotinib (Tarceva). In other parts of the world, gefitinib (Iressa) is used. Cheryl would now be treated with a "targeted therapy" that hones in on one specific target in a cancer cell and has fewer side effects than most chemotherapy drugs. She could not believe her ears when I told her that she would soon be feeling much better again. She was in disbelief and cried tears of joy when I told her that her life would now be measured in years, rather than months.
Welcome to the ever-evolving landscape of cancer care. Old therapies are being replaced by newer and smarter ones. Cancers that were previously considered "terminal" from the start are now being converted into chronic diseases in many situations. The therapies are more sophisticated, the care is more complicated, and the needs of patients are greater than ever. It is critical for all cancer patients to be involved in their own care, to understand all their options, and to aim to live life to its fullest throughout their battle with cancer.
When First Diagnosed: "What Should I Ask?"
A NOTE OF CAUTION: The following questions should be asked of your surgical, medical, and radiation oncologists. Although other types of doctors may actually make the diagnosis of cancer, they are not expert in determining treatment strategies and prognoses for cancer. More in-depth answers to each question are contained in the chapters that follow.
1. What is the specific type of cancer I have? Most types of cancer come in different varieties. For example, in non-small cell lung cancer, it may be adenocarcinoma, squamous carcinoma, or another type. A breast cancer is typically invasive ductal or lobular carcinoma or ductal carcinoma in situ. A non-Hodgkin's lymphoma can be one of thirty varieties, the most common two being follicular lymphoma and diffuse large cell lymphoma. The cancer discussion must start with the specific diagnosis. Know yours.
Helpful hint: Obtain a copy of the pathology report and go through it with your oncologist.
Advice: Ask if it would be helpful for the pathology specimen to be reviewed by a pathologist at another center, referred to as a "second opinion in pathology." Sometimes, these reviews lead to changes in the type or extent of a cancer, resulting in a change in its management. This is especially important for some types of lymphoma and breast cancer.
What is the grade of the cancer? Pathologists will often assign a "grade" to cancer that is an indication of its aggressiveness. Grade has to do with how closely the cancer resembles the normal tissue from which it is derived or how "differentiated" the tumor is. Grade is typically divided into three categories: low, intermediate, or high grade, or well, moderately, or poorly differentiated. The higher the grade, the less differentiated the cancer, the more aggressive it tends to be. In prostate cancer, grade is given a number, called the Gleason score.
Helpful hint: The pathology report will indicate the grade of the cancer.
2. What are the molecular markers of my cancer? The molecular markers of a cancer refer to the higher-level analysis of a cancer specimen, testing it for specific genes and proteins. These markers are usually performed because they help guide the type of therapy recommended. (Consult chapter 9, "Personalized Cancer Medicine," for discussion of this complicated issue.)
* Is a breast cancer hormone positive (ER and PR), Her2 positive, or triple negative? For each situation, treatment would involve hormone therapies (such as tamoxifen), Her2-directed therapies (such as trastuzumab or Herceptin), or chemotherapy, respectively. Should a genetic test, such as an Oncotype DX, be performed in order to determine whether chemotherapy is necessary?
* Is a lung cancer EGFR or ALK gene positive (in other words, do the genes contain a mutation)? If so, then treatment might involve erlotinib (Tarceva) or crizotinib (Xalkori), respectively, rather than chemotherapy.
* In advanced colon cancer, is it KRAS gene wild type or mutated? If KRAS wild type, then cetuximab (Erbitux) or panitumomab (Vectibix) may be of benefit; if KRAS is mutated, these drugs would not work.
Advice: Ask your oncologist if molecular testing has been or will be done on the cancer specimen and what the results mean in terms of prognosis and treatment.
3. What stage is the cancer in? The stage of a cancer has to do with its extent in the body. For most of the common cancers, stage is divided into I, II, III, and IV. The higher the stage, the further the cancer has traveled in the body from its original starting location. Stage is determined after a "staging workup" that may include surgery, imaging tests such as CT, MRI, and PET scans, or other specialized tests, such as endoscopic ultrasound for gastrointestinal cancers (esophagus, stomach, rectum) or a bone marrow biopsy for those diagnosed with a blood cancer.
