Navigating the Patient Journey: Neuroendocrine Tumors
We hope this guide will be a useful roadmap for navigating your unique neuroendocrine journey. There are many reasons to be hopeful as there have been multiple advances in neuroendocrine cancer care in the past several years. We wish you strength throughout your journey. Know that you are not alone.
You are one of a kind.
Like fingerprints and zebras, no two neuroendocrine patients are the same. That’s why many organizations that advocate on behalf of these patients display zebra stripes as their symbol. Another reason we use the zebra symbol is to raise awareness in the medical profession. During their medical school training, doctors are taught the old adage, “If you hear hoofbeats, think horses, not zebras,” meaning that common symptoms usually are caused by common diseases. In NETs, however, we need our doctors to be prepared to “think zebra” as well.
Navigating the Many Decisions Involved in Cancer Care
- Steps to Working Through Treatment Decisions
- Navigating Roadblocks
- Advance Directives
- Being Ready in Case of Emergencies
Managing NET as a Chronic Disease
- Surveillance and Monitoring
- Functional Tumors
- Carcinoid Crisis and Carcinoid Heart Disease
- Emotions as Part of the Journey
- Supportive Care Throughout the Journey
- Supportive Care Nearing the End of the Journey
Arriving at Whole-Self Perspective
Choosing a Provider
Here are some questions you might consider asking to understand if the provider has the necessary expertise in NETs:
- How many NET patients are you currently treating? A low number may raise concerns about experience level.
- Would you be willing to consult or collaborate with an NET expert?
- Are you familiar with and are you willing to consult the latest expert guidelines for treating neuroendocrine tumors by the National Comprehensive Cancer Network (NCCN) and the North American Neuroendocrine Tumor Society (NANETS)?
- Do you have a multidisciplinary team to collaborate with on my case? Will my case be discussed by a tumor board (a regular meeting of specialists who discuss treatment options for cancer patients)?
Establishing a Treatment Plan
When establishing a treatment plan, it is very important to know as much as possible about the tumor itself—grade, origin, stage, organs involved by the cancer, whether the tumor makes any biochemical substances (hormones), and if you have had any symptoms from the cancer. Additionally, it is important to consider any previous history of cancer and related treatments, as well as your overall state of health.
GRADE: These tumors do not all behave or grow in the same way.
Grade refers to how fast or how slow the tumor is growing. Doctors use a sample of the tumor obtained through a biopsy or through surgery and examine it to determine how quickly the tumor cells are multiplying. The result is the Ki-67 Index (Mitotic Index), and the higher the measure, the higher the grade, on a range from Grade 1 or G1 (slower growing) to Grade 3 or G3 (faster growing).
STAGE: A tumor can start in one place and then spread (metastasize) to other parts of the body.
Stage refers to how advanced the cancer is—how far it has spread, or traveled, from where it began in the body. Using scans or imaging (CTs, MRIs, or various types of PET scans), doctors can “see” where the tumors are in your body. The stages range from Stage 1 (has not spread) to Stage 4 (spread to other organs or parts of the body).
DIFFERENTIATION: The structure of the cells that make up NET tumors is variable.
Differentiation refers to how the tumor cells look when compared to normal cells. Well-differentiated means the cells generally resemble normal cells, and these are usually slower growing tumors. Poorly-differentiated means the cell structures appear abnormally jumbled or disordered as compared to normal cells, and these are typically faster growing, more aggressive tumors.
In lung NETs there are additional classifications: well-differentiated lung NETs are further classified as typical or atypical, with the typical ones being more slow growing than the atypical ones. Atypical lung tumors are sometimes called moderately differentiated or poorly differentiated. Poorly differentiated lung neuroendocrine tumors may also be called large cell neuroendocrine tumors of the lung.
Documenting the Journey
There is a lot to remember, and terms are confusing. If you track and record things as you go, you will not miss important information along the way. Here are some record-keeping tips:
- To capture key information about your disease in one place, find an app or a tool you like, such as NET VITALS from LACNETS.
- You can use Dropbox, Box, Google Drive, etc., to store and share reports, labs, notes, advanced directives and other documents in the cloud.
- Organize every piece of paper related to your care, for example, by year or by practitioner, and keep these documents in one place.
- Take the same notebook with you to every appointment so that you can refer to your notes from previous visits.
- Keep detailed records of all communications with your insurance company, including dates and times, the name, title, and contact information of the person you spoke with, and the information you received.
