Diagnosed with carcinoid/NETs: What now?

Another person diagnosed with Jane’s cancer

Every day, 33 people are diagnosed with carcinoid/NETs. Every day 33 people with that diagnosis die from the disease and its complications. Most live for several years–even decades–between the two events. Others, like Jane, for a variety of reasons, are gone in what seems like a heartbeat.

 You face a real struggle in dealing with this disease, but there is hope.

A friend was diagnosed with carcinoid/NETs in recent weeks. I only learned about it this weekend–and when I did it brought back all the memories of our first weeks dealing with the disease in 2010. I wish we’d known then what I know now. But so much of what I wish we’d known did not exist then.

Newly diagnosed need to learn the basics

That–and some posts by people newly diagnosed with carcinoid/NETs on the various online support groups–got me thinking about the need for a primer for patients on what resources are available and background materials. Sites like this one have lots of information about the disease, but in the panicky first weeks after diagnosis no one is in a mental state to sift through  dozens of sites trying to figure out what is useful and what isn’t.

I wish we’d known then what I know now.

I quickly put together a note to my friend about the things I thought she needed to know–the things I wish we had known at the beginning. What follows is based on what I told her. If you are recently diagnosed, it may prove useful to you, as well.

The moments after diagnosis are scary

First, take a deep breath. You are probably feeling a thousand different emotions this point–and your mind is likely chasing off in an equal number of directions. Your doctor has likely told you this is a rare disease–may even have told you he has never seen a case of it before. Your oncologist has likely told you something similar. Those are scary things to hear from the people you view as the experts you have put in charge of your health.

Sites like this one have lots of information…

We don’t train doctors to deal with unusual diseases very well. They are taught from the beginning of medical school that when they hear hoofbeats they should think horses, not zebras. What that means is that a particular set of symptoms most likely is the result of the most common illnesses (horses) associated with those symptoms, not some other disease that is relatively rare (zebras).

The newly diagnosed zebra

Carcinoid/NETs is a zebra even in the cancer community. We diagnose about 12,000 cases in the US every year. There are about 120,000 people living in this country who have been diagnosed with it at any given time. Both those numbers have been rising in recent years, though whether that is because there are more people getting it or whether our ability to detect it has gotten better is unclear. What is clear is that about 12,000 people who know they have the disease die of it every year.

First, take a deep breath.

If your cancer was detected early, there is a good chance surgery will cure you. Unfortunately, this cancer is rarely detected early. If the cancer has spread beyond the primary site, we currently have no cure for the disease. We do have  therapies that will often slow it down and alleviate some of the symptoms. And we do have some things in trials that offer the possibility of a cure for at least some patients.

Newly diagnosed have reasons for hope

In most cases, this is a fairly slow-growing cancer. The therapies we do have may buy you enough time and quality of life for better therapies to come along. Things are changing rapidly in this field and you have reason for hope.

Carcinoid/NETs is a zebra even in the cancer community.

Carcinoid/NETs is unlike any cancer you have heard of before. Most tumor cancers take up space and consume resources. Eventually, they are consuming more resources than your body has or begin to impinge of the spaces of vital organs and destroy their functions.

This cancer is radically different

The primary tumor for most carcinoids is very small–often getting no larger than a lentil. The daughter cancers–the metastases–may get much larger, especially those in the liver, but even those are harder to detect than the more normal cancers. Regular scans often fail to pick up even the larger carcinoid tumors. Every tumor has what are called receptors on them. Contrasting agents–essentially radioactive dyes–are picked up by those receptors, making the cancer visible.

Things are changing rapidly…

But carcinoid tumors lack the common receptors so the dyes don’t stick to them–sort of like the wax on the Easter eggs refusing to pick up the color. This helps keep the tumors seemingly invisible. Two new types of scan can see carcinoid/NETs tumors by using radioactive agents that are traced by their decay —octreoscan and Gallium-68. Unfortunately, some carcinoid tumors are not revealed by those either–and remain invisible.

But just because you can’t see them doesn’t mean they are not there.