Helpful hint: Ask the doctor if other tests are needed to complete the staging workup.
4. What is the prognosis? Is it likely, possible, or unlikely that the cancer can be cured? These questions take bravery to ask at first, but it is vital to have some sense of the realistic curability of the cancer. Even if a cancer is not curable, that does not mean that it is not treatable or that the affected person cannot live for years with the cancer. At some point, these questions must be confronted or the patient and family will enter a cancer pitfall.
Advice: Asking about prognosis helps you and your loved ones realistically come to terms with the type of battle you are up against and what time frame you are dealing with. Try your best to hear it as information, as an estimation, not as a fate or guarantee, because every patient is unique. Most important, the answer should never extinguish hope.
5. What treatments are recommended, and what are their possible side effects? This discussion should include the different options for treatment, such as different chemotherapy regimens or surgery versus radiation for prostate cancer, and which might be the best fit for you. You and your oncologist will take into consideration your medical condition, the particular side effects that you wish to avoid, treatment logistics, and what you are willing to do and put up with.
Helpful hint: When educating yourself about cancer therapies, there are no dumb questions! Make sure all your questions are answered by the doctors, nurses, and other professionals at your disposal. And although friends and family undoubtedly mean well, it is best not to rely on them for medical information.
6. Should I get a second opinion? A second opinion may be helpful for peace of mind to confirm the results and recommendations you have heard. If you are dealing with an uncommon cancer, then a second opinion is essential. But it is not necessary if the recommendations are straightforward and the oncologists and treatment center in your community are well-established and trusted.
Helpful hint: The decision to seek a second opinion is a personal one. Trust your own judgment about your health care providers. If you don't feel you need one, then you probably don't. If one is medically necessary, your oncologist will suggest it or help make arrangements for one.
Advice: If you desire a second opinion, get one from a different type of treatment center than the one you will receive treatment at. If you intend on being treated at a large cancer center, obtain a second opinion with an oncologist in your community to see what he or she can offer. If you will receive treatment in a community setting, get a second opinion at a large cancer center to take advantage of any expertise that may be found there. It may turn out that the doctors cooperate in your treatment and you get the best of both worlds.
Once you have committed to a second opinion, you must make three calls: (1) to the office of the oncologist you have already seen, asking for their records to be faxed to the doctor providing the second opinion; (2) to the pathology department or laboratory that processed your biopsies or tumor specimen, asking them to send the pathology slides and a tissue block (if requested) to the referral doctor or his or her pathology department; and (3) to the radiology department or office where you had your imaging tests performed, requesting that they make CD-ROM copies of your X-rays and CT, MRI, or PET scans. Carry these CDs to your appointment for the referral doctor to review.
7. Where should I be treated? Advertisements abound in every media outlet in the United States about the superiority of one cancer center over another. The message that some centers have a cure that others do not is false hope and very detrimental to patients and families, willing to go to any lengths to help their loved ones with cancer. I have seen more than a few patients and families greatly inconvenience themselves for care, only to regret their decisions later. Sometimes more is less. Be aware that there are cancer treatment standards and guidelines that all oncologists follow (see the resources listed in appendix 2 to learn about these). On the other hand, there are reasons to travel for treatment as outlined below. As you make treatment decisions, keep in mind:
Surgery: Outcomes are better with experienced and highly trained cancer surgeons for most cancer surgeries, especially esophagectomy for esophageal cancer, the Whipple procedure for pancreatic cancer, and gynecologic cancers (ovarian, uterine, cervical). Patients with uncommon cancers, such as brain tumors, sarcomas, and head and neck cancers, should have surgical second opinions at high-volume centers before choosing a surgeon.