- Ask for and keep at least one CD of every scan that you get. Many NET patients survive for years, even decades, and it is quite possible that earlier scans can be helpful later. Get two copies of scans, if possible, so you can share one with additional providers.
Navigating the Many Decisions Involved in Cancer Care
Dealing with a serious disease means making multiple decisions and choices that can be quite difficult. Who do you tell about your diagnosis? How can you handle work and family responsibilities? Who should be your caregiver? How do you navigate all this?
It may help to consider a few things about yourself and how you make decisions. Do you prefer control or do you prefer others to step in and help you make decisions? What type of information and level of detail would be helpful to you? Are you comfortable discussing sensitive things with others, including those close to you?
Steps to Working Through Treatment Decisions
Caring doctors will always support your efforts to understand your disease. Work to foster open, honest communication with them. Share your hesitations and struggles. Don’t be afraid to ask questions or request a second medical opinion (or even a third) before making a big treatment decision. Patients who are secure in their treatment decisions often report improved outcomes, including better quality of life. Remember, you are a vital part of your care, and what you bring to the process is the most important filter of all.
“The more quality information you have, the better decision you can make. Working with a specialist helps get more information, detail, and experience about the different treatment options, which can fill out the picture and help you evaluate what is right for you. Such choices can be very personal too. Your thoughts and feelings about treatment choice are critical. The most important thing is to remember that we are here to help you.” Eric Liu, MD, FACS
Following this process may help with your decision-making:
- Start with yourself. List your goals for treatment and preferences for your quality of life, and anything else that is important to you. Do you prefer to be treated close to home? Do you want to continue working during treatment?
- List any financial, insurance, and logistical considerations that might affect your decisions.
- Ask your medical team about the effectiveness and side effects of treatment and the time it will take to recover.
- Ask what else is out there. Clinical trials can be an avenue to therapies that show promise but are still considered to be “investigational.” It is important to remember that statistical data from scientific studies is averaged across a large patient population, and your experience with a particular treatment might be different from the “average.”
- In addition to getting a second opinion, request that your case be reviewed by a multidisciplinary tumor board if available.
While consultations can be extremely helpful, they can also create confusion if your consultants recommend different courses of action. You may feel pressured by loved ones to choose a particular medical intervention. This can lead to tremendous stress and discomfort. Everyone involved needs to respect your decision—a decision based on your goals and what you can tolerate. Having respectful and caring conversations with your loved ones may help them understand your decision and the reasoning behind it. Social workers and therapists can help navigate these conversations if emotions run high.
You may get many suggestions from friends and family. Whether it is finding a clinical trial, taking a dietary supplement, or following a special diet, everyone seems to have an opinion. During this difficult time of adjustment to your rare cancer, unsolicited advice can be a drain on your energy. If so, ask a trusted friend or relative to run interference for you. Or redirect the conversation by saying, “What I really need now is...”
The fear and anxiety that can accompany a cancer diagnosis can be very limiting and hard to overcome. Decision fatigue can set in. Consider asking to speak with a social worker or psychologist if you find things overwhelming and need more help.
As adults, there are decisions that we all need to think about in the case of sudden impairment due to accidents, or health crises, or serious illness, but these are often neglected until it is too late. Every adult in a family should prepare advance directives.
Advance directives allow you to communicate the type of medical care you want if you become too sick to make your own decisions. Yes, preparing these documents ahead of time is a chore, but think of it as a gift to your family members. Without an advance directive, they might have to decide what is best for you without your input.
Be prepared to be asked if you have these documents when you go in for a surgery or a procedure requiring anesthesia.
- A living will states your wishes should various medical scenarios occur, like stroke or coma.
- A medical power of attorney (sometimes known as a durable power of attorney), appoints someone as your health care proxy or a healthcare representative to make healthcare decisions for you when you are unable to speak for yourself. A proxy can be a family member or trusted individual who will carry out your wishes. Your proxy is also allowed to access your medical records.
You should also let those close to you know where your important papers are stored and what your wishes are for burial, memorial services, or celebrations. Conversations surrounding these difficult decisions are sensitive and deeply personal, and there are cancer organizations that provide resources or counseling to help you have these conversations with loved ones.
Being Ready in Case of Emergencies
It is always advisable to have an “emergency preparedness kit” at the ready. Here are some important items to keep in your kit:
- People to reach: Carry emergency contact information, including treating physicians and their contact information.