The hormone/peptide difference

Another difference between this form of cancer and others is that these tumors can–and often do–produce any of the more than 24,000 hormones and peptides the body produces. Increasing the amount of any hormone can have a huge impact on your life because each is involved in body chemistry.

just because you can’t see them doesn’t mean they are not there.

For example, my wife’s tumors were producing serotonin. Serotonin is involved in controlling digestion, sleep, respiration and blood pressure among other things. Too much of it results in food being digested too quickly for the body to absorb its nutrients–and results in frequent diarrhea; in greater and greater difficulty falling asleep and staying asleep; in dangerously low blood pressure and respiration.

Diagnosing hormone issues

For a long time we had only one test for excess serotonin levels: the 5-hiaa urine test, which requires the patient collect all their urine over a 24 hour period. It is a cumbersome test and doctors, not wanting to put patients through it, can be slow to order it–especially when the disease it tests for is rare. A blood test for excess serotonin appears to have finished trials and is now available, but its broad use is still some years away because few doctors know about it.

Increasing the amount of any hormone can have a huge impact…

But the hormone or peptide being produced doesn’t have to be serotonin–it’s just the one we check for most often. It can be any of those 24,000+ hormones and peptides–and we only have tests for a relative handful of them. And the result of those excess hormones can also result in carcinoid syndrome.

Unique tumor biology complicates life

This complex biology is the reason a mainstream oncologist is often not the best choice for dealing with this disease. I can’t tell you how often I’ve heard things like, “Your 5-HIAA urine test came back negative, so we can rule out carcinoid/NETs;” or “The scans all came back negative, so it can’t be cancer.” The doctors involved were excellent doctors. Their lack of experience with this particular cancer left them prone to mistakes.

…we only have tests for a relative handful of them.

I can’t say strongly enough how important it is to find an oncologist who is familiar with this disease and who can put together the right team for you. Jane and I were lucky to have two of the best people in the field virtually in our back yard–and a local oncologist knowledgeable enough to send us to them.

Medical resources for newly diagnosed

There is a list on our resources page of some of the places that have carcinoid centers at them. That list is not exhaustive, however, because new centers pop up with some frequency. Nor can I recommend any of them, save one, based on personal experience because I have not worked with them. They are often worth the trip because the doctors working at them should have both the expertise and the resources available to make things the best they can be for you. There are also many fine individual doctors with experience with this disease outside those centers. The Carcinoid Cancer Foundation maintains an extensive list of doctors who report they have expertise in this area.

Jane and I were lucky to have two of the best people in the field…

That specialist should put together a team of specialists in other medical areas your cancer will have an impact on. In Jane’s case, because the serotonin was affecting her heart, we also had an oncocardiologist and a heart surgeon on our team–and would have added a liver specialist in due time if she had lived.

Support resources for the newly diagnosed

Just as important as finding the right team of doctors is finding others with the disease to talk to. When Jane was diagnosed, the nearest group we could have found was in New Jersey. Honestly, even had we found the New England group I discovered 18 months after Jane’s death, their meetings would have been too far away for her to get to.

There are also many fine individual doctors with experience…

Now, several groups have moved online. There are several support groups on Facebook who function very well. They are private groups open only to patients and caregivers, but all you need to do is ask to be admitted. All the groups below are useful but each has a slightly different personality. I spend time in each regularly, trying to be useful for both caregivers and patients alike.

Carcinoid Cancer Awareness Network

Carcinoid Coffee Cafe

Carcinoid Neuroendocrine Cancer Awareness

ZEBRA Awareness Global NETS

Lovable Lungnoids  (This group deals specifically with carcinoid lung cancer)

The Carcinoid Cancer Foundation also maintains an extensive list of support groups.

Information resources for the newly diagnosed

You can also find additional information and support though a number of other organizations. First, walkingwithjane.org is the website of Walking with Jane, the foundation I set up shortly after Jane’s death to raise money for research and raise awareness about carcinoid/NETs. It has lots of information, but is also in the midst of a renovation project, so I am a bit behind on some things. Use its Resources section to find other groups and organizations, as well as some basic information about the disease. The News Archive at bottom of every page may also prove useful to you.