Chemotherapy: The administration of chemotherapy is supervised by a medical oncologist. Although every doctor is different, chemo is chemo wherever it's given. There is no good reason to travel further than you need to for standard chemotherapy unless guided by strong preferences for treatment setting or a specific doctor. Make this as convenient as possible. If you take toxic therapies far from home, you may enter a cancer pitfall.
Radiation therapy: You should feel confident about the experience of the radiation oncologist supervising your radiation therapy. Inquire about the age of the equipment at their facility, ensuring that it is updated and current. Ask about quality and safety measures and make sure that these are monitored throughout your treatments. Radiation therapy is often a daily venture over several weeks so only travel if medically necessary.
Clinical trials: Participation in a cancer research study or clinical trial is the only way to access promising new therapies not yet approved for general use. They may be good options as the first treatment or later in the course of your cancer. Be aware that high quality cancer research occurs in the community as well as at larger centers. Inquire with the treatment centers around you by calling their research departments. You may also search on the Internet to learn what is available in any region: an excellent starting point is www.clinicaltrials.gov (and see the resources listed in appendix 2). Discuss the merits and risks of participating in a research study with your oncologist.
Helpful hint: All of your treatments need not be in one hospital. You may choose to have surgery at one hospital and chemotherapy and radiation at another without burning any bridges, offending anyone, or compromising your care.
Advice: Keep abreast of advances in cancer medicine by listening to the nightly news and by subscribing to reputable newspapers and websites (see the resources listed in appendix 2). Bring items that you hear about to the attention of your oncologist.
8. What else should I ask? What have I missed? In the frantic first steps of learning about the cancer you have and how best to treat it, you will forget about one of the most pressing concerns that go with the diagnosis: the psychological and emotional impact that cancer will exert on you and your loved ones. You cannot do this alone, meaning only with doctors and nurses. You must have a support system of family, friends and ideally at least one counselor or therapist who is skilled in caring for your spirit. You may also benefit from talking with others in a similar situation as yourself. If you do not nurture your soul along the way, you will run into serious trouble down the line. If your treatment center does not offer these services to you, seek them out (see the resources listed in appendix 2).
Advice: Regardless of how strong you are, I advise you to speak to a counselor about your life situation, hopes, and fears. You have no experience with being a cancer patient, and it is important to have a lifeline to call on when the need arises within you. The only guarantees about cancer are that it causes stress and that you need love and support to get through it successfully.
Every cancer patient is at risk for a cancer pitfall. I have compiled this list during my twenty years' practice as an oncologist at a world-famous cancer center and an excellent community cancer center. Being aware of them is the best way to avoid them.
* Thinking the grass is greener. It is human nature to take for granted what we have in our own backyard. When it comes to cancer care, some people think they have to travel and inconvenience themselves in order to receive great care. Cancer care can be very stressful, inconvenient, time consuming and costly. My advice is to first meet with your local cancer providers and then seek a second opinion or treatment elsewhere if that suits you better.
* Listening to others instead of yourself. "You can't go to that hospital! My friend Jack's aunt Millie had a horrible experience there a few years ago." Sadly, this would be enough to steer some people away from a hospital or a doctor's office. A cancer patient has to be on guard for the well-meaning but often unhelpful advice of others, some they may not even know. My advice is to make decisions about care for yourself. Your family and loved ones should be part of the decision, but in the end they should respect your decision, whatever it is. It is your life, after all.
* Accepting poor communication. It is vital to have good communication with your oncologist. This may take some time, as in any relationship, but eventually there should be a familiar bond that enables you to express your thoughts and feelings about any aspect of your cancer care. If your doctor is too busy to address your needs, ask to set up an appointment with more time allotted. If that doesn't work, it may be time to interview another oncologist.
* Accepting poor care. Wherever you receive treatment, you should be made to feel that you are an individual and not just a number. Your caregivers should have an appreciation of your lifestyle and life situation and the challenges you may face outside of cancer. Your care should be compassionate and, to every extent possible, attentive to your needs. Although few medical offices run perfectly, do not accept two- or three-hour waits to see a doctor or receive a treatment.