- Emergency bracelets or cards: Wearing an emergency bracelet or keeping an emergency card in your wallet or billfold can alert first responders including police and paramedics to any life-threatening conditions you have, including allergies and risk of carcinoid crisis, in case a medical emergency
prevents you from communicating.
- Smart devices: Smartphones and smart devices often have a feature that allows you to set up your emergency contacts and add essential information such as medical conditions, allergies, and current medications. This allows first responders to access important medical information from the lock screen, even without your passcode.
- “Go kit” or “Go bag”: Pack a casual travel bag with supplies you’d need if you were admitted to the hospital, such as a spare change of clothes, extra medications, a flash drive or other record of your current medical information and history, people to contact in case of emergency, and your advance directives.
Managing NET as a Chronic Disease
For the most part, this section focuses on the journeys of those with slow(er) growing disease and not all will apply to those with more aggressive, high-grade NETs and NEC.
Coping with a serious disease, especially if you will always have it, is very challenging. The terrain can feel foreign until you adjust to where you are now. It is important to watch for and manage issues that are related but not directly tied to your cancer. Such difficulties could include sleep problems, digestion issues, nutritional deficiencies, pain, fatigue, as well as anxiety and depression. Do not be afraid to ask your doctor for a referral to other providers for help with these issues.
Surveillance and Monitoring
Most likely you will be dealing with NET cancer in some way or another for the rest of your life. It is important to understand that NETs recur very often, sometimes years later, even if a patient is determined NED (No Evidence of Disease) at one point. Patients who are living with existing disease should be checked at regular intervals for any changes. A provider with NET expertise will know what to look for and how often to check.
Surveillance is watching for disease recurrence once all visible signs of the cancer have been removed by surgery or other therapy and the patient is considered NED (no evidence of disease). Even in this case, there should always be a physician-monitored schedule of surveillance. This schedule of surveillance may differ in how (which modalities) and when (how often) depending on individual circumstances.
Monitoring is watching existing disease by comparing the results from the latest tests and scans to prior results. How you are monitored will depend on the type of NET you have and the location and extent of the cancer in your body. It may include blood tests, 24-hour urine collections, and imaging scans such as CT, MRI and/or nuclear medicine scans such as the Gallium-68 DOTATATE PET scan. If you have any questions about your monitoring plan, ask your doctor. It is important to have confidence that you are being managed well.
Experiencing bouts of anxiety can occur as part of having these regular scans and check-ins with your oncologist. Support groups often discuss the ups and downs of living between scans. It is part of the reality that you will adjust to in your own way, which is not to say it is easy. Keep in mind that the same adage “knowledge is power” is true here as well as during your first diagnostic work-up. When you are worried, remind yourself that early detection of changes leads to earlier intervention, which offers the best shot at controlling disease!
A certain proportion of NET patients will have to adjust to living with symptoms that the cancer is actively causing. NETs can upset your normal hormone balance; your doctor can help discern if your symptoms result from your tumors and, if so, how best to address them. It can take a long time to sort this out, which can be frustrating, but keep at it.
NETs that actively produce and release excess hormones into the body are called functional tumors. It is generally understood that about 20 to 35% of all patients may have some level of functional disease. This causes imbalances that affect your body and how it functions and may create dangerous, health threatening issues.
Non-functional tumors do not cause imbalances, either because they do not release hormones or they release such small amounts. However, it has been observed that some tumors that are originally non-functional can change after a while and begin to produce and release hormones.
Excess hormones can cause a myriad of symptoms referred to as a syndrome. Symptoms will differ depending upon where the tumor arises and which hormone is overproduced. The most common is carcinoid syndrome caused by excess serotonin. Patients will differ in the frequency and intensity of symptoms they experience. Some may require constant management of their symptoms while others may experience symptoms only occasionally. If you have any of the syndromes that can be caused by a functional NET, you may need the attention and care of a knowledgeable endocrinologist (a specialist in the endocrine, or hormone-releasing system) to help your oncologist sort out, treat, and monitor your symptoms.
A typical treatment for the syndromes caused by functional NETs is prescribing somatostatin analogs (SSAs). Your doctor can discuss all the available variations of these medications with you. A medication called telotristat ethyl can be given along with SSAs to help control carcinoid syndrome diarrhea that is not managed by SSAs alone. In some cases, surgery to remove as much of the tumor load as possible is recommended. This is referred to as debulking surgery and this can help reduce the effects of functional tumors.