…several groups have moved online.

The organization that has dealt with this the longest is the Carcinoid Cancer Foundation. Their site has lists of doctors who have dealt with the disease before, as well as national and regional support groups. The Caring for Carcinoid Foundation in Boston has similar, though less extensive, listings. They have a strong research focus.

There are many good videos online the Carcinoid Cancer Foundation has posted–including all the sessions from some excellent patient conferences. Walking with Jane has also posted some, though our collection is dwarfed by theirs. Here is a basic FAQ video we did last fall.

Some final thoughts

I hope this has helped you get through some of the initial shock of being diagnosed. Things are much better now than they were four years ago when we were just beginning to try to figure out what was going on with Jane. There are some therapies in the pipeline that offer hope of a complete cure for many patients. And our increasing knowledge of how this beastie works offers hope of even better treatments in the near future.

There are many good videos online…

Be optimistic, then. You face a real struggle in dealing with this disease, but there is hope. You will often have to be your own advocate, but the doctors you will need to find will understand that sometimes you will have heard something before they have. One of the standard openings at patient conferences is that anyone who has dealt with this disease for more than six months has the equivalent of a Ph.d in the subject. There is more than a little truth in that statement.

Editor’s note: None of the above constitutes medical advice. I am not a doctor–and don’t pretend to be. The information above is based on my experience dealing with my wife’s battle with carcinoid/NETs and what I have learned since from talking with her doctors, other patients and caregivers, and reading everything I can find on the subject. Before making any medical decision you should speak with your doctor about your particular situation and circumstances. They are qualified to help you make decisions on these things. I am not.

At first, being diagnosed with carcinoid/NETs can feel like you are facing a horrible obstacle course. The search for information about the disease can be difficult and confusing.

At first, being diagnosed with carcinoid/NETs can feel like you are facing a horrible obstacle course. The search for information about the disease can be difficult and confusing.

Posted by walking with jane on April 14, 2014

4 responses to “Diagnosed with carcinoid/NETs: What now?”

  1. Karen Avery says:

    I am still quite new to all of this so can’t help with info but I found it quite helpful even with what I have already learnt the last few months. I think people who can find this once you have got your final version will find it a useful tool. Sorry to hear about your wife but well done and Thankyou for your continued support of the rest of us.
    Karen x

    • Thanks, Karen. You are exactly the kind of patient I needed to hear from on this. Your diagnosis is close enough that you remember the things you really wanted to know at the beginning. If you think of anything else it would have been good to know in those first days, let me know. Thanks again.

      • Julie Moore says:

        Thank you so very much for distilling this ocean of information in a succinct, informative and hopeful manner. I know it has been a journey that I never expected, but even after only six months, I feel connected to a group of people that may have the answers, the questions, the experiences and the compassion to share with the newly diagnosed like myself.

        Your eloquence and empathy is appreciated.
        I will look foward to following WALKING WITH JANE

  2. Wayne Beetge says:

    I was diagnosed with a pancreatic NET with metastasis in the liver in April 2011. The 4cm NET in the pancreas had grown into the slpeenic vein making surgery impossible.I am 35 years old and live in Johannesburg, South Africa.
    I started monthly sandostatin injections with my wife making enquiries on recommendation from Prof José Ramos on PRRT treatments available in Rotterdam Holland and Basel Switzerland. Rotterdam didn’t take foreign patients anymore. Basel university hospital accepted me for two Lu177 PRRT treatments starting Aug 2011 and 0ct 2011. I stopped sandostatin after one injection and went to Basel. After fundraising, a work dread policy I was able to go. Thanks to my wife Steph and we only got married in Sep 2010.
    After the 1st trip I started to feel better and pick up weight, then started to feel much better after my October treatment. I only weighed 57kgs and now weigh 80kgs. The metastasis in the liver are gone and the tumour is stable
    I only have pain 3 years later and go for tests once a year. Prof Ramos says I must live with PNET like diabetes. I’m seeing Prof Ramos on the 15th May to see what tests are needed. People always ask me how I can be so strong but this is something you learn to live with and CARRY ON with your life