Functional Tumor Syndromes
Carcinoid Syndrome: usually arises from NETs in the small intestine or lung due to the overproduction of serotonin, which may trigger severe diarrhea, flushing of the skin, night sweats, and/or wheezing. Carcinoid syndrome diarrhea and its treatments can lead to pellagra, a niacin-deficiency disorder.
Zollinger Ellison Syndrome: results from an NET of pancreas or duodenum and produces excessive levels of the digestive hormone gastrin. This tumor is also called a gastrinoma. Symptoms include diarrhea, severe ulceration of the small bowel, heartburn, and abdominal pain.
Glucagonoma: an NET that usually begins in the pancreas. The overproduction of the hormone glucagon raises the concentration of glucose and fatty acids in the bloodstream and can cause diabetes, severe weight loss, severe rashes, and blood clots.
Insulinoma: an NET that almost always begins in the pancreas. It overproduces the hormone insulin. Uncontrolled insulin levels can cause hypoglycemia which can lead to serious medical conditions.
VIPoma: an NET that usually arises in the pancreas, may overproduce a hormone that can cause prolonged watery diarrhea and dehydration. Patients may also experience muscle weakness, nausea, vomiting, and crampy abdominal pain.
Pheochromocytoma: an NET that arises in the adrenal glands above the kidneys, can release biologically active compounds, which in turn can raise blood pressure and cause heart palpitations, headaches, and excessive sweating.
Carcinoid Crisis and Carcinoid Heart Disease
Carcinoid Crisis is an uncommon but dangerous situation that is different from carcinoid syndrome. It can arise from a sudden over-release of hormones from your tumors, causing intense flushing, wheezing, and dangerous fluctuations in blood pressure. Severe stress or anesthetics used in surgical or dental procedures are two known causes. Even if you do not have carcinoid syndrome (your tumors are non-functioning) you are at risk of carcinoid crisis. Always reduce this risk by informing your doctor or dentist of your situation prior to undergoing surgery or dental procedures. If needed, the swift administration of octreotide and/or other medications (held on standby during procedures) can combat this crisis should it occur. Octreotide can also be administered preventively during such invasive treatments to avoid this complication.
Carcinoid Heart Disease is one of the major features of carcinoid syndrome. It is caused by excessive release of serotonin usually by metastatic tumors in the liver. When carried through the bloodstream into the heart, the excessive serotonin can cause scarring of the heart’s inner lining, including the two right heart valves, the tricuspid, and pulmonary. Carcinoid heart disease occurs in 50 to 70% of people with carcinoid syndrome. Symptoms may or may not be apparent and can include: fatigue, difficult or labored breathing, and/or fluid build up and swelling in the body. It is important for you to report ALL of your symptoms to your doctors. This condition may be treated by medications and, if needed, cardiac surgery.
The Five E's: To Help with Managing Carcinoid Syndrome Symptoms
Eating: Large meals, spicy foods, and foods high in nitrogen-containing compounds called amines can trigger symptoms of carcinoid syndrome. Tomato-based foods, caffeine, chocolate, nuts, and raw vegetables are common triggers.
Exercise: This trigger is highly variable from person to person, but in general physical stress may activate symptoms. The trick here is not to overdo. If you are not used to a particular exercise regimen, take it slowly and work up your tolerance.
Emotions: Strong emotions such as anger, stress, and anxiety can trigger symptoms. While it is impossible to completely avoid stress in everyday life, patients with carcinoid syndrome should work to reduce stress levels.
Epinephrine: This medication can cause flare-ups and can possibly contribute to carcinoid crisis. Certain medical procedures may require anesthetics, and these may contain epinephrine. Epinephrine-containing numbing agents are commonly given during dental procedures. Inform your providers so they can consider alternatives when possible. If this is an issue for you, you may want to wear a “No EPI” medical ID bracelet.
Ethanol (Alcohol): Some people cannot tolerate any alcohol because it can exacerbate carcinoid syndrome. Monitor your own reaction and adjust accordingly.
Emotions as Part of the Journey
While it is heartening to realize that it is possible to live a long time with this disease, and to live well, it is also daunting to wrap your head around the fact that you will always have cancer and that it needs to be managed and controlled. It’s important to give your emotional and mental health needs the same level of attention that you give your disease. Don’t be hesitant or embarrassed about asking for help when things become too heavy.
Since slow-growing cancers typically do not benefit from chemotherapy and are treated in other ways, NET patients do not have the visible signs of cancer treatment, like hair loss. They also may remain stable for a number of years—something that people don’t associate with cancer. While that is a welcome scenario (comparatively), it can be frustrating to patients when those around them, including those closest to them, don’t recognize or acknowledge the worry and discomfort of living with cancer. The familiar term “remission” really does not apply to stable, progression-free NET cancer, and sometimes this is difficult to explain.
Supportive Care Throughout the Journey
If you are lucky enough to be near a center that offers palliative care, integrative medicine, and/or survivorship programs, you may want to explore how they might serve your needs. Eligibility for these programs begins at diagnosis and can provide the most benefit if taken advantage of from the start, but you can access them and enjoy their benefits at any point along the way.
Palliative Care: Palliative care is not hospice care. It is focused on improving quality of life for people with serious illness. Experts in palliative care can help with things such as relaxation techniques, psychological counseling, nutrition, sleep issues, and pain control.
Integrative Medicine: Similarly, integrative medicine can address and help alleviate other issues associated with serious disease by promoting stress reduction and relaxation through such offerings as acupuncture, exercise classes, mindfulness, and massage.
Survivorship or Wellness Programs: These programs are designed to help patients manage the transition from active treatment to recovery. They generally take an extensive assessment that details the patient’s diagnosis, treatment and monitoring protocols, any physical and emotional needs resulting from treatment, as well as the patient’s hopes and desires. The assessments are periodically updated.
While formal palliative care, integrative medicine, and survivorship programs may not be available in your area, your primary physician or a hospital social worker might help you find providers and services that can address your needs.
Supportive Care Nearing the End of the Journey
Hospice Care: Some cancer patients and their families will be confronted with one of the most difficult decisions of all: the decision to stop treatment. Hospice programs are invaluable in helping to shepherd families and their loved ones through this time by providing a medical team, equipment, home or inpatient treatments, hospice pharmacy, assistance with personal care, grief, end of life planning, spiritual guidance, and counseling. This support and care is for patients and their family and friends who are helping on the journey. Some hospice programs provide music, pets, massage, aromatherapy, or other complementary therapies.
Arriving at a Whole-Self Perspective
The Importance of Maintaining Good Health
Protecting your health is more important now than ever before. To maintain your ability to live well after treatment it is important to improve, or avoid developing, other health conditions to the extent this is within your control. Do you smoke? Do you have a sedentary lifestyle? Do you have a poor diet or drink to excess?
One positive effect of making changes to improve (or maintain) your health is that this commitment to better health can also boost your emotional wellbeing. Even doing small things to take care of yourself and making time for yourself can increase your confidence and feelings of self-worth.
Many wellness and cancer patient support programs offer meditation, yoga, and other classes geared toward stress reduction. Exercise classes can be fun and are wonderful ways to connect with others and relieve stress. Spiritual communities can be extremely comforting to their members, providing assistance and emotional support, as well as continuing to offer purposeful opportunities to help others, which can be very gratifying.
Part and parcel of self-care is being honest with yourself about your own needs, especially those involved in getting you to your goals. This may involve setting limits on your time and responsibilities with both family and friends and choosing to make time and space for yourself when you might have neglected to do this in the past.
If you have an issue that needs attention, or a self-improvement goal, use the “4 I’s” below to help you focus on what is most important to you right now. The goal of such an exercise is to increase self awareness and prioritize goals for improvement, whether spiritual, emotional, physical, or interpersonal.
The 4 “I’s” to a Healthier Life:
- Identify: What issues am I struggling with? What would I like to improve?
- Investigate: How can I address my issues? Who can help?
- Initiate: Develop a plan. Take the first small steps.
- Integrate: Make these changes a part of my life.
As you go through the steps, consider writing them out, and sharing your responses with a friend or loved one. Support can be a powerful way to reinforce your commitment to change. If you have access to a professional like a certified health coach, counselor, or social worker, they can help you with this self-assessment.
It is important to take your self-care, especially exercise, at a pace that is sustainable. Being a patient can be exhausting in many ways, and you might find yourself with limited energy even after treatment ends. You would not be alone in this. The most common symptom and the one identified as most troublesome to all cancer patients, including NETs, is fatigue. Yet fatigue will get worse if ignored. One of the best ways to combat it is to get moving. Moving your body can hasten healing and help you recover strength that may have been lost during treatment and recovery.
Of course, you will need to check with your physician before starting any activities after surgery, treatments, or interventions. But once you’ve been cleared, just start, one step at a time, maybe with short walks inside your home. Any gentle movement builds a foundation for more exercise over time. Cooking and house-work can be exercise—put on your favorite tunes and move to the